25
chapter
Surgical Anatomy
Physiology
1009
1015
Swallowing Mechanism / 1015
Physiologic Reflux / 1017
Assessment of Esophageal
Function
1018
Tests to Detect Structural
Abnormalities / 1018
Tests to Detect Functional
Abnormalities / 1019
Video- and Cineradiography / 1028
Tests to Detect Increased
Exposure to Gastric Juice / 1028
Tests of Duodenogastric Function / 1030
Gastroesophageal Reflux
Disease
1031
The Human Antireflux Mechanism
and the Pathophysiology of
Gastroesophageal Reflux Disease / 1032
Complications Associated With
Gastroesophageal Reflux Disease / 1033
Metaplastic (Barrett’s Esophagus)
and Neoplastic (Adenocarcinoma)
Complications / 1035
Respiratory Complications / 1035
Surgical Therapy for Gastroesophageal
Reflux Disease / 1038
Primary Antireflux Repairs / 1040
Giant Diaphragmatic
(Hiatal) Hernias
Incidence and Etiology / 1045
Clinical Manifestations / 1047
Diagnosis / 1047
Pathophysiology / 1048
Treatment / 1048
1045
Esophagus and Diaphragmatic
Hernia
Blair A. Jobe, John G. Hunter, and David I. Watson
Diaphragmatic Repair / 1048
The Short Esophagus and PEH / 1049
Results / 1049
Schatzki’s Ring
Scleroderma
Eosinophilic Esophagitis
1049
1050
1051
Symptoms / 1051
Signs / 1051
Pathology / 1051
Treatment / 1051
Motility Disorders of the
Pharynx and Esophagus
1052
Clinical Manifestations / 1052
Motility Disorders of the Pharynx and
Upper Esophagus—Transit
Dysphagia / 1052
Diagnostic Assessment of the
Cricopharyngeal Segment / 1052
Motility Disorders of the Esophageal
Body and Lower Esophageal
Sphincter / 1055
Operations for Esophageal Motor
Disorders and Diverticula
1060
Long Esophageal Myotomy for Motor
Disorders of the Esophageal Body / 1060
Myotomy of the Lower Esophageal
Sphincter (Heller Myotomy) / 1063
Open Esophageal Myotomy / 1065
Laparoscopic Cardiomyotomy / 1065
Per Oral Endoscopic
Myotomy (POEM) / 1065
Outcome Assessment of the Therapy for
Achalasia / 1065
Esophageal Resection for End-Stage Motor
Disorders of the Esophagus / 1068
SURGICAL ANATOMY
The esophagus is a muscular tube that starts as the continuation of the pharynx and ends as the cardia of the stomach.
When the head is in a normal anatomic position, the transition from pharynx to esophagus occurs at the lower border of
the sixth cervical vertebra. Topographically this corresponds
to the cricoid cartilage anteriorly and the palpable transverse
process of the sixth cervical vertebra laterally (Fig. 25-1). The
esophagus is firmly attached at its upper end to the cricoid
Carcinoma of the Esophagus
1068
Clinical Manifestations / 1068
General Approach to
Esophageal Cancer / 1069
Staging of Esophageal Cancer / 1069
Clinical Approach to Carcinoma of the
Esophagus and Cardia / 1070
Palliation of Esophageal Cancer / 1074
Surgical Treatment / 1074
Comparative Studies of Esophagectomy
Technique / 1077
Alternative Therapies / 1077
Sarcoma of the Esophagus
Benign Tumors and Cysts
1078
1080
Leiomyoma / 1081
Esophageal Cyst / 1083
Esophageal Perforation
1083
Diagnosis / 1083
Management / 1084
Mallory-Weiss Syndrome
Caustic Injury
1085
1086
Pathology / 1086
Clinical Manifestations / 1086
Treatment / 1086
Acquired Fistula
Techniques of Esophageal
Reconstruction
1088
1089
Partial Esophageal Resection / 1089
Reconstruction After Total
Esophagectomy / 1089
Composite Reconstruction / 1090
Vagal Sparing Esophagectomy
With Colon Interposition / 1090
cartilage and at its lower end to the diaphragm; during swallowing, the proximal points of fixation move craniad the distance of one cervical vertebral body.
The esophagus lies in the midline, with a deviation to the
left in the lower portion of the neck and upper portion of the
thorax, and returns to the midline in the midportion of the thorax near the bifurcation of the trachea (Fig. 25-2). In the lower
portion of the thorax, the esophagus again deviates to the left
and anteriorly to pass through the diaphragmatic hiatus.
Key Points
1
Barrett’s esophagus is the transformation of the distal esophageal epithelium from squamous to a specialized columnar
epithelium capable of further neoplastic progression. The
detection of Barrett’s esophagus on endoscopy and biopsy
increases the future risk of cancer by >40x compared to individuals without Barrett’s esophagus.
Giant hiatal hernia, otherwise known as paraesophageal hernia, should be repaired when symptomatic or associated with
iron deficiency anemia. Laparoscopic hiatal hernia repair
with fundoplication is the most common approach to repair.
Achalasia is the most common primary esophageal motor
disorder. It is characterized by an absence of peristalsis and
a hypertensive nonrelaxing lower esophageal sphincter. It is
best treated with laparoscopic Heller myotomy and partial
fundoplication.
Most esophageal cancer presents with dysphagia, at which
time it has invaded the muscularis of the esophagus and is
often associated with lymph node metastases. The preferred
treatment at this stage is multimodality therapy with chemoradiation therapy followed by open or minimally invasive
esophagectomy.
Three normal areas of esophageal narrowing are evident
on the barium esophagogram or during esophagoscopy. The
uppermost narrowing is located at the entrance into the esophagus and is caused by the cricopharyngeal muscle. Its luminal
diameter is 1.5 cm, and it is the narrowest point of the esophagus. The middle narrowing is due to an indentation of the anterior and left lateral esophageal wall caused by the crossing of the
left main stem bronchus and aortic arch. The luminal diameter at
this point is 1.6 cm. The lowermost narrowing is at the hiatus of
the diaphragm and is caused by the gastroesophageal sphincter
mechanism. The luminal diameter at this point varies somewhat,
depending on the distention of the esophagus by the passage
of food, but has been measured at 1.6 to 1.9 cm. These normal
constrictions tend to hold up swallowed foreign objects, and the
overlying mucosa is subject to injury by swallowed corrosive
liquids due to their slow passage through these areas.
Figure 25-3 shows the average distance in centimeters
measured during endoscopic examination between the incisor
teeth and the cricopharyngeus, aortic arch, and cardia of the
stomach. Manometrically, the length of the esophagus between
the lower border of the cricopharyngeus and upper border of the
lower sphincter varies according to the height of the individual.
2
3
Benign esophageal disease is common and is best evaluated
with thorough physiologic testing (high resolution esophageal motility, 24-hour ambulatory pH measurement, and/or
esophageal impedance testing) and anatomic testing (esophagoscopy, video esophagography, and/or computed tomography [CT] scanning).
Gastroesophageal reflux disease (GERD) is the most common disease of the gastrointestinal tract for which patients
seek medical therapy. When GERD symptoms (heartburn,
regurgitation, chest pain, and/or supraesophageal symptoms)
are troublesome despite adequately dosed PPI, surgical correction may be indicated.
a
b
c
d
e
A
1010
B
4
5
6
Figure 25-1. A. Topographic relationships of the cervical esophagus:
(a) hyoid bone, (b) thyroid
cartilage, (c) cricoid cartilage, (d)
thyroid gland, (e) sternoclavicular.
B. Lateral radio-graphic appearance with landmarks identified as
labeled in A. The location of C6
is also included (f). (Reproduced
with permission from Shields TW:
General Thoracic Surgery, 3rd ed.
Philadelphia, PA: Lea & Febiger;
1989.)
1011
Figure 25-2. Barium esophagogram. A. Posterior-anterior view. White arrow shows deviation to left. Black arrow shows return to midline.
B. Lateral view. Black arrow shows anterior deviation. (Reproduced with permission from Shields TW: General Thoracic Surgery, 3rd ed.
Philadelphia, PA: Lea & Febiger; 1989.)
Incisor teeth
Pharynx
15cm
14cm
The pharyngeal musculature consists of three broad, flat,
overlapping fan-shaped constrictors (Fig. 25-4). The opening
of the esophagus is collared by the cricopharyngeal muscle,
which arises from both sides of the cricoid cartilage of the larynx and forms a continuous transverse muscle band without
an interruption by a median raphe. The fibers of this muscle
24–26cm
Upper sphincter
(C6)
Superior pharyngeal
constrictor m.
Aortic arch
(T4)
40cm
38cm
Middle pharyngeal
constrictor m.
25cm
23cm
Inferior pharyngeal
constrictor m.
Cricopharyngeus m.
Lower sphincter
(T11)
Esophagus
A
Figure 25-3. Important clinical endoscopic measurements of the
esophagus in adults. (Reproduced with permission from Shields
TW: General Thoracic Surgery, 3rd ed. Philadelphia, PA: Lea &
Febiger; 1989.)
B
Figure 25-4. External muscles of the pharynx. A. Posterolateral
view. B. Posterior view. Dotted line represents usual site of myotomy.
(Reproduced with permission from Shields TW: General Thoracic
Surgery, 3rd ed. Philadelphia, PA: Lea & Febiger; 1989.)
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
B
A
1012
PART II
SPECIFIC CONSIDERATIONS
blend inseparably with those of the inferior pharyngeal constrictor above and the inner circular muscle fibers of the esophagus
below. Some investigators believe that the cricopharyngeus is
part of the inferior constrictor; that is, that the inferior constrictor has two parts, an upper or retrothyroid portion having diagonal fibers, and a lower or retrocricoid portion having transverse
fibers. Keith in 1910 showed that these two parts of the same
muscle serve totally different functions. The retrocricoid portion
serves as the upper sphincter of the esophagus and relaxes when
the retrothyroid portion contracts, to force the swallowed bolus
from the pharynx into the esophagus.
The cervical portion of the esophagus is approximately
5 cm long and descends between the trachea and the vertebral
column, from the level of the sixth cervical vertebra to the level
of the interspace between the first and second thoracic vertebrae posteriorly, or the level of the suprasternal notch anteriorly.
The recurrent laryngeal nerves lie in the right and left grooves
between the trachea and the esophagus. The left recurrent nerve
lies somewhat closer to the esophagus than the right, owing to
the slight deviation of the esophagus to the left, and the more
lateral course of the right recurrent nerve around the right subclavian artery. Laterally, on the left and right sides of the cervical esophagus are the carotid sheaths and the lobes of the thyroid
gland.
The thoracic portion of the esophagus is approximately
20 cm long. It starts at the thoracic inlet. In the upper portion
of the thorax, it is in intimate relationship with the posterior
wall of the trachea and the prevertebral fascia. Just above the
tracheal bifurcation, the esophagus passes to the right of the
aorta. This anatomic positioning can cause a notch indentation
in its left lateral wall on a barium swallow radiogram.
Immediately below this notch, the esophagus crosses both the
bifurcation of the trachea and the left main stem bronchus,
owing to the slight deviation of the terminal portion of the
trachea to the right by the aorta (Fig. 25-5). From there
down, the esophagus passes over the posterior surface of
the subcarinal lymph nodes (LNs), and then descends over the
pericardium of the left atrium to reach the diaphragmatic hiatus
(Fig. 25-6). From the bifurcation of the trachea downward,
Ascending aorta
both the vagal nerves and the esophageal nerve plexus lie on
the muscular wall of the esophagus.
Dorsally, the thoracic esophagus follows the curvature of
the spine and remains in close contact with the vertebral bodies. From the eighth thoracic vertebra downward, the esophagus moves vertically away from the spine to pass through the
hiatus of the diaphragm. The thoracic duct passes through the
hiatus of the diaphragm on the anterior surface of the vertebral column behind the aorta and under the right crus. In the
thorax, the thoracic duct lies dorsal to the esophagus between
the azygos vein on the right and the descending thoracic aorta
on the left.
The abdominal portion of the esophagus is approximately
2 cm long and includes a portion of the lower esophageal
sphincter (LES). It starts as the esophagus passes through the
diaphragmatic hiatus and is surrounded by the phrenoesophageal membrane, a fibroelastic ligament arising from the subdiaphragmatic fascia as a continuation of the transversalis fascia
lining the abdomen (Fig. 25-7). The upper leaf of the membrane
attaches itself in a circumferential fashion around the esophagus, about 1 to 2 cm above the level of the hiatus. These fibers
blend in with the elastic-containing adventitia of the abdominal
esophagus and the cardia of the stomach. This portion of the
esophagus is subjected to the positive-pressure environment of
the abdomen.
The musculature of the esophagus can be divided into an
outer longitudinal and an inner circular layer. The upper 2 to
6 cm of the esophagus contains only striated muscle fibers.
From then on, smooth muscle fibers gradually become more
abundant. Most clinically significant esophageal motility disorders involve only the smooth muscle in the lower two-thirds
of the esophagus. When a long surgical esophageal myotomy is
indicated, the incision needs to extend only this distance.
The longitudinal muscle fibers originate from a cricoesophageal tendon arising from the dorsal upper edge of the
anteriorly located cricoid cartilage. The two bundles of muscle diverge and meet in the midline on the posterior wall of
the esophagus about 3 cm below the cricoid (see Fig. 25-4).
From this point on, the entire circumference of the esophagus is
Thymus
Left main stem bronchus
Pericardium
Superior vena cava
Bottom of aortic arch
a
e
Tracheal carina
Descending
aorta
b
c
d
Right main stem
bronchus
IV
Esophagus
B
A
Figure 25-5. A. Cross-section of the thorax at the level of the tracheal bifurcation. B. Computed tomographic scan at same level viewed from
above: (a) ascending aorta, (b) descending aorta, (c) tracheal carina, (d) esophagus, (e) pulmonary artery. (Reproduced with permission from
Shields TW: General Thoracic Surgery, 3rd ed. Philadelphia, PA: Lea & Febiger; 1989.)
1013
Pericardium
Right ventricle
Left ventricle
f
Right atrium
Left atrium
d
e
Pericardium
Esophagus
c
Pleura
Aorta
g
b
a
VII
B
A
Figure 25-6. A. Cross-section of the thorax at the midleft atrial level. B. Computed tomographic scan at same level viewed from above: (a)
aorta, (b) esophagus, (c) left atrium, (d) right atrium, (e) left ventricle, (f) right ventricle, (g) pulmonary vein. (Reproduced with permission
from Shields TW: General Thoracic Surgery, 3rd ed. Philadelphia, PA: Lea & Febiger; 1989.)
covered by a layer of longitudinal muscle fibers. This configuration of the longitudinal muscle fibers around the most proximal
part of the esophagus leaves a V-shaped area in the posterior
wall covered only with circular muscle fibers. Contraction of
the longitudinal muscle fibers shortens the esophagus. The circular muscle layer of the esophagus is thicker than the outer
longitudinal layer. In situ, the geometry of the circular muscle
is helical and makes the peristalsis of the esophagus assume a
wormlike drive, as opposed to segmental and sequential squeezing. As a consequence, severe motor abnormalities of the esophagus assume a corkscrew-like pattern on the barium swallow
radiogram.
The cervical portion of the esophagus receives its main
blood supply from the inferior thyroid artery. The thoracic portion receives its blood supply from the bronchial arteries, with
75% of individuals having one right-sided and two left-sided
branches. Two esophageal branches arise directly from the
aorta. The abdominal portion of the esophagus receives its blood
supply from the ascending branch of the left gastric artery and
from inferior phrenic arteries (Fig. 25-8). On entering the wall
of the esophagus, the arteries assume a T-shaped division to
form a longitudinal plexus, giving rise to an intramural vascular
network in the muscular and submucosal layers. As a consequence, the esophagus can be mobilized from the stomach to
the level of the aortic arch without fear of devascularization and
ischemic necrosis. Caution, however, should be exercised as to
the extent of esophageal mobilization in patients who have had
a previous thyroidectomy with ligation of the inferior thyroid
arteries proximal to the origin of the esophageal branches.
Blood from the capillaries of the esophagus flows into
a submucosal venous plexus, and then into a periesophageal
Esophageal branch
Inferior thyroid
artery
Right bronchial
artery
Diaphragm
Phreno-esophageal membrane
(Descending leaf)
Superior left
bronchial artery
Inferior left
bronchial artery
Aortic esophageal
arteries
Phreno-esophageal membrane
(Ascending leaf)
Parietal
peritoneum
Visceral
peritoneum
Para-esophageal fat pad
Ascending branches of
left gastric artery
Left gastric artery
Figure 25-7. Attachments and structure of the phrenoesophageal
membrane. Transversalis fascia lies just above the parietal peritoneum. (Reproduced with permission from Shields TW: General
Thoracic Surgery, 3rd ed. Philadelphia, PA: Lea & Febiger; 1989.)
Figure 25-8. Arterial blood supply of the esophagus. (Reproduced
with permission from Shields TW: General Thoracic Surgery, 3rd ed.
Philadelphia, PA: Lea & Febiger; 1989.)
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Pleura
1014
PART II
SPECIFIC CONSIDERATIONS
venous plexus from which the esophageal veins originate. In the
cervical region, the esophageal veins empty into the inferior thyroid vein; in the thoracic region, they empty into the bronchial,
azygos, or hemiazygos veins; and in the abdominal region,
they empty into the coronary vein (Fig. 25-9). The submucosal
venous networks of the esophagus and stomach are in continuity
with each other, and, in patients with portal venous obstruction,
this communication functions as a collateral pathway for portal
blood to enter the superior vena cava via the azygos vein.
The parasympathetic innervation of the pharynx and
esophagus is provided mainly by the vagus nerves. The constrictor muscles of the pharynx receive branches from the
pharyngeal plexus, which is on the posterior lateral surface of
the middle constrictor muscle, and is formed by pharyngeal
branches of the vagus nerves with a small contribution from cranial nerves IX and XI (Fig. 25-10). The cricopharyngeal sphincter and the cervical portion of the esophagus receive branches
from both recurrent laryngeal nerves, which originate from the
vagus nerves—the right recurrent nerve at the lower margin of
the subclavian artery and the left at the lower margin of the
aortic arch. They are slung dorsally around these vessels and
ascend in the groove between the esophagus and trachea, giving
branches to each. Damage to these nerves interferes not only
with the function of the vocal cords but also with the function
of the cricopharyngeal sphincter and the motility of the cervical
esophagus, predisposing the individual to pulmonary aspiration
on swallowing.
Afferent visceral sensory pain fibers from the esophagus
end without synapse in the first four segments of the thoracic
spinal cord, using a combination of sympathetic and vagal pathways. These pathways are also occupied by afferent visceral
sensory fibers from the heart; hence, both organs have similar
symptomatology.
The lymphatics located in the submucosa of the esophagus are so dense and interconnected that they constitute a single
Right vagus nerve
Recurrent
laryngeal
nerves
Left vagus nerve
Right recurrent
laryngeal nerve
Left recurrent
laryngeal nerve
Anterior esophageal
plexus
Thoracic chain
Left or anterior
vagal trunk
Right or posterior
vagal trunk
Figure 25-10. Innervation of the esophagus. (Reproduced with
permission from Shields TW: General Thoracic Surgery, 3rd ed.
Philadelphia, PA: Lea & Febiger; 1989.)
plexus (Fig. 25-11). There are more lymph vessels than blood
capillaries in the submucosa. Lymph flow in the submucosal
plexus runs in a longitudinal direction, and, on injection of a
contrast medium, the longitudinal spread is seen to be about six
times that of the transverse spread. In the upper two-thirds of
the esophagus, the lymphatic flow is mostly cephalad, and, in
the lower third, caudad. In the thoracic portion of the esophagus,
Inferior thyroid veins
Superior
paraesophageal nodes
Accessory azygous vein
Hemiazygous vein
Paratracheal
nodes
Pulmonary hilar
nodes
Azygous vein
Subcarinal nodes
Inferior paraesophageal
nodes
Parahiatal nodes
Short
gastric
veins
Coronary vein
Portal vein
Superior mesenteric
vein
Internal jugular
nodes
Splenic artery
nodes
Left gastric artery nodes
Hepatic artery
nodes
Celiac artery nodes
Splenic vein
Figure 25-9. Venous drainage of the esophagus. (Reproduced with
permission from Shields TW: General Thoracic Surgery, 3rd ed.
Philadelphia, PA: Lea & Febiger; 1989.)
Figure 25-11. Lymphatic drainage of the esophagus. (Reproduced
with permission from DeMeester TR, Barlow AP. Surgery and current management for cancer of the esophagus and cardia: Part I,
Curr Probl Surg. 1988 Jul;25(7):475-531.)
The act of alimentation requires the passage of food and drink
from the mouth into the stomach. One-third of this distance consists of the mouth and hypopharynx, and two-thirds is made up
by the esophagus. To comprehend the mechanics of alimentation, it is useful to visualize the gullet as a mechanical model
in which the tongue and pharynx function as a piston pump
with three valves, and the body of the esophagus and cardia
function as a worm-drive pump with a single valve. The three
valves in the pharyngeal cylinder are the soft palate, epiglottis,
and cricopharyngeus. The valve of the esophageal pump is the
LES. Failure of the valves or the pumps leads to abnormalities in swallowing—that is, difficulty in food propulsion from
mouth to stomach—or regurgitation of gastric contents into the
esophagus or pharynx.
Food is taken into the mouth in a variety of bite sizes,
where it is broken up, mixed with saliva, and lubricated. Once
initiated, swallowing is entirely a reflex act. When food is
ready for swallowing, the tongue, acting like a piston, moves
the bolus into the posterior oropharynx and forces it into the
hypopharynx (Fig. 25-12). Concomitantly with the posterior
movement of the tongue, the soft palate is elevated, thereby
closing the passage between the oropharynx and nasopharynx.
This partitioning prevents pressure generated in the oropharynx
from being dissipated through the nose. When the soft palate is
paralyzed, for example, after a cerebrovascular accident, food
is commonly regurgitated into the nasopharynx. During swallowing, the hyoid bone moves upward and anteriorly, elevating
the larynx and opening the retrolaryngeal space, bringing the
epiglottis under the tongue (see Fig. 25-12). The backward tilt
of the epiglottis covers the opening of the larynx to prevent aspiration. The entire pharyngeal part of swallowing occurs within
1.5 seconds.
During swallowing, the pressure in the hypopharynx rises
abruptly, to at least 60 mmHg, due to the backward movement
of the tongue and contraction of the posterior pharyngeal constrictors. A sizable pressure difference develops between the
hypopharyngeal pressure and the less-than-atmospheric midesophageal or intrathoracic pressure (Fig. 25-13). This pressure
1
3
2
4
6
5
1. Elevation of tongue
2. Posterior movement of tongue
3. Elevation of soft palate
4. Elevation of hyoid
5. Elevation of larynx
6. Tilting of epiglottis
Figure 25-12. Sequence of events during the oropharyngeal phase
of swallowing. (Reproduced with permission from Zuidema GD,
Orringer MB: Shackelford’s Surgery of the Alimentary Tract, 3rd ed.
Vol 1. Philadelphia, PA: Elsevier/Saunders; 1991.)
gradient speeds the movement of food from the hypopharynx
into the esophagus when the cricopharyngeus or upper esophageal sphincter relaxes. The bolus is both propelled by peristaltic
contraction of the posterior pharyngeal constrictors and sucked
into the thoracic esophagus. Critical to receiving the bolus is
the compliance of the cervical esophagus; when compliance is
lost due to muscle pathology, dysphagia can result. The upper
esophageal sphincter closes within 0.5 seconds of the initiation
of the swallow, with the immediate closing pressure reaching
P
C
E
DES
G
0
% Esophagus length
PHYSIOLOGY
Swallowing Mechanism
1015
20
Upright position
40
60
Air
80
100
–10 –5 0
5 10 15 20 25 30 35 40
Pressure (mm Hg)
Figure 25-13. Resting pressure profile of the foregut showing the
pressure differential between the atmospheric pharyngeal pressure
(P) and the less-than-atmospheric midesophageal pressure (E) and
greater-than-atmospheric intragastric pressure (G), with the interposed high-pressure zones of the cricopharyngeus (C) and distal
esophageal sphincter (DES). The necessity for relaxation of the cricopharyngeus and DES pressure to move a bolus into the stomach
is apparent. Esophageal work occurs when a bolus is pushed from
the midesophageal area (E), with a pressure less than atmospheric,
into the stomach, which has a pressure greater than atmospheric
(G). (Reproduced with permission from Waters PF, DeMeester TR:
Foregut motor disorders and their surgical managemen, Med Clin
North Am. 1981 Nov;65(6):1235-1268.)
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
the submucosal lymph plexus extends over a long distance in
a longitudinal direction before penetrating the muscle layer to
enter lymph vessels in the adventitia. As a consequence of this
nonsegmental lymph drainage, a primary tumor can extend for
a considerable length superiorly or inferiorly in the submucosal
plexus. Consequently, free tumor cells can follow the submucosal lymphatic plexus in either direction for a long distance
before they pass through the muscularis and on into the regional
LNs. The cervical esophagus has more direct segmental lymph
drainage into the regional nodes, and, as a result, lesions in this
portion of the esophagus have less submucosal extension and a
more regionalized lymphatic spread.
The efferent lymphatics from the cervical esophagus drain
into the paratracheal and deep cervical LNs, and those from the
upper thoracic esophagus empty mainly into the paratracheal
LNs. Efferent lymphatics from the lower thoracic esophagus
drain into the subcarinal nodes and nodes in the inferior pulmonary ligaments. The superior gastric nodes receive lymph not
only from the abdominal portion of the esophagus, but also from
the adjacent lower thoracic segment.
1016
60
50
40
30
20
10
0
Pharynx
Cricopharyngeus
50
40
30
20
10
0
PART II
50
40
30
20
10
0
Esophageal body
SPECIFIC CONSIDERATIONS
50
40
30
20
10
0
High pressure zone
Stomach
50
40
30
20
10
0
mm Swallow
Hg
Seconds
Seconds
Seconds
Seconds
Seconds
Figure 25-14. Intraluminal esophageal pressures in response
to swallowing. (Reproduced with permission from Waters PF,
DeMeester TR: Foregut motor disorders and their surgical managemen, Med Clin North Am. 1981 Nov;65(6):1235-1268.)
approximately twice the resting level of 30 mmHg. The postrelaxation contraction continues down the esophagus as a peristaltic wave (Fig. 25-14). The high closing pressure and the
initiation of the peristaltic wave prevents reflux of the bolus
from the esophagus back into the pharynx. After the peristaltic
wave has passed farther down the esophagus, the pressure in the
upper esophageal sphincter returns to its resting level.
Swallowing can be started at will, or it can be reflexively
elicited by the stimulation of areas in the mouth and pharynx,
among them the anterior and posterior tonsillar pillars or the
posterior lateral walls of the hypopharynx. The afferent sensory nerves of the pharynx are the glossopharyngeal nerves
and the superior laryngeal branches of the vagus nerves. Once
aroused by stimuli entering via these nerves, the swallowing
center in the medulla coordinates the complete act of swallowing by discharging impulses through cranial nerves V, VII, X,
XI, and XII, as well as the motor neurons of C1 to C3. Discharges through these nerves occur in a rather specific pattern
and last for approximately 0.5 seconds. Little is known about the
organization of the swallowing center, except that it can trigger
swallowing after a variety of different inputs, but the response
is always a rigidly ordered pattern of outflow. Following a cerebrovascular accident, this coordinated outflow may be altered,
causing mild to severe abnormalities of swallowing. In more
severe injury, swallowing can be grossly disrupted, leading to
repetitive aspiration.
The striated muscles of the cricopharyngeus and the upper
one-third of the esophagus are activated by efferent motor fibers
distributed through the vagus nerve and its recurrent laryngeal
branches. The integrity of innervation is required for the cricopharyngeus to relax in coordination with the pharyngeal
contraction, and resume its resting tone once a bolus has entered
the upper esophagus. Operative damage to the innervation can
interfere with laryngeal, cricopharyngeal, and upper esophageal
function, and predispose the patient to aspiration.
The pharyngeal activity in swallowing initiates the esophageal phase. The body of the esophagus functions as a wormdrive propulsive pump due to the helical arrangement of its
circular muscles, and it is responsible for transferring a bolus
of food into the stomach. The esophageal phases of swallowing represent esophageal work done during alimentation, in
that food is moved into the stomach from a negative-pressure
environment of –6 mmHg intrathoracic pressure, to a positivepressure environment of 6 mmHg intra-abdominal pressure, or
over a gradient of 12 mmHg (see Fig. 25-13). Effective and
coordinated smooth muscle function in the lower one-third of
the esophagus is therefore important in pumping the food across
this gradient.
The peristaltic wave generates an occlusive pressure varying from 30 to 120 mmHg (see Fig. 25-14). The wave rises
to a peak in 1 second, lasts at the peak for about 0.5 seconds,
and then subsides in about 1.5 seconds. The whole course of
the rise and fall of occlusive pressure may occupy one point in
the esophagus for 3 to 5 seconds. The peak of a primary peristaltic contraction initiated by a swallow (primary peristalsis)
moves down the esophagus at 2 to 4 cm/s and reaches the distal
esophagus about 9 seconds after swallowing starts. Consecutive
swallows produce similar primary peristaltic waves, but when
the act of swallowing is rapidly repeated, the esophagus remains
relaxed and the peristaltic wave occurs only after the last movement of the pharynx. Progress of the wave in the esophagus is
caused by sequential activation of its muscles, initiated by efferent vagal nerve fibers arising in the swallowing center.
Continuity of the esophageal muscle is not necessary for
sequential activation if the nerves are intact. If the muscles, but
not the nerves, are cut across, the pressure wave begins distally below the cut as it dies out at the proximal end above the
cut. This allows a sleeve resection of the esophagus to be done
without destroying its normal function. Afferent impulses from
receptors within the esophageal wall are not essential for progress of the coordinated wave. Afferent nerves, however, do go to
the swallowing center from the esophagus because if the esophagus is distended at any point, a contraction wave begins with a
forceful closure of the upper esophageal sphincter and sweeps
down the esophagus. This secondary contraction occurs without
any movements of the mouth or pharynx. Secondary peristalsis
can occur as an independent local reflex to clear the esophagus
of ingested material left behind after the passage of the primary
wave. Current studies suggest that secondary peristalsis is not
as common as once thought.
Despite the powerful occlusive pressure, the propulsive
force of the esophagus is relatively feeble. If a subject attempts
to swallow a bolus attached by a string to a counterweight, the
maximum weight that can be overcome is 5 to 10 g. Orderly
contractions of the muscular wall and anchoring of the esophagus at its inferior end are necessary for efficient aboral propulsion to occur. Loss of the inferior anchor, as occurs with a large
hiatal hernia, can lead to inefficient propulsion.
The LES provides a pressure barrier between the esophagus and stomach and acts as the valve on the worm-drive pump
of the esophageal body. Although an anatomically distinct LES
has been difficult to identify, microdissection studies show
that, in humans, the sphincter-like function is related to the
-50
50-
0-
Phrenoesophageal
membrane
Semi-circular
fibers
Gastro-esophageal
muscular ring
-0 mm
Oblique
fibers
-50
-0 mm
-20
Anterior wall thickness
Figure 25-15. Wall thickness and orientation of fibers on microdissection of the cardia. At the junction of the esophageal tube
and gastric pouch, there is an oblique muscular ring composed of
an increased muscle mass inside the inner muscular layer. On the
lesser curve side of the cardia, the muscle fibers of the inner layer
are oriented transversely and form semicircular muscle clasps. On
the greater curve side of the cardia, these muscle fibers form oblique
loops that encircle the distal end of the cardia and gastric fundus.
Both the semicircular muscle clasps and the oblique fibers of the
fundus contract in a circular manner to close the cardia. (Reproduced
with permission from Glenn WWL: Thoracic and Cardiovascular
Surgery, 4th ed. Norwalk, CT: Appleton-Century-Crofts; 1983.)
architecture of the muscle fibers at the junction of the esophageal tube with the gastric pouch (Fig. 25-15). The sphincter
actively remains closed to prevent reflux of gastric contents into
the esophagus and opens by a relaxation that coincides with a
pharyngeal swallow (see Fig. 25-14). The LES pressure returns
to its resting level after the peristaltic wave has passed through
the esophagus. Consequently, reflux of gastric juice that may
occur through the open valve during a swallow is cleared back
into the stomach.
If the pharyngeal swallow does not initiate a peristaltic contraction, then the coincident relaxation of the LES is unguarded
and reflux of gastric juice can occur. This may be an explanation
for the observation of spontaneous lower esophageal relaxation,
thought by some to be a causative factor in gastroesophageal
reflux disease (GERD). The power of the worm-drive pump of
the esophageal body is insufficient to force open a valve that
does not relax. In dogs, a bilateral cervical parasympathetic
blockade abolishes the relaxation of the LES that occurs with
pharyngeal swallowing or distention of the esophagus. Consequently, vagal function appears to be important in coordinating
the relaxation of the LES with esophageal contraction.
The antireflux mechanism in human beings is composed
of three components: a mechanically effective LES, efficient
esophageal clearance, and an adequately functioning gastric
reservoir. A defect of any one of these three components can
lead to increased esophageal exposure to gastric juice and the
development of mucosal injury.
On 24-hour esophageal pH monitoring, healthy individuals have
occasional episodes of gastroesophageal reflux. This physiologic reflux is more common when awake and in the upright
position than during sleep in the supine position. When reflux
of gastric juice occurs, normal subjects rapidly clear the acid
gastric juice from the esophagus regardless of their position.
There are several explanations for the observation that
physiologic reflux in normal subjects is more common when
they are awake and in the upright position than during sleep in
the supine position. First, reflux episodes occur in healthy volunteers primarily during transient losses of the gastroesophageal
barrier, which may be due to a relaxation of the LES or intragastric pressure overcoming sphincter pressure. Gastric juice
can also reflux when a swallow-induced relaxation of the LES
is not protected by an oncoming peristaltic wave. The average
frequency of these “unguarded moments” or of transient losses
of the gastroesophageal barrier is far less while asleep and in
the supine position than while awake and in the upright position. Consequently, there are fewer opportunities for reflux to
occur in the supine position. Second, in the upright position,
there is a 12-mmHg pressure gradient between the resting, positive intra-abdominal pressure measured in the stomach and the
most negative intrathoracic pressure measured in the esophagus
at midthoracic level. This gradient favors the flow of gastric
juice up into the thoracic esophagus when upright. The gradient diminishes in the supine position. Third, the LES pressure
in normal subjects is significantly higher in the supine position than in the upright position. This is due to the apposition
of the hydrostatic pressure of the abdomen to the abdominal
portion of the sphincter when supine. In the upright position,
the abdominal pressure surrounding the sphincter is negative
compared with atmospheric pressure, and, as expected, the
abdominal pressure gradually increases the more caudally it is
measured. This pressure gradient tends to move the gastric contents toward the cardia and encourages the occurrence of reflux
into the esophagus when the individual is upright. In contrast,
in the supine position, the gastroesophageal pressure gradient
diminishes, and the abdominal hydrostatic pressure under the
diaphragm increases, causing an increase in sphincter pressure
and a more competent cardia.
The LES has intrinsic myogenic tone, which is modulated by neural and hormonal mechanisms. α-Adrenergic neurotransmitters or β-blockers stimulate the LES, and α-blockers
and β-stimulants decrease its pressure. It is not clear to what
extent cholinergic nerve activity controls LES pressure. The
vagus nerve carries both excitatory and inhibitory fibers to the
esophagus and sphincter. The hormones gastrin and motilin
have been shown to increase LES pressure; and cholecystokinin,
estrogen, glucagon, progesterone, somatostatin, and secretin
decrease LES pressure. The peptides bombesin, l-enkephalin,
and substance P increase LES pressure; and calcitonin generelated peptide, gastric inhibitory peptide, neuropeptide Y, and
vasoactive intestinal polypeptide decrease LES pressure. Some
pharmacologic agents such as antacids, cholinergics, agonists,
domperidone, metoclopramide, and prostaglandin F2 are known
to increase LES pressure; and anticholinergics, barbiturates, calcium channel blockers, caffeine, diazepam, dopamine, meperidine, prostaglandin E1 and E2, and theophylline decrease LES
pressure. Peppermint, chocolate, coffee, ethanol, and fat are all
associated with decreased LES pressure and may be responsible
for esophageal symptoms after a sumptuous meal.
1017
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Greater curvature
wall thickness
20-
Lesser curvature
wall thickness
-20
Physiologic Reflux
1018
ASSESSMENT OF ESOPHAGEAL FUNCTION
A thorough understanding of the patient’s underlying anatomic
and functional deficits before making therapeutic decisions is
fundamental to the successful treatment of esophageal disease.
The diagnostic tests, as presently used, may be divided into four
groups: (a) tests to detect structural abnormalities of
1 broad
the esophagus; (b) tests to detect functional abnormalities
of the esophagus; (c) tests to detect increased esophageal exposure to gastric juice; and (d) tests of duodenogastric function as
they relate to esophageal disease.
PART II
Tests to Detect Structural Abnormalities
SPECIFIC CONSIDERATIONS
Endoscopic Evaluation. The first diagnostic test in patients
with suspected esophageal disease is usually upper gastrointestinal endoscopy. This allows assessment and biopsy of the mucosa
of the stomach and the esophagus, as well as the diagnosis and
assessment of obstructing lesions in the upper gastrointestinal
tract. In any patient complaining of dysphagia, esophagoscopy
is indicated, even in the face of a normal radiographic study.
For the initial endoscopic assessment, the flexible fiberoptic esophagoscope is the instrument of choice because of its
technical ease, patient acceptance, and the ability to simultaneously assess the stomach and duodenum. Rigid endoscopy is
now only rarely required, mainly for the disimpaction of difficult foreign bodies impacted in the esophagus, and few individuals now have the skill set and experience to use this equipment.
When GERD is the suspected diagnosis, particular attention should be paid to detecting the presence of esophagitis and
Barrett’s columnar-lined esophagus (CLE). When endoscopic
esophagitis is seen, severity and the length of esophagitis
involved are recorded. Whilst many different grading systems
have been proposed, the commonest system now in use is the
Los Angeles (LA) grading system. In this system, mild esophagitis is classified LA grade A or B—one or more erosions limited to the mucosal fold(s) and either less than or greater than
5 mm in longitudinal extent respectively (Fig. 25-16). More
severe esophagitis is classified LA grade C or D. In grade C,
erosions extend over the mucosal folds but over less than threequarters of the esophageal circumference; in grade D, confluent
erosions extend across more than three-quarters of the esophageal circumference. In addition to these grades, more severe
damage can lead to the formation of a stricture. A stricture’s
severity can be assessed by the ease of passing a standard endoscope. When a stricture is observed, the severity of the esophagitis above it should be recorded. The absence of esophagitis
above a stricture suggests the possibility of a chemical-induced
injury or a neoplasm as a cause. The latter should always be
considered and is ruled out only by evaluation of a tissue biopsy
of adequate size. It should be remembered that gastroesophageal
reflux is not always associated with visible mucosal abnormalities, and patients can experience significant reflux symptoms,
despite an apparently normal endoscopy examination.
Barrett’s esophagus (BE) is a condition in which the tubular esophagus is lined with columnar epithelium, as opposed to
the normal squamous epithelium (see Fig. 25-16). Histologically, it appears as intestinal metaplasia (IM). It is suspected at
endoscopy when there is difficulty in visualizing the squamocolumnar junction at its normal location, and by the appearance of
a redder, salmon-colored mucosa in the lower esophagus, with
a clearly visible line of demarcation at the top of the Barrett’s
esophagus segment. Its presence is confirmed by biopsy. Multiple biopsy specimens should be taken in a cephalad direction
to confirm the presence of IM, and to evaluate the Barrett’s epithelium for dysplastic changes. BE is susceptible to ulceration,
bleeding, stricture formation, and, most important, malignant
degeneration. The earliest sign of the latter is high grade dysplasia or intramucosal adenocarcinoma (see Fig. 25-16). These
dysplastic changes have a patchy distribution, so a minimum
of four biopsy samples spaced 2 cm apart should be taken from
the Barrett’s-lined portion of the esophagus. Changes seen in
one biopsy are significant. Nishimaki has determined that the
tumors occur in an area of specialized columnar epithelium near
the squamocolumnar junction in 85% of patients, and within
2 cm of the squamocolumnar junction in virtually all patients.
Particular attention should be focused on this area in patients
suspected of harboring a carcinoma.
Abnormalities of the gastroesophageal flap valve can be
visualized by retroflexion of the endoscope. Hill has graded the
appearance of the gastroesophageal valve from I to IV according
to the degree of unfolding or deterioration of the normal valve
architecture (Fig. 25-17). The appearance of the valve correlates
with the presence of increased esophageal acid exposure, occurring predominantly in patients with grade III and IV valves.
A hiatal hernia is endoscopically confirmed by finding a
pouch lined with gastric rugal folds lying 2 cm or more above
the margins of the diaphragmatic crura, identified by having the
patient sniff. A hernia is best demonstrated with the stomach
fully insufflated and the gastroesophageal junction observed
with a retroflexed endoscope. A prominent sliding hiatal hernia
frequently is associated with increased esophageal exposure to
gastric juice. When a paraesophageal hernia (PEH) is observed,
particular attention is taken to exclude gastric (Cameron’s)
ulcers or gastritis within the pouch. The intragastric retroflex or
J maneuver is important in evaluating the full circumference of
the mucosal lining of the herniated stomach.
When an esophageal diverticulum is seen, it should
be carefully explored with the flexible endoscope to exclude
ulceration or neoplasia. When a submucosal mass is identified,
biopsy specimens are usually not performed. At the time of surgical resection, a submucosal leiomyoma or reduplication cyst
can generally be dissected away from the intact mucosa, but if
a biopsy sample is taken, the mucosa may become fixed to the
underlying abnormality. This complicates the surgical dissection by increasing the risk of mucosal perforation. Endoscopic
ultrasound provides a better method for evaluating these lesions.
Radiographic Evaluation. Barium swallow evaluation is undertaken selectively to assess anatomy and motility. The anatomy of
large hiatal hernias is more clearly demonstrated by contrast radiology than endoscopy, and the presence of coordinated esophageal peristalsis can be determined by observing several individual
swallows of barium traversing the entire length of the organ,
with the patient in the horizontal position. Hiatal hernias are best
demonstrated with the patient prone because the increased intraabdominal pressure produced in this position promotes displacement of the esophagogastric junction above the diaphragm. To
detect lower esophageal narrowing, such as rings and strictures,
fully distended views of the esophagogastric region are crucial.
The density of the barium used to study the esophagus can potentially affect the accuracy of the examination. Esophageal disorders
shown clearly by a full-column technique include circumferential
carcinomas, peptic strictures, large esophageal ulcers, and hiatal hernias. A small hiatal hernia is usually not associated with
significant symptoms or illness, and its presence is an irrelevant
finding unless the hiatal hernia is large (Fig. 25-18) or the hernia
1019
B
C
D
Figure 25-16. Complications of reflux disease as seen on endoscopy. A. Linear erosions of LA grade B esophagitis. B. Uncomplicated
Barrett’s mucosa. C. High-grade dysplasia in Barrett’s mucosa. D. Early adenocarcinoma arising in Barrett’s mucosa.
is of the paraesophageal variety. Lesions extrinsic but adjacent to
the esophagus can be reliably detected by the full-column technique if they contact the distended esophageal wall. Conversely,
a number of important disorders may go undetected if this is the
sole technique used to examine the esophagus. These include
small esophageal neoplasms, mild esophagitis, and esophageal
varices. Thus, the full-column technique should be supplemented
with mucosal relief or double-contrast films to enhance detection
of these smaller or more subtle lesions.
Motion-recording techniques greatly aid in evaluating
functional disorders of the pharyngoesophageal and esophageal
phases of swallowing. The technique and indications for cineand videoradiography will be discussed in the section entitled
“Video- and Cineradiography,” as they are more useful to evaluate function and seldom used to detect structural abnormalities.
The radiographic assessment of the esophagus is not complete unless the entire stomach and duodenum have been examined.
A gastric or duodenal ulcer, partially obstructing gastric neoplasm,
or scarred duodenum and pylorus may contribute significantly to
symptoms otherwise attributable to an esophageal abnormality.
When a patient’s complaints include dysphagia and no
obstructing lesion is seen on the barium swallow, it is useful to
have the patient swallow a barium-impregnated marshmallow, a
barium-soaked piece of bread, or a hamburger mixed with barium. This test may bring out a functional disturbance in esophageal transport that can be missed when liquid barium is used.
Tests to Detect Functional Abnormalities
In many patients with symptoms of an esophageal disorder,
standard radiographic and endoscopic evaluation fails to demonstrate a structural abnormality. In these situations, esophageal
function tests are necessary to identify a functional disorder.
Esophageal Motility. Esophageal motility is a widely used
technique to examine the motor function of the esophagus and
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
A
1020
PART II
SPECIFIC CONSIDERATIONS
A
B
C
Figure 25-17. A. Grade I flap valve appearance. Note the ridge of tissue that is closely approximated to the shaft of the retroflexed endoscope.
It extends 3 to 4 cm along the lesser curve. B. Grade II flap valve appearance. The ridge is slightly less well defined than in grade I and it
opens rarely with respiration and closes promptly. C. Grade III flap valve appearance. The ridge is barely present, and there is often failure
to close around the endoscope. It is nearly always accompanied by a hiatal hernia. D. Grade IV flap valve appearance. There is no muscular
ridge at all. The gastroesophageal valve stays open all the time, and squamous epithelium can often be seen from the retroflexed position. A
hiatal hernia is always present. (Reproduced with permission from Hill LD, Kozarek RA, Kraemer SJ, et al: The gastroesophageal flap valve:
in vitro and in vivo observations, Gastrointest Endosc. 1996 Nov;44(5):541-547.)
1021
its sphincters. The esophageal motility study (EMS) is indicated
whenever a motor abnormality of the esophagus is suspected on
the basis of complaints of dysphagia, odynophagia, or noncardiac chest pain, and the barium swallow or endoscopy does not
show a clear structural abnormality. EMS is particularly necessary to confirm the diagnosis of specific primary esophageal
motility disorders (i.e., achalasia, diffuse esophageal spasm
[DES], nutcracker esophagus, and hypertensive LES). It also
identifies nonspecific esophageal motility abnormalities and
motility disorders secondary to systemic disease such as scleroderma, dermatomyositis, polymyositis, or mixed connective tissue disease. In patients with symptomatic GERD, manometry
of the esophageal body can identify a mechanically defective
LES and evaluate the adequacy of esophageal peristalsis and
contraction amplitude. EMS has become an essential tool in the
preoperative evaluation of patients before antireflux surgery,
guiding selection of the appropriate procedure based upon the
patient’s underlying esophageal function and excluding patients
with achalasia who can be misdiagnosed with gastroesophageal
reflux when clinical and endoscopic parameters alone are used
for diagnosis.
EMS is performed using electronic, pressure-sensitive
transducers located within the catheter, or water-perfused catheters with lateral side holes attached to transducers outside the
body. The traditional water perfused catheter has largely been
replaced by high resolution motility (HRM), but knowledge of
traditional methods of assessing esophageal motility is helpful
for understanding esophageal physiology.
As the pressure-sensitive station is brought across the gastroesophageal junction (GEJ), a rise in pressure above the gastric baseline signals the beginning of the LES. The respiratory
inversion point is identified when the positive excursions that
occur in the abdominal cavity with breathing change to negative
deflections in the thorax. The respiratory inversion point serves
as a reference point at which the amplitude of LES pressure
and the length of the sphincter exposed to abdominal pressure
are measured. As the pressure-sensitive station is withdrawn
into the body of the esophagus, the upper border of the LES is
identified by the drop in pressure to the esophageal baseline.
From these measurements, the pressure, abdominal length, and
overall length of the sphincter are determined (Fig. 25-19). To
Overall length
Gastric
baseline
pressure
43
Pressure
42
41
40
10 sec
Abdominal length
39
RIP
38
37 cm
Esophageal
baseline
pressure
RIP = Respiratory inversion point
Figure 25-18. Radiogram of an intrathoracic stomach. This
is the end stage of a large hiatal hernia, regardless of its initial
classification.
Figure 25-19. Manometric pressure profile of the lower esophageal
sphincter. The distances are measured from the nares. (Reproduced
with permission from Zaninotto G, DeMeester TR, Schwizer W,
et al: The lower esophageal sphincter in health and disease, Am J
Surg. 1988 Jan;155(1):104-11.)
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
D
Figure 25-17. (Continued )
1022
foregut disorder. A mechanically defective sphincter is identified
by having one or more of the following characteristics: an
average LES pressure of <6 mmHg, an average length exposed
to the positive-pressure environment in the abdomen of 1 cm or
less, and/or an average overall sphincter length of 2 cm or less.
LP
P
0
25
RP
L
50
PART II
LA
R
SPECIFIC CONSIDERATIONS
RA
A
Figure 25-20. Radial configuration of the lower esophageal
sphincter. A = anterior; L = left; LA = left anterior; LP = left posterior; P = posterior; R = right; RA = right anterior; RP = right posterior. (Reproduced with permission from Winans CS: Manometric
asymmetry of the lower-esophageal high-pressure zone, Am J Dig
Dis. 1977 Apr;22(4):348-354.)
account for the asymmetry of the sphincter (Fig. 25-20), the
pressure profile is repeated with each of the five radially oriented transducers, and the average values for sphincter pressure
above gastric baseline, overall sphincter length, and abdominal
length of the sphincter are calculated.
Table 25-1 shows the values for these parameters in 50
normal volunteers without subjective or objective evidence of a
Table 25-1
Normal manometric values of the distal esophageal
sphincter, n = 50
PERCENTILE
MEDIAN
2.5
97.5
13
5.8
27.7
Overall length (cm) 3.6
2.1
5.6
Abdominal
length (cm)
2
0.9
4.7
MEAN
MEAN – 2 SD MEAN + 2 SD
Pressure (mmHg)
Pressure (mmHg)
13.8 ± 4.6 4.6
23.0
Overall length (cm) 3.7 ± 0.8
2.1
5.3
Abdominal
length (cm)
0.6
3.8
2.2 ± 0.8
SD = standard deviation.
Reproduced with permission from Moody FG, Carey LC, Jones RS,
et al: Surgical Treatment of Digestive Disease. Chicago, IL: Year Book
Medical; 1990.
High-Resolution Manometry. Esophageal manometry was
introduced into clinical practice in the 1970s and, until recently,
has changed little. In 1991, Ray Clouse introduced the concept of
improving conventional manometry by increasing the number of
recording sites and adding a three-dimensional assessment. This
“high-resolution manometry” is a variant of the conventional
manometry in which multiple, circumferential recording sites
are used, in essence creating a “map” of the esophagus and its
sphincters. High-resolution catheters contain 36 miniaturized
pressure sensors positioned every centimeter along the length
of the catheter. The vast amount of data generated by these
sensors is then processed and presented in traditional linear
plots or as a visually enhanced spatiotemporal video tracing that
is readily interpreted. The function of the esophageal body is
assessed with 10 to 15 wet swallows. Amplitude, duration, and
morphology of contractions following each swallow are visually
displayed (Fig. 25-21).
The relationship of the esophageal contractions following
a swallow is classified as peristaltic or simultaneous. The data
are used to identify motor disorders of the esophagus.
The position, length, and function of the lower esophageal sphincter (LES) are demonstrated by a high-pressure zone
that should relax at the inception of swallowing and contract
after the water or solid bolus passes through the LES. Simultaneous acquisition of data for the upper esophageal sphincter, esophageal body, LES, and gastric pressure minimizes the
movement artifacts and study time associated with conventional esophageal manometry. This technology significantly
enhances esophageal diagnostics, bringing it into the realm
of “image”-based studies. High-resolution manometry may
allow the identification of focal motor abnormalities previously overlooked. It has enhanced the ability to predict bolus
propagation and increased sensitivity in the measurement of
pressure gradients.
Esophageal Impedance. Newer technology introduced
into the clinical realm a decade ago allows measurement of
esophageal function and gastroesophageal reflux in a way that
was previously not possible. An intraluminal electrical impedance catheter is used to measure GI function. Impedance is the
ratio of voltage to current, and is a measure of the electrical
conductivity of a hollow organ and its contents. Intraluminal
electrical impedance is inversely proportional to the electrical
conductivity of the luminal contents and the cross-sectional area
of the lumen. Air has a very low electrical conductivity and,
therefore, high impedance. Saliva and food cause an impedance decrease because of their increased conductivity. Luminal
dilatation results in a decrease in impedance, whereas luminal
contraction yields an impedance increase. Investigators have
established the impedance waveform characteristics that define
esophageal bolus transport. This allows for the characterization
of both esophageal function, via quantification of bolus transport, and gastroesophageal reflux (Fig. 25-22). The probe measures impedance between adjacent electrodes, with measuring
segments located at 2, 4, 6, 8, 14, and 16 cm from the distal tip.
An extremely low electric current of 0.00025 μW is transmitted
across the electrodes at a frequency of 1 to 2 kHz and is limited
Pharynx
Esophagus
40.3
43.7
PIP
42.3
Gastric 46.2
Stomac
1A. Normal high-resolution manometry motility study. Pressure measurements are recorded with color coding (red = high; blue = low). LES = lower esophageal sphincter; P
int; UES = upper esophageal sphincter.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
PART II
Pharynx
Esophagus
41.3 PIP
42.7 41.8
Stomach
Gastric 50.3
SPECIFIC CONSIDERATIONS
1B. High-resolution manometry motility study in patient with mechanically defective lower esophageal sphincter. Note the absence of lower esophageal sphincter tone. Press
corded with color coding (red = high; blue = low). LES = lower esophageal sphincter; PIP = pressure inversion point; UES = upper esophageal sphincter.
Pharynx
Esophagus
40.9
PIP
42.3
44.6
Gastric 47.5
Stomach
1C. High-resolution manometry motility study in patient with deficient esophageal body peristalsis. Note the very weak peristalsis in the lower two-thirds of the esopha
ts are recorded with color coding (red = high; blue = low). LES = lower esophageal sphincter; PIP = pressure inversion point; UES = upper esophageal sphincter.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
PART II
Pharynx
Esophagus
42.7
45.7
PIP
44.1
Stomach
Gastric 48.5
SPECIFIC CONSIDERATIONS
1D. High-resolution manometry motility study in patient with achalasia. Note the complete absence of esophageal body peristalsis, and the lack of relaxation of the lowe
essure measurements are recorded with color coding (red = high; blue = low). LES = lower esophageal sphincter; PIP = pressure inversion point; UES = upper esophageal
Pharynx
Esophagus
45.6
PIP
47.1
49.7 Stoma
Gastric 51.7
1E. High-resolution manometry motility study in patient with diffuse esophageal spasm. Note the very high amplitude contractions in the esophageal body. Pressure meas
h color coding (red = high; blue = low). LES = lower esophageal sphincter; PIP = pressure inversion point; UES = upper esophageal sphincter.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
1028
Impedence site
pH site
17cm
Distance above LES
PART II
Distance above LES
15cm
9cm
7cm
5cm
5cm
3cm
SPECIFIC CONSIDERATIONS
LES
Figure 25-22. Esophageal impedance probe measures electrical
resistance between evenly spaced electrodes. LES = lower esophageal sphincter.
to 8 μA. This is below the stimulation threshold for nerves and
muscles and is three orders of magnitude below the threshold of cardiac stimulation. A standard pH electrode is located
5 cm from the distal tip so that the acidic or nonacidic nature
of refluxate can be correlated with the number of reflux events.
Esophageal impedance has been validated as an appropriate method for the evaluation of GI function and is used selectively for the diagnosis of gastroesophageal reflux. It has been
compared to cineradiography showing that impedance waves
correspond well with actual bolus transport illustrated by radiography. Bolus entry, transit, and exit can be clearly identified
by impedance changes in the corresponding measuring segments. Studies comparing standard esophageal manometry with
impedance measurements in healthy volunteers have shown that
esophageal impedance correlates with peristaltic wave progression and bolus length.
Twenty-four-hour pH monitoring, the historical gold standard for diagnosing and quantifying gastroesophageal reflux,
has some significant limitations. With 24-hour ambulatory pH
testing, reflux is defined as a drop in the pH below 4, which
effectively “blinds” the test to reflux occurring at higher pH
values. Furthermore, in patients with persistent symptoms on
proton pump inhibitor (PPI) therapy, pH monitoring has limited use as it can only detect abnormal acid reflux (pH <4), the
occurrence of which has been altered by the antisecretory medication. Given that PPI antisecretory therapy is highly effective
in neutralizing gastric acid, the question of whether persistent
symptoms are a result of persistent acid reflux, nonacid reflux,
or are not reflux related becomes a key issue in surgical decision
making. Until recently, this differentiation could not be made.
Detection of both acid and nonacid reflux has potential to define
these populations of patients and thus improve patient selection
for antireflux surgery. Multichannel intraluminal impedance
technology allows the measurement of both acid and nonacid
reflux, with potential to enhance diagnostic accuracy.
Using this technology, Balaji and colleagues showed that
most gastroesophageal reflux remains despite acid suppression.
Impedance pH may be particularly useful in evaluating patients
with persistent symptoms despite PPI treatment, patients with
respiratory symptoms, and postoperative patients who are having symptoms that are elusive to diagnosis.
Esophageal Transit Scintigraphy. The esophageal transit of
a 10-mL water bolus containing technetium-99m (99mTc) sulfur
colloid can be recorded with a gamma camera. Using this technique, delayed bolus transit has been shown in patients with
a variety of esophageal motor disorders, including achalasia,
scleroderma, DES, and nutcracker esophagus.
Video- and Cineradiography
High-speed cinematic or video recording of radiographic studies
allows re-evaluation by reviewing the studies at various speeds.
This technique is more useful than manometry in the evaluation
of the pharyngeal phase of swallowing. Observations suggesting
oropharyngeal or cricopharyngeal dysfunction include misdirection of barium into the trachea or nasopharynx, prominence of
the cricopharyngeal muscle, a Zenker’s diverticulum, a narrow
pharyngoesophageal segment, and stasis of the contrast medium
in the valleculae or hypopharyngeal recesses (Fig. 25-23). These
findings are usually not specific, but rather common manifestations of neuromuscular disorders affecting the pharyngoesophageal area. Studies using liquid barium, barium-impregnated
solids, or radiopaque pills aid the evaluation of normal and
abnormal motility in the esophageal body. Loss of the normal
stripping wave or segmentation of the barium column with
the patient in the recumbent position correlates with abnormal
motility of the esophageal body. In addition, structural abnormalities such as small diverticula, webs, and minimal extrinsic impressions of the esophagus may be recognized only with
motion-recording techniques. The simultaneous computerized
capture of videofluoroscopic images and manometric tracings
is now available and is referred to as manofluorography. Manofluorographic studies allow precise correlation of the anatomic
events, such as opening of the upper esophageal sphincter, with
manometric observations, such as sphincter relaxation. Manofluorography, although not widely available, is presently the best
means available to evaluate complex functional abnormalities.
Tests to Detect Increased Exposure to
Gastric Juice
Twenty-Four-Hour Ambulatory pH Monitoring. The most
direct method of measuring increased esophageal exposure to gastric juice is by an indwelling pH electrode, or, more recently, via a
radiotelemetric pH monitoring capsule that can be clipped to the
esophageal mucosa. The latter consists of an antimony pH electrode fitted inside a small, capsule-shaped device accompanied
by a battery and electronics that allow 48-hour monitoring and
transmission of the pH data via transcutaneous radio telemetry to
a waist-mounted data logger. The device can be introduced either
transorally or transnasally, and it can be clipped to the esophageal
mucosa using endoscopic fastening techniques. It passes spontaneously within 1 to 2 weeks. Prolonged monitoring of esophageal
pH is performed by placing the pH probe or telemetry capsule
5 cm above the manometrically measured upper border of the distal sphincter for 24 hours. It measures the actual time the esophageal mucosa is exposed to gastric juice, measures the ability of the
esophagus to clear refluxed acid, and correlates esophageal acid
exposure with the patient’s symptoms. A 24- to 48-hour period
is necessary so that measurements can be made over one or two
complete circadian cycles. This allows measuring the effect of
physiologic activity, such as eating or sleeping, on the reflux of
gastric juice into the esophagus (Fig. 25-24).
1029
B
The 24-hour esophageal pH monitoring should not be considered a test for reflux, but rather a measurement of the esophageal exposure to gastric juice. The measurement is expressed
by the time the esophageal pH was below a given threshold
during the 24-hour period (Table 25-3). This single assessment, although concise, does not reflect how the exposure has
occurred; that is, did it occur in a few long episodes or several
pH
8
mp
mp
6
4
2
14:00
16:00
18:00
20:00
22:00
pH sp
8
short episodes? Consequently, two other assessments are necessary: the frequency of the reflux episodes and their duration.
The units used to express esophageal exposure to gastric
juice are: (a) cumulative time the esophageal pH is below a chosen threshold, expressed as the percentage of the total, upright,
and supine monitored time; (b) frequency of reflux episodes
below a chosen threshold, expressed as number of episodes
per 24 hours; and (c) duration of the episodes, expressed as the
number of episodes >5 minutes per 24 hours, and the time in
minutes of the longest episode recorded. Table 25-2 shows the
normal values for these components of the 24-hour record at the
whole-number pH threshold derived from 50 normal asymptomatic subjects. The upper limits of normal were established at the
95th percentile. Most centers use pH 4 as the threshold.
Based on these studies and extensive clinical experience,
48-hour esophageal pH monitoring is considered to be the gold
standard for the diagnosis of GERD.
The Bravo pH Capsule (Medtronics, Minneapolis, MN)
measures pH levels in the esophagus and transmits continuous
6
4
2
Table 25-2
22:00
pH
8
00:00
02:00
04:00
06:00
Normal values for esophageal exposure to pH <4 (n = 50)
COMPONENT
MEAN
SD
95%
6
Total time
1.51
1.36
4.45
4
Upright time
2.34
2.34
8.42
2
Supine time
0.63
1.0
3.45
No. of episodes
19.00
12.76
46.90
No. >5 min
0.84
1.18
3.45
Longest episode
6.74
7.85
19.80
06:00
mp
08:00
10:00
12:00
14:00
Figure 25-24. Strip chart display of a 24-hour esophageal pH
monitoring study in a patient with increased esophageal acid exposure. mp = meal period; sp = supine period. (Reproduced with permission from Zuidema GD, Orringer MB: Shackelford’s Surgery
of the Alimentary Tract, 3rd ed. Vol 1. Philadelphia, PA: Elsevier/
Saunders; 1991.)
SD = standard deviation.
Reproduced with permission from Moody FG, Carey LC, Jones RS,
et al: Surgical Treatment of Digestive Disease. Chicago, IL: Year Book
Medical; 1990.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
A
Figure 25-23. Esophagograms from a patient
with cricopharyngeal achalasia. A. Anteroposterior film showing retention of the contrast
medium at the level of the vallecula and piriform
recesses, with no barium passing into the esophagus. B. Lateral film, taken opposite the C5–C6
vertebrae, showing posterior indentation of the
cricopharyngeus, retention in the hypopharynx,
and tracheal aspiration. (Reproduced with permission from DeMeester TR, Matthews H: International Trends in General Thoracic Surgery.
Vol 3. Benign Esophageal Disease. St. Louis, Mo:
Mosby; 1987.)
1030
Table 25-3
Normal composite score for various pH thresholds:
upper level of normal value
PART II
pH THRESHOLD
95TH PERCENTILE
<1
14.2
<2
17.37
<3
14.10
<4
14.72
<5
15.76
<6
12.76
>7
14.90
>8
8.50
SPECIFIC CONSIDERATIONS
Reproduced with permission from Moody FG, Carey LC, Jones RS,
et al: Surgical Treatment of Digestive Disease. Chicago, IL: Year Book
Medical; 1990.
esophageal pH readings to a receiver worn on the patient’s belt
or waistband (Fig. 25-25). Symptoms that the patient experiences are recorded in a diary and/or by pressing buttons on
the receiver unit. Generally, 48 hours of pH data are measured
with this probe. A recent study has shown that the addition of
a second day of pH monitoring increased the sensitivity of pH
measurement by 22%. The capsule eventually detaches and
passes through the digestive tract in 5 to 7 days.
Radiographic Detection of Gastroesophageal Reflux. The
definition of radiographic gastroesophageal reflux varies depending on whether reflux is spontaneous or induced by various maneuvers. In only about 40% of patients with classic symptoms of
GERD is spontaneous reflux (i.e., reflux of barium from the stomach into the esophagus with the patient in the upright position)
by the radiologist. In most patients who show spon2 observed
taneous reflux on radiography, the diagnosis of increased
esophageal acid exposure is confirmed by 24-hour esophageal pH
monitoring. Therefore, the radiographic demonstration of spontaneous regurgitation of barium into the esophagus in the upright
position is a reliable indicator that reflux is present. However, failure to see this does not indicate the absence of disease, and for this
reason this test is rarely used for clinical diagnosis.
Tests of Duodenogastric Function
Esophageal disorders are frequently associated with abnormalities of duodenogastric function. Abnormalities of the gastric reservoir or increased gastric acid secretion can be responsible for
increased esophageal exposure to gastric juice. Reflux of alkaline duodenal juice, including bile salts, pancreatic enzymes,
and bicarbonate, is thought to have a role in the pathogenesis of
esophagitis and complicated Barrett’s esophagus. Furthermore,
functional disorders of the esophagus are often not confined to
pH
7
4
2
10:00
pH probe
12:00
14:00
16:00
18:00
20:00
22:00
00:00
02:00
04:00
06:00
08:00
10:00
7
4
5 cm
above
2
18:00
Combined 24-hour
gastric and esophageal
pH monitoring
5 cm
below
7
4
2
02:00
A
B
Figure 25-25. A. Combined esophageal and gastric pH monitoring showing position of probes in relation to the lower esophageal sphincter.
B. Combined ambulatory esophageal (upper tracing) and gastric (lower tracing) pH monitoring showing duodenogastric reflux (arrows) with
propagation of the alkaline juice into the esophagus of a patient with complicated Barrett’s esophagus. The gastric tracing (lower) is taken
from a probe lying 5 cm below the upper esophageal sphincter. The esophageal tracing (upper) is taken from a probe lying 5 cm above the
lower esophageal sphincter. Note that in only a small proportion of time does duodenogastric reflux move the pH of the esophagus above
the threshold of 7, causing the iceberg effect. (Reproduced with permission from Zuidema GD, Orringer MB: Shackelford’s Surgery of the
Alimentary Tract, 3rd ed. Vol 1. Philadelphia, PA: Elsevier/Saunders; 1991.)
the esophagus alone, but are associated with functional disorders of the rest of the foregut (i.e., stomach and duodenum).
Tests of duodenogastric function that are helpful to investigate
esophageal symptoms include gastric emptying studies, gastric
acid analysis, and cholescintigraphy (for the diagnosis of pathologic duodenogastric and/or duodenogastroesophageal reflux).
GASTROESOPHAGEAL REFLUX DISEASE
GERD was not recognized as a significant clinical problem until
the mid-1930s and was not identified as a precipitating cause for
esophagitis until after World War II. In the early 21st century,
it has grown to be a very common problem and now accounts
for a majority of esophageal pathology. It is recognized as a
chronic disease, and when medical therapy is required, it is often
lifelong treatment. Recent efforts at the development of various
endoscopic antireflux interventions, although innovative, have
not been successful in consistently controlling gastroesophageal
reflux. Antireflux surgery is an effective and long-term therapy
and is the only treatment that is able to restore the gastroesophageal barrier. Despite the common prevalence of GERD, it can be
one of the most challenging diagnostic and therapeutic problems
in clinical medicine. A contributing factor to this is the lack of
a universally accepted definition of the disease.
The most simplistic approach is to define the disease by
its symptoms. However, symptoms thought to be indicative of
GERD, such as heartburn or acid regurgitation, are very common in the general population and many individuals consider
them to be normal and do not seek medical attention. Even when
excessive, these symptoms are not specific for gastroesophageal
reflux. They can be caused by other diseases such as achalasia,
DES, esophageal carcinoma, pyloric stenosis, cholelithiasis,
gastritis, gastric or duodenal ulcer, and coronary artery disease.
A thorough, structured evaluation of the patient’s symptoms
is essential before any therapy, particularly any form of esophageal surgery. The presence and severity of both typical symptoms of heartburn, regurgitation, and dysphagia, and atypical
symptoms of cough, hoarseness, chest pain, asthma, and aspiration should be discussed with the patient in detail. Many of these
atypical symptoms may not be esophageal related and hence will
not improve and may even worsen with antireflux surgery.
Heartburn is generally defined as a substernal burningtype discomfort, beginning in the epigastrium and radiating
upward. It is often aggravated by meals, spicy or fatty foods,
chocolate, alcohol, and coffee and can be worse in the supine
position. It is commonly, although not universally, relieved by
antacid or antisecretory medications. Epidemiologic studies
have shown that heartburn occurs monthly in as many as 40%
1031
American Gastroenterologic Association Gallup poll on
nighttime gastroesophageal reflux disease symptoms
• 50 million Americans have nighttime heartburn at least 1/wk
• 80% of heartburn sufferers had nocturnal symptoms—65%
both day & night
• 63% report that it affects their ability to sleep and impacts
their work the next day
• 72% are on prescription medications
• Nearly half (45%) report that current remedies do not
relieve all symptoms
to 50% of the Western population. The occurrence of heartburn
at night and its effect on quality of life have recently been highlighted by a Gallup poll conducted by the American Gastroenterologic Society (Table 25-4).
Regurgitation, the effortless return of acid or bitter gastric
contents into the chest, pharynx, or mouth, is highly suggestive
of foregut pathology. It is often particularly severe at night when
supine or when bending over and can be secondary to either an
incompetent or obstructed GEJ. With the latter, as in achalasia,
the regurgitant is often bland, as if food was put into a blender.
When questioned, most patients can distinguish the two. It is the
regurgitation of gastric contents that may result in associated
pulmonary symptoms, including cough, hoarseness, asthma,
and recurrent pneumonia. Bronchospasm can be precipitated by
esophageal acidification and cough by either acid stimulation or
distention of the esophagus.
Dysphagia, or difficulty swallowing, is a relatively nonspecific term but arguably the most specific symptom of foregut
disease. It can be a sign of underlying malignancy and should be
aggressively investigated until a diagnosis is established. Dysphagia refers to the sensation of difficulty in the passage of food from
the mouth to the stomach and can be divided into oropharyngeal
and esophageal etiologies. Oropharyngeal dysphagia is characterized by difficulty transferring food out of the mouth into the
esophagus, nasal regurgitation, and/or aspiration. Esophageal dysphagia refers to the sensation of food sticking in the lower chest or
epigastrium. This may or may not be accompanied by pain (odynophagia) that will be relieved by the passage of the bolus.
Chest pain, although commonly and appropriately attributed to cardiac disease, is frequently secondary to esophageal
pathology as well. Nearly 50% of patients with severe chest
pain, normal cardiac function, and normal coronary arteriograms have positive 24-hour pH studies, implicating gastroesophageal reflux as the underlying etiology. Exercise-induced
gastroesophageal reflux is well known to occur, and may result
in exertional chest pain similar to angina. It can be quite difficult, if not impossible, to distinguish between the two etiologies,
particularly on clinical grounds alone. Nevens and colleagues
evaluated the ability of experienced cardiologists to differentiate
pain of cardiac vs. esophageal origin. Of 248 patients initially
seen by cardiologists, 185 were thought to have typical angina,
and 63 were thought to have atypical chest pain. Forty-eight
(26%) of those thought to have classic angina had normal coronary angiograms, and 16 of the 63 with atypical pain had abnormal angiogram. Thus, the cardiologists’ clinical impression was
wrong 25% of the time. Finally, Pope and associates investigated the ultimate diagnosis in 10,689 patients presenting to an
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Gastric Emptying Study. Gastric emptying studies are performed
with radionuclide-labeled meals. Emptying of solids and liquids
can be assessed simultaneously when both phases are marked with
different tracers. After ingestion of a labeled standard meal, gamma
camera images of the stomach are obtained at 5- to 15-minute intervals for 2 to 4 hours. After correction for decay, the counts in the
gastric area are plotted as the percentage of total counts at the start
of the imaging. The resulting emptying curve can be compared with
data obtained in normal volunteers. In general, normal subjects will
empty 59% of a meal within 90 minutes. Although delayed gastric emptying is often associated with gastroesophageal reflux, in
general delayed emptying does not correlate with a poorer clinical
outcome after antireflux surgery, and it should not be considered a
contraindication to surgical treatment.
Table 25-4
PART II
SPECIFIC CONSIDERATIONS
emergency department with acute chest pain. Approximately
17% were found to have acute ischemia, 6% had stable angina,
21% had other cardiac causes, and 55% had noncardiac causes.
The investigators concluded that the majority of people presenting to the emergency department with chest pain do not have an
underlying cardiac etiology for their symptoms. Chest pain precipitated by meals, occurring at night while supine, nonradiating, responsive to antacid medication, or accompanied by other
symptoms suggesting esophageal disease such as dysphagia or
regurgitation should trigger the thought of possible esophageal
origin. Furthermore, the distinction between heartburn and chest
pain is also difficult and largely dependent upon the individual
patient. One person’s heartburn is another’s chest pain.
The precise mechanisms accounting for the generation of
symptoms secondary to esophageal pathology remain unclear.
Considerable insight has been acquired, however. Investigations into the effect of luminal content, esophageal distention
and muscular function, neural pathways, and brain localization
have provided a basic understanding of the stimuli responsible
for symptom generation. It is also clear that the visceroneural
pathways of the foregut are complexly intertwined with that of
the tracheobronchial tree and heart. This fact accounts for the
common overlap of clinical presentations with diverse disease
processes in upper GI, cardiac, and pulmonary systems.
The Human Antireflux Mechanism and the
Pathophysiology of Gastroesophageal Reflux
Disease
There is a high-pressure zone located at the esophagogastric junction in humans. Although this is typically referred to as the lower
esophageal “sphincter,” there are no distinct anatomical landmarks that define its beginning and end. Architecturally speaking, there is a specialized thickening in this region that is made
up of the collar sling musculature and the clasp fibers. The collar
sling is located on the greater curvature side of the junction, and
the clasp fibers are located on the lesser curvature side. These
muscles remain in tonic opposition until the act of swallowing,
whereupon receptive relaxation occurs allowing passage of a food
bolus into the stomach. In addition, the LES will also open when
the gastric fundus is distended with gas and liquid, thus resulting
in an unfolding of the valve and enabling venting of gas (a belch).
Whether physiologic or pathologic, the common denominator for
most episodes of gastroesophageal reflux is the loss of the highpressure zone and thus a decrease in the resistance it imparts to
the retrograde flow of gastric juice into the esophageal body.
Table 25-5
Normal manometric values of the distal esophageal
sphincter, n = 50
PARAMETER
MEDIAN 2.5TH
97.5TH
VALUE PERCENTILE PERCENTILE
Pressure (mmHg)
13
5.8
27.7
Overall length (cm)
3.6
2.1
5.6
0.9
4.7
Abdominal length (cm) 2
A third characteristic of the LES that impacts its ability to
prevent reflux is its position about the diaphragm. It is important
that a portion of the total length of the LES be exposed to the
effects of an intra-abdominal pressure. That is, during periods of
elevated intra-abdominal pressure, the resistance of the barrier
would be overcome if pressure were not applied equally to both
the LES and stomach simultaneously. Thus, in the presence of
a hiatal hernia, the sphincter resides entirely within the chest
cavity and cannot respond to an increase in intra-abdominal
pressure because the pinch valve mechanism is lost and gastroesophageal reflux is more liable to occur.
Therefore, a permanently defective sphincter is defined
by one or more of the following characteristics: an LES with a
mean resting pressure of less than 6 mmHg, an overall sphincter
length of <2 cm, and intra-abdominal sphincter length of
<1 cm. Compared to normal subjects without GERD these values
are below the 2.5 percentile for each parameter. The most common cause of a defective sphincter is an inadequate abdominal
length.
Once the sphincter is permanently defective, this condition is irreversible, and although esophageal mucosal injury may
be healed with antisecretory medication, reflux will continue
to occur. Additionally, the presence of a defective LES may
be associated with reduced esophageal body function and thus
decrease clearance times of refluxed material. In addition, the
progressive loss of effective esophageal clearance may predispose the patient to severe mucosal injury, volume regurgitation,
aspiration, and pulmonary injury. Reflux may occur in the face
of a normal LES resting pressure. This condition is usually
due to a functional problem of gastric emptying or excessive
air swallowing. These conditions may lead to gastric distention, increased intra-gastric pressure, a resultant shortening or
The Lower Esophageal Sphincter. As defined by esophageal
manometry, there are three characteristics of the LES that work
in unison to maintain its barrier function. These characteristics
include the resting LES pressure, its overall length, and the
intra-abdominal length that is exposed to the positive pressure
environment of the abdomen (Table 25-5). The resistance to
gastroesophageal reflux is a function of both the resting LES
pressure and length over which this pressure is exerted. Thus, as
the sphincter becomes shorter, a higher pressure will be required
in order to prevent a given amount of reflux (Fig. 25-26). Much
like the neck of a balloon as it is inflated, as the stomach fills
and distends, sphincter length decreases. Therefore, if the overall length of the sphincter is permanently short from repeated
distention of the fundus secondary to large volume meals, then
with minimal episodes of gastric distention and pressure, there
will be insufficient sphincter length for the barrier to remain
competent, and reflux will occur.
24
LES pressure (mmHg)
1032
18
Competent
12
Incompetent
6
0
0
1
2
3
LES length (cm)
4
5
Figure 25-26. As the esophageal sphincter becomes shorter,
increased pressure is necessary to maintain competence. LES =
lower esophageal sphincter.
Relationship Between Hiatal Hernia and Gastroesophageal Reflux Disease. As the collar sling musculature and
clasp fibers become attenuated with repeated gastric distention,
the esophagogastric junction begins to assume an “upside down
funnel” appearance, with progressive opening of the acute angle
of His. This in turn may result in attenuation and stretching of
the phrenoesophageal ligament, with subsequent enlargement of
the hiatal opening and axial herniation. There is a high degree
of correlation between reflux threshold and the degree of hiatal
herniation (Fig. 25-27).
Summary. It is believed that GERD has its origins within the
stomach. Distention of the fundus occurs because of overeating and delayed gastric emptying secondary to a high-fat diet.
The resultant distention causes “unrolling” of the sphincter by
the expanding fundus, and this subsequently exposes the squamous epithelium in the region of the distal LES to gastric juice.
Repeated exposure results in inflammation and the development
of columnar epithelium at the cardia. This is the initial step of
the development of carditis and explains why in early disease
esophagitis is mild and commonly limited to the very distal
aspect of the esophagus. The patient attempts to compensate for
this by increased swallowing, allowing the saliva to neutralize the
refluxed gastric juice and thus, alleviate the discomfort induced
by the reflux event. The increased swallowing results in aerophagia, bloating, and belching. This in turn creates a vicious cycle of
increased gastric distention and thus further exposure and repetitive injury to the distal esophagus. The development of carditis
explains the complaint of epigastric pain often experienced by
patients with early reflux disease. Additionally, this process can
lead to a fibrotic mucosal ring located at the squamocolumnar
junction, which is termed a “Schatzki ring” and which may result
in dysphagia. This inflammatory process may extend into muscularis propria and thus result in a progressive loss in the length and
pressure of the LES. This explanation for the pathophysiology of
GERD is supported by the observation that severe esophagitis is
almost always associated with a defective LES.
Complications Associated With
Gastroesophageal Reflux Disease
The complications of gastroesophageal reflux disease may result
from the direct injurious effects of gastric fluid on the mucosa,
larynx, or respiratory epithelium. Complications due to repetitive
reflux are esophagitis, stricture, and BE; repetitive aspiration
may lead to progressive pulmonary fibrosis. The severity of the
complications is directly related to the prevalence of a structurally
defective sphincter (Table 25-6). The observation that a
structurally defective sphincter occurs in 42% of patients without
complications (most of whom have one or two components
failed) suggests that disease may be confined to the sphincter
due to compensation by a vigorously contracting esophageal
body. Eventually, all three components of the sphincter fail,
allowing unrestricted reflux of gastric juice into the esophagus
and overwhelming its normal clearance mechanisms. This leads
to esophageal mucosal injury with progressive deterioration of
esophageal contractility, as is commonly seen in patients with
strictures and BE. The loss of esophageal clearance increases the
potential for regurgitation into the pharynx with aspiration.
Table 25-6
Complications of gastroesophageal reflux disease: 150
consecutive cases with proven gastroesophageal reflux
disease (24-hour esophageal pH monitoring endoscopy,
and motility)
40
36
Yield pressure (mmHg)
32
24
COMPLICATION
NO.
STRUCTURALLY
NORMAL
SPHINCTER (%)
20
None
59
58
42
16
Erosive
esophagitis
47
23
77a
12
Stricture
19
11
89
8
Barrett’s
esophagus
25
0
100
Total
150
28
4
0
No hernia
< 3 cm hernia
3 cm hernia
Figure 25-27. Yield pressure of the lower esophageal sphincter
decreases as hiatal hernia size increases.
STRUCTURALLY
DEFECTIVE
SPHINCTER (%)
Grade more severe with defective cardia.
Reproduced with permission from Moody FG, Carey LC, Jones RS,
et al: Surgical Treatment of Digestive Disease. Chicago, IL: Year Book
Medical; 1990.
a
1033
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
unfolding of the LES, and subsequent reflux. The mechanism
by which gastric distention contributes to LES unfolding provides a mechanical explanation for “transient LES relaxation.”
It is thought that with repeated gastric distention secondary to
large meal volume or chronic air swallowing, there is repeated
unfolding of the LES and subsequent attenuation of the collar
sling musculature. It is at this point that the physiologic and normal mechanism of gastric venting is replaced with pathologic
and severe postprandial reflux disease. In addition, patients with
GERD will increase the frequency of swallowing in an effort to
neutralize the refluxed acid with their saliva (pH 7.0). This phenomenon leads to increased air swallowing and further gastric
distention, thus compounding the problem. Therefore, GERD
may have its origins in the stomach secondary to gastric distention due to overeating/drinking, air swallowing, or consumption of carbonated liquids, and this may be further compounded
by the ingestion of fatty meals, which result in delayed gastric
emptying.
70
Prevalence
60
50
%
40
30
20
10
0
Gastric reflux
(n = 22)
Mixed reflux
(n = 31)
A
20
% Time
SPECIFIC CONSIDERATIONS
15
10
5
0
pH
<4
pH
4–7
pH
>7
B
Figure 25-29. A. Prevalence of reflux types in 53 patients with
gastroesophageal reflux disease. B. Esophageal luminal pH during bilirubin exposure. (Reproduced with permission from Kauer
WK, Peters JH, DeMeester TR, etal: Mixed reflux of gastric and
duodenal juices is more harmful to the esophagus than gastric juice
alone. The need for surgical therapy re-emphasized, Ann Surg.
1995 Oct;222(4):525-531.)
Prevalence of patients with increased bilirubin
350
10
9
300
80
8
250
7
200
6
150
4
5
60
3
100
50
0
100
pH
PART II
The potential injurious components that reflux into the
esophagus include gastric secretions such as acid and pepsin,
as well as biliary and pancreatic secretions that regurgitate from
the duodenum into the stomach. There is a considerable body of
experimental evidence to indicate that maximal epithelial injury
occurs during exposure to bile salts combined with acid and
pepsin. These studies have shown that while acid alone does
minimal damage to the esophageal mucosa, the combination of
acid and pepsin is highly deleterious. Similarly, the reflux of
duodenal juice alone does little damage to the mucosa, although
the combination of duodenal juice and gastric acid is particularly noxious.
Complications of gastroesophageal reflux such as esophagitis, stricture, and Barrett’s metaplasia occur in the presence
of two predisposing factors: a mechanically defective LES and
an increased esophageal exposure to fluid containing duodenal
content that includes bile and pancreatic juice. The duodenal
origin of esophageal contents in patients with an increased
exposure to a pH >7 has previously been confirmed by esophageal aspiration studies (Fig. 25-28). Studies have clarified and
expanded these observations by measuring esophageal bilirubin
exposure over a 24-hour period as a marker for the presence
of duodenal juice. Direct measurement of esophageal bilirubin
exposure as a marker for duodenal juice has shown that 58%
of patients with GERD have increased esophageal exposure to
duodenal juice and that this exposure occurs most commonly
when the esophageal pH is between 4 and 7 (Fig. 25-29). These
earlier studies have been confirmed by other studies that measure volume reflux using impedance technology (Fig. 25-30).
If reflux of gastric juice is allowed to persist and sustained
or repetitive esophageal injury occurs, two sequelae can result.
First, a luminal stricture can develop from submucosal and eventually intramural fibrosis. Second, the tubular esophagus may
become replaced with columnar epithelium. The columnar epithelium is resistant to acid and is associated with the alleviation
of the complaint of heartburn. This columnar epithelium often
becomes intestinalized, identified histologically by the presence
Bile acid conc. umol/l
1034
18:00
06:00
2
1
0
Time
Figure 25-28. Sample bile acid concentration and esophageal pH
plotted against time to obtain detailed profiles; in this case showing
both significant bile acid (vertical bars) and acid (linear plot) reflux.
(Reproduced with permission from Nehra D, Watt P, Pye JK, et al.
Automated oesophageal reflux sampler: a new device used to monitor bile acid reflux in patients with gastroesophageal reflux disease,
J Med Eng Technol. 1997 Jan-Feb;21(1):1-9.)
40
20
0
Normal
subjects
n = 25
No mucosal
Erosive
injury
esophagitis
n = 16
n = 10
Barrett’s
esophagus
n = 27
Figure 25-30. Prevalence of abnormal esophageal bilirubin exposure in healthy subjects and in patients with gastroesophageal reflux
disease with varied degrees of mucosal injury. (*P <.03 vs. all other
groups; **P <.03 vs. healthy subjects.) (Reproduced with permission from Kauer WK, Peters JH, DeMeester TR, et al: Mixed reflux
of gastric and duodenal juices is more harmful to the esophagus
than gastric juice alone. The need for surgical therapy re-emphasized,
Ann Surg. 1995 Oct;222(4):525-531.)
Metaplastic (Barrett’s Esophagus) and
Neoplastic (Adenocarcinoma) Complications
The condition whereby the tubular esophagus is lined with
columnar epithelium rather than squamous epithelium was first
by Norman Barrett in 1950. He incorrectly
3 described
believed it to be congenital in origin. It is now realized that
it is an acquired abnormality, occurs in 10% to 15% of patients
with GERD, and represents the end stage of the natural history
of this disease. It is also distinctly different from the congenital
condition in which islands of gastric fundic epithelium are
found in the upper half of the esophagus.
The definition of BE has evolved considerably over the
past decade. Traditionally, BE was identified by the presence
of columnar mucosa extending at least 3 cm into the esophagus.
It is now recognized that the specialized, intestinal-type epithelium, or intestinal metaplasia (IM) found in the Barrett’s
mucosa, is the only tissue predisposed to malignant degeneration. Consequently, the diagnosis of BE is presently made given
any length of endoscopically identifiable columnar mucosa
that proves, on biopsy, to show IM. Although long segments
of columnar mucosa without IM do occur, they are uncommon
and might be congenital in origin.
The hallmark of IM is the presence of intestinal goblet
cells. There is a high prevalence of biopsy-demonstrated IM at
the cardia, on the gastric side of the squamocolumnar junction,
in the absence of endoscopic evidence of a CLE. Evidence is
accumulating that these patches of what appears to be Barrett’s
in the cardia have a similar malignant potential as in the longer
segments, and are precursors for carcinoma of the cardia.
The long-term relief of symptoms remains the primary reason for performing antireflux surgery in patients with BE. Healing of esophageal mucosal injury and the prevention of disease
progression are important secondary goals. In this regard,
patients with BE are no different than the broader population
of patients with gastroesophageal reflux. They should be considered for antireflux surgery when patient data suggest severe
disease or predict the need for long-term medical management.
Most patients with BE are symptomatic. Although it has been
argued that some patients with BE may not have symptoms,
careful history taking will reveal the presence of symptoms in
most, if not all, patients.
Patients with BE have a spectrum of disease ranging from
visually identifiable but short segments, to long segments of
classic BE. In general, however, they represent a relatively
severe stage of gastroesophageal reflux, usually with markedly
increased esophageal acid exposure, deficient LES characteristics, poor esophageal body function, and a high prevalence of
duodenogastroesophageal reflux. Gastric hypersecretion occurs
in 44% of patients. Most will require long-term PPI therapy for
relief of symptoms and control of coexistent esophageal mucosal injury. Given such profound deficits in esophageal physiology, antireflux surgery is an excellent means of long-term
control of reflux symptoms for most patients with BE.
The typical complications in BE include ulceration in the
columnar-lined segment, stricture formation, and a dysplasiacancer sequence. Barrett’s ulceration is unlike the erosive
ulceration of reflux esophagitis in that it more closely resembles peptic ulceration in the stomach or duodenum, and has the
same propensity to bleed, penetrate, or perforate. Fortunately,
this complication occurs very rarely. The strictures found in BE
occur at the squamocolumnar junction, and they are typically
higher than peptic strictures in the absence of BE. Ulceration
and stricture in association with BE were commonly reported
before 1975, but with the advent of potent acid suppression
medication, they have become less common. In contrast, the
complication of adenocarcinoma developing in Barrett’s mucosa
has become more common. Adenocarcinoma developing in Barrett’s mucosa was considered a rare tumor before 1975. Today,
it occurs at approximately 0.2% to 0.5% per year of followup, which represents a risk 40 times that of the general population. Most, if not all, cases of adenocarcinoma of the esophagus
arise in Barrett’s epithelium (Fig. 25-31). About one-third of all
patients with BE present with malignancy.
The long-term risk of progression to dysplasia and adenocarcinoma, although not the driving force behind the decision to perform antireflux surgery, is a significant concern for
both patient and physician. Although to date, there have been
no prospective randomized studies documenting that antireflux
surgery has an effect on the risk of progression to dysplasia and
carcinoma, complete control of reflux of gastric juice into the
esophagus is clearly a desirable goal.
Respiratory Complications
A significant proportion of patients with GERD will have
associated respiratory symptoms. These patients may have
laryngopharyngeal reflux-type symptoms, adult-onset asthma,
or even idiopathic pulmonary fibrosis. These symptoms and
organ injury may occur in isolation or in conjunction with typical reflux symptoms such as heartburn and regurgitation. Several studies have demonstrated that up to 50% of patients with
asthma have either endoscopically evident esophagitis or abnormal distal esophageal acid exposure. These findings support a
causal relationship between GERD and aerodigestive symptoms
and complications in a proportion of patients.
1035
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
of goblet cells. This specialized IM is currently required for the
diagnosis of BE. Endoscopically, BE can be quiescent or associated with complications of esophagitis, stricture, Barrett’s ulceration, and dysplasia. The complications associated with BE may
be due to the continuous irritation from refluxed duodenogastric
juice. This continued injury is pH dependent and may be modified by medical therapy. The incidence of metaplastic Barrett’s
epithelium becoming dysplastic and progressing to adenocarcinoma is approximately 0.2% to 0.5% per year.
An esophageal stricture can be associated with severe
esophagitis or BE. In the latter situation, it occurs at the site of
maximal inflammatory injury (i.e., the columnar-squamous epithelial interface). Patients who have a stricture in the absence of
Barrett’s esophagus should have the presence of gastroesophageal reflux documented before the presence of the stricture
is ascribed to reflux esophagitis. In patients with normal acid
exposure and no endoscopic or CT evidence of cancer, the stricture may be a result of a drug-induced chemical injury, the latter
resulting from the lodgment of a capsule or tablet in the distal
esophagus. In such patients, dilation usually corrects the problem of dysphagia. It is also possible for drug-induced injuries to
occur in patients who have underlying esophagitis and a distal
esophageal stricture secondary to gastroesophageal reflux. In
this situation, a long, string-like stricture progressively develops as a result of repetitive caustic injury from capsule or tablet
lodgment on top of an initial reflux stricture. These strictures
are often resistant to dilation. The incidence of this problem
has lessened since the introduction of proton pump inhibitor
medication.
1036
PART II
SPECIFIC CONSIDERATIONS
A
B
Figure 25-31. Photomicrographs. A. Barrett’s epithelium with severe dysplasia. (×200.) Note nuclear irregularity, stratification, and loss of
polarity. B. Barrett’s epithelium with intramucosal carcinoma. (×66.) Note malignant cells in the mucosa (upper arrow), but not invading the
muscularis mucosae (bottom arrow). (Reproduced with permission from Zuidema GD, Orringer MB: Shackelford’s Surgery of the Alimentary
Tract, 3rd ed. Vol 1. Philadelphia, PA: Elsevier/Saunders; 1991.)
Etiology of Reflux-Induced Respiratory Symptoms. There
are two mechanisms that have been proposed as the cause of
reflux-induced respiratory symptoms. The reflux theory suggests that these symptoms are the direct result of laryngopharyngeal exposure and aspiration of gastric contents. The reflex
theory suggests that the vagal-mediated afferent fibers result
in bronchoconstriction during episodes of distal esophageal
acidification. The evidence supporting a mechanism of direct
exposure to the aerodigestive system is based in clinical studies
that have documented a strong correlation between idiopathic
pulmonary fibrosis and hiatal hernia. In addition, the presence
of GERD was demonstrated to be highly associated with several
pulmonary diseases in a recent Department of Veteran Affairs
multivariate analysis. Next, with ambulatory pH testing, acid
exposure within the proximal esophagus is more frequently
identified in patients with gastroesophageal reflux and respiratory symptoms than in patients who have gastroesophageal
reflux symptoms alone. These findings are supported by scintigraphic studies, which have demonstrated aspiration of ingested
radioisotope in patients with both gastroesophageal reflux and
pulmonary symptoms. In animal studies, tracheal instillation of
acid has been demonstrated to profoundly increase airway resistance. Finally, in patients who have undergone multichannel
intraluminal impedance testing with a catheter configured to
detect laryngopharyngeal reflux, a correlation between proximal fluid movement and laryngopharyngeal symptoms, such as
cough, can be demonstrated.
The reflex mechanism is supported by the bronchoconstriction that occurs with the infusion of acid into the distal
esophagus. There is a shared embryologic origin of the tracheoesophageal tract and vagus nerve, and this reflex is thought to be
an afferent fiber–mediated reflex that protects the aerodigestive
system from the aspiration of refluxate. In patients with respiratory symptoms and documented gastroesophageal reflux without proximal esophageal acid exposure, pulmonary symptoms
will often times significantly improve or completely resolve
after undergoing laparoscopic fundoplication. It is likely that
both of the proposed mechanisms work simultaneously to cause
these symptoms in the face of GERD.
The most difficult clinical challenge in formulating a treatment plan for reflux-associated respiratory symptoms resides
in establishing the diagnosis. Although the diagnosis may be
straightforward in patients with predominately typical reflux
symptoms and secondary respiratory complaints, a substantial number of patients will have respiratory symptoms that
dominate the clinical scenario. Typical gastroesophageal reflux
Treatment. Once the diagnosis is established, treatment may
be initiated with either PPI therapy or antireflux surgery. A trial
of high-dose PPI therapy may help establish that reflux is partly
or completely responsible for the respiratory symptoms. It is
important to note that the persistence of symptoms in the face
of aggressive PPI treatment does not necessarily rule out reflux
as a possible cofactor or sole etiology.
Although there is probably some element of a placebo
effect, relief of respiratory symptoms can be anticipated in up
to 50% of patients with reflux-induced asthma treated with antisecretory medications. However, when examined objectively,
<15% of patients can be expected to have improvement in their
pulmonary function with medical therapy. In properly selected
patients, antireflux surgery improves respiratory symptoms
in nearly 90% of children and 70% of adults with asthma and
reflux disease. Improvements in pulmonary function can be
demonstrated in around 30% of patients. Uncontrolled studies
of the two forms of therapy (PPI and surgery) and the evidence
from the two randomized controlled trials of medical vs. surgical therapy indicate that surgical valve reconstruction is the
most effective therapy for reflux-induced asthma. The superiority of the surgery over PPI is most noticeable in the supine
position, which corresponds with the nadir of PPI blood levels
and resultant acid breakthrough and is the time in the circadian
cycle when asthma symptoms are at their worst.
In asthmatic patients with an esophageal motility disorder,
performing an antireflux operation will not prevent the regurgitation and possible aspiration of swallowed liquid or food
“upstream” to the valve reconstruction. It is critical that esophageal body function be considered prior to surgical intervention
in this patient population.
Medical Therapy for Gastroesophageal Reflux Disease.
With the widespread availability of over-the-counter antisecretory medications, most patients with mild or moderate symptoms will carry self-medication. When initially identified with
mild symptoms of uncomplicated GERD, patients can be placed
on 12 weeks of simple antacids before diagnostic testing is initiated. This approach may successfully and completely resolve
the symptoms. Patients should be counseled to elevate the head
of the bed; avoid tight-fitting clothing; eat small, frequent meals;
avoid eating the nighttime meal immediately prior to bedtime;
and avoid alcohol, coffee, chocolate, and peppermint, which
are known to reduce resting LES pressure and may aggravate
symptoms.
Used in combination with simple antacids, alginic acid
may augment the relief of symptoms by creating a physical barrier to reflux, as well as by acid reduction. Alginic acid reacts
with sodium bicarbonate in the presence of saliva to form a
highly viscous solution that floats like a raft on the surface of
the gastric contents. When reflux occurs, this protective layer
is refluxed into the esophagus, and acts as a protective barrier
against the noxious gastric contents. Medications to promote
gastric emptying, such as metoclopramide or domperidone,
are beneficial in early disease but of little value in more severe
disease.
In patients with persistent symptoms, the mainstay of
medical therapy is acid suppression. High-dosage regimens of
hydrogen potassium PPIs, such as omeprazole (up to 40 mg/d),
can reduce gastric acidity by as much as 80% to 90%. This usually heals mild esophagitis. In severe esophagitis, healing may
occur in only one-half of the patients. In patients who reflux
a combination of gastric and duodenal juice, acid-suppression
therapy may give relief of symptoms, while still allowing mixed
reflux to occur. This can allow persistent mucosal damage in
an asymptomatic patient. Unfortunately, within 6 months of
discontinuation of any form of medical therapy for GERD,
80% of patients have a recurrence of symptoms, and 40% of
individuals with daily GERD eventually develop symptoms
that “breakthrough” adequately dosed PPIs. Once initiated,
most patients with GERD will require lifelong treatment with
PPIs, both to relieve symptoms and to control any coexistent
esophagitis or stricture. Although control of symptoms has historically served as the endpoint of therapy, the wisdom of this
approach has recently been questioned, particularly in patients
with BE. Evidence suggesting that reflux control may prevent
the development of adenocarcinoma and lead to regression of
dysplastic and nondysplastic Barrett’s segments has led many
to consider control of reflux, and not symptom control, a better
therapeutic endpoint. However, this hypothesis remains controversial. It should be noted that complete control of reflux using
PPIs can be difficult, as has been highlighted by studies of acid
breakthrough while on PPI therapy and of persistent reflux following antireflux surgery. Castell, Triadafilopoulos, and others
have shown that 40% to 80% of patients with BE continue to
have abnormal esophageal acid exposure despite up to 20 mg
twice daily of PPIs. Ablation trials have shown that mean doses
of 56 mg of omeprazole were necessary to normalize 24-hour
esophageal pH studies. It is likely that antireflux surgery results
in more reproducible and reliable elimination of reflux of both
acid and duodenal contents, although long-term outcome studies
suggest that as many as 25% of postfundoplication patients will
have persistent pathologic esophageal acid exposure confirmed
by positive 24-hour pH studies.
Suggested Therapeutic Approach. Traditionally a stepwise
approach is used for the treatment of GERD. First-line therapy
entails antisecretory medication, usually PPIs, in most patients.
Failure of medication to adequately control GERD symptoms
suggests either that the patient may have relatively severe disease or a non-GERD cause for his or her symptoms. Endoscopic
examination at this stage of the patient’s evaluation is recommended and will provide the opportunity to assess the degree of
mucosal injury and presence of BE. Treatment options for these
patients entails either long term PPI use vs. antireflux surgery.
Laparoscopic antireflux surgery in these patients achieves longterm control of symptoms in 85% to 90%. The measurement
1037
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
symptoms, such as heartburn and regurgitation, may often be
completely absent only to be uncovered with objective esophageal physiology testing. Traditionally, the diagnosis of refluxinduced respiratory injury is established using ambulatory dual
probe pH monitoring, with one probe positioned within the distal esophagus and the other at a proximal location. Proximal
probe positioning has included multiple locations such as the
trachea, pharynx, and proximal esophagus. Although ambulatory esophageal pH monitoring allows a direct correlation
between esophageal acidification and respiratory symptoms,
sensitivity of this testing modality is poor, and the temporal relationship between laryngeal or pulmonary symptoms and reflux
events is complex. In addition, as the refluxed gastric fluid travels proximally, it may be neutralized by saliva and therefore
go undetected with pH monitoring. Impedance testing may also
be used to detect the movement of fluid throughout the entire
esophageal column regardless of pH content.
1038
of esophageal acid exposure via 24-hour pH should be undertaken when patients are considered for surgery. The status of
the LES and esophageal body function with esophageal manometry should also be performed at this stage. These studies will
serve to establish the diagnosis and assess esophageal body
dysfunction.
Surgical Therapy for Gastroesophageal
Reflux Disease
Selection of Patients for Surgery. Studies of the natural
PART II
SPECIFIC CONSIDERATIONS
history of GERD indicate that most patients have a relatively
benign form of the disease that is responsive to lifestyle changes
and dietary and medical therapy and do not need surgical treatment. Approximately 25% to 50% of the patients with GERD
have persistent or progressive disease, and it is this patient population that is best suited to surgical therapy. In the past, the
presence of esophagitis and a structurally defective LES were
the primary indications for surgical treatment, and many internists and surgeons were reluctant to recommend operative procedures in their absence. However, one should not be deterred
from considering antireflux surgery in a symptomatic patient
with or without esophagitis or a defective sphincter, provided
the disease process has been objectively documented by 24-hour
pH monitoring. This is particularly true in patients who have
become dependent upon therapy with PPIs, or require increasing
doses to control their symptoms. It is important to note that a
good response to medical therapy in this group of patients predicts an excellent outcome following antireflux surgery.
In general, the key indications for antireflux surgery are
(a) objectively proven gastroesophageal reflux disease, and (b)
typical symptoms of gastroesophageal reflux disease (heartburn
and/or regurgitation) despite adequate medical management, or
(c) a younger patient unwilling to take lifelong medication. In
addition, a structurally defective LES can also predict which
patients are more likely to fail with medical therapy. Patients
with normal sphincter pressures tend to remain well controlled
with medical therapy, whereas patients with a structurally defective LES may not respond as well to medical therapy, and often
develop recurrent symptoms within 1 to 2 years of beginning
therapy. Such patients should be considered for an antireflux
operation, regardless of the presence or absence of endoscopic
esophagitis.
Young patients with documented reflux disease with or
without a defective LES are also excellent candidates for antireflux surgery. They usually will require long-term medical
therapy for control of their symptoms, and some will go on to
develop complications of the disease. An analysis of the cost of
therapy based on data from the Veterans Administration Cooperative trial indicates that surgery has a cost advantage over
medical therapy in patients <49 years of age.
Severe endoscopic esophagitis in a symptomatic patient
with a structurally defective LES is also an indication for early
surgical therapy. These patients are prone to breakthrough of
their symptoms while receiving medical therapy. Symptoms and
mucosal injury can be controlled in such patients, but careful
monitoring is required, and increasing dosages of PPIs are necessary. In everyday clinical practice, however, such treatment
can be both difficult and impractical, and, in such cases, antireflux surgery can be considered early, especially if PPI therapy
is problematic.
The development of a stricture in a patient represents a failure of medical therapy, and it is also an indication for a surgical
antireflux procedure. In addition, strictures are often associated
with a structurally defective sphincter and loss of esophageal
contractility. Before proceeding with surgical treatment, malignancy and a drug-related etiology of the stricture should be
excluded, and the stricture should be progressively dilated up
to a 50 to 60F bougie. When the stricture is fully dilated, the
relief of dysphagia is evaluated, and esophageal manometry is
performed to determine the adequacy of peristalsis in the distal
esophagus. If dysphagia is relieved and the amplitude of esophageal contractions is adequate, an antireflux procedure should be
performed; if there is a global loss of esophageal contractility,
caution should be exercised in performing an antireflux procedure with a complete fundoplication, and a partial fundoplication should be considered.
Barrett’s CLE is commonly associated with a severe
structural defect of the LES and often poor contractility of the
esophageal body. Patients with BE are at risk of the development
of an adenocarcinoma. Whilst surgeons would like to think that an
antireflux procedure can reduce the risk of progression to cancer,
the evidence supporting this is relatively weak, and for now
Barrett’s esophagus should be considered to be evidence
that the patient has gastroesophageal reflux, and progression
to antireflux surgery is indicated for the treatment of reflux
symptoms, not cancer progression. If, however, high grade
dysplasia or intramucosal carcinoma is found on mucosal biopsy
specimens, treatment should then be directed at the BE and the
lesion, using either evaluation endoscopic ablation, endoscopic
resection, or esophageal resection.
The majority of patients requiring treatment for reflux
have a relatively mild form of disease and will respond to antisecretory medications. Patients with more severe forms of disease,
particularly those who develop persistent or progressive disease,
should be considered for definitive therapy. Laparoscopic fundoplication will provide a long-term cure in the majority of
these patients, with minimal discomfort and an early return to
normal activity.
Preoperative Evaluation. Before proceeding with an antireflux operation, several factors should be evaluated. The clinical
symptoms should be consistent with the diagnosis of gastroesophageal reflux. Patients presenting with the typical symptoms of heartburn and/or regurgitation which have responded,
at least partly, to PPI therapy, will generally do well following
surgery, whereas patients with atypical symptoms have a less
predictable response. Reflux should also be objectively confirmed by either the presence of ulcerative esophagitis or an
abnormal 24-hour pH study.
The propulsive force of the body of the esophagus should
be evaluated by esophageal manometry to determine if it has
sufficient power to propel a bolus of food through a newly
reconstructed valve. Patients with normal peristaltic contractions can be considered for a 360° Nissen fundoplication or a
partial fundoplication, depending on patient and surgeon preferences. When peristalsis is absent, a partial fundoplication is
probably the procedure of choice, but only if achalasia has been
ruled out.
Hiatal anatomy should also be assessed. In patients with
smaller hiatal hernias, endoscopy evaluation usually provides
sufficient information. However, when patients present with a
very large hiatus hernia or for revision surgery after previous
antireflux surgery, contrast radiology provides better anatomical
information. The concept of anatomic shortening of the esophagus is controversial, with divergent opinions held about how
Principles of Surgical Therapy. The primary goal of antireflux surgery is to safely create a new antireflux valve at the
gastroesophageal junction, while preserving the patient’s ability to swallow normally and to belch to relieve gaseous distention. Regardless of the choice of the procedure, this goal can
be achieved if attention is paid to some basic principles when
reconstructing the antireflux mechanism. First, the operation
should create a flap valve which prevents regurgitation of gastric contents into the esophagus. This will result in an increase
in the pressure of the distal esophageal sphincter region. Following a Nissen fundoplication the expected increase is to a level
twice the resting gastric pressure (i.e., 12 mmHg for a gastric
pressure of 6 mmHg). The extent of the pressure rise is often
less following a partial fundoplication, although with all types
of fundoplication the length of the reconstructed valve should be
at least 3 cm. This not only augments sphincter characteristics
in patients in whom they are reduced before surgery but also
prevents unfolding of a normal sphincter in response to gastric
distention (Fig. 25-32). Preoperative and postoperative esophageal manometry measurements have shown that the resting
sphincter pressure and the overall sphincter length can be surgically augmented over preoperative values, and that the change
in the former is a function of the degree of gastric wrap around
the esophagus (Fig. 25-33). However, the aim of any fundoplication is to create a loose wrap and to maintain the position of
the gastric fundus close to the distal intra-abdominal esophagus,
in a flap valve arrangement. The efficacy of this relies on the
close relationship between the fundus and the esophagus, not the
“tightness” of the wrap.
Second, the operation should place an adequate length
of the distal esophageal sphincter in the positive-pressure
Distention
Figure 25-32. A graphic illustration of the shortening of the lower
esophageal sphincter that occurs as the sphincter is “taken up” by
the cardia as the stomach distends.
∆ P mmHg
20
Hill
Belsey
Nissen
N=15
N=15
N=15
1039
15
10
5
Y = 4.63 + .023 (x)
P < .01
0
240
Degree of wrap
360
Figure 25-33. The relationship between the augmentation of
sphincter pressure over preoperative pressure (∆P) and the degree of
gastric fundic wrap in three different antireflux procedures. (Reproduced with permission from O’Sullivan GC, DeMeester TR, Joelsson BE, et al: Interaction of lower esophageal sphincter pressure
and length of sphincter in the abdomen as determinants of gastroesophageal competence, Am J Surg. 1982 Jan;143(1):40-47.)
environment of the abdomen by a method that ensures its
response to changes in intra-abdominal pressure. The permanent
restoration of 2 or more cm of abdominal esophagus ensures
the preservation of the relationship between the fundus and the
esophagus. All of the popular antireflux procedures increase the
length of the sphincter exposed to abdominal pressure by an
average of at least 1 cm.
Third, the operation should allow the reconstructed cardia to relax on deglutition. In normal swallowing, a vagally
mediated relaxation of the distal esophageal sphincter and the
gastric fundus occurs. The relaxation lasts for approximately
10 seconds and is followed by a rapid recovery to the former
tonicity. To ensure relaxation of the sphincter, three factors are
important: (a) Only the fundus of the stomach should be used
to buttress the sphincter, because it is known to relax in concert with the sphincter; (b) the gastric wrap should be properly
placed around the sphincter and not incorporate a portion of the
stomach or be placed around the stomach itself, because the
body of the stomach does not relax with swallowing; and (c)
damage to the vagal nerves during dissection of the thoracic
esophagus should be avoided because it may result in failure of
the sphincter to relax.
Fourth, the fundoplication should not increase the resistance of the relaxed sphincter to a level that exceeds the peristaltic power of the body of the esophagus. The resistance of the
relaxed sphincter depends on the degree, length, and diameter of
the gastric fundic wrap, and on the variation in intra-abdominal
pressure. A 360° gastric wrap should be no longer than 2 cm and
constructed over a large (50 to 60F) bougie. This will ensure
that the relaxed sphincter will have an adequate diameter with
minimal resistance. A bougie is not necessary when constructing a partial wrap.
Fifth, the operation should ensure that the fundoplication
can be placed in the abdomen without undue tension and maintained there by approximating the crura of the diaphragm above
the repair. Leaving the fundoplication in the thorax converts a
sliding hernia into a PEH, with all the complications associated with that condition. Maintaining the repair in the abdomen
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
common this problem is. Believers claim that anatomic shortening of the esophagus compromises the ability of the surgeon
to perform an adequate repair without tension and that this can
lead to an increased incidence of breakdown or thoracic displacement of the repair. Some of those who hold this view claim that
esophageal shortening is present when a barium swallow X-ray
identifies a sliding hiatal hernia that will not reduce in the upright
position or that measures more than 5 cm in length at endoscopy.
When such identification is made, these surgeons usually add a
gastroplasty to the antireflux procedure. Others claim that esophageal shortening is overdiagnosed and rarely seen, and that the
morbidity of adding a gastroplasty outweighs any benefits. These
surgeons would recommend a standard antireflux procedure in
all patients undergoing primary surgery.
1040
under tension predisposes to an increased incidence of recurrence. How common this problem is encountered is disputed,
with some surgeons advocating lengthening the esophagus by
gastroplasty and constructing a partial fundoplication, and others claiming that this issue is now rarely encountered.
PART II
SPECIFIC CONSIDERATIONS
Procedure Selection. A laparoscopic approach is now used
routinely in all patients undergoing primary antireflux surgery.
Some surgeons advocate the use of a single antireflux procedure
for all patients, whereas others advocate a tailored approach.
Advocates of the laparoscopic Nissen fundoplication as the procedure of choice for a primary antireflux repair would generally
apply this procedure in all patients with normal or near normal
esophageal motility, and they would reserve a partial fundoplication for use in individuals with poor esophageal body motility.
Others, based on the good longer-term outcomes now reported
following partial fundoplication procedures, advocate the routine application of a partial fundoplication procedure, thereby
avoiding any concerns about constructing a fundoplication in
individuals with poor esophageal motility.
Experience and randomized studies have shown that both
the Nissen fundoplication and various partial fundoplication
procedures are all effective and durable antireflux repairs that
generate an excellent outcome in approximately 90% of patients
at longer-term follow-up.
L
R
Primary Antireflux Repairs
Nissen Fundoplication. The most common antireflux procedure is the Nissen fundoplication. In the past, this procedure has
been performed through an open abdominal or a chest incision,
but with the development of laparoscopic approaches primary
antireflux surgery is now routinely undertaken using the laparoscope. Rudolph Nissen described this procedure as a 360° fundoplication around the lower esophagus for a distance of 4 to
5 cm, without division of the short gastric blood vessels.
Although this provided good control of reflux, it was associated
with a number of side effects that have encouraged modifications of the procedure as originally described. These include
using only the gastric fundus to envelop the esophagus in a fashion analogous to a Witzel jejunostomy, sizing the fundoplication
with a large (50 to 60F) bougie, limiting the length of the fundoplication to 1 to 2 cm, and dividing the short gastric vessels.
The essential elements necessary for the performance of a transabdominal fundoplication are common to both the laparoscopic
and open procedures and include the following:
1. Hiatal dissection and preservation of both vagi along their
entire length
2. Circumferential esophageal mobilization
3. Hiatal closure, usually posterior to the esophagus
4. Creation of a short and floppy fundoplication over an esophageal dilator
In addition, many surgeons also routinely divide the
short gastric blood vessels, although this step is not universally
applied, and the results of several randomized trials have failed
to show that this step yields any benefit.
The laparoscopic approach to fundoplication has now
replaced the open abdominal Nissen fundoplication as the procedure of choice. Five ports are usually used (Fig. 25-34), and
dissection is begun by incising the gastrohepatic omentum above
and below the hepatic branch of the anterior vagus nerve, which
is usually preserved. The circumference of the diaphragmatic
Figure 25-34. Patient positioning and trocar placement for laparoscopic antireflux surgery. The patient is placed with the head
elevated approximately 30° in the modified lithotomy position.
The surgeon stands between the patient’s legs, and the procedure is
completed using five abdominal access ports.
hiatus is dissected and the esophagus is mobilized by careful dissection of the anterior and posterior soft tissues within the hiatus.
The esophagus is held anterior and to the left and the hiatal pillars
are approximated with interrupted nonabsorbable sutures, starting
posteriorly and working anteriorly. A tension-free fundoplication
should be constructed. This can usually be achieved either with
or without division of the short gastric blood vessels, according to surgeon preference. If the vessels are divided, the upper
one-third of the greater curvature is mobilized by sequentially
dissecting and dividing these vessels, commencing distally and
working proximally. Following complete fundal mobilization,
the posterior wall of the fundus is brought behind the esophagus
to the right side, and the anterior wall of the fundus is brought
anterior to the esophagus. The fundic lips are manipulated to
allow the fundus to envelop the esophagus without twisting. A 50
to 60F bougie is passed to properly size the fundoplication, and
it is sutured using nonabsorbable sutures. Some surgeons use a
single U-stitch of 2-0 polypropylene buttressed with felt pledgets
(Fig. 25-35), and others use 2-4 interrupted sutures.
Posterior Partial Fundoplication. Partial fundoplications were
obstruction of a complete fundoplication. The Toupet posterior
partial fundoplication consists of a 270° gastric fundoplication
around the distal 4 cm of esophagus (Fig. 25-36). It is usually
stabilized by anchoring the wrap posteriorly to the hiatal rim.
1041
Anterior Partial Fundoplication. An alternative approach to
partial fundoplication is to construct an anterior partial fundoplication. Following posterior hiatal repair, the anterior fundus is
rolled over the front of the esophagus and sutured to the hiatal
rim and the esophageal wall. Division of the short gastric vessels
Figure 25-35. A. Laparoscopic Nissen fundoplication is performed with a five-trocar technique. B. The liver retractor is affixed to a mechanical arm to hold it in place throughout the operation. C. After division of the gastrohepatic omentum above the hepatic branch of the vagus (pars
flaccida), the surgeon places a blunt atraumatic grasper beneath the phrenoesophageal ligament. D. After completion of the crural closure,
an atraumatic grasper is placed right to left behind the gastroesophageal junction. The grasper is withdrawn, pulling the posterior aspect of
the gastric fundus behind the esophagus. E. Once the suture positions are chosen, the first stitch (2-0 silk, 20 cm long) is introduced through
the 10-mm trocar, and the needle is passed first through the left limb of the fundus, then the esophagus (2.5 cm above the gastroesophageal
junction), then through the right limb of the fundus. F. Final position of the fundoplication.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
developed as an alternative to the Nissen procedure in an attempt
to minimize the risk of postfundoplication side effects, such as
dysphagia, inability to belch, and flatulence. The commonest
approach has been a posterior partial or Toupet fundoplication.
Some surgeons use this type of procedure for all patients presenting for antireflux surgery, whereas others apply a tailored approach
in which a partial fundoplication is constructed in patients with
impaired esophageal motility, in which the propulsive force of the
esophagus is thought to be insufficient to overcome the outflow
1042
PART II
SPECIFIC CONSIDERATIONS
Figure 25-35. (Continued )
is never needed when constructing this type of fundoplication.
Various degrees of anterior partial fundoplication have been
described—90°, 120°, 180°. The anterior 180° partial fundoplication (Fig. 25-37) provides a more robust fundoplication and
achieves an excellent longer-term outcome in approximately 90%
of patients at follow-up of at least 10 years. With this procedure,
the fundus and esophagus are sutured to the right side of the hiatal
rim to create a flap valve at the gastroesophageal junction and to
stabilize a 3 to 4 cm length of intra-abdominal esophagus.
Collis Gastroplasty. When a shortened esophagus is encountered, many surgeons choose to add an esophageal lengthening
procedure before fundoplication, to reduce the tension on the gastroesophageal junction, believing this will minimize the risk of failure
due to postoperative hiatus hernia. The commonest approach to
this is the Collis gastroplasty. This entails using a stapler to divide
the cardia and upper stomach, parallel to the lesser curvature of
Figure 25-36. Completed laparoscopic posterior partial (Toupet)
fundoplication. The fundoplication does not cover the anterior surface of the esophagus, and it is stabilized by suturing the fundus to
the side of the esophagus, and posteriorly to the right hiatal pillar.
the stomach, thereby creating a gastric tube in continuity with the
esophagus, and effectively lengthening the esophagus by several
centimeters. Laparoscopic techniques for Collis gastroplasty have
been described (Fig. 25-38). Following gastroplasty a fundoplication is constructed, with the highest suture is placed on the native
esophagus when constructing a Nissen fundoplication. Not all surgeons choose to undertake a Collis procedure, however, as there is
controversy about the actual incidence of the shortened esophagus
and widely divergent views are held about how often this problem is encountered. In addition, some surgeons have questioned
the wisdom of creating an amotile tube of gastric wall, which can
secrete acid, and then placing a Nissen fundoplication below this.
Outcome After Fundoplication. Studies of long-term outcome
following both open and laparoscopic fundoplication document
the ability of laparoscopic fundoplication to relieve typical reflux
symptoms (heartburn, regurgitation, and dysphagia) in more than
Figure 25-37. Completed laparoscopic anterior 180° partial fundoplication. The fundoplication fully covers the anterior surface of
the esophagus, and it is stabilized by suturing the fundus to the
right side of the esophagus, and to the right hiatal pillar. Unlike the
Nissen procedure, the fundus is not pulled behind the esophagus.
1043
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Figure 25-38. A. After removal of the fat pad and release of tension on the Penrose drain, the gastroesophageal junction (GES) retracts to
the level of the hiatus. The interior end of the staple line is marked 2/5 cm below the angle of His. B. The first horizontal firing of the stapler
occurs by maximally articulating the stapler to the left, aiming toward the previously marked spot adjacent to the dilator. C. The vertical staple
line is created by a single firing of the GIA placed parallel and flush against the 48F dilator. D. The highest Nissen fundoplication suture is
placed on the native esophagus, and the second suture tucks in the apex of the staple line.
90% of patients at follow-up intervals averaging 2 to 3 years
and 80% to 90% of patients 5 years or more following surgery.
This includes evidence-based reviews of antireflux surgery, prospective randomized trials comparing antireflux surgery to PPI
therapy and open to laparoscopic fundoplication and analysis of
U.S. national trends in use and outcomes. Postoperative pH studies indicate that more than 90% of patients will normalize their
pH tracings. The results of laparoscopic fundoplication compare
favorably with those of the “modern” era of open fundoplication. They also indicate the less predictable outcome of atypical
reflux symptoms (cough, asthma, laryngitis) after surgery, being
relieved in only two-thirds of patients.
The goal of surgical treatment for GERD is to relieve
the symptoms of reflux by reestablishing the gastroesophageal
barrier. The challenge is to accomplish this without inducing
dysphagia or other untoward side effects. Dysphagia, existing
before surgery, usually improves following laparoscopic fundoplication. Temporary dysphagia is common after surgery
and generally resolves within 3 months, but it can take up to
12 months in some individuals, and dysphagia sufficient to
require ongoing dietary modification persists in up to 5% of
individuals following Nissen fundoplication. Other side effects
common to antireflux surgery include the inability to belch and
vomit and increased flatulence. Most patients cannot vomit
through an intact wrap, though this is rarely clinically relevant.
Most patients are unable to belch gas from the stomach in the
first 3 to 6 months after fundoplication, but 80% to 90% regain
the ability to belch normally beyond the first 12 months of follow-up. Hyperflatulence is a common and noticeable problem,
likely related to increased air swallowing that is present in most
patients with reflux disease, aggravated by the inability to belch
in some patients.
1044
Randomized Controlled Trials Addressing Surgical
Technique
Division of the Short Gastric Blood Vessels Originally,
PART II
SPECIFIC CONSIDERATIONS
Nissen’s description of a total fundoplication entailed a 360°
fundoplication during which the short gastric blood vessels were
left intact. However, with reports of troublesome postoperative
dysphagia, division of these vessels—to achieve full fundal
mobilization and thereby ensure a loose fundoplication—was
promoted and has entered common practice. The evidence supporting dividing these vessels has been based on the outcomes
from uncontrolled case series of patients undergoing Nissen
fundoplication either with vs. without division of the short gastric vessels. However, the results from these studies have been
conflicting, with different proponents reporting good results
irrespective of whether these vessels have been divided or not.
To address this issue, six randomized trials that enrolled a total
of 438 patients have been reported. None of these trials demonstrated any differences for the postoperative dysphagia or recurrent gastro-esophageal reflux. However, in the three largest of
the six trials an increased incidence of flatulence and bloating
symptoms, as well as greater difficulty with belching, was seen
in patients in whom the short gastric vessels were divided.
A recent meta-analysis from Engstrom et al, generated by
combining the raw data from Australian and Swedish trials, evaluated a larger cohort of 201 patients, with 12 years of follow-up
in 170, and also confirmed equivalent reflux control but found
more abdominal bloating after division of the short gastric vessels. Overall, these trials fail to support the belief that dividing the
short gastric vessels improves any outcome following Nissen fundoplication. The trials actually suggest that dividing the vessels
increases the complexity of the procedure and leads to a poorer
outcome due to the increase in bloating symptoms.
Nissen vs. Posterior Partial Fundoplication Eleven
randomized trials have compared Nissen vs. posterior partial
fundoplication. Some of the trials contributed little to the pool of
evidence, as they are either small or underpowered, and failed to
show significant outcome differences. The larger trials, however,
have consistently demonstrated equivalent reflux control, but
they also show a reduced incidence of wind-related side-effects
(flatulence, bloating, and inability to belch) following posterior
partial fundoplication procedures, although less dysphagia following a posterior fundoplication was only demonstrated in 2 of
the 11 trials. Lundell et al reported the outcomes of Nissen vs.
Toupet partial fundoplication in a trial that enrolled 137 patients
with reported follow-up to 18 years. Reflux control and dysphagia symptoms were similar, but flatulence was commoner after
Nissen fundoplication at some medium-term follow-up time
points, and revision surgery was more common following Nissen
fundoplication, mainly to correct postoperative paraoesophageal
herniation. At 18 years follow-up, success rates of more than
80% were reported for both procedures, as well as no significant
differences in the incidence of side effects. The data from this
trial suggested that the mechanical side effects following Nissen fundoplication progressively improve with very long-term
follow-up. Strate et al reported 2-year follow-up in a trial that
enrolled 200 patients. Approximately 85% of each group was
satisfied with the clinical outcome, but dysphagia was significantly more common following Nissen fundoplication (19 vs.
8 patients).
Other trials (Guérin et al–140 patients, Booth et al–127,
Khan et al–121, Shaw et al–100) also report similar reflux
control within the first few years of follow-up. Only Booth et
al demonstrated less dysphagia following posterior fundoplication. Subgroup analysis in 3 trials (Booth, Shaw, Zornig) did not
reveal differences between patients with vs. without poor preoperative oesophageal motility. Overall these trials suggest that
some side-effects, mainly wind-related issues, are less common
following posterior partial fundoplication. However, the hypothesis that dysphagia is less of a problem following posterior partial fundoplication has only been substantiated in 2 of 11 trials.
Nissen vs. Anterior Fundoplication Six trials have evaluated
Nissen vs. anterior partial fundoplication variants. Four have
assessed Nissen vs. anterior 180° partial fundoplication (Watson
et al–107 patients, Baigrie et al–161, Cao et al–100, Raue et
al–64). These trials all demonstrated equivalent reflux control,
but less dysphagia and less wind-related side effects after anterior 180° partial fundoplication at up to 5 years follow-up. Only
the study from Watson et al has reported follow-up to 10 years, and
at late follow-up in their trial there were no significant outcome
differences for the two procedures, with equivalent control of
reflux, and no differences for side effects due to a progressive
decline in dysphagia as follow-up extended beyond 5 years.
Two trials compared laparoscopic anterior 90° partial
fundoplication vs. Nissen fundoplication (Watson et al–112
patients, Spence et al–79). In both of these trials, side-effects
were less common following anterior 90° fundoplication, but
this was offset by a slightly higher incidence of recurrent reflux
at up to 5 years follow-up. Satisfaction with the overall outcome
was similar for both fundoplication variants.
Anterior vs. Posterior Partial Fundoplication Two randomized trials have directly compared anterior vs. posterior
partial fundoplication. Hagedorn et al randomized 95 patients
to undergo either Toupet vs. anterior 120° partial fundoplication, and Khan et al enrolled 103 patients to anterior 180° vs.
posterior partial fundoplication. Both studies demonstrated better reflux control, offset by more side effects following posterior
partial fundoplication. The anterior 120° partial fundoplication
performed by Hagedorn et al was similar to the anterior 90° variant described above. However, the outcomes following this procedure were much worse in this trial than the outcomes in other
studies, with the average exposure time to acid (pH <4%–5.6%)
following anterior fundoplication in their study unusually high
compared to other studies. Khan et al only reported 6 months
follow-up, and longer-term outcomes are awaited before drawing firm conclusions. The overall results from all eight trials
that included an anterior fundoplication variant suggest that this
type of fundoplication achieves satisfactory reflux control, with
less dysphagia and other side-effects, yielding a good overall
outcome. However, the reduced incidence of troublesome sideeffects is traded off against a higher risk of recurrent reflux.
Outcome of Antireflux Surgery in Patients With Barrett’s
Esophagus. Few studies have focused on the alleviation of symptoms after antireflux surgery in patients with BE (Table 25-7).
Those that are available document excellent to good results in
72% to 95% of patients at 5 years following surgery. Several
nonrandomized studies have compared medical and surgical
therapy and report better outcomes after antireflux surgery. Parrilla and colleagues reported the only randomized trial to evaluate
this issue. They enrolled 101 patients over 18 years, and median
follow-up was 6 years. Medical therapy consisted of 20 mg
of omeprazole (PPI) twice daily since 1992 in all medically
treated patients, and surgical therapy consisted of an open Nissen
Reoperation for Failed Antireflux Repairs. Failure of an
Table 25-7
Symptomatic outcome of surgical therapy for Barrett’s
esophagus
% EXCELLENT MEAN
TO GOOD
FOLLOW-UP,
RESPONSE
YEARS
YEAR
Starnes
1984
8
75
2
Williamson
1990
37
92
3
DeMeester
1990
35
77
3
McDonald
1996
113
82.2
6.5
Ortiz
1996
32
90.6
5
fundoplication. The symptomatic outcome in the two groups was
nearly identical, although esophagitis and/or stricture persisted
in 20% of the medically treated patients, compared to only 3%
to 7% of patients following antireflux surgery. About 15% of
patients had abnormal acid exposure after surgery. Although pH
data were not routinely collected in patients on PPI therapy, in the
subgroup of 12 patients that did have 24-hour monitoring on treatment, 3 of 12 (25%) had persistently high esophageal acid exposure, and most (75%) had persistently high bilirubin exposure.
The common belief that Barrett’s epithelium cannot be
reversed by antireflux surgery may not be correct. Within the
control arm of a randomized trial of ablation vs. surveillance,
Bright and associates identified approximately 50% regression
in the length of Barrett’s esophagus in 20 patients within the
control arm of a randomized trial of ablation vs. surveillance.
Current data indicate that patients with BE should remain
in an endoscopic surveillance program following antireflux
surgery. Biopsy specimens should be reviewed by a pathologist with expertise in the field. If low-grade dysplasia is confirmed, biopsy specimens should be repeated after 12 weeks of
high-dose acid suppression therapy. If high-grade dysplasia or
intramucosal cancer is evident on more than one biopsy specimen, then treatment is escalated. Treatment options include
endoscopic mucosal resection, endoscopic ablation of the BE,
or esophageal resection. Esophageal resection is advisable when
an invasive cancer (stage T1b or deeper) is present, or for multifocal long segment BE in younger and fit patients in whom
endoscopic treatments are unlikely to be adequate. Endoscopic
mucosal resection allows smaller intramucosal tumors to be
removed with clear pathology margins, and it can be used as a
“big biopsy” to obtain better pathological staging, and even to
excise shorter segments of BE in a piecemeal fashion. Ablation,
commonly using radiofrequency ablation, has been shown at
short-term follow-up in a randomized trial to reduce the rate
of progression from high grade dysplasia to invasive cancer
by approximately 50%. However, following any endoscopic
treatment, patients need to continue with close endoscopic surveillance as recurrence can occur and the longer-term outcome
following these treatments remains uncertain. Early detection
and treatment have been shown to decrease the mortality rate
from esophageal cancer in these patients.
If the dysplasia is reported as lower grade or indeterminant, then inflammatory change that is often confused with
dysplasia should be suppressed by a course of acid suppression
therapy in high doses for 2 to 3 months, followed by rebiopsy
of the Barrett’s segment.
GIANT DIAPHRAGMATIC (HIATAL) HERNIAS
With the advent of clinical radiology, it became evident that
a diaphragmatic hernia was a relatively common abnormality
and was not always accompanied by symptoms. Three types
of esophageal hiatal hernia were identified: (a) the sliding
hernia, type I, characterized by an upward dislocation of the
cardia in the posterior mediastinum (Fig. 25-39A); (b) the rolling or PEH, type II, characterized by an upward dislocation
of the gastric fundus alongside a normally positioned cardia
(Fig. 25-39B); and (c) the combined sliding-rolling or mixed
hernia, type III, characterized by an upward dislocation of both
the cardia and the gastric fundus (Fig. 25-39C). The end stage
of type I and type II hernias occurs when the whole stomach
migrates up into the chest by rotating 180° around its longitudinal axis, with the cardia and pylorus as fixed points. In this
situation, the abnormality is usually referred to as an intrathoracic stomach (Fig. 25-39D). In some taxonomies, a type IV
hiatal hernia is declared when an additional organ, usually the
colon, herniates as well. Types II–IV hiatal hernias are also
referred to as paraesophageal hernia (PEH), as a portion of
the stomach is situated adjacent to the esophagus, above the
gastroesophageal junction.
Incidence and Etiology
The true incidence of a hiatal hernia is difficult to determine
because of the absence of symptoms in a large number of
patients who are subsequently shown to have a hernia. When
radiographic examinations are done in response to GI symptoms,
1045
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
AUTHOR
NO. OF
PATIENTS
antireflux procedure occurs when, after the repair, the patient is
unable to swallow normally, experiences upper abdominal discomfort during and after meals, or has recurrence or persistence
of reflux symptoms. The assessment of these symptoms and the
selection of patients who need further surgery are challenging
problems. Functional assessment of patients who have recurrent, persistent, or emergent new symptoms following a primary
antireflux repair is critical to identifying the cause of the failure.
Analysis of patients requiring reoperation after a previous antireflux procedure shows that placement of the wrap around the
stomach is the most frequent cause for failure after open procedures, while herniation of the repair into the chest is the most
frequent cause of failure after a laparoscopic procedure. Partial
or complete breakdown of the fundoplication and construction
of a too-tight a fundoplication or overnarrowing the esophageal
hiatus occurs with both open and closed procedures.
Patients who have recurrence of heartburn and regurgitation
without dysphagia and have good esophageal motility are most
amenable to reoperation, and they can be expected to have an
excellent outcome. When dysphagia is the cause of failure, the situation can be more difficult to manage. If the dysphagia occurred
immediately following the repair, it is usually due to a technical
failure, most commonly a misplaced fundoplication around the
upper stomach, or overnarrowing of the esophageal diaphragmatic
hiatus and reoperation is usually satisfactory. When dysphagia is
associated with poor motility and multiple previous repairs, further revision fundoplication is unlikely to be successful, and in
otherwise fit patients it is appropriate to seriously consider esophageal resection. With each reoperation, the esophagus is damaged
further, and the chance of preserving function is decreased. Also,
blood supply is reduced, and ischemic necrosis of the esophagus
can occur after several previous mobilizations.
1046
PART II
SPECIFIC CONSIDERATIONS
A
B
C
D
Figure 25-39. A. Radiogram of a type I (sliding) hiatal hernia. B. Radiogram of a type II (rolling or paraesophageal) hernia. C. Radiogram of
a type III (combined sliding-rolling or mixed) hernia. D. Radiogram of an intrathoracic stomach. This is the end stage of a large hiatal hernia
regardless of its initial classification. Note that the stomach has rotated 180° around its longitudinal axis, with the cardia and pylorus as fixed
points. (Reproduced with permission from Nyhus LM, Condon RE: Hernia, 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1989.)
Clinical Manifestations
The clinical presentation of a giant hiatal (paraesophageal) hernia differs from that of a sliding hernia. There is usually a higher
prevalence of symptoms of dysphagia and postprandial fullness
with PEHs, but the typical symptoms of heartburn and regurgitation present in sliding hiatal hernias can also occur. Both are
caused by gastroesophageal reflux secondary to an underlying
mechanical deficiency of the cardia. The symptoms of dysphagia
and postprandial fullness in patients with a PEH are explained
by the compression of the adjacent esophagus by a distended
cardia, or twisting of the GEJ by the torsion of the stomach that
occurs as it becomes progressively displaced in the chest. The
postprandial fullness or retrosternal chest pain is a thought to be
a result of distension of the stomach with gas or food in the hiatal
hernia. Many patients with sliding hernias and reflux symptoms
will lose the reflux symptoms when the hernia evolves into the
paraesophageal variety. This can be explained by the recreation
of the cardiophrenic angle when the stomach herniates alongside the GEJ or becomes twisted in the sac. Repair of the hernia
without addressing the reflux can create extremely bothersome
heartburn. Respiratory complications are frequently associated
with a PEH and consist of dyspnea and recurrent pneumonia
from aspiration. New research demonstrates that the cause of
dyspnea in the presence of a giant PEH is more likely to be left
atrial compression, decreasing cardiac output, than a restrictive
pulmonary effect, as has been hypothesized for many years.
Approximately one-third of patients with a PEH are found
to be anemic, which is due to recurrent bleeding from ulceration
of the gastric mucosa in the herniated portion of the stomach,
even if ulcerations are not detected at the time of endoscopy.
The association of anemia and PEH is best proven by fixing
the hernia. Anemia is corrected in >90% of patients with this
condition. With time, more and more stomach migrates into the
chest and can cause intermittent foregut obstruction due to the
rotation that has occurred. In contrast, many patients with PEH
are asymptomatic or complain of minor symptoms. However,
the presence of a PEH can be life-threatening in that the hernia
can lead to sudden catastrophic events, such as excessive bleeding or volvulus with acute gastric obstruction or infarction. With
mild dilatation of the stomach, the gastric blood supply can be
markedly reduced, causing gastric ischemia, ulceration, perforation, and sepsis. The probability of incarceration/strangulation is
not well known, although recent studies suggest that the lifetime
risk is less than 5%, making this concern an insufficient concern
for routine repair of the asymptomatic PEH.
The symptoms of sliding hiatal hernias are usually due
to functional abnormalities associated with gastroesophageal
reflux and include heartburn, regurgitation, and dysphagia.
These patients have a mechanically defective LES, giving rise
to the reflux of gastric juice into the esophagus and the symptoms of heartburn and regurgitation. The symptom of dysphagia
occurs from the presence of mucosal edema, Schatzki’s ring,
stricture, or the inability to organize peristaltic activity in the
body of the esophagus as a consequence of the disease.
There is a group of patients with sliding hiatal hernias not
associated with reflux disease who have dysphagia without any
obvious endoscopic or manometric explanation. Video barium
radiograms have shown that the cause of dysphagia in these
patients is an obstruction of the swallowed bolus by diaphragmatic impingement on the herniated stomach. Manometrically,
this is reflected by a double-humped high-pressure zone at the
GEJ. The first pressure rise is due to diaphragmatic impingement on the herniated stomach, and the second is due to the
true distal esophageal sphincter. These patients usually have a
mechanically competent sphincter, but the impingement of the
diaphragm on the stomach can result in propelling the contents
of the supradiaphragmatic portion of the stomach up into the
esophagus and pharynx, resulting in complaints of pharyngeal
regurgitation and aspiration. Consequently, this abnormality is
often confused with typical GERD. Surgical reduction of the
hernia results in relief of the dysphagia in 91% of patients.
Diagnosis
A chest X-ray with the patient in the upright position can diagnose a hiatal hernia if it shows an air-fluid level behind the cardiac shadow. This is usually caused by a PEH or an intrathoracic
stomach. The accuracy of the upper GI barium study in detecting a paraesophageal hiatal hernia is greater than for a sliding
hernia because the latter can often spontaneously reduce. The
paraesophageal hiatal hernia is a permanent herniation of the
stomach into the thoracic cavity, so a barium swallow provides
the diagnosis in virtually every case. Attention should be focused
on the position of the GEJ, when seen, to differentiate it from a
type II hernia (see Fig. 25-39B and C). Fiber-optic esophagoscopy is useful in the diagnosis and classification of a hiatal hernia
because the scope can be retroflexed. In this position, a sliding
hiatal hernia can be identified by noting a gastric pouch lined
with rugal folds extending above the impression caused by the
crura of the diaphragm, or measuring at least 2 cm between the
crura, identified by having the patient sniff, and the squamocolumnar junction on withdrawal of the scope (Fig. 25-40). A PEH
is identified on retroversion of the scope by noting a separate
orifice adjacent to the GEJ into which gastric rugal folds ascend.
A sliding-rolling or mixed hernia can be identified by noting a
gastric pouch lined with rugal folds above the diaphragm, with
the GEJ entering about midway up the side of the pouch.
1047
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
the incidence of a sliding hiatal hernia is seven times higher than
that of a PEH. The PEH is also known as the giant hiatal hernia.
Over time the pressure gradient between the abdomen and chest
enlarges the hiatal hernia. In many cases the type 1 sliding hernia
will evolve into a type III mixed hernia. Type II hernias are quite
rare. The age distribution of patients with PEHs is significantly
different from that observed in sliding hiatal hernias. The median
age of the former is 61 years old; of the latter, 48 years old. PEHs
are more likely to occur in women by a ratio of 4:1.
Structural deterioration of the phrenoesophageal membrane over time may explain the higher incidence of hiatal hernias in the older age group. These changes involve thinning of
the upper fascial layer of the phrenoesophageal membrane (i.e.,
the supradiaphragmatic continuation of the endothoracic fascia)
and loss of elasticity in the lower fascial layer (i.e., the infradiaphragmatic continuation of the transversalis fascia). Consequently, the phrenoesophageal membrane yields to stretching in
the cranial direction due to the persistent intra-abdominal pressure and the tug of esophageal shortening on swallowing. Interestingly, the stretching and thinning occurs more anteriorly and
posteriorly, with fixation of the left crus of the diaphragm to the
stomach at the 3 o’clock position, as viewed from the foot. This
creates an anterior and posterior hernia sac, the latter of which is
often filled with epiphrenic and retroperitoneal fat. These observations point to the conclusion that the development of a hiatal
hernia is an age-related phenomenon secondary to repetitive
upward stretching of the phrenoesophageal membrane.
1048
PART II
SPECIFIC CONSIDERATIONS
Figure 25-40. Endoscopic view through a retroflexed fiber-optic
gastroscope showing the shaft of the scope (arrow) coming down
through a sliding hernia. Note the gastric rugal folds extending
above the impression caused by the crura of the diaphragm. (Reproduced with permission from Nyhus LM, Condon RE: Hernia, 3rd ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 1989.)
Pathophysiology
Physiologic testing with 24-hour esophageal pH monitoring has
shown increased esophageal exposure to acid gastric juice in
60% of the patients with a paraesophageal hiatal hernia, compared with the observed 71% incidence in patients with a sliding
hiatal hernia. It is now recognized that paraesophageal hiatal hernia can be associated with pathologic gastroesophageal reflux.
Physiologic studies have also shown that the competency
of the cardia depends on an interrelationship between distal
esophageal sphincter pressure, the length of the sphincter that is
exposed to the positive-pressure environment of the abdomen,
and the overall length of the sphincter. A deficiency in any one
of these manometric characteristics of the sphincter is associated
with incompetency of the cardia regardless of whether a hernia
is present. Patients with a PEH who have an incompetent cardia
have been shown to have a distal esophageal sphincter with normal pressure, but a shortened overall length and displacement
outside the positive-pressure environment of the abdomen. One
might expect esophageal body function to be diminished with
the esophagus “accordioned” up into the chest. Surprisingly,
esophageal peristalsis in patients with PEH is normal in 88%.
Treatment
The treatment of paraesophageal hiatal hernia is largely surgical. Controversial aspects include: (a) indications for repair, (b)
diaphragmatic repair, (c) role of fundoplication, and (d) existence and treatment of the short esophagus.
Indications and Surgical Approach. The presence of a
paraesophageal hiatal hernia has traditionally been considered an indication for surgical repair. This recommendation is
largely based upon two clinical observations. First, retrospective studies have shown a significant incidence of catastrophic,
life-threatening complications of bleeding, infarction, and perforation in patients being followed with known paraesophageal
herniation. Second, emergency repair carries a high mortality. In
the classic report of Skinner and Belsey, six of 21 patients with
a PEH, treated medically because of minimal symptoms, died
from the complications of strangulation, perforation, exsanguinating hemorrhage, or acute dilatation of the herniated intrathoracic stomach. For the most part, these catastrophes occurred
without warning. Others have reported similar findings.
Recent studies suggest that catastrophic complications may
be somewhat less common. Allen and colleagues followed 23
patients for a median of 78 months with only four patients progressively worsening. There was a single mortality secondary to
aspiration that occurred during a barium swallow examination to
investigate progressive symptoms. Although emergency repairs
had a median hospital stay of 48 days compared to a stay of
9 days in those having elective repair, there were only three cases
of gastric strangulation in 735 patient-years of follow-up.
If surgery is delayed and repair is done on an emergency
basis, operative mortality is high, compared to <1% for an elective repair. With this in mind, patients with a PEH are generally
counseled to have elective repair of their hernia, particularly if
they are symptomatic. Watchful waiting of asymptomatic PEHs
may be an acceptable option.
The surgical approach to repair of a paraesophageal hiatal
hernia may be either transabdominal (laparoscopic or open) or
Each has its advantages and disadvantages. A
4 transthoracic.
transthoracic approach facilitates complete esophageal mobilization but is rarely used because the access trauma and postoperative pain are significantly greater than a laparoscopic approach.
The transabdominal approach facilitates reduction of the
volvulus that is often associated with PEHs. Although some
degree of esophageal mobilization can be accomplished transhiatally, complete mobilization to the aortic arch is difficult or
impossible without risk of injury to the vagal nerves.
Laparoscopic repair of PEH would appear to have become
the standard approach. Laparoscopic repair of a pure type II, or
mixed type III PEH is an order of magnitude more difficult than
a standard laparoscopic Nissen fundoplication. Most would recommend that these procedures are best avoided until the surgeon
has accumulated considerable experience with laparoscopic
antireflux surgery. There are several reasons for this. First, the
vertical and horizontal volvulus of the stomach often associated
with PEHs makes identification of the anatomy, in particular
the location of the esophagus, difficult. Second, dissection of a
large PEH sac may result in significant bleeding if the surgeon
deviates from the correct plane of dissection between the peritoneal sac and the endothoracic fascia. Finally, redundant tissue
present at the GEJ following dissection of the sac frustrates the
creation of a fundoplication. This tissue, which includes the epiphrenic fat pad and hernia sac should be removed at the time of
PEH repair. Mindful of these difficulties, and given appropriate
experience, patients with PEH may be approached laparoscopically, with expectation of success in the majority.
Diaphragmatic Repair
It has been shown that PEH repair has a relatively high incidence
of recurrence (10–40%) when the crura is closed primarily with
permanent suture. Techniques to reduce hernia recurrence continue to evolve. Most surgeons believe that recurrence may be
reduced with the use of synthetic or biologic mesh to reinforce
the standard crural closure. Randomized controlled studies have
demonstrated a reduction in PEH recurrence rate when mesh
was used. Nonabsorbable synthetic mesh must be used carefully
and not in a keyhole fashion at the hiatus because of a potential
risk of esophagus or gastric erosion and mesh infection. Biologic mesh (acellular porcine dermis, acellular human dermis,
porcine small intestinal submucosa) has become more widely
used, but these meshes are significantly more expensive than
synthetic mesh, and the only randomized study supporting biologic mesh usage failed to demonstrate superiority over suture
alone after 5 years of rigorous follow-up.
Controversy remains as to whether to perform an antireflux
procedure at all, in selected cases only, or in all patients. Most
advocate the routine addition of an antireflux procedure following repair of the hernia defect. There are several reasons for this.
Physiologic testing with 24-hour esophageal pH monitoring has
shown increased esophageal exposure to acid gastric juice in
60% to 70% of patients with a paraesophageal hiatal hernia,
nearly identical to the observed 71% incidence in patients with
a sliding hiatal hernia. Furthermore, there is no relation between
the symptoms experienced by the patient with a PEH and the
competency of the cardia. Finally, dissection of the gastroesophageal esophagus may lead to postoperative reflux despite
a negative preoperative pH score.
The Short Esophagus and PEH
Giant PEH can be associated with a short esophagus in up to 5%
to 20% of patients as a result of chronic cephalad displacement
of the GEJ. The presence of a short esophagus increases the difficulty of laparoscopic PEH repair. Approximately 10% to 20%
of surgical failures with PEH repair is due to the lack of recognition of a short esophagus. Preoperative results of barium swallow
and esophagogastroduodenoscopy may provide an indication of
short esophagus, but no combination of preoperative clinical variables reliably predict the presence of short esophagus, defined as
the failure to achieve 2.5 cm of intra-abdominal esophagus with
standard mediastinal dissection techniques. Hence, the diagnosis of this entity continues to be made definitively only in the
operating room. Collis gastroplasty achieves esophageal lengthening by creation of a neoesophagus using the gastric cardia. The
totally laparoscopic approach to the short esophagus has evolved
from a method using an end-to-end anastomosis circular stapler
to the current approach that uses a linear stapler creating a stapled wedge gastroplasty. Elements of importance in fashioning
the fundoplication after Collis gastroplasty include placement of
the initial suture of the fundoplication on the esophagus, immediately above the GEJ to ensure that acid-secreting (gastric) mucosa
does not reside above the fundoplication. A second element that
ensures safety and avoids wrap deformation is to place the gastric
portion of the staple line against the neoesophagus, such that the
tip of the gastric staple line sits adjacent to the middle suture of
the fundoplication on the right side of the esophagus.
SCHATZKI’S RING
Schatzki’s ring is a thin submucosal circumferential ring in the
lower esophagus at the squamocolumnar junction, often associated with a hiatal hernia. Its significance and pathogenesis are
unclear (Fig. 25-41). The ring was first noted by Templeton,
but Schatzki and Gary defined it as a distinct entity in 1953. Its
prevalence varies from 0.2% to 14% in the general population,
depending on the technique of diagnosis and the criteria used.
Stiennon believed the ring to be a pleat of mucosa formed by
infolding of redundant esophageal mucosa due to shortening of
the esophagus. Others believe the ring to be congenital, and still
others suggest it is an early stricture resulting from inflammation of the esophageal mucosa caused by chronic reflux.
Schatzki’s ring is a distinct clinical entity having different
symptoms, upper GI function studies, and response to treatment
compared with patients with a hiatal hernia, but without a ring.
Twenty-four-hour esophageal pH monitoring has shown that
patients with a Schatzki’s ring have a lower incidence of reflux
than hiatal hernia controls. They also have better LES function.
This, together with the presence of a ring, could represent a protective mechanism to prevent gastroesophageal reflux.
Results
Most outcome studies report relief of symptoms following surgical repair of PEHs in more than 90% of patients. The current
literature suggests that laparoscopic repair of a paraesophageal
hiatal hernia can be successful. Most authors report symptomatic improvement in 80% to 90% of patients, and <10% to
15% prevalence of recurrent symptomatic hernia. However,
the problem of recurrent asymptomatic or minimally symptomatic hernia following PEH repair, open or laparoscopic, is
Figure 25-41. Barium esophagogram showing Schatzki’s ring
(i.e., a thin circumferential ring in the distal esophagus at the squamocolumnar junction). Below the ring is a hiatal hernia.
1049
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Role of Fundoplication in Giant Hiatal Hernia Repair.
becoming increasingly appreciated. Recurrent hiatal hernia is the
most common cause of anatomic failure following laparoscopic
Nissen fundoplication done for GERD (5–10%), but this risk is
compounded for the giant hernia where radiologic recurrence
is detected in 25% to 40% of patients. It appears that optimal
results with open or laparoscopic giant hiatal hernia repair should
include options for mesh buttressing of hiatal closure and selective esophageal lengthening with one of the many techniques
developed for the creation of a Collis gastroplasty. Despite this
high incidence of radiologic recurrence, and the surgical pursuit
of a remedy, it must be reinforced that asymptomatic recurrent
hernias, like primary PEH, do not need to be repaired. The risk of
incarceration, strangulation, or obstruction is minimal.
1050
PART II
SPECIFIC CONSIDERATIONS
Symptoms associated with Schatzki’s ring are brief episodes of dysphagia during hurried ingestion of solid foods. Its
treatment has varied from dilation alone to dilation with antireflux measures, antireflux procedure alone, incision, and even
excision of the ring. Little is known about the natural progression of Schatzki’s rings. Using radiologic techniques, Chen
and colleagues showed progressive stenosis of rings in 59%
of patients, whereas Schatzki found that the rings decreased in
diameter in 29% of patients and remained unchanged in the rest.
Symptoms in patients with a ring are caused more by the
presence of the ring than by gastroesophageal reflux. Most patients
with a ring but without proven reflux respond to one dilation, while
most patients with proven reflux require repeated dilations. In this
regard, the majority of Schatzki’s ring patients without proven
reflux have a history of ingestion of drugs known to be damaging
to the esophageal mucosa. Bonavina and associates have suggested
drug-induced injury as the cause of stenosis in patients with a ring,
but without a history of reflux. Because rings also occur in patients
with proven reflux, it is likely that gastroesophageal reflux also
plays a part. This is supported by the fact that there is less drug
ingestion in the history of these patients. Schatzki’s ring is probably an acquired lesion that can lead to stenosis from chemicalinduced injury by pill lodgment in the distal esophagus, or from
reflux-induced injury to the lower esophageal mucosa.
The best form of treatment of a symptomatic Schatzki’s
ring in patients who do not have reflux consists of esophageal
dilation for relief of the obstructive symptoms. In patients with
a ring who have proven reflux and a mechanically defective
sphincter, an antireflux procedure is necessary to obtain relief
and avoid repeated dilation.
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal
abnormalities in approximately 80% of patients. In most, the
disease follows a prolonged course. Renal involvement occurs
in a small percentage of patients and signals a poor prognosis.
The onset of the disease is usually in the third or fourth decade
of life, occurring twice as frequently in women as in men.
Small vessel inflammation appears to be an initiating
event, with subsequent perivascular deposition of normal collagen, which may lead to vascular compromise. In the GI tract,
the predominant feature is smooth muscle atrophy. Whether
the atrophy in the esophageal musculature is a primary effect
or occurs secondary to a neurogenic disorder is unknown.
The results of pharmacologic and hormonal manipulation,
with agents that act either indirectly via neural mechanisms
or directly on the muscle, suggest that scleroderma is a primary neurogenic disorder. Methacholine, which acts directly
on smooth muscle receptors, causes a similar increase in LES
pressure in normal controls and in patients with scleroderma.
Edrophonium, a cholinesterase inhibitor that enhances the
effect of acetylcholine when given to patients with scleroderma, causes an increase in LES pressure that is less marked in
these patients than in normal controls, suggesting a neurogenic
rather than myogenic etiology. Muscle ischemia due to perivascular compression has been suggested as a possible mechanism for the motility abnormality in scleroderma. Others have
observed that in the early stage of the disease, the manometric abnormalities may be reversed by reserpine, an agent that
depletes catecholamines from the adrenergic system. This suggests that, in early scleroderma, an adrenergic overactivity may
be present that causes a parasympathetic inhibition, supporting
Scleroderma
Esophagus
25 cm
S
mmHg
S S
S
35 –
0
35 –
Esophagus
30 cm
0
35 –
Esophagus
35 cm
0
Figure 25-42. Esophageal motility record in a patient with scleroderma showing aperistalsis in the distal two-thirds of the esophageal body with peristalsis in the proximal portion. (Reproduced
with permission from Waters PF, DeMeester TR: Foregut motor
disorders and their surgical management, Med Clin North Am.
1981 Nov;65(6):1235-1268.)
a neurogenic mechanism for the disease. In advanced disease
manifested by smooth muscle atrophy and collagen deposition,
reserpine no longer produces this reversal. Consequently, from
a clinical perspective, the patient can be described as having a
poor esophageal pump and a poor valve.
The diagnosis of scleroderma can be made manometrically
by the observation of normal peristalsis in the proximal striated
esophagus, with absent peristalsis in the distal smooth muscle portion (Fig. 25-42). The LES pressure is progressively weakened
as the disease advances. Because many of the systemic sequelae
of the disease may be nondiagnostic, the motility pattern is frequently used as a specific diagnostic indicator. Gastroesophageal
reflux commonly occurs in patients with scleroderma because they
have both hypotensive sphincters and poor esophageal clearance.
This combined defect can lead to severe esophagitis and stricture
formation. The typical barium swallow shows a dilated, bariumfilled esophagus, stomach, and duodenum, or a hiatal hernia with
distal esophageal stricture and proximal dilatation (Fig. 25-43).
Traditionally, esophageal symptoms have been treated
with PPIs, antacids, elevation of the head of the bed, and
multiple dilations for strictures, with generally unsatisfactory results. The degree of esophagitis is usually severe and
may lead to marked esophageal shortening as well as stricture. Scleroderma patients have frequently had numerous
dilations before they are referred to the surgeon. The surgical management is somewhat controversial, but the majority of opinion suggests that a partial fundoplication (anterior
or posterior) performed laparoscopically is the procedure of
choice. The need for a partial fundoplication is dictated by
the likelihood of severe dysphagia if a total fundoplication is
performed in the presence of aperistalsis. Esophageal shortening may require a Collis gastroplasty in combination with
a partial fundoplication. Surgery reduces esophageal acid
exposure but does not return it to normal because of the poor
EOSINOPHILIC ESOPHAGITIS
1051
Eosinophilic esophagitis (EE) was first described in 1977, but it
has become well known only in the last two decades. The condition is characterized by a constellation of symptoms, endoscopic
and radiologic findings, and distinctive pathology. The etiology
of eosinophilic esophagitis is not entirely known but its similarities, immunologically, to asthma suggest that it is a form of
“allergic esophagitis.”
The presentation of eosinophilic esophagitis is chest pain (often
postprandial) and dysphagia. Dysphagia may occur with liquids
or solids, but solid food dysphagia is most common. Because
dysphagia and chest pain are characteristic of GERD, EE is
often confused with GERD; however, EE does not respond to
proton pump inhibitors. The evaluation of the patient with EE
and dysphagia and chest pain with esophagram and endoscopy
usually reveals the diagnosis.
Signs
A barium swallow should be the first test obtained in the patient
with dysphagia. EE has a characteristic finding often called the
“ringed esophagus” or the “feline esophagus,” as the esophageal
rings are felt to look like the stripes on a housecat (Fig. 25-44).
The endoscopic appearance of EE is also characteristic, and also
appears as a series of rings (Fig. 25-45).
Pathology
Figure 25-43. Barium esophagogram of a patient with scleroderma and stricture. Note the markedly dilated esophagus and
retained food material. (Reproduced with permission from Waters
PF, DeMeester TR: Foregut motor disorders and their surgical
management, Med Clin North Am. 1981 Nov;65(6):1235-1268.)
clearance function of the body of the esophagus. Only 50%
of the patients have a good-to-excellent result. If the esophagitis is severe, or there has been a previous failed antireflux
procedure and the disease is associated with delayed gastric
emptying, a gastric resection with Roux-en-Y gastrojejunostomy has proved the best option.
Endoscopic biopsy specimens should be taken when eosinophilic esophagus is suspected. To make the diagnosis of
EE, the pathologist should see a minimum of 15 eosinophils
per high powered field, usually at the base of the epithelium
(Fig. 25-46).
Treatment
The treatment of EE is largely symptomatic and includes testing for food allergies and elimination of identified items from
the diet. Second-line therapy includes inhaled or ingested corticosteroids, as would be used to treat asthma. If dysphagia
is not relieved with steroids, it may be necessary to dilate the
Figure 25-44. The esophagus on the left shows a stacking of rings, demonstrating eosinophilic esophagus. The esophagus on the right is a
normal barium swallow.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Symptoms
1052
the meal, is the last to finish, or is forced to interrupt or avoid a
social meal; and whether he or she has been admitted to the hospital for food impaction. These assessments, plus an evaluation
of the patient’s nutritional status, help to determine how severe
the dysphagia is and judge the need for surgical intervention,
rather than more conservative methods of treating dysphagia.
Motility Disorders of the Pharynx and Upper
Esophagus—Transit Dysphagia
PART II
SPECIFIC CONSIDERATIONS
Figure 25-45. The endoscopic appearance of eosinophilic esophagitis is characteristically a series of stacked mucosal rings.
esophagus. Because of the length of esophageal involvement,
rigid dilators (Maloney or Savary) are often used. Great care
must be exercised, as the inflamed EE is quite friable. The
mucosal tears easily, and esophageal perforation (full thickness
laceration) has been reported with EE dilation.
MOTILITY DISORDERS OF THE PHARYNX AND
ESOPHAGUS
Clinical Manifestations
Dysphagia (i.e., difficulty in swallowing) is the primary symptom of esophageal motor disorders. Its perception by the patient
is a balance between the severity of the underlying abnormality
causing the dysphagia and the adjustment made by the patient in
altering eating habits. Consequently, any complaint of dysphagia must include an assessment of the patient’s dietary history.
It must be known whether the patient experiences pain, chokes,
or vomits with eating; whether the patient requires liquids with
Disorders of the pharyngeal phase of swallowing result from a
discoordination of the neuromuscular events involved in chewing, initiation of swallowing, and propulsion of the material
from the oropharynx into the cervical esophagus. They can be
categorized into one or a combination of the following abnormalities: (a) inadequate oropharyngeal bolus transport; (b)
inability to pressurize the pharynx; (c) inability to elevate the
larynx; (d) discoordination of pharyngeal contraction and cricopharyngeal relaxation; and (e) decreased compliance of the
pharyngoesophageal segment secondary to neuromuscular disease. The latter may result in incomplete relaxation of the cricopharyngeus and cervical esophagus during swallowing. Taken
together, these disorders are termed transit dysphagia by many.
Transit dysphagia is usually congenital or results from
acquired disease involving the central and peripheral nervous
system. This includes cerebrovascular accidents, brain stem
tumors, poliomyelitis, multiple sclerosis, Parkinson’s disease,
pseudobulbar palsy, peripheral neuropathy, and operative damage to the cranial nerves involved in swallowing. Pure muscular
diseases such as radiation-induced myopathy, dermatomyositis,
myotonic dystrophy, and myasthenia gravis are less common
causes. Rarely, extrinsic compression of the cervical esophagus
by thyromegaly, lymphadenopathy, or hyperostosis of the cervical spine can cause transit dysphagia.
Diagnostic Assessment of the Cricopharyngeal
Segment
Transit dysphagia difficult to assess with standard manometric
techniques because of the rapidity of the oropharyngeal phase of
swallowing, the elevation of the larynx, and the asymmetry of
the cricopharyngeus. Video- or cineradiography is currently the
Figure 25-46. A cluster of eosinophils are visualized in the esophageal epithelium in a patient with EE.
Time 0
Peak pharyngeal
pressure
1053
Atmospheric
pressure
Bolus pressure
final
initial
A
B
Figure 25-47. A. Zenker’s diverticulum, initially discovered
15 years ago and left untreated. B. Note its marked enlargement
and evidence of laryngeal inlet aspiration on recent esophagogram.
(Reproduced with permission from Waters PF, DeMeester TR:
Foregut motor disorders and their surgical management, Med Clin
North Am. 1981 Nov;65(6):1235-1268.)
Maximum residual
(MaxR)
contraction B0
Minimum Residual
(MinR)
Subatomic pressure
B
most objective test to evaluate oropharyngeal bolus transport,
pharyngeal compression, relaxation of the pharyngoesophageal
segment, and the dynamics of airway protection during swallowing. It readily identifies a diverticulum (Fig. 25-47), stasis
of the contrast medium in the valleculae, a cricopharyngeal bar,
and/or narrowing of the pharyngoesophageal segment. These
are anatomic manifestations of neuromuscular disease, and they
result from the loss of muscle compliance in portions of the
pharynx and esophagus composed of skeletal muscle.
Careful analysis of video- or cineradiographic studies combined with manometry using specially designed catheters can
identify the cause of a pharyngoesophageal dysfunction in most situations (Fig. 25-48). Motility studies may demonstrate inadequate
pharyngeal pressurization, insufficient or lack of cricopharyngeal
relaxation, marked discoordination of pharyngeal pressurization,
cricopharyngeal relaxation and cervical esophageal contraction,
or a hypopharyngeal bolus pressure suggesting decreased compliance of the skeletal portion of the cervical esophagus.
In many patients with cricopharyngeal dysfunction,
including those with Zenker’s diverticulum, it has been difficult
to consistently demonstrate a motility abnormality or discoordination of pharyngoesophageal events. The abnormality most
apt to be present is a loss of compliance in the pharyngoesophageal segment manifested by an increased bolus pressure. Cook
and colleagues have demonstrated an increased resistance to the
movement of a bolus through what appears on manometry to be
a completely relaxed cricopharyngeal sphincter. Using simultaneous manometry and videofluoroscopy, they showed that, in
these patients, the cricopharyngeus is only partially relaxed; that
is, the sphincter is relaxed enough to allow a drop of its pressure
to esophageal baseline on manometry, but insufficiently relaxed
to allow unimpaired passage of the bolus into the esophagus.
This incomplete relaxation is due to a loss of compliance of the
muscle in the pharyngoesophageal segment, and may be associated with a cricopharyngeal bar or Zenker’s diverticulum. This
decreased compliance of the cricopharyngeal sphincter can be
Figure 25-48. A. Schematic drawing of a pharyngeal pressure
wave indicating the presence of the bolus pressure. B. Schematic
drawing of the manometric recording typically seen during cricopharyngeal sphincter relaxation.
recognized on esophageal manometry by a “shoulder” on the
pharyngeal pressure wave, the amplitude of which correlates
directly with the degree of outflow obstruction (Fig. 25-49).
Increasing the diameter of this noncompliant segment reduces
the resistance imposed on the passage of a bolus. Consequently,
patients with low pharyngeal pressure (i.e., poor piston function
of the pharynx), or patients with increased resistance of the pharyngocervical esophageal segment from loss of skeletal muscle
compliance, are improved by a cricopharyngeal myotomy. This
enlarges the pharyngoesophageal segment and reduces outflow
resistance. Esophageal muscle biopsy specimens from patients
with Zenker’s diverticulum have shown histologic evidence of
the restrictive myopathy in the cricophayngeous muscle. These
findings correlate well with the observation of a decreased compliance of the upper esophagus demonstrated by videoradiography and the findings on detailed manometric studies of the
pharynx and cervical esophagus. They suggest that the diverticulum develops as a consequence of the outflow resistance to
bolus transport through the noncompliant muscle of the pharyngoesophageal segment.
The requirements for a successful pharyngoesophageal
myotomy are (a) adequate oropharyngeal bolus transport; (b) the
presence of an intact swallowing reflex; (c) reasonable coordination of pharyngeal pressurization with cricopharyngeal relaxation; and (d) a cricopharyngeal bar, Zenker’s diverticulum, or a
narrowed pharyngoesophageal segment on videoesophagogram
and/or the presence of excessive pharyngoesophageal shoulder
pressure on motility study.
Zenker’s Diverticulum. In the past, the most common recognized sign of cricopharyngeal dysfunction was the presence of a
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
A
40
200
30
150
Zenker’s
20
10
UES area mm2
Pharyngeal shoulder
pressure mmHg
1054
Controls
0
5
10
15
Controls
100
Zenker’s
50
PART II
0
20
Swallow volume
5
10
15
20
SPECIFIC CONSIDERATIONS
Zenker’s diverticulum, originally described by Ludlow in 1769.
The eponym resulted from Zenker’s classic clinicopathologic
descriptions of 34 cases published in 1878. Pharyngoesophageal
diverticula have been reported to occur in 1 of 1000 routine
barium examinations, and classically occur in elderly, white
males. Zenker’s diverticula tend to enlarge progressively with
time due to the decreased compliance of the skeletal portion of
the cervical esophagus that occurs with aging.
Presenting symptoms include dysphagia associated with
the spontaneous regurgitation of undigested, bland material,
often interrupting eating or drinking. On occasion, the dysphagia can be severe enough to cause debilitation and significant
weight loss. Chronic aspiration and repetitive respiratory infection are common associated complaints. Once suspected, the
diagnosis is established by a barium swallow. Endoscopy is
usually difficult in the presence of a cricopharyngeal diverticulum, and potentially dangerous, owing to obstruction of the true
esophageal lumen by the diverticulum and the attendant risk of
diverticular perforation.
Cricopharyngeal Myotomy. The low morbidity and mortality associated with cricopharyngeal and upper esophageal
myotomy have encouraged a liberal approach toward its use for
almost any problem in the oropharyngeal phase of swallowing.
This attitude has resulted in an overall success rate in the relief
of symptoms of only 64%. When patients are selected for surgery using radiographic or motility markers of disease, a much
higher proportion will benefit. Two methods of cricopharyngoesophageal myotomy are in common use, one using traditional
surgical approaches, and one using rigid laryngoscopy and a
linear cutting stapler.
mm Hg
40–
Figure 25-49. Pharyngeal shoulder pressures and diameter
of the pharyngoesophageal segment in controls and patients
with Zenker’s diverticulum. UES = upper esophageal
sphincter. (Data from Cook IJ, et al. Zenker’s diverticulum: evidence for a restrictive cricopharyngeal myopathy.
Gastroenterology. 1989;96:A98.)
Open Cricopharyngeal Myotomy, Diverticulopexy, and
Diverticulectomy. The myotomy can be performed under local
or general anesthesia through an incision along the anterior border
of the left sternocleidomastoid muscle. The pharynx and cervical esophagus are exposed by retracting the sternocleidomastoid
muscle and carotid sheath laterally and the thyroid, trachea,
and larynx medially (Fig. 25-50). When a pharyngoesophageal
diverticulum is present, localization of the pharyngoesophageal
segment is easy. The diverticulum is carefully freed from the
overlying areolar tissue to expose its neck, just below the inferior
pharyngeal constrictor and above the cricopharyngeus muscle.
It can be difficult to identify the cricopharyngeus muscle in the
absence of a diverticulum. A benefit of local anesthesia is that the
patient can swallow and demonstrate an area of persistent narrowing at the pharyngoesophageal junction. Furthermore, before
closing the incision, gelatin can be fed to the patient to ascertain
whether the symptoms have been relieved, and to inspect the
opening of the previously narrowed pharyngoesophageal segment. Under general anesthesia, and in the absence of a diverticulum, the placement of a nasogastric tube to the level of the
manometrically determined cricopharyngeal sphincter helps in
localization of the structures. The myotomy is extended cephalad
by dividing 1 to 2 cm of inferior constrictor muscle of the pharynx, and caudad by dividing the cricopharyngeal muscle and the
cervical esophagus for a length of 4 to 5 cm. The cervical wound
is closed only when all oozing of blood has ceased because a
hematoma after this procedure is common and is often associated
with temporary dysphagia while the hematoma absorbs. Oral alimentation is started the day after surgery. The patient is usually
discharged on the first or second postoperative day.
Hypopharynx
0
40
30
20
10
0
Cricopharyngeus
Figure 25-50. Cross-section of the neck at the
level of the thyroid isthmus that shows the surgical approach to the hypopharynx and cervical
esophagus. (Reproduced with permission from
Waters PF, DeMeester TR: Foregut motor disorders and their surgical management, Med
Clin North Am. 1981 Nov;65(6):1235-1268.)
1055
Zenker’s
diverticulum
If a diverticulum is present and is large enough to persist
after a myotomy, it may be sutured in the inverted position to
the prevertebral fascia using a permanent suture (i.e., diverticulopexy) (Fig. 25-51). If the diverticulum is excessively large so
that it would be redundant if suspended, or if its walls are thickened, a diverticulectomy should be performed. This is best performed under general anesthesia by placing a Maloney dilator
(48F) in the esophagus, after controlling the neck of the diverticulum and after myotomy. A linear stapler is placed across the
neck of the diverticulum, and the diverticulum is excised distal
to the staple line. The security of this staple line and effectiveness of the myotomy may be tested before hospital discharge
with a water-soluble contrast esophagogram. Postoperative
complications include fistula formation, abscess, hematoma,
recurrent nerve paralysis, difficulties in phonation, and Horner’s
syndrome. The incidence of the first two can be reduced by performing a diverticulopexy rather than diverticulectomy.
Endoscopic Cricopharyngotomy. Endoscopic stapled cricopharyngotomy and diverticulotomy recently has been described.
This procedure is most effective for larger diverticula (>2 cm)
and may be impossible to perform for the small diverticulum.
The procedure uses a specialized “diverticuloscope” with two
retractable valves passed into the hypopharynx. The lips of the
diverticuloscope are positioned so that one lip lies in the esophageal lumen and the other in the diverticular lumen. The valves
of the diverticuloscope are retracted appropriately so as to visualize the septum interposed between the diverticulum and the
esophagus. An endoscopic linear stapler is introduced into the
diverticuloscope and positioned against the common septum
with the anvil in the diverticulum and the cartridge in the esophageal lumen. Firing of the stapler divides the common septum
between the posterior esophageal and the diverticular wall over
a length of 30 mm, placing three rows of staples on each side.
More than one stapler application may be needed, depending on
the size of the diverticulum (Fig. 25-52). The patient is allowed
to resume liquid feeds immediately and is usually discharged
the day after surgery. Complications are rare and may include
perforation at the apex of the diverticulum and failure to relieve
dysphagia resulting from incomplete myotomy. The former
complication can usually be treated with antibiotics, but it may,
rarely, require neck drainage.
Recurrence of a Zenker’s diverticulum may occur with
long follow-up and is more common after diverticulectomy
without myotomy, presumably due to persistence of the underlying loss of compliance of the cervical esophagus when a myotomy is not performed. After endoscopic cricopharyngotomy
Prevertebral
fascia
Figure 25-51. Posterior of the anatomy
of the pharynx and cervical esophagus
showing pharyngoesophageal myotomy
and pexing of the diverticulum to the
prevertebral fascia.
lateral residual “pouches” may be seen on radiographs, but they
are rarely responsible for residual or recurrent symptoms if the
myotomy has been complete.
Postoperative motility studies have shown that the peak
pharyngeal pressure generated on swallowing is not affected,
the resting cricopharyngeal pressure is reduced but not eliminated, and the cricopharyngeal sphincter length is shortened.
Consequently, after myotomy, there is protection against esophagopharyngeal regurgitation.
Motility Disorders of the Esophageal Body and
Lower Esophageal Sphincter
Disorders of the esophageal phase of swallowing result from
abnormalities in the propulsive pump action of the esophageal
body or the relaxation of the LES. These disorders result from
either primary esophageal abnormalities, or from generalized
neural, muscular, or collagen vascular disease (Table 25-8).
The use of standard and high-resolution esophageal manometry
techniques has allowed specific primary esophageal motility
disorders to be identified out of a pool of nonspecific motility abnormalities. Primary esophageal motor disorders include
achalasia, DES, nutcracker esophagus, and the hypertensive
LES. The manometric characteristics of these disorders are
shown in Table 25-9.
The boundaries between the primary esophageal motor
disorders are vague, and intermediate types exist, some of which
may combine more than one type of motility pattern. These
findings indicate that esophageal motility disorders should be
looked at as a spectrum of abnormalities that reflects various
stages of destruction of esophageal motor function.
Achalasia. The best known and best understood primary motility disorder of the esophagus is achalasia, with an incidence of six
Figure 25-52. The technique for transoral cricopharyngotomy and
Zenker’s diverticulotomy.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Myotomy
1056
Table 25-8
Esophageal motility disorders
Primary esophageal motility disorders
Achalasia, “vigorous” achalasia
Diffuse and segmental esophageal spasm
Nutcracker esophagus
Hypertensive lower esophageal sphincter
Nonspecific esophageal motility disorders
PART II
SPECIFIC CONSIDERATIONS
Secondary esophageal motility disorders
Collagen vascular diseases: progressive systemic sclerosis,
polymyositis and dermatomyositis, mixed connective
tissue disease, systemic lupus erythematosus, etc.
Chronic idiopathic intestinal pseudoobstruction
Neuromuscular diseases
Endocrine and metastatic disorders
per 100,000 population per year. Although complete absence of
in the esophageal body has been proposed as the
5 peristalsis
major abnormality, present evidence indicates achalasia is a
primary disorder of the LES. This is based on 24-hour outpatient
esophageal motility monitoring, which shows that, even in
advanced disease, up to 5% of contractions can be peristaltic.
Table 25-9
Manometric characteristics of the primary esophageal
motility disorders
Achalasia
Incomplete lower esophageal sphincter (LES) relaxation
(<75% relaxation)
Aperistalsis in the esophageal body
Elevated LES pressure ≤26 mmHg
Increased intraesophageal baseline pressures relative to
gastric baseline
Diffuse esophageal spasm (DES)
Simultaneous (nonperistaltic contractions) (>20% of wet
swallows)
Repetitive and multipeaked contractions
Spontaneous contractions
Intermittent normal peristalsis
Contractions may be of increased amplitude and duration
Nutcracker esophagus
Mean peristaltic amplitude (10 wet swallows) in distal
esophagus ≥180 mmHg
Increased mean duration of contractions (>7.0 s)
Normal peristaltic sequence
Hypertensive lower esophageal sphincter
Elevated LES pressure (≥26 mmHg)
Normal LES relaxation
Normal peristalsis in the esophageal body
Ineffective esophageal motility disorders
Decreased or absent amplitude of esophageal peristalsis
(<30 mmHg)
Increased number of nontransmitted contractions
Reproduced with permission from Zuidema GD, Orringer MB:
Shackelford’s Surgery of the Alimentary Tract, 3rd ed. Vol 1.
Philadelphia, PA: Elsevier/Saunders; 1991.
Simultaneous esophageal waves develop as a result of the
increased resistance to esophageal emptying caused by the nonrelaxing LES. This conclusion is supported by experimental studies
in which a band placed loosely around the GEJ in experimental
models did not change sphincter pressures but resulted in impaired
relaxation of the LES and outflow resistance. This led to a markedly increased frequency of simultaneous waveforms and a
decrease in contraction amplitude. The changes were associated
with radiographic dilation of the esophagus and were reversible
after removal of the band. Observations in patients with pseudoachalasia due to tumor infiltration, a tight stricture in the distal
esophagus, or an antireflux procedure that is too tight also provide
evidence that dysfunction of the esophageal body can be caused
by the increased outflow obstruction of a nonrelaxing LES. The
observation that esophageal peristalsis can return in patients with
classic achalasia following dilation or myotomy provides further
support that achalasia is a primary disease of the LES.
The pathogenesis of achalasia is presumed to be a neurogenic degeneration, which is either idiopathic or due to infection. In experimental animals, the disease has been reproduced by
destruction of the nucleus ambiguus and the dorsal motor nucleus
of the vagus nerve. In patients with the disease, degenerative
changes have been shown in the vagus nerve and in the ganglia
in the myenteric plexus of the esophagus itself. This degeneration
results in hypertension of the LES, a failure of the sphincter to
relax on swallowing, elevation of intraluminal esophageal pressure, esophageal dilatation, and a subsequent loss of progressive
peristalsis in the body of the esophagus. The esophageal dilatation
results from the combination of a nonrelaxing sphincter, which
causes a functional retention of ingested material in the esophagus, and elevation of intraluminal pressure from repetitive pharyngeal air swallowing (Fig. 25-53). With time, the functional
disorder results in anatomic alterations seen on radiographic studies, such as a dilated esophagus with a tapering, “bird’s beak”-like
narrowing of the distal end (Fig. 25-54). There is usually an airfluid level in the esophagus from the retained food and saliva, the
height of which reflects the degree of resistance imposed by the
nonrelaxing sphincter. As the disease progresses, the esophagus
becomes massively dilated and tortuous.
A subgroup of patients with otherwise typical features of
classic achalasia has simultaneous contractions of their esophageal body that can be of high amplitude. This manometric pattern
has been termed vigorous achalasia, and chest pain episodes are a
common finding in these patients. Since the development of high
resolution esophageal manometry technology, the term vigorous
achalasia has been replaced with Chicago type 3 achalasia. Differentiation of type 3 achalasia from DES can be difficult. In
both diseases, videoradiographic examination may show a corkscrew deformity of the esophagus and diverticulum formation.
Diffuse and Segmental Esophageal Spasm. DES is characterized by substernal chest pain and/or dysphagia. DES differs
from classic achalasia in that it is primarily a disease of the
esophageal body, produces a lesser degree of dysphagia, causes
more chest pain, and has less effect on the patient’s general condition. Nonetheless, it is impossible to differentiate achalasia
from DES on the basis of symptoms alone. Esophagogram and
esophageal manometry are required to distinguish these two
entities. True symptomatic DES is a rare condition, occurring
about five times less frequently than achalasia.
The causation and neuromuscular pathophysiology of
DES are unclear. The basic motor abnormality is rapid wave
progression down the esophagus secondary to an abnormality in
1057
100 mmHg
10 mins
3 140
120
100
80
60
40
20
0
–20
10 secs
4 60
50
40
30
20
10
0
–10
–20
100 mmHg
6 45
35
* 25
15
9
–5
–15
–25
–35
A
3150
140
* 120
100
80
60
40
20
0
Meal
4150
140
* 120
100
80
60
40
20
0
5150
140
* 120
100
80
60
40
20
0
6145
125
* 105
100
85
65
45
5
–15
B
Figure 25-53. Pressurization of esophagus: ambulatory motility
tracing of a patient with achalasia. A. Before esophageal myotomy.
B. After esophageal myotomy. The tracings have been compressed
to exaggerate the motility spikes and baseline elevations. Note the
rise in esophageal baseline pressure during a meal represented by
the rise off the baseline to the left of panel A. No such rise occurs
postmyotomy (B).
the latency gradient. Hypertrophy of the muscular layer of the
esophageal wall and degeneration of the esophageal branches
of the vagus nerve have been observed in this disease, although
these are not constant findings. Manometric abnormalities in
DES may be present over the total length of the esophageal body
but usually are confined to the distal two-thirds. In segmental
esophageal spasm, the manometric abnormalities are confined
to a short segment of the esophagus.
The classic manometric findings in these patients are
characterized by the frequent occurrence of simultaneous waveforms and multipeaked esophageal contractions, which may be
of abnormally high amplitude or long duration. Key to the diagnosis of DES is that there remain some peristaltic waveforms in
excess of those seen in achalasia. A criterion of 30% or more
peristaltic waveforms out of 10 wet swallows has been used to
differentiate DES from vigorous achalasia. However, this figure
is arbitrary and often debated.
The LES in patients with DES usually shows a normal
resting pressure and relaxation on swallowing. A hypertensive
sphincter with poor relaxation may also be present. In patients
with advanced disease, the radiographic appearance of tertiary
contractions appears helical and has been termed corkscrew
Figure 25-54. Barium esophagogram showing a markedly dilated
esophagus and characteristic “bird’s beak” in achalasia. (Reproduced with permission from Waters PF, DeMeester TR: Foregut
motor disorders and their surgical management, Med Clin North
Am. 1981 Nov;65(6):1235-1268.)
esophagus or pseudodiverticulosis (Fig. 25-55). Patients with
segmental or diffuse esophageal spasm can compartmentalize
the esophagus and develop an epiphrenic or midesophageal
diverticulum between two areas of high pressure occurring
simultaneously (Fig. 25-56).
Nutcracker Esophagus. The disorder, termed nutcracker or
supersqueezeresophagus, was recognized in the late 1970s.
Other terms used to describe this entity are hypertensive peristalsis or high-amplitude peristaltic contractions. It is the most
common of the primary esophageal motility disorders. By
definition the so-called nutcracker esophagus is a manometric abnormality in patients who are characterized by peristaltic esophageal contractions with peak amplitudes greater than
two SDs above the normal values in individual laboratories.
Contraction amplitudes in these patients can easily be above
400 mmHg. At the lower end of peak pressure, it is unclear
whether nutcracker esophagus causes any symptoms. In fact,
chest pain symptoms in nutcracker esophagus patients may
be related to GERD rather than intraluminal hypertension.
Treatment in these patients should be aimed at the treatment
of GERD. At the high end (peak pressures >300 mmHg) chest
pain may be the result of the nutcracker physiology, as treatment
directed at reducing intraluminal pressure is more effective than
when used for those with lower peak pressures.
Hypertensive Lower Esophageal Sphincter. Hypertensive lower esophageal sphincter (LES) in patients with chest
pain or dysphagia was first described as a separate entity by
Code and associates. This disorder is characterized by an elevated basal pressure of the LES with normal relaxation and
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
5 60
50
40
30
20
10
0
–10
–20
1058
PART II
normal propulsion in the esophageal body. About one-half of
these patients, however, have associated motility disorders of
the esophageal body, particularly hypertensive peristalsis and
simultaneous waveforms. In the remainder, the disorder exists
as an isolated abnormality. Dysphagia in these patients may
be caused by a lack of compliance of the sphincter, even in its
relaxed state. Myotomy of the LES may be indicated in patients
not responding to medical therapy or dilation. When the symptom contribution of the hypertensive sphincter is in doubt, it is
possible to inject the LES with botulinum toxin, endoscopically.
If symptoms are relieved (temporarily) with this technique, then
it is likely that myotomy will provide more permanent benefit.
SPECIFIC CONSIDERATIONS
Secondary Esophageal Motility Disorders. Connective
tissue disease, particularly scleroderma and the CREST syndrome, exhibits severe esophageal motility disorders. Additionally, patients treated as infants for esophageal atresia will
often develop secondary motility disorders manifest later in
life. Symptoms of these disorders are heartburn and dysphagia.
The latter may be a result of a peptic stricture rather than the
esophageal dysmotility. An esophageal motility study will usually show severely reduced or absent peristalsis with severely
reduced or absent LES pressure. The role of antireflux surgery
under these conditions is controversial but, if performed, should
be limited to partial fundoplication, as full (Nissen) fundoplication may result in severe dysphagia.
Figure 25-55. Barium esophagogram of patient with diffuse
spasm showing the corkscrew deformity.
Nonspecific Esophageal Motor Disorders and Ineffective
Esophageal Motility. Many patients complaining of dys-
Figure 25-56. Barium esophagogram showing a high epiphrenic
diverticulum in a patient with diffuse esophageal spasm. (Reproduced with permission from Castell DO: The Esophagus. Boston,
MA: Little, Brown; 1992.)
Diverticula of the Esophageal Body. Diverticula of the
esophagus may be characterized by their location in the esophagus (proximal, mid-, or distal esophagus), or by the nature of
phagia or chest pain of noncardiac origin demonstrate a variety of wave patterns and contraction amplitudes on esophageal
manometry that are clearly out of the normal range, but do
not meet the criteria of a primary esophageal motility disorder. Esophageal motility in these patients frequently shows an
increased number of multipeaked or repetitive contractions,
contractions of prolonged duration, nontransmitted contractions, an interruption of a peristaltic wave at various levels of
the esophagus, or contractions of low amplitude. These motility
abnormalities have been termed nonspecific esophageal motility
disorders. Their significance in the causation of chest pain or
dysphagia is still unclear. Surgery plays no role in the treatment
of these disorders unless there is an associated diverticulum.
A clear distinction between primary esophageal motility
disorders and nonspecific esophageal motility disorders is often
not possible. Patients diagnosed as having nonspecific esophageal
motility abnormalities on repeated studies will occasionally show
abnormalities consistent with nutcracker esophagus. Similarly,
progression from a nonspecific esophageal motility disorder to
classic DES has been demonstrated. Therefore, the finding of a
nonspecific esophageal motility disorder may represent only a
manometric marker of an intermittent, more severe esophageal
motor abnormality. Combined ambulatory 24-hour esophageal
pH and motility monitoring has shown that an increased esophageal exposure to gastric juice is common in patients diagnosed as
having a nonspecific esophageal motility disorder. In some situations, the motor abnormalities may be induced by the irritation
of refluxed gastric juice; in other situations, it may be a primary
event unrelated to the presence of reflux. High-amplitude peristalsis (nutcracker esophagus) and low-amplitude peristalsis (ineffective esophageal motility) are frequently associated with GERD.
1059
Figure 25-57. Barium esophagogram showing a midesophageal
diverticulum. Despite the anatomic distortion, the patient was
asymptomatic. (Reproduced with permission from Waters PF,
DeMeester TR: Foregut motor disorders and their surgical management, Med Clin North Am. 1981 Nov;65(6):1235-1268.)
sarcoid, may create traction esophageal diverticula after successful treatment. Rarely, when no underlying inflammatory pathology is identified, a motility disorder may be identified.
Most midesophageal diverticula are asymptomatic and
incidentally discovered during investigation for nonesophageal
complaints. In such patients, the radiologic abnormality may
Inflamed
nodes
Traction diverticulum
Figure 25-58. Illustration of the pathophysiology of midesophageal diverticulum showing traction on the esophageal wall from
adhesions to inflamed subcarinal lymph nodes.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
concomitant pathology. Diverticula associated with motor disorders are termed pulsion diverticula and those associated with
inflammatory conditions are termed traction diverticula. Pulsion
diverticula occur most commonly with nonspecific motility disorders, but they can occur with all of the primary motility disorders.
In the latter situation, the motility disorder is usually diagnosed
before the development of the diverticulum. When associated
with achalasia, the development of a diverticulum may temporarily alleviate the symptom of dysphagia by becoming a receptacle
for ingested food and substitute the symptom of dysphagia for
postprandial pain and regurgitation of undigested food. If a motility abnormality of the esophageal body or LES cannot be identified, a traction or congenital cause for the diverticulum should be
considered.
Because development in radiology preceded development in motility monitoring, diverticula of the esophagus were
considered historically to be a primary abnormality, the cause,
rather than the consequence, of motility disorders. Consequently, earlier texts focused on them as specific entities based
upon their location.
Epiphrenic diverticula arise from the terminal third of
the thoracic esophagus and are usually found adjacent to the
diaphragm. They have been associated with distal esophageal
muscular hypertrophy, esophageal motility abnormalities, and
increased luminal pressure. They are “pulsion” diverticula, and
they are associated with diffuse spasm, achalasia, or nonspecific
motor abnormalities in the body of the esophagus.
Whether the diverticulum should be surgically resected
or suspended depends on its size and proximity to the vertebral
body. When diverticula are associated with esophageal motility
disorders, esophageal myotomy from the proximal extent of the
diverticulum to the stomach should be combined with diverticulectomy. If diverticulectomy alone is performed, one can expect
a high incidence of suture line rupture due to the same intraluminal pressure that initially gave rise to the diverticulum. If the
diverticulum is suspended to the prevertebral fascia of the thoracic vertebra, a myotomy is begun at the neck of the diverticulum and extended across the LES. If the diverticulum is excised
by dividing the neck, the muscle is closed over the excision site,
and a myotomy is performed on the opposite esophageal wall,
starting just above the level of the diverticulum or at the proximal
extent of the spastic segment of the esophagus if high resolution
motility is used. If complete, the myotomy will cross the LES,
reducing distal esophageal peak pressure, and it will increase the
likelihood that dysphagia will be replaced with GERD symptoms. Increasingly, partial fundoplication (anterior or posterior)
is performed after LES myotomy to decrease the frequency of
disabling GERD developing after myotomy and diverticulectomy. When a large diverticulum is associated with a hiatal hernia, then hiatal hernia repair is added. All these procedures may
be performed with traditional or minimally invasive techniques.
Midesophageal or traction diverticula were first described
in the 19th century (Fig. 25-57). At that time, they were frequently noted in patients who had mediastinal LN involvement with tuberculosis. It was theorized that adhesions formed
between the inflamed mediastinal nodes and the esophagus. By
contraction, the adhesions exerted traction on the esophageal
wall and led to a localized diverticulum (Fig. 25-58). This theory
was based on the findings of early dissections, where adhesions
between diverticula and LNs were commonly found. Other conditions associated with mediastinal lymphadenopathy, such as
pulmonary fungal infections (e.g., aspergillosis), lymphoma, or
1060
PART II
be ignored. Patients with symptoms of dysphagia, regurgitation, chest pain, or aspiration, in whom a diverticulum is discovered, should be thoroughly investigated for an esophageal
motor abnormality. Occasionally, a patient will present with a
bronchoesophageal fistula manifested by a chronic cough on
ingestion of meals. The diverticulum in such patients is most
likely to have an inflammatory etiology.
The indication for surgical intervention is dictated by
the degree of symptomatic disability. Usually, midesophageal
diverticula can be suspended due to their proximity to the spine.
If a motor abnormality is documented, a myotomy should be
performed as described for an epiphrenic diverticulum.
OPERATIONS FOR ESOPHAGEAL MOTOR
DISORDERS AND DIVERTICULA
SPECIFIC CONSIDERATIONS
Long Esophageal Myotomy for Motor Disorders
of the Esophageal Body
A long esophageal myotomy is indicated for dysphagia caused
by any motor disorder characterized by segmental or generalized simultaneous waveforms in a patient whose symptoms are
not relieved by medical therapy. Such disorders include diffuse
and segmental esophageal spasm, vigorous or type 3 achalasia,
and nonspecific motility disorders associated with a mid- or
epiphrenic esophageal diverticulum. However, the decision to
operate must be made by a balanced evaluation of the patient’s
symptoms, diet, lifestyle adjustments, and nutritional status,
with the most important factor being the possibility of improving the patient’s swallowing disability. The symptom of chest
pain alone is not an indication for a surgical procedure.
The identification of patients with symptoms of dysphagia and chest pain who might benefit from a surgical myotomy
is difficult. Ambulatory motility studies have shown that when
the prevalence of “effective contractions” (i.e., peristaltic
waveforms consisting of contractions with an amplitude above
30 mmHg) drops below 50% during meals, the patient is likely
to experience dysphagia (Fig. 25-59). This would suggest that
relief from the symptom can be expected with an improvement
of esophageal contraction amplitude or amelioration of nonperistaltic waveforms. Prokinetic agents may increase esophageal contraction amplitude, but they do not alter the prevalence
of simultaneous waveforms. Patients in whom the efficacy of
esophageal propulsion is severely compromised because of a
100%
80%
60%
40%
20%
0%
Normal volunteers
Pat, no dysphagia
Pat, dysphagia
Figure 25-59. Prevalence of effective contractions (i.e., peristaltic
contractions with an amplitude >30 mmHg) during meal periods in
individual normal volunteers, patients (Pat) without dysphagia, and
patients with nonobstructive dysphagia.
100%
% Symptomatic
80%
60%
40%
10 cm
% Retention
20%
Eso. diameter
0%
N
Pre Rx 0–24 25–48 49–72 73–120
mo
mo
mo
mo
17
17
16
14
12
5 cm
0 cm
Figure 25-60. Esophageal (Eso.) diameter, dysphagia, and esophageal retention in patients with achalasia treated with myotomy and
Nissen fundoplication, 10 years after treatment (Rx). (Data from
Topart P, Deschamps C, Taillefer R, et al: Long-term effect of total
fundoplication on the myotomized esophagus, Ann Thorac Surg.
1992 Dec;54(6):1046-1051.)
high prevalence of simultaneous waveforms usually receive
little benefit from medical therapy. In these patients, a surgical myotomy of the esophageal body can improve the patients’
dysphagia, provided the loss of contraction amplitude in the
remaining peristaltic waveforms, caused by the myotomy, has
less effect on swallowing function than the presence of the
excessive simultaneous contractions. This situation is reached
when the prevalence of effective waveforms during meals drops
below 30% (i.e., 70% of esophageal waveforms are ineffective).
In patients selected for surgery, preoperative highresolution manometry is essential to determine the proximal
extent of the esophageal myotomy. Most surgeons extend the
myotomy distally across the LES to reduce outflow resistance.
Consequently, some form of antireflux protection is needed
to avoid gastroesophageal reflux if there has been extensive
dissection of the cardia. In this situation, most authors prefer
a partial, rather than a full, fundoplication, in order not to add
back-resistance that will further interfere with the ability of the
myotomized esophagus to empty (Fig. 25-60). If the symptoms
of reflux are present preoperatively, 24-hour pH monitoring is
required to confirm its presence.
The procedure may be performed either open or via
thoracoscopy. The open technique is performed through a
left thoracotomy in the sixth intercostal space (Fig. 25-61).
An incision is made in the posterior mediastinal pleura over
the esophagus, and the left lateral wall of the esophagus is
exposed. The esophagus is not circumferentially dissected
unless necessary. A 2-cm incision is made into the abdomen
through the parietal peritoneum at the midportion of the left
crus. A tongue of gastric fundus is pulled into the chest. This
exposes the GEJ and its associated fat pad. The latter is excised
to give a clear view of the junction. A myotomy is performed
through all muscle layers, extending distally over the stomach
1 to 2 cm below the GEJ, and proximally on the esophagus over
the distance of the manometric abnormality. The muscle layer
is dissected from the mucosa laterally for a distance of 1 cm.
Care is taken to divide all minute muscle bands, particularly
in the area of the GEJ. The gastric fundic tongue is sutured to
the margins of the myotomy over a distance of 3 to 4 cm and
replaced into the abdomen. This maintains separation of the
muscle and acts as a partial fundoplication to prevent reflux.
1061
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Figure 25-61. Technique of long myotomy: A. Exposure of the lower esophagus through the left sixth intercostal space and incision of
the mediastinal pleura in preparation for surgical myotomy. B. Location of a 2-cm incision made through the phrenoesophageal membrane into the abdomen along the midlateral border of the left crus. C. Retraction of tongue of gastric fundus into the chest through the
previously made incision. D. Removal of the gastroesophageal fat pad to expose the gastroesophageal junction. E. A myotomy down
to the mucosa is started on the esophageal body. F. Completed myotomy extending over the stomach for 1 cm. G. Reconstruction of
the cardia after a myotomy, illustrating the position of the sutures used to stitch the gastric fundic flap to the margins of the myotomy.
H. Reconstruction of the cardia after a myotomy, illustrating the intra-abdominal position of the gastric tongue covering the distal
4 cm of the myotomy.
1062
PART II
SPECIFIC CONSIDERATIONS
Figure 25-61. (Continued )
If an epiphrenic diverticulum is present, it is excised by
dividing the neck with a stapler sized for the thickness of the
diverticulum (2.0- to 4.8-mm staple leg length) followed by a
closure of the muscle over the staple line, when possible. The
myotomy is then performed on the opposite esophageal wall. If
a midesophageal diverticulum is present, the myotomy is made
so that it includes the muscle around the neck, and the diverticulum is suspended by attaching it to the paravertebral fascia
of the thoracic vertebra above the level of the diverticular neck.
Before performing any operation for an esophageal diverticulum, it is wise to endoscope the patient to wash all food and
other debris from the diverticulum.
The results of myotomy for motor disorders of the esophageal body have improved in parallel with the improved preoperative diagnosis afforded by manometry. Previous published
series report between 40% and 92% improvement of symptoms,
but interpretation is difficult due to the small number of patients
involved and the varying criteria for diagnosis of the primary
motor abnormality. When myotomy is accurately done, 93% of the
patients have effective palliation of dysphagia after a mean followup of 5 years, and 89% would have the procedure again, if it was
necessary. Most patients gain or maintain rather than lose weight
after the operation. Postoperative motility studies show that the
myotomy reduces the amplitude of esophageal contractions to near
zero and eliminates simultaneous peristaltic waves. If the benefit
of obliterating the simultaneous waves exceeds the adverse effect
on bolus propulsion caused by the loss of peristaltic waveforms,
the patient’s dysphagia is likely to be improved by the procedure.
If not, the patient is likely to continue to complain of dysphagia
and to have little improvement as a result of the operation.
The thoracoscopic technique may be performed through
the left or right chest. There has been little experience gained
with doing adequate operations (as described previously with
the open exposure) through left thoracoscopy, so most surgeons
will combine a right thoracoscopic long myotomy with an
abdominal approach for Heller myotomy and partial fundoplication. These two procedures may be done at the same setting,
by double positioning the patient, or they may be done at two
operations. If this is the case, it is best to do the abdominal component first, as the esophageal outflow obstruction is the source
of most of the symptoms. Performing abdominal myotomy (and
diverticulectomy, if present) may be all that is required.
A new procedure, peroral endoscopic myotomy
(POEM) allows a long myotomy to be performed from the
lumen of the esophagus with an endoscope. This procedure
is attractive for, at a minimum, those with type 3 achalasia
(vigorous achalasia), where it is necessary to divide esophagogastric circular muscle on both sides of the diaphragm to
the extent that might not be possible with laparoscopy or
thoracoscopy alone. The POEM procedure is started by opening the esophageal mucosa several centimeters above the
spastic segment with a needle–knife electrosurgery device
passed through an endoscope. A long submucosal plane is
developed with the endoscope, down to and below the LES.
The circular muscle of the LES and the esophagus is divided
with endoscopic electrosurgery all the way back until normal
(nonspastic) esophagus is reached. The submucosal entry site
in the esophagus is then closed with endoscopic clips. While
the results of POEM are still accumulating, the procedure is
attractive because it is extremely minimally invasive and can
be done on an outpatient basis.
Epiphrenic diverticula cannot be treated with POEM and
are most frequently addressed with laparoscopic access, in
combination with a laparoscopic division of the LES (Heller
myotomy) (Fig. 25-62). If the diverticulum can be completely
mobilized through the hiatus, it may be safely excised from
below. The neck of the diverticulum is transected with a GIA
stapler after passage of a 48F dilator. Not infrequently, the
diverticulum is sufficiently large that access to the neck of
the diverticulum across the hiatus is quite difficult. Additionally, the inflammatory reaction to the diverticulum may
further make the transhiatal dissection difficult. Under these
circumstances, it is safer to perform the diverticulectomy
through a right thoracoscopic approach either at the time of
the initial procedure or at a later date, depending upon the
frailty of the patient. Following diverticulectomy, it is critical
that the esophageal staple line be treated with a great deal of
care. Closure of the muscle over the staple line is preferable.
Additionally, the patient is kept NPO or on clear liquids for
5 to 7 days, and a contrast study is obtained before advancing
to a full liquid or “mushy food” diet. Solid foods are withheld
for 2 weeks to decrease the likelihood of staple line leak. Buttressing or sealing the staple line with fibrin glue is also an
attractive option.
1063
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Figure 25-62. A. Epiphrenic diverticula are situated above the lower esophageal sphincter on right side of esophagus. B. Stapler amputates
neck of diverticulum. C. Muscle reapproximated over staple line, and Heller myotomy is performed.
Myotomy of the Lower Esophageal Sphincter
(Heller Myotomy)
Second only to reflux disease, achalasia is the most common
functional disorder of the esophagus to require surgical
intervention. The goal of treatment is to relieve the functional
outflow obstruction secondary to the loss of relaxation and
compliance of the LES. This requires disrupting the LES
muscle. When performed adequately (i.e., reducing sphincter
pressure to <10 mmHg), and done early in the course of disease,
LES myotomy results in symptomatic improvement with the
occasional return of esophageal peristalsis. Reduction in LES
resistance can be accomplished intraluminally by hydrostatic
balloon dilation, which ruptures the sphincter muscle, by
botulinum toxin injection, or by a surgical myotomy that cuts
the sphincter. The difference between these three methods
appears to be the greater likelihood of reducing sphincter
pressure to <10 mmHg by surgical myotomy compared with
hydrostatic balloon dilation. However, patients whose sphincter
pressure has been reduced by hydrostatic balloon dilation to
<10 mmHg have an outcome similar to those after surgical
myotomy (Fig. 25-63). Botulinum toxin injection may achieve
similar results, but it has a longer duration of action that may
be measured in weeks or months, rather than years. Botulinum
toxin injection may best be used as a diagnostic tool, when it is
not clear whether a hypertensive LES is the primary cause of
dysphagia. Responsiveness to botulinum toxin injection may
predict a good response to Heller myotomy.
The therapeutic decisions regarding the treatment of
patients with achalasia center on four issues. The first issue is
the question of whether newly diagnosed patients should be
treated with pneumatic dilation or a surgical myotomy. Longterm follow-up studies have shown that pneumatic dilation
1064
1
% in remission
0.8
0.6
LES < 10 mmHg
0.53
LES > 10 mmHg
0.23
0.4
0.2
PART II
0
0
12
24
26
48
Months
60
72
84
96
SPECIFIC CONSIDERATIONS
Figure 25-63. Prevalence of clinical remission in 122 patients
stratified according to postdilatation lower esophageal sphincter
(LES) pressures greater than or <10 mmHg. (Reproduced with permission from Ponce J, Garrigues V, Pertejo V, et al: Individual prediction of response to pneumatic dilation in patients with achalasia,
Dig Dis Sci. 1996 Nov;41(11):2135-2141.)
achieves adequate relief of dysphagia and pharyngeal regurgitation in 50% to 60% of patients (Fig. 25-64). Close follow-up
is required, and if dilation fails, myotomy is indicated. For those
patients who have a dilated and tortuous esophagus or an associated hiatal hernia, balloon dilation is dangerous and surgery is
the better option. The outcome of the one controlled randomized study (38 patients) comparing the two modes of therapy
suggests that surgical myotomy as a primary treatment gives
better long-term results. Several randomized trials comparing
laparoscopic cardiomyotomy with balloon dilation or botulinum toxin injection have favored the surgical approach as well.
100
90
80
70
60
% 50
40
30
20
10
0
Myotomy n = 81
Myotomy n = 65
Myotomy + antireflux n = 22
Pneumatic dilatation n = 122
Pneumatic dilatation n = 54
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
Years
Figure 25-64. Summary of long-term studies reporting the
proportion of patients with complete relief or minimal dysphagia
(Stage 0–1) stratified according to type of treatment. (Data
from: Ellis FH, Jr. Oesophagomyotomy for achalasia: a 22-year
experience. Br J Surg. 1993;80:882; Goulbourne IA, Walbaum PR.
Long-term results of Heller’s operation for achalasia. J Royal Coll
Surg. 1985;30:101; Malthaner RA, Todd TR, Miller L, et al. Longterm results in surgically managed esophageal achalasia. Ann
Thorac Surg. 1994;58:1343; Ponce J, Garrigues V, Pertejo V, et al.
Individual prediction of response to pneumatic dilation in patients
with achalasia. Dig Dis Sci. 1996;41:2135; Eckardt V, Aignherr
C, Bernhard G. Predictors of outcome in patients with achalasia
treated by pneumatic dilation. Gastroenterology. 1992;103:1732.)
Although it has been reported that a myotomy after previous
balloon dilation is more difficult, this has not been the experience of these authors unless the cardia has been ruptured in a
sawtooth manner. In this situation, operative intervention, either
immediately or after healing has occurred, can be difficult. Similarly, myotomy after botulinum toxin injection has reported to
be more difficult, but this is largely a function of the submucosal
inflammatory response, which may be a bit unpredictable, and is
most intense in the first 6 to 12 weeks after injection. It is important to wait at least 3 months after botulinum toxin injection to
perform cardiomyotomy to minimize the risk of encountering
dense inflammation.
The second issue is the question of whether a surgical
myotomy should be performed through the abdomen or the
chest. Myotomy of the LES can be accomplished via either an
abdominal or thoracic approach. In the absence of a previous
upper abdominal surgery, most surgeons prefer the abdominal
approach to LES myotomy as laparoscopy results in less pain
and a shorter length of stay than thoracoscopy. In addition, it is
a bit easier to ensure a long gastric myotomy when the approach
is transabdominal.
The third issue—and one that has been long debated—is
the question of whether an antireflux procedure should be added
to a surgical myotomy. Excellent results have been reported following meticulously performed myotomy without an antireflux
component. Retrospective studies, with long-term follow-up of
large cohorts of patients undergoing Heller myotomy demonstrated that, after 10 years, more than 50% of patients had reflux
symptoms without a fundoplication. In a recent randomized clinical trial, 7% of patients undergoing Dor fundoplication following LES myotomy had abnormal 24-hour pH probes, and 42%
of patients with a myotomy only had abnormal reflux profiles.
If an antireflux procedure is used as an adjunct to esophageal
myotomy, a complete 360° fundoplication should be avoided.
Rather, a 270° Belsey fundoplication, a Toupet posterior 180°
fundoplication, or a Dor anterior 180° fundoplication should be
used to avoid the long-term esophageal dysfunction secondary
to the outflow obstruction afforded by the fundoplication itself.
The fourth issue centers on whether or not a cure of this
disease is achievable. Long-term follow-up studies after surgical
myotomy have shown that late deterioration in results occurs
after this procedure, regardless of whether an antireflux procedure is done, and also after balloon dilation, even when the
sphincter pressure is reduced to below 10 mmHg. It may be that,
even though a myotomy or balloon rupture of the LES muscle
reduces the outflow obstruction at the cardia, the underlying
motor disorder in the body of the esophagus persists and deteriorates further with the passage of time, leading to increased
impairment of esophageal emptying. The earlier an effective
reduction in outflow resistance can be accomplished, the better
the outcome will be, and the more likely some esophageal body
function can be restored.
In performing a surgical myotomy of the LES, there are
four important principles: (a) complete division of all circular
and collar-sling muscle fibers, (b) adequate distal myotomy to
reduce outflow resistance, (c) “undermining” of the muscularis
to allow wide separation of the esophageal muscle, and (d) prevention of postoperative reflux. In the past, the drawback of a
surgical myotomy was the need for an open procedure, which
often deterred patients from choosing the best treatment option
for achalasia. With the advent of minimally invasive surgical techniques two decades ago, laparoscopic cardiomyotomy
(Heller myotomy) has become the treatment of choice for most
patients with achalasia.
Open Esophageal Myotomy
Laparoscopic Cardiomyotomy
More commonly known as a laparoscopic Heller myotomy,
after Ernst Heller, a German surgeon who described a “double myotomy” in 1913, the laparoscopic approach is similar to
the Nissen fundoplication in terms of the trocar placement and
exposure and dissection of the esophageal hiatus (Fig. 25-65).
The procedure begins by division of the short gastric vessels
Per Oral Endoscopic Myotomy (POEM)
The POEM procedure was developed in Japan. It is the ultimate
minimally invasive myotomy as it requires no incisions through
the skin. With the POEM procedure, a very effective myotomy
is performed entirely from the lumen of the esophagus. The
POEM procedure is started by opening the esophageal mucosa
10 cm above the lower esophageal sphincter with a needle–knife
electrosurgery device passed through an endoscope. A long
submucosal plane is developed with the endoscope, down to
and below the LES. The circular muscle of the LES, above and
below the gastroesophageal junction, is divided with endoscopic
electrosurgery. The submucosal entry site in the esophagus is
then closed with endoscopic clips. While the results of POEM
are still accumulating, the procedure is attractive because it is
extremely minimally invasive, and can be done on an outpatient
basis. The major downside of POEM is that an effective antireflux valve cannot be created, exposing the patient to a 40% to
50% risk of GERD post procedure.
Outcome Assessment of the Therapy
for Achalasia
Critical analysis of the results of therapy for motor disorders of the esophagus requires objective measurement. The
use of symptoms alone as an endpoint to evaluate therapy for
achalasia may be misleading. The propensity for patients to
unconsciously modify their diet to avoid difficulty swallowing
is underestimated, making an assessment of results based on
symptoms unreliable. Insufficient reduction in outflow resistance may allow progressive esophageal dilation to develop
slowly, giving the impression of improvement because the
volume of food able to be ingested with comfort increases.
A variety of objective measurements may be used to assess
success, including LES pressure, esophageal baseline pressure,
and scintigraphic assessment of esophageal emptying time.
Esophageal baseline pressure is usually negative compared to
gastric pressure. Given that the goal of therapy is to eliminate
the outflow resistance of a nonrelaxing sphincter, measurements of improvements in esophageal baseline pressure and
scintigraphic transit time may be better indicators of success,
but these are rarely reported.
1065
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Open techniques of distal esophageal myotomy are rarely used
outside reoperations. In fact, primary procedures can almost
always be successfully completed via laparoscopy. A modified
Heller myotomy can be performed through a left thoracotomy
incision in the sixth intercostal space along the upper border
of the seventh rib. The esophagus and a tongue of gastric fundus are exposed as described for a long myotomy. A myotomy
through all muscle layers is performed, extending distally over
the stomach to 1 to 2 cm below the junction, and proximally
on the esophagus for 4 to 5 cm. The cardia is reconstructed
by suturing the tongue of gastric fundus to the margins of the
myotomy to prevent rehealing of the myotomy site and to provide reflux protection in the area of the divided sphincter. If an
extensive dissection of the cardia has been done, a more formal Belsey repair is performed. The tongue of gastric fundus is
allowed to retract into the abdomen. Traditionally, nasogastric
drainage is maintained for 6 days to prevent distention of the
stomach during healing. An oral diet is resumed on the seventh
day, after a barium swallow study shows unobstructed passage
of the bolus into the stomach without extravasation.
In a randomized, long-term follow-up by Csendes and
colleagues of 81 patients treated for achalasia, either by forceful
dilation or by surgical myotomy, myotomy was associated with
a significant increase in the diameter at the GEJ and a decrease
in the diameter at the middle third of the esophagus on follow-up
radiographic studies. There was a greater reduction in sphincter
pressure and improvement in the amplitude of esophageal
contractions after myotomy. After dilation, 13% of patients
regained some peristalsis, compared with 28% after surgery.
These findings were shown to persist over a 5-year follow-up
period, at which time 95% of those treated with surgical myotomy
were doing well. Of those who were treated with dilation, only
54% were doing well, while 16% required redilation, and 22%
eventually required surgical myotomy to obtain relief.
If simultaneous esophageal contractions are associated
with the sphincter abnormality, the so-called vigorous achalasia, then the myotomy should extend over the distance of the
abnormal motility as mapped by the preoperative motility study.
Failure to do this will result in continuing dysphagia and a dissatisfied patient. The best objective evaluation of improvement
in the patient following either balloon dilation or myotomy
is a scintigraphic measurement of esophageal emptying time.
A good therapeutic response improves esophageal emptying
toward normal. However, some degree of dysphagia may persist despite improved esophageal emptying, due to disturbances
in esophageal body function. When an antireflux procedure is
added to the myotomy, it should be a partial fundoplication. A
360° fundoplication is associated with progressive retention of
swallowed food, regurgitation, and aspiration to a degree that
exceeds the patient’s preoperative symptoms.
in preparation for fundoplication. Exposure of the GEJ via
removal of the gastroesophageal fat pad follows. The anterior
vagus nerve is swept right laterally along with the fat pad. Once
completed, the GEJ and distal 4 to 5 cm of esophagus should be
bared of any overlying tissue, and generally follows dissection
of the GEJ. A distal esophageal myotomy is performed. It is
generally easiest to begin the myotomy 1 to 2 cm above the GEJ,
in an area above that of previous botulinum toxin injections or
balloon dilation. Either scissors or a hook-type electrocautery
can be used to initiate the incision in the longitudinal and circular muscle. Distally, the myotomy is carried across the GEJ and
onto the proximal stomach for approximately 2 to 3 cm. After
completion, the muscle edges are separated bluntly from the
esophageal mucosa for approximately 50% of the esophageal
circumference. An antireflux procedure follows completion of
the myotomy. Either an anterior hemifundoplication augmenting the angle of His (Dor) or posterior partial fundoplication
(Toupet) can be performed. The Dor type fundoplication is
slightly easier to perform, and it does not require disruption of
the normal posterior gastroesophageal attachments (a theoretical
advantage in preventing postoperative reflux).
1066
PART II
SPECIFIC CONSIDERATIONS
Eckardt and associates investigated whether the outcome
of pneumatic dilation in patients with achalasia could be predicted on the basis of objective measurements. Postdilation
LES pressure was the most valuable measurement for predicting long-term clinical response. A postdilatation sphincter pressure <10 mmHg predicted a good response. Approximately 50%
of the patients studied had postdilatation sphincter pressures
between 10 and 20 mmHg, with a 2-year remission rate of 71%.
More important, 16 of 46 patients were left with a postdilatation
sphincter pressure of >20 mmHg and had an unacceptable outcome. Overall, only 30% of patients dilated remained in symptomatic remission at 5 years.
Bonavina and colleagues reported good to excellent results
with transabdominal myotomy and Dor fundoplication in 94%
of patients after a mean follow-up of 5.4 years. No operative
mortality occurred in either of these series, attesting to the
safety of the procedure. Malthaner and Pearson reported the
long-term clinical results in 35 patients with achalasia, having
a minimum follow-up of 10 years (Table 25-10). Twenty-two
of these patients underwent primary esophageal myotomy and
Belsey hemifundoplication at the Toronto General Hospital.
Excellent to good results were noted in 95% of patients at
1 year, declining to 68%, 69%, and 67% at 10, 15, and 20 years,
respectively. Two patients underwent early reoperation for an
incomplete myotomy, and three underwent an esophagectomy
for progressive disease. They concluded that there was a
deterioration of the initially good results after surgical myotomy
and hiatal repair for achalasia, which is due to late complications
of gastroesophageal reflux.
Ellis reported his lifetime experience with transthoracic
short esophageal myotomy without an antireflux procedure. One
hundred seventy-nine patients were analyzed at a mean followup of 9 years, ranging from 6 months to 20 years. Overall, 89%
of patients were improved at the 9-year mark. He also observed
that the level of improvement deteriorated with time, with excellent results (patients continuing to be symptom free) decreasing
from 54% at 10 years to 32% at 20 years. He concluded that a
short transthoracic myotomy without an antireflux procedure
provides excellent long-term relief of dysphagia, and, contrary
to Malthaner and Pearson’s experience, does not result in complications of gastroesophageal reflux. Both studies document
nearly identical results 10 to 15 years following the procedure,
and both report deterioration over time, probably due to progression of the underlying disease. The addition of an antireflux
procedure if the operation is performed transthoracically has no
significant effect on the outcome.
Figure 25-65. A. Longitudinal muscle is divided. B. Mechanical disruption of lower esophageal sphincter muscle fibers. C. Myotomy must
be carried across gastroesophageal junction. D. Gastric extension should equal 2 to 3 cm. E. Anterior (Dor) fundoplication is sutured to the
diaphragmatic arch. F. Posterior (Toupet) fundoplication is sutured to cut edges of myotomy. EG jct = esophagogastric junction.
1067
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Figure 25-65. (Continued )
Table 25-10
Reasons for failure of esophageal myotomy
AUTHOR, PROCEDURE (N)
REASON
ELLIS, MYOTOMY
ONLY (N = 81)
GOULBOURNE, MYOTOMY
ONLY (N = 65)
MALTHANER, MYOTOMY +
ANTIREFLUX (N = 22)
Reflux
4%
5%
18%
Inadequate myotomy
2%
—
9%
Megaesophagus
2%
—
—
Poor emptying
4%
3%
—
Persistent chest pain
1%
—
—
Data from Malthaner RA, et al. Long-term results in surgically managed esophageal achalasia. Ann Thorac Surg. 1994;58:1343; Ellis FH, Jr.
Oesophagomyotomy for achalasia: a 22-year experience. Br J Surg. 1993;80:882; and Goulbourne IA, et al. Long-term results of Heller’s operation for
achalasia. J R Coll Surg Edinb. 1985;30:101.
1068
PART II
SPECIFIC CONSIDERATIONS
The outcome of laparoscopic myotomy and hemifundoplication has been well documented. Two reports of over
100 patients have documented relief of dysphagia in 93% of
patients. Richter and coworkers reviewed published reports to
date, including 254 patients with an average success rate of 93%
at 2.5 years. Conversion to an open procedure occurs in 0%
to 5% of patients. Complications are uncommon, occurring in
<5% of patients. Intraoperative complications consist largely of
mucosal perforation, and have been more likely to occur after
botulinum toxin injection. The incidence of objective reflux disease as evidenced by abnormal acid exposure is <10%.
A number of randomized clinical trials in the past decade
have compared the outcomes of laparoscopic Heller myotomy
to pneumatic dilation and to botulinum toxin injection. In each
of these trials, laparoscopic Heller myotomy and partial fundoplication was superior to the alternative treatment. Lastly, a
randomized clinical trial examining the need for fundoplication following Heller myotomy demonstrated a great deal more
reflux in patients without fundoplication, and no better swallowing in the Heller-only group. The best treatment for achalasia
is a laparoscopic Heller myotomy and partial fundoplication.
The role of POEM in the management of classic (nonspastic)
achalasia is yet to be established.
Esophageal Resection for End-Stage Motor
Disorders of the Esophagus
Patients with dysphagia and long-standing benign disease,
whose esophageal function has been destroyed by the disease
process or multiple previous surgical procedures, are best managed by esophagectomy. Fibrosis of the esophagus and cardia
can result in weak contractions and failure of the distal esophageal sphincter to relax. The loss of esophageal contractions can
result in the stasis of food, esophageal dilatation, regurgitation,
and aspiration. The presence of these abnormalities signals endstage motor disease. In these situations, esophageal replacement is usually required to establish normal alimentation.
Before proceeding with esophageal resection for patients with
end-stage benign disease, the choice of the organ to substitute
for the esophagus (i.e., stomach, jejunum, or colon) should be
considered. The choice of replacement is affected by a number of factors, as described later in “Techniques of Esophageal
Reconstruction.” If minimally invasive esophagectomy is to be
performed, thoracoscopic dissection should be combined with
abdominal dissection. Attempts at MIS transhiatal esophagectomy for the massively dilated esophagus may result in large
volume bleeding from mediastinal vessels that become enlarged
with esophageal dilation, and such bleeding must be directly
controlled for hemostasis to be adequate and the operation to
be safe.
CARCINOMA OF THE ESOPHAGUS
Squamous carcinoma accounts for the majority of esophageal
carcinomas worldwide. Its incidence is highly variable, ranging
from approximately 20 per 100,000 in the United States and
Britain, to 160 per 100,000 in certain parts of South Africa
and the Henan Province of China, and even 540 per 100,000
in the Guriev district of Kazakhstan. The environmental
factors responsible for these localized high-incidence areas
have not been conclusively identified, though additives to
local foodstuffs (nitroso compounds in pickled vegetables and
smoked meats) and mineral deficiencies (zinc and molybdenum)
have been suggested. In Western societies, smoking and alcohol
consumption are strongly linked with squamous carcinoma.
Other definite associations link squamous carcinoma with
long-standing achalasia, lye strictures, tylosis (an autosomal
dominant disorder characterized by hyperkeratosis of the palms
and soles), and human papillomavirus.
Adenocarcinoma of the esophagus, once an unusual malignancy, is diagnosed with increasing frequency (Fig. 25-66) and
now accounts for more than 50% of esophageal cancer in most
Western countries. The shift in the epidemiology of esophageal
cancer from predominantly squamous carcinoma seen in association with smoking and alcohol to adenocarcinoma in the setting
of BE is one of the most dramatic changes that has occurred in
the history of human neoplasia. Although esophageal carcinoma
is a relatively uncommon malignancy, its prevalence is exploding, largely secondary to the well-established association among
gastroesophageal reflux, BE, and esophageal adenocarcinoma.
Although BE was once a nearly uniformly lethal disease, survival has improved slightly because of advances in the understanding of its molecular biology, screening and surveillance
practices, improved staging, minimally invasive surgical techniques, and neoadjuvant therapy.
Furthermore, the clinical picture of esophageal adenocarcinoma is changing. It now occurs not only considerably more
frequently but also in younger patients, and it is often detected
at an earlier stage. These facts support rethinking the traditional
approach of assuming palliation is appropriate in all patients.
The historical focus on palliation of dysphagia in an elderly
patient with comorbidities should change when dealing with
a young patient with dependent children and a productive life
ahead. The potential for cure becomes of paramount importance.
The gross appearance resembles that of squamous cell carcinoma. Microscopically, adenocarcinoma almost always originates in Barrett’s mucosa and resembles gastric cancer. Rarely,
it arises in the submucosal glands and forms intramural growths
that resemble the mucoepidermal and adenoid cystic carcinomas
of the salivary glands.
The most important etiologic factor in the development
of primary adenocarcinoma of the esophagus is a metaplastic
columnar-lined or Barrett’s esophagus, which occurs in approximately 10% to 15% of patients with GERD. When studied prospectively, the incidence of adenocarcinoma in a patient with
BE is one in 100 to 200 patient-years of follow-up (i.e., for
every 100 patients with BE followed for 1 year, one will develop
adenocarcinoma). Although this risk appears to be small, it is
at least 40 to 60 times that expected for a similar population
without BE. This risk is similar to the risk for developing lung
cancer in a person with a 20-pack-per-year history of smoking.
Endoscopic surveillance for patients with BE is recommended
for two reasons: (a) at present there is no reliable evidence that
medical therapy removes the risk of neoplastic transformation,
and (b) malignancy in BE is curable if detected at an early stage.
Clinical Manifestations
Esophageal cancer generally presents with dysphagia, although
increasing numbers of relatively asymptomatic patients are
now identified on surveillance endoscopy, or present with
nonspecific upper GI symptoms and undergo screening
Extension of the primary tumor into the
6 endoscopy.
tracheobronchial tree can occur primarily with squamous
cell carcinoma and can cause stridor, tracheoesophageal
fistula, and resultant coughing, choking, and aspiration
U.S. esophageal cancer incidence
20
15
10
5
0
1985
1993
1989
1997
2001
2005
U.S. esophageal cancer mortality
25
Mortality per 100,000
20
15
10
General Approach to Esophageal Cancer
5
0
1985
1993
1989
1997
2001
2005
White females
Overall rate
White males
African American males
African American females
NCI esophageal cancer research investment
$4.6B
$21.8M
$4.7B
$21.7M
$4.8B
$22.7M
$4.7B
$21.6M
$4.8B
$22.3M
4
20
Millions of dollars
5
3
15
10
2
5
1
0
2003
2004
2005
2006
2007
Billions of dollars
25
pneumonia. Rarely, severe bleeding from the primary tumor or
from erosion into the aorta or pulmonary vessels occurs. Either
vocal cord may be invaded, causing paralysis, but most
commonly, paralysis is caused by invasion of the left recurrent
laryngeal nerve by the primary tumor or LN metastasis.
Systemic organ metastases are usually manifested by jaundice
or bone pain. The situation is different in high-incidence areas
where screening is practiced. In these communities, the most
prominent early symptom is pain on swallowing rough or dry
food. In patients that present with back pain at the time of
esophageal cancer diagnosis, there is usually distant metastasis
or celiac encasement.
Dysphagia usually presents late in the natural history of
the disease because the lack of a serosal layer on the esophagus allows the smooth muscle to dilate with ease. As a result,
the dysphagia becomes severe enough for the patient to seek
medical advice only when more than 60% of the esophageal
circumference is infiltrated with cancer. Consequently, the disease is usually advanced if symptoms herald its presence. Tracheoesophageal fistula may be present in some patients on their
first visit to the hospital, and more than 40% will have evidence
of distant metastases. With tumors of the cardia, anorexia and
weight loss usually precede the onset of dysphagia. The physical
signs of esophageal tumors are those associated with the presence of distant metastases.
0
Fiscal year
Esophageal cancer funding
Total NCI budget
Figure 25-66. Incidence and mortality rate trends for esophageal
cancer. NCI = National Cancer Institute. (Reproduced with permission from the National Cancer Institute. Last updated September,
2008.)
Therapy of esophageal cancer is dictated by the stage of the cancer at the time of diagnosis. Put simply, one needs to determine
if the disease is confined to the esophagus, (T1–T2, N0), locally
advanced (T1–3, N1), or disseminated (any T, any N, M1). If
cancer is confined to the esophagus, removal of the tumor with
adjacent lymph nodes may be curative. Very early tumors confined to the mucosa (T in situ, T1a, intramucosal cancer) may be
addressed with endoscopic treatment. When the tumor is locally
aggressive, modern therapy dictates a multimodality approach
in a surgically fit patient. Multimodality therapy is either chemotherapy followed by surgery or radiation and chemotherapy
followed by surgery. When given before surgery, these treatments are referred to as neoadjuvant or induction therapy. For
disseminated cancer, treatment is aimed at palliation of symptoms. If the patient has dysphagia, as many do, the most rapid
form of palliation is the endoscopic placement of an expandable
esophageal stent. For palliation of GEJ cancer, radiation may be
the first choice, as stents placed across the GEJ create a great
deal of gastroesophageal reflux.
Staging of Esophageal Cancer
Choosing the best therapy for an individual patient requires
accurate staging. Staging starts with the history and physical.
LN disease remote from the tumor, particularly in the cervical region, may be palpable on neck examination and generally
indicates cancer dissemination. This is often referred to as M1a
disease, indicating that these patients should not be treated with
therapy directed toward locally advanced cancer. Other metastatic LNs are rarely palpable but are equally ominous, especially the umbilical LN in GEJ cancer.
Computed tomographic (CT) scanning of the chest, abdomen, and pelvis provides information on local invasion of the
primary cancer, LN involvement, or disseminated disease.
The most common sites of esophageal cancer metastases are
lung, liver, and peritoneal surfaces, including the omentum
and small bowel mesentery. If masses are identified that are
1069
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Incidence per 100,000
25
1070
PART II
SPECIFIC CONSIDERATIONS
not characteristic for cancer or are in a location that precludes
resection with the cancer specimen, positron emission tomography (PET) scanning may be able to tell whether the masses are
metabolically active (likely to be cancer) or not. A PET active
focus corresponding to a mass on CT scan outside of the field
of esophageal resection should be biopsied before resection is
performed.
The introduction of endoscopic ultrasound (EUS) has
made it possible to identify patients who are potentially curable
before surgical therapy. Using an endoscope, the depth of the
wall penetration by the tumor and the presence of LN metastases can be determined with 80% accuracy. A curative resection
should be encouraged if EUS indicates that the tumor has not
invaded adjacent organs (T4b), and/or fewer than six enlarged
LNs are imaged. Thoracoscopic and laparoscopic staging of
esophageal cancer may add benefit when the nature of enlarged
LNs remote from the cancer cannot be determined or when
advanced imaging systems (PET and high-resolution spiral CT)
are not available.
Occasionally, diagnostic laparoscopy and jejunostomy tube
placement may precede induction chemoradiation in the patient
with severe dysphagia and weight loss from a locally advanced
cancer. In summary, esophageal cancer is diagnosed with
endoscopic biopsy and is staged with CT scanning of the chest
and abdomen, EUS, and PET scan for all patients with CT or
EUS evidence of advanced disease (T2 or greater, N1-2 or NX).
Experience with esophageal resection in patients with early
stage disease has identified characteristics of esophageal cancer
that are associated with improved survival. A number of studies
suggest that only metastasis to LNs and tumor penetration of the
esophageal wall have a significant and independent influence
on prognosis. Factors known to be important in the survival
of patients with advanced disease, such as cell type, degree of
cellular differentiation, or location of tumor in the esophagus,
have no effect on survival of patients who have undergone
resection for early disease. Studies also showed that patients
having five or fewer LN metastases have a better outcome.
Using these data, Skinner developed the wall penetration, LN,
and distant organ metastases system for staging.
The wall penetration, LN, and distant organ metastases
system differed somewhat from the previous efforts to
develop a satisfactory staging criteria for carcinoma of the
esophagus. Most surgeons agreed that the 1983 tumor, nodes,
and metastasis system left much to be desired. In the third
edition of the manual for Staging of Cancer of the American
Joint Committee on Cancer (AJCC) in 1988, an effort was
made to provide a finer discrimination between stages than
had been contained in the previous edition in 1983. In 2016,
further refinements of the staging system of esophageal cancer
were approved by the AJCC, recognizing the difference in
survival afforded by resection of limited LN disease adjacent
to the tumor, compared to multilevel LN disease and positive
LNs remote from the primary. Table 25-11 shows the AJCC
definitions for the primary tumor, lymph nodes, distant
metastasis, and overall staging schema for both squamous cell
carcinoma and adenocarcinoma.
Clinical Approach to Carcinoma of the
Esophagus and Cardia
The selection of a curative vs. a palliative operation for cancer of
the esophagus is based on the location of the tumor, the patient’s
age and health, the extent of the disease, and preoperative staging. Figure 25-67 shows an algorithm of the clinical decisions
important in the selection of curative or palliative therapy.
Tumor Location. The selection of surgical therapy for patients
with carcinoma of the esophagus depends not only on the anatomic stage of the disease and an assessment of the swallowing
capacity of the patient but also on the location of the primary
tumor.
It is estimated that 8% of the primary malignant tumors of
the esophagus occur in the cervical portion (Fig. 25-68). They
are almost always squamous cell cancer, with a rare adenocarcinoma arising from a congenital inlet patch of columnar lining.
These tumors, particularly those in the postcricoid area, represent a separate pathologic entity for two reasons: (a) they are
more common in females and appear to be a unique entity in
this regard; and (b) the efferent lymphatics from the cervical
esophagus drain completely differently from those of the thoracic esophagus. The latter drain directly into the paratracheal
and deep cervical or internal jugular LNs with minimal flow in a
longitudinal direction. Except in advanced disease, it is unusual
for intrathoracic LNs to be involved.
Cervical esophageal cancer is frequently unresectable
because of early invasion of the larynx, great vessels, or trachea.
Radical surgery, including esophagolaryngectomy may occasionally be performed for these lesions, but the ensuing morbidity makes this a less than desirable approach in the face of
uncertain cure. Thus, for most patients with cervical esophageal
cancer, stereotactic radiation with concomitant chemotherapy is
the most desirable treatment.
Tumors that arise within the middle third of the esophagus are squamous carcinomas most commonly and are frequently associated with LN metastasis, which are usually in
the thorax but may be in the neck or abdomen, and may skip
areas in between. Although it is generally felt that individuals with midthoracic cancer and abdominal LN metastases
are incurable with surgery, there are some emerging data
that suggest that cervical LN metastases, if isolated, can be
resected with benefit. Generally, T1 and T2 cancers without LN metastases are treated with resection only, but there
is more and more data to suggest that LN involvement or
transmural cancer (T3) warrants treatment with neoadjuvant
chemoradiation therapy followed by resection. Although
some surgeons prefer a transhiatal esophagectomy for all
tumor locations, most surgeons believe that resection of midesophageal cancer should be performed under direct vision
with either thoracoscopy (video-assisted thoracic surgery
[VATS]) or with thoracotomy.
Tumors of the lower esophagus and cardia are usually
adenocarcinomas. Unless preoperative and intraoperative staging clearly demonstrate an incurable lesion, resection in continuity with a LN dissection should be performed. Because of
the propensity of GI tumors to spread for long distances submucosally, long lengths of grossly normal GI tract should be
resected. The longitudinal lymph flow in the esophagus can
result in skip areas, with small foci of tumor above the primary
lesion, which underscores the importance of a wide resection of
esophageal tumors. Wong has shown that local recurrence at the
anastomosis can be prevented by obtaining a 10-cm margin of
normal esophagus above the tumor. Anatomic studies have also
shown that there is no submucosal lymphatic barrier between
the esophagus and the stomach at the cardia, and Wong has
1071
Table 25-11
American Joint Committee on Cancer (AJCC) Staging Schema for Esophageal Cancer
Primary tumor cannot be assessed.
No evidence of primary tumor.
High-grade dysplasia.
Tumor invades lamina propria, muscularis mucosae, or submucosa.
Tumor invades lamina propria or muscularis mucosae.
Tumor invades submucosa.
Tumor invades muscularis propria.
Tumor invades adventitia.
Tumor invades adjacent structures.
Resectable tumor invading pleura, pericardium, or diaphragm.
Unresectable tumor invading other adjacent structures, such as aorta, vertebral body, trachea, etc.
Regional lymph nodes cannot be assessed.
No regional lymph node metastasis.
Metastases in 1–2 regional lymph nodes.
Metastases in 3–6 regional lymph nodes.
Metastases in ≥7 regional lymph nodes.
No distant metastasis.
Distant metastasis.
SQUAMOUS CELL CARCINOMA
Clinical (cTNM)
When
cT is...
Tis
T1
T2
T3
T3
T1–3
T4
Any T
Any T
And
cN is...
N0
N0–1
N0–1
N0
N1
N2
N0–2
N3
Any N
Pathological (pTNM)
When And
And And
pT is... pN is... M is... G is...
Tis
N0
M0
N/A
T1a
N0
M0
G1
T1a
N0
M0
G2–3
T1a
N0
M0
GX
T1b
N0
M0
G1–3
T1b
N0
M0
GX
T2
N0
M0
G1
T2
N0
M0
G2–3
T2
N0
M0
GX
T3
N0
M0
G1–3
T3
N0
M0
G1
T3
N0
M0
G2–3
And
M is...
M0
M0
M0
M0
M0
M0
M0
M0
M1
Then the
stage group is...
0
I
II
II
III
III
IVA
IVA
IVB
T3
T3
T1
T1
T2
T2
T3
T4a
T4a
T4b
Any T
Any T
N0
M0
N0
M0
N1
M0
N2
M0
N1
M0
N2
M0
N1–2 M0
N0–1 M0
N2
M0
N0–2 M0
N3
M0
Any N M1
GX
Any
Any
Any
Any
Any
Any
Any
Any
Any
Any
Any
Lower/upper/middle IIB
Location X
IIB
Any
IIB
Any
IIIA
Any
IIIA
Any
IIIB
Any
IIIB
Any
IIIB
Any
IVA
Any
IVA
Any
IVA
Any
IVB
Postneoadjuvant Therapy (ypTNM)
And
location is...
Any
Any
Any
Any
Any
Any
Any
Any
Any
Lower
Upper/middle
Upper/middle
Then the stage
group is...
0
IA
IB
IA
IB
IB
IB
IIA
IIA
IIA
IIA
IIB
When yp
T is...
T0–2
T3
T0–2
T3
T0–3
T4a
T4a
T4a
T4b
Any T
Any T
And yp
N is...
N0
N0
N1
N1
N2
N0
N1–2
NX
N0–2
N3
Any N
And
M is...
M0
M0
M0
M0
M0
M0
M0
M0
M0
M0
M1
Then the stage
group is...
I
II
IIIA
IIIB
IIIB
IIIB
IVA
IVA
IVA
IVA
IVB
ADENOCARCINOMA
Clinical (cTNM)
When
And
cT is...
cN is...
Tis
N0
T1
N0
T1
N1
T2
N0
And
M is...
M0
M0
M0
M0
Then the
stage group is...
0
I
IIA
IIB
T2
T3
T4a
T1–4a
T4b
Any T
Any T
N1
N0–1
N0–1
N2
N0–2
N3
Any N
M0
M0
M0
M0
M0
M0
M1
III
III
III
IVA
IVA
IVA
IVB
(Continued)
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
TX
T0
Tis
T1
T1a
T1b
T2
T3
T4
T4a
T4b
NX
N0
N1
N2
N3
M0
M1
1072
Table 25-11
American Joint Committee on Cancer (AJCC) Staging Schema for Esophageal Cancer (Continued)
Pathological (pTNM)
PART II
SPECIFIC CONSIDERATIONS
When
pT is...
Tis
T1a
T1a
T1a
T1b
T1b
T1
T2
T2
T2
T1
T3
T1
T2
T2
T3
And
pN is...
N0
N0
N0
N0
N0
N0
N0
N0
N0
N0
N1
N0
N2
N1
N2
N1–2
And
M is...
M0
M0
M0
M0
M0
M0
M0
M0
M0
M0
M0
M0
M0
M0
M0
M0
And
G is...
N/A
G1
GX
G2
G1–2
GX
G3
G1–2
G3
GX
Any
Any
Any
Any
Any
Any
Then the stage
group is...
0
IA
IA
IB
IB
IB
IC
IC
IIA
IIA
IIB
IIB
IIIA
IIIA
IIIB
IIIB
T4a
T4a
T4b
Any T
Any T
N0–1
N2
N0–2
N3
Any N
M0
M0
M0
M0
M1
Postneoadjuvant Therapy (ypTNM)
When yp
And yp
And
T is...
N is...
M is...
T0–2
N0
M0
T3
N0
M0
T0–2
N1
M0
T3
N1
M0
T0–3
N2
M0
T4a
N0
M0
T4a
N1–2
M0
T4a
NX
M0
T4b
N0–2
M0
Any T
N3
M0
Any T
Any N
M1
Any
Any
Any
Any
Any
IIIB
IVA
IVA
IVA
IVB
Then the stage
group is...
I
II
IIIA
IIIB
IIIB
IIIB
IVA
IVA
IVA
IVA
IVB
Used with the permission of the American College of Surgeons. Amin MB, Edge SB, Greene FL, et al. (Eds.) AJCC Cancer Staging Manual, 8th Ed.
Springer New York, 2017.
shown that 50% of the local recurrences in patients with esophageal cancer who are resected for cure occur in the intrathoracic
stomach along the line of the gastric resection. Considering that
the length of the esophagus ranges from 17 to 25 cm, and the
length of the lesser curvature of the stomach is approximately
12 cm, a curative resection requires a cervical division of the
esophagus and a >50% proximal gastrectomy in most patients
with carcinoma of the distal esophagus or cardia.
Age
Physiologic
fitness
Clinical staging
patient older than 80 years is rarely indicated because of the
additional operative risk and the shorter life expectancy. Despite
this general guideline, octogenarians with a high-performance
status and excellent cardiopulmonary reserve may be considered candidates for esophagectomy, and recent case series have
established its success in highly selected patients. It is in this
group of patients that the lesser physiologic impact of minimally
Palliation
75 years
Palliation
FEV1 1.25
Ejection fraction
40%
Palliation
Recurrent nerve paralysis
Horner's syndrome
Persistent spinal pain
Paralysis of diaphragm
Fistula formation
Malignant pleural effusion
Endoscopic tumor length 9 cm
Abnormal esophageal axis
Multiple enlarged nodes or distant
organ metastasis on CT
More than 20% weight loss
Loss of appetite (relative)
Endoscopic
ultrasound
Palliation
Transmural tumors
with 4 enlarged nodes
Intraoperative
staging
Palliation
Unresectable primary
Cavitary spread
Distant metastasis
Extension through mediastinal wall
Multiple gross lymph node metastases
Microscopic nodal metastasis at margins of
the en bloc dissection
Curative en
bloc resection
Age. Resection for cure of carcinoma of the esophagus in a
Palliative symptoms
Dysphagia
Obstruction
Pain of ulceration
Bleeding
Infection
Anxiety
Requirements for
palliative transhiatal
resection*
- Free of distant organ
metastases
- Complete excision of
primary tumor possible
Nonsurgical
palliation
*Could include combined Rx and chemo neoadjuvant therapy
prior to resection to increase resectability and potential
survival in patients 75 or under.
Figure 25-67. Algorithm for the evaluation of esophageal cancer patients to select the proper therapy: curative en bloc resection, palliative
transhiatal resection, or nonsurgical palliation. CT = computed tomography; FEV1 = forced expiratory volume in 1 second. (Reproduced with
permission from DeMeester TR: Esophageal carcinoma: current controversies, Semin Surg Oncol. 1997 Jul-Aug;13(4):217-233.)
Location
Incidence
Cervical
8%
Upper
thoracic
3%
32%
Lower
thoracic
25%
Cardia
32%
Figure 25-68. Incidence of carcinoma of the esophagus and cardia
based on tumor location.
invasive surgery may reduce the morbidity and mortality associated with open two- or three-field esophagectomy.
Cardiopulmonary Reserve. Patients undergoing esophageal
resection should have sufficient cardiopulmonary reserve to tolerate the proposed procedure. The respiratory function is best
assessed with the forced expiratory volume in 1 second, which
ideally should be 2 L or more. Any patient with a forced expiratory volume in 1 second of <1.25 L is a poor candidate for
thoracotomy because he or she has a 40% risk of dying from
respiratory insufficiency within 4 years. In patients with poor
pulmonary reserve, the transhiatal esophagectomy should be
considered, as the pulmonary morbidity of this operation is
less than is seen following thoracotomy. Clinical evaluation
and electrocardiogram are not sufficient indicators of cardiac
reserve. Echocardiography and dipyridamole thallium imaging
provide accurate information on wall motion, ejection fraction,
and myocardial blood flow. A defect on thallium imaging may
require further evaluation with preoperative coronary angiography. A resting ejection fraction of <40%, particularly if there is
no increase with exercise, is an ominous sign. In the absence of
invasive testing, observed stair-climbing is an economical (albeit
not quantitative) method of assessing cardiopulmonary reserve.
Most individuals who can climb three flights of stairs without
stopping will do well with two-field open esophagectomy, especially if an epidural catheter is used for postoperative pain relief.
Nutritional Status. The factor most predictive of postoperative
complication is the nutritional status of the patient. Profound
weight loss, more than 20 lb, associated with hypoalbuminemia
(albumin <3.5 g/dL) is associated with a much higher rate of
complications and mortality than patients who enter curative
surgery in better nutritional condition. Because malnourished
patients generally have locally advanced esophageal cancer, if
not metastatic disease, one should consider the placement of a
feeding tube before the beginning of induction chemoradiation
therapy. Although mild amounts of dysphagia are improved by
1073
Clinical Staging. Clinical factors that indicate an advanced
stage of carcinoma and exclude surgery with curative intent are
recurrent nerve paralysis, Horner’s syndrome, persistent spinal
pain, paralysis of the diaphragm, fistula formation, and malignant pleural effusion. Factors that make surgical cure unlikely
include a tumor >8 cm in length, abnormal axis of the esophagus on a barium radiogram, more than four enlarged LNs on
CT, a weight loss more than 20%, and loss of appetite. Studies indicate that there are several favorable parameters associated with tumors <4 cm in length, there are fewer with tumors
between 4 and 8 cm, and there are no favorable criteria for
tumors >8 cm in length. Consequently, the finding of a tumor
>8 cm in length should exclude curative resection; the finding
of a smaller tumor should encourage an aggressive approach.
Preoperative Staging With Advanced Imaging. For years,
clinical staging, contrast radiography, endoscopy, and CT scanning formed the backbone of esophageal cancer staging. More
recently, preoperative decision making is guided by endoscopic
ultrasonography and PET scanning.
EUS provides the most reliable method of determining
depth of cancer invasion. In the absence of enlarged LNs,
the degree of wall invasion dictates surgical therapy. If a
small focus of esophageal cancer is confined to the mucosa,
endoscopic mucosal resection (EMR) is a preferable option.
If the tumor invades into the submucosa, without visible
lymph node involvement, most individuals would suggest
esophagectomy with LN dissection, as positive nodes can
be found in 20% to 25% of those with cancer limited to the
mucosa and submucosa. If EUS demonstrates spread through
the wall of the esophagus, especially if LNs are enlarged, then
induction chemoradiation therapy (neoadjuvant therapy) should
be strongly considered. Lastly, when the EUS demonstrates
invasion of the trachea, bronchus, aorta, or spine, then surgical
resection is rarely indicated. If there is invasion into the pleura
(T4a), then surgical resection can be considered in the absence
of a malignant effusion. Thus, it can be seen that the therapy
of esophageal cancer is largely driven by the findings of an
endoscopic ultrasonography. It is difficult to provide modern
treatment of esophageal cancer without access to this modality.
PET scanning, usually combined with an axial CT scan
(CTPET), usually is performed on patients with locally
advanced cancer or questionable lesions on CT scan to determine whether metastases are present. The PET scan uses the
injection of radiolabeled deoxyglucose, which is taken up in
metabolically active tissues such as cancer. PET-positive areas
must be correlated with the CT scan findings to assess the significance of “hot spots.” CTPET scanning has been especially
useful before the initiation of chemoradiation therapy. An early
response to chemoradiotherapy, by PET scan, improves the
prognosis whether or not resection is ultimately performed.
Conversely, if a PET-avid tumor shows no change in metabolic
activity after 2 weeks of induction chemoradiation therapy, it
is unlikely that further chemo- or radiation therapy will be of
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Middle
thoracic
induction chemoradiation therapy, more pronounced dysphagia
and associated malnutrition should be addressed before the
initiation of chemoradiation. A laparoscopic jejunostomy
tube can be placed prior to induction therapy or at the time
of esophagectomy. There are emerging data that 5 days’
pretreatment with immune-enhancing nutrition, rich in fish
oils, decreases cardiac and other complications, following
esophagectomy.
1074
any benefit. These patients have a worse prognosis and may be
referred for resection or palliation without incurring the morbidity or expense of a full course of chemo- and radiation therapy.
Palliation of Esophageal Cancer
PART II
SPECIFIC CONSIDERATIONS
Palliation of esophageal cancer is indicated for individuals with
metastatic esophageal cancer or cancer invading adjacent organs
(T4b) who are unable to swallow, or individuals with fistulae
into the tracheobronchial tree. Aortic esophageal fistulas are
extremely rare and nearly 100% lethal. Dysphagia as a result of
esophageal cancer can be graded from grade I, eating normally,
to grade VI, unable to swallow saliva (Table 25-12). Grades I
to III often can be managed with radiation therapy, usually in
combination with chemotherapy. When surgical resection is not
anticipated in the future, this is termed definitive chemoradiation therapy and usually is palliative. Radiation dose is increased
from 45 Gy to 60 Gy administered over 8 weeks, rather than the
4 weeks given for chemoradiation induction therapy. In 20% of
patients, a complete response to chemoradiation therapy will
not only palliate the symptoms but will also leave the patient
with undetectable cancer of the esophagus. Although some of
these patients are truly cured, cancer will recur in many either
locally or systemically 1 to 5 years following definitive chemoradiation. In a few patients, definitive chemoradiation will be
successful in all sites but the esophagus. After a 12-month wait
from initial treatment and no other sites of tumor detectable
except the esophagus, some of these patients may be candidates
for salvage esophagectomy.
For individuals with dysphagia grades IV and higher, additional treatment generally is necessary. The mainstay of therapy
is in-dwelling esophageal stents. Covered removable stents may
be used to seal fistulae or when stent removal becomes desirable in the future. When large, locally invasive tumors or metastatic esophageal cancer precludes any future hope of resection,
uncovered expandable metal stents are the treatment of choice.
The major limitations to stenting exist in cancers at the GEJ. A
stent placed across the GEJ will result in severe gastroesophageal reflux and heartburn that can be quite disabling. In cancers
at this level, radiation therapy alone may be preferable. If feeding access is desirable, a laparoscopic jejunostomy is usually the
procedure of choice.
Table 25-12
Functional grades of dysphagia
GRADE
DEFINITION
INCIDENCE AT
DIAGNOSIS (%)
I
Eating normally
11
II
Requires liquids with meals
21
III
Able to take semisolids but
unable to take any solid food
30
IV
Able to take liquids only
40
V
Unable to take liquids, but able
to swallow saliva
7
VI
Unable to swallow saliva
12
Data from Takita H, Vincent RG, Caicedo V, et al. Squamous cell
carcinoma of the esophagus: a study of 153 cases, J Surg Oncol.
1977;9(6):547-554.
Surgical Treatment
The surgical treatment of esophageal cancer is dependent upon
the location of the cancer, the depth of invasion, LN metastases,
the fitness of the patient for operation, and the culture and
beliefs of the individuals and institutions in which the treatment
is performed. In an ideal world, there would be a single,
stage-specific method of treating esophageal cancer because
the evidence would be unassailable and noncontroversial.
Randomized clinical trials and meta-analyses would prove
beyond a shadow of a doubt the value of surgery vs. nonoperative
therapy and would dictate the type and extent of surgery that
would optimally balance immediate morbidity and mortality
with duration and quality of life conferred by the procedure and
the perioperative management of the esophagectomy patient.
Despite many noble attempts to establish this high level of
evidence, many questions relating to the appropriate therapy
of esophageal cancer remain. About the only area of complete
agreement is that esophagectomy should not be performed if an
R0 resection is not possible. In other words, if the surgeon does
not believe he or she can remove all LNs invaded by cancer and
provide a tumor-free radial margin and esophagus and stomach
margins that are tumor free, then a resection should not be
performed.
Mucosally Based Cancer. In patients with BE, and especially
those with high-grade dysplasia, subcentimeter nodules are
frequently discovered. Nodules should be resected in entirety,
as they often harbor adenocarcinoma. Five years ago, such
resection was performed with a transhiatal esophagectomy,
but more recently EMR offers another method for removing
intramucosal cancer. In this clinical situation, EMR is typically combined with EUS to rule out more invasive disease.
EUS, however, is unable to differentiate between cancer that is
confined to the mucosa (T1a) and that which invades the submucosa (T1b). Tumors invading the submucosa are not amenable
to endoscopic mucosal resection because of the high-frequency
(20–25%) concurrent finding of positive LNs, which cannot be
removed without esophagectomy. On the other hand, intramucosal cancers have little risk of spreading to regional LNs. The
current approach used involves performing EMR on all nodules
identified in a field of Barrett’s esophagus, and then T staging
is performed by histologic analysis. This approach dictates the
need for future therapy such as esophagectomy.
For this reason, small intramucosal carcinomas may be
removed with EMR in the following manner. The area beneath
the nodule is infiltrated with saline through a sclerotherapy
needle. A specialized suction cap is mounted on the end of the
endoscope, and the nodule is drawn up into the cap; a snare
is then applied to resect the tissue. Alternatively, a rubber
band can be delivered, and the snare can be used to resect
above the level of the rubber band. This specimen is then
removed and sent to pathology. As long as the tumor is found
to be confined to the mucosa and all margins are negative,
the resection is complete. A positive margin or involvement
of the submucosa warrants esophagectomy. Most importantly,
these patients are at high risk for developing small nodular
carcinomas elsewhere in their Barrett’s segment, and routine
surveillance on a 3- to 6-month basis must be continued
indefinitely. Alternatively, one can consider radiofrequency
ablation of the remainder of the high-grade dysplasia after
careful surveillance biopsy specimens demonstrate no further
sign of cancer. This approach to the early esophageal cancer
1075
should not be used when there is any suspicion of mediastinal
or abdominal lymphadenopathy. Although it is currently
rare that EMR provides definitive therapy of small nodular
esophageal cancers, this may become more of the norm as
greater surveillance reveals earlier cancers and proficiency of
the technique by surgeons and gastroenterologists increases.
Minimally Invasive Transhiatal Esophagectomy.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Minimally invasive transhiatal esophagectomy is an increasingly
popular procedure; however, the number of these operations
performed around the world remains small. Mini-invasive
surgery (MIS) transhiatal esophagectomy was first performed
by Aureo DePaula in Brazil and has been modified and adopted
by many individuals around the world. This operation combines
the advantages of transhiatal esophagectomy at minimizing
pulmonary complications with the advantages of laparoscopy
(less pain, quicker rehabilitation). Several variations of MIS
transhiatal esophagectomy have been developed. For the
earliest lesions, such as high-grade dysplasia or intramucosal
carcinoma, a vagal sparing procedure can be entertained. In such
a procedure, the vagal trunks are separated from the esophagus
at the level of the diaphragm and the lesser curvature dissection
of the stomach allows the vagus and left gastric pedicle to
remain intact. Clearly, this dissection, which hugs the stomach
and esophagus, provides no LN staging and is thus inadequate
for all high-grade dysplasia and intramucosal cancer.
MIS transhiatal esophagectomy is usually performed
through five or six small incisions in the upper abdomen and
a transverse cervical incision for removing the specimen and
performing the cervical esophagogastrostomy. To remove
the esophagus from the posterior mediastinum, especially the
area behind the pulmonary vessels and the tracheal bifurcation,
which cannot be visualized even with a long laparoscope
placed in the posterior mediastinum, it is preferred to use a
vein stripping “inversion” technique (Fig. 25-69A). The details
of this operation are too lengthy to include in this text, but
include the laparoscopic creation of a neo-esophagus (gastric
conduit) along the greater curvature of the stomach using the
right gastroepiploic artery as the primary vascular pedicle.
The conduit can be created through a mini-laparotomy or
laparoscopically. A Kocher maneuver releases the duodenum,
and a pyloroplasty may be performed (optional). Retrograde
esophageal stripping is performed by dividing the esophagus
below the GEJ and sliding a vein stripper from the neck down
into the abdomen followed by an inversion of the esophagus
in the posterior mediastinum and removal through the
neck (Fig. 25-69B). This technique is reserved for patients
with high-grade dysplasia. For small cancers at the GEJ, the
esophagus can be stripped in an antegrade fashion by sliding
the vein stripper down from the cervical incision and out the
tail of the lesser curvature (Fig. 25-69C). The tail of the lesser
curvature is pulled out a port site high in the epigastrium while
the esophagus is inverted into itself. For GEJ cancers, a wide
celiac access LN dissection, splenic artery, hepatic artery, and
posterior mediastinal LN dissection can be performed as well
or better than through a laparotomy. The gastric conduit is
pulled up to the neck with a chest tube and anastomosed to the
cervical esophagus in an end-to-side fashion using a surgical
stapler or with a handsewn anastomosis. Complications
of this technique are primarily limited to leak from the
esophagogastric anastomosis, which is self-limited and usually
heals within 1 to 3 weeks, spontaneously.
Figure 25-69. A. Laparoscopic retrograde inversion. B. Laparoscopic antegrade inversion. A silk suture holds the tunnel after the
esophagus is removed. C. The esophageal conduit is returned to
the neck after passing a chest tube down the tunnel and suturing the
conduit to the chest tube.
1076
PART II
SPECIFIC CONSIDERATIONS
Open Transhiatal Esophagectomy. Transhiatal esophagectomy, also known as blunt esophagectomy or esophagectomy
without a thoracotomy, was first performed in 1933 by a British
surgeon, but was popularized in the last quarter of the 20th century
by Mark Orringer from the University of Michigan. Although
this operation may violate many of the principles of cancer resection, including extended radical LN dissection, this operation
has performed as well as any of the more radical procedures in
randomized trials, and in large database analyses. With transhiatal esophagectomy, the elements of dissection are similar to that
described in the section entitled Minimally Invasive Transhiatal
Esophagectomy, including the creation of the gastric tube and the
posterior mediastinal dissection through the hiatus. Because this
dissection is performed with the fingertips rather than under direct
vision with surgical instruments, it requires an enlargement of the
diaphragmatic hiatus. The lower mediastinal LN basins can be
resected as can the upper abdominal LNs, making this an attractive option for GEJ cancers. The mediastinal LNs above the inferior pulmonary vein are not removed with this technique, but they
rarely result in a point of isolated cancer recurrence.
Of all procedures for esophageal cancer, this operation is the
quickest to perform in experienced hands and lies in an intermediate position between minimally invasive esophagectomy and the
Ivor Lewis procedure with respect to complications and recovery.
Minimally Invasive Two- and Three-Field Esophagectomy.
After a rocky start, minimally invasive esophagectomy using
a thoracic dissection through VATS has become reasonably
popular. In general, this operation is performed with an
anastomosis created in the neck (three-field), but it may be
performed with the anastomosis stapled in the high thorax (twofield). Both procedures will be described.
With a minimally invasive three-field esophagectomy,
the patient is placed in the left lateral decubitus position.
Double lumen intubation is required. Videoscopic access
to the thorax is obtained in the midaxillary line in the ninth
intercostal space and an angled telescope illuminates the chest
superiorly. A mini-thoracotomy at about the sixth intercostal
space anteriorly allows introduction of conventional surgical
instruments, and a high trocar allows retraction of the lung
away from the esophagus. In a three-field approach, the
esophagus is dissected along its length to include division
of the azygos vein and harvesting of the LNs in the upper,
middle, and lower posterior mediastinum. Hilar, and posterior
mediastinal nodes are all removed and sent with the specimen
or individually. The thoracic duct is divided at the level of the
diaphragm and removed with the specimen.
Following complete intrathoracic dissection, the patient
is placed in the supine position and five laparoscopic ports
are placed as with the MIS transhiatal esophagectomy. The
abdominal portions of the operation are identical to those
described previously in the section entitled “Minimally Invasive
Transhiatal Esophagectomy,” and the gastric conduit is then
sewn to the tip of the fully mobilized GEJ and lesser curvature
sleeve. A feeding tube is placed, and the pyloroplasty may be
performed laparoscopically. A transverse cervical incision and
dissection between the sternocleidomastoid and the anterior
strap muscles allows access to the cervical esophagus. Great
care is made to avoid stretching the recurrent laryngeal nerve.
The esophagus and proximal stomach is then pulled up into the
neck with the gastric conduit following. Cervical anastomosis
is then performed.
The MIS transthoracic two-field esophagectomy is
slightly different. In this operation, the abdominal portions of
the operation are done first, including placement of the feeding
tube, the creation of the conduit, and the sewing of the tip
of the conduit to the fully dissected GEJ. The patient is then
rolled into the left lateral decubitus position and, through right
thoracoscopy, the esophagus is dissected and divided 10 cm
above the tumor. Once freed, the specimen is pulled out through
the mini-thoracotomy, and an end-to-end anastomosis stapler is
introduced through the high corner of the gastric conduit and out
a stab wound along the greater curvature. The anvil of the stapler
is placed in the proximal esophagus and held with a pursestring, the stapler is docked, the anastomosis is created, and a
gastrotomy is then closed with another firing of the GIA stapler.
The three-field esophagectomy has the advantage of placing the
anastomosis in the neck where leakage is unlikely to create a
severe systemic consequence. On the other hand, placement of
the anastomosis in the high chest minimizes the risks of injury
to structures in the neck, particularly the recurrent laryngeal
nerve. Although the leak of the intrathoracic anastomosis may
be more likely to bear septic consequences, the incidence of leak
is diminished. Other complications of this approach relate to
pulmonary and cardiac status. In many series, the most common
complication is pneumonia, the second is atrial fibrillation, and
the third is anastomotic leak.
Ivor Lewis (En Bloc) Esophagectomy. The theory behind
radical transthoracic esophagectomy is that greater removal of
LNs and periesophageal tissues diminishes the chance of a positive radial margin and LN recurrence. Although there are no randomized data demonstrating this to be superior to other forms of
esophagectomy, there are many retrospective data demonstrating improved survival with greater numbers of LNs harvested.
A recent study from Sloan-Kettering demonstrates a direct relationship between the number of negative nodes harvested and
long-term survival. Although such a survival advantage may be
related to the completeness of resection, extended radical resections may also be a surrogate for experienced surgeons working
in great institutions. As a time-honored operation, there is no
doubt that en bloc esophagectomy is the standard to which less
radical techniques must be compared.
Generally, this operation is started in the abdomen with
an upper midline laparotomy and extensive LN dissection in
and about the celiac access and its branches, extending into the
porta hepatis and along the splenic artery to the tail of the pancreas. All LNs are removed en bloc with the lesser curvature of
the stomach. Unless the tumor extends into the stomach, reconstruction is performed with a greater curvature gastric tube. For
GEJ cancers extending significantly into the gastric cardia or
fundus, the proximal stomach is removed, and reconstruction
is performed with an isoperistaltic section of left colon between
the upper esophagus and the remnant stomach, or the colon is
connected to a Roux-en-Y limb of jejunum, if total gastrectomy
is necessary. In the majority of cases, colon interposition is
unnecessary, and a gastric conduit is used.
Following closure of the abdominal incision, the patient is
placed in the left lateral decubitus position and an anterolateral
thoracotomy is performed through the sixth intercostal space.
The azygos vein is divided and the posterior mediastinum is
entirely cleaned out to include the thoracic duct, all periaortic tissues, and all tissue in the upper mediastinum along the
course of the current laryngeal nerves and in the peribronchial,
but when adjusted by cancer stage, this survival benefit disappeared. The mortality and morbidity after transhiatal esophagectomy appeared to be less. Suffice it to say that this debate
over the best procedure for esophagectomy remains an open
question.
The role of the minimally invasive surgical procedures for
a cancer cure will require further study and longer follow-up.
It would appear from preliminary analysis that the transhiatal
esophagectomy, like its open cousin, may be performed with
less morbidity and mortality than the VATS procedure. Longterm survival analyses will require careful follow-up for at least
5 to 10 years after cancer treatment. A recent European multicenter randomized trial comparing open and minimally invasive
approaches revealed a highly significant reduction in pulmonary complications in the patients who underwent the minimally
invasive approach. There was no difference in procedure-related
mortality between the approaches.
Three-Field Open Esophagectomy. Three-field open esoph-
Radiation Therapy. Primary treatment with radiation ther-
agectomy is very similar to a minimally invasive three-field
except that all access is through open incisions. This procedure is preferred by certain Japanese surgeons and LN counts
achieved through this kind of operation may run from 45 to
60 LNs. Most Western surgeons question the benefit of such
radical surgery when it is hard to define a survival advantage.
Nonetheless, high intrathoracic cancers probably deserve such
an aggressive approach if cure is the goal.
Salvage Esophagectomy. Salvage esophagectomy is the
nomenclature applied to esophagectomy performed after
failure of definitive radiation and chemotherapy. The most
frequent scenario is one in which distant disease (bone, lung,
brain, or wide LN metastases) renders the patient nonoperable
at initial presentation. Then, systemic chemotherapy, usually
with radiation of the primary tumor, destroys all foci of
metastasis, as demonstrated by CT and CT-PET, but the
primary remains present and symptomatic. Following a period
of observation, to make sure no new disease will become
evident, salvage esophagectomy is performed, usually with
an open two-field approach. Surprisingly, the cure rate of
salvage esophagectomy is not inconsequential. One in four
patients undergoing this operation will be disease free 5 years
later, despite the presence of residual cancer in the operative
specimen. Because of the dense scarring created by radiation
treatment, this procedure is the most technically challenging
of all esophagectomy techniques.
Comparative Studies of Esophagectomy
Technique
Transthoracic vs. Transhiatal Esophagectomy. There has
been a great debate as to whether en bloc esophagectomy will
provide a greater long-term benefit and cure rate in esophageal
cancer than transhiatal esophagectomy. In a recent 7-year follow-up of a Dutch study addressing GEJ and lower esophageal
cancers, there does not appear to be any benefit to the more
extensive dissection despite higher morbidity and mortality. In
a subgroup analysis of those with one to eight positive LNs,
it did appear that the en bloc transthoracic resection may add
to longevity. In another large database analysis of the Surveillance, Epidemiology, and End Results database, transthoracic
and transhiatal esophagectomy were compared. In this study,
the transhiatal esophagectomy had a greater long-term survival,
Alternative Therapies
apy does not produce results comparable with those obtained
with surgery. Currently, the use of radiotherapy is restricted to
patients who are not candidates for surgery, and it is usually
combined with chemotherapy. Radiation alone is used for palliation of dysphagia, but the benefit is short lived, lasting only
2 to 3 months. Furthermore, the length and course of treatment
are difficult to justify in patients with a limited life expectancy.
Radiation is effective in patients who have hemorrhage from
the primary tumor.
Adjuvant Chemotherapy. The proposal to use adjuvant chemotherapy in the treatment of esophageal cancer began when it
became evident that most patients develop postoperative systemic metastasis without local recurrence. This observation
led to the hypothesis that undetected systemic micrometastasis had been present at the time of diagnosis, and if effective
systemic therapy was added to local regional therapy, survival
should improve.
Recently, this hypothesis has been supported by the observation of epithelial tumor cells in the bone marrow in 37% of
patients with esophageal cancer who were resected for cure.
These patients had a greater prevalence of relapse at 9 months
after surgery compared to those patients without such cells.
Such studies emphasize that hematogenous dissemination of
viable malignant cells occurs early in the disease, and that systemic chemotherapy may be helpful if the cells are sensitive to
the agent. On the other hand, systemic chemotherapy may be a
hindrance, because of its immunosuppressive properties, if the
cells are resistant. Unfortunately, current technology is not able
to test tumor cell sensitivity to chemotherapeutic drugs. This
requires that the choice of drugs be made solely on the basis of
their clinical effectiveness against grossly similar tumors.
The decision to use preoperative rather than postoperative chemotherapy was based on the ineffectiveness of chemotherapeutic agents when used after surgery, and animal studies
suggesting that agents given before surgery were more effective. The claim that patients who receive chemotherapy before
resection are less likely to develop resistance to the drugs is
unsupported by hard evidence. The claim that drug delivery is
enhanced because blood flow is more robust before patients
undergo surgical dissection is similarly flawed, due to the
fact that if enough blood reaches the operative site to heal the
wound or anastomosis, then the flow should be sufficient to
1077
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
hilar, and tracheal LN stations. The proximal stomach is pulled
up into the thorax where a conduit is created (if not performed
previously) and a handsewn or stapled anastomosis is made
between the upper thoracic esophagus and the gastric conduit
or transverse colon. Chest tubes are placed, and the patient is
taken to the intensive care unit.
Because this is the most radical of dissections, complications are most common, including pneumonia, respiratory failure, atrial fibrillation, chylothorax, anastomotic leak,
conduit necrosis, gastrocutaneous fistula, and, if dissection is
too near the recurrent laryngeal nerves, hoarseness will occur
with an increased risk of aspiration. Tracheobronchial injury
resulting in fistulas between the bronchus and conduit may
also occur, however rarely. Although this procedure and threefield esophagectomy are fraught with the highest complication rate, the long-term outcome of this procedure provides the
greatest survival in many single-center series and retrospective
reviews.
deliver chemotherapeutic drugs. There are, however, data supporting the claim that preoperative chemotherapy in patients
with esophageal carcinoma can, if effective, facilitate surgical
resection by reducing the size of the tumor. This is particularly
beneficial in the case of squamous cell tumors above the level
of the carina. Reducing the size of the tumor may provide a
safer margin between the tumor and the trachea and allow an
anastomosis to a tumor-free cervical esophagus just below the
cricopharyngeus. Involved margin at this level usually requires
a laryngectomy to prevent subsequent local recurrence.
PART II
Preoperative Chemotherapy. Eight randomized prospective studies of neoadjuvant chemotherapy vs. surgery alone
have demonstrated mixed results. For adenocarcinomas of the
distal esophagus and proximal stomach, preoperative neoadjuvant 5-fluorouracil (5-FU) and cisplatin chemotherapy has been
shown to provide a survival advantage over surgery alone in a
well-powered study from the United Kingdom (MRC trial). This
trial is one of the few to include enough patients (800) to detect
small differences. The trial had a 10% absolute survival benefit
at 2 years for the neoadjuvant chemotherapy group. In a second
trial from the United Kingdom (MAGIC trial) of distal esophageal and proximal gastric adenocarcinomas, the use of epirubicin in combination with cisplatin and 5-FU also demonstrated
a survival advantage for the induction chemotherapy arm with
4 years median follow-up. As a result of these two trials, standard treatment of locally advanced adenocarcinoma in Europe
calls for neoadjuvant chemotherapy with one of these two regimens. Most failures are due to distant metastatic disease, underscoring the need for improved systemic therapy. Postoperative
septic and respiratory complications may be more common in
patients receiving chemotherapy.
SPECIFIC CONSIDERATIONS
1078
Preoperative Combination Chemo- and Radiotherapy.
Preoperative chemoradiotherapy using cisplatin and 5-FU in
combination with radiotherapy has been reported by several
investigators to be beneficial in both adenocarcinoma and squamous cell carcinoma of the esophagus. There have been 10
randomized prospective studies (Table 25-13). A recent metaanalysis of these trials demonstrates a 13% survival advantage
for neoadjuvant chemoradiation therapy, which is more pronounced for patients with adenocarcinoma than for those with
squamous carcinoma (Table 25-14). It was also observed that
the benefit for chemotherapy alone (7%) was not as dramatic as
for chemoradiotherapy used in the neoadjuvant setting. Additionally, other work has demonstrated the importance of obtaining an R0 (tumor-free) resection as the most important variable
determining long-term survival. Although there are no direct,
randomized comparisons between chemotherapy and chemoradiation therapy, it appears that the addition of radiation may
improve local response of the tumor and may allow a greater
opportunity for the surgeon to obtain an R0 resection.
The timing of surgery after chemoradiation induction is
generally felt to be optimal between 6 and 8 weeks following
the completion of induction therapy. Earlier than this time,
active inflammation may make the resection hazardous, and the
patients have not had time to recover fully from the chemoradiation. After 8 weeks, edema in the periesophageal tissue starts to
turn to scar tissue, making dissection more difficult.
With chemoradiation, the complete response rates for adenocarcinoma range from 17% to 24% (Table 25-15). No tumor
is detected in the specimen after esophagectomy. Patients demonstrating a complete response to chemoradiation have a better
survival rate than those without complete response, but distant
failure remains common.
At present, the strongest predictors of outcome of patients
with esophageal cancer are the anatomic extent of the tumor at
diagnosis and the completeness of tumor removal by surgical
resection. After incomplete resection of an esophageal cancer,
the 5-year survival rates are 0% to 5%. In contrast, after complete resection, independent of stage of disease, 5-year survival ranges from 15% to 40%, according to selection criteria
and stage distribution. The importance of early recognition
and adequate surgical resection cannot be overemphasized.
Figure 25-70 is a global algorithm for the management of
esophageal carcinoma.
SARCOMA OF THE ESOPHAGUS
Sarcomas and carcinosarcomas are rare neoplasms, accounting for approximately 0.1% to 1.5% of all esophageal tumors.
They present with the symptom of dysphagia, which does not
differ from the dysphagia associated with the more common
epithelial carcinoma. Tumors located within the cervical or
high thoracic esophagus can cause symptoms of pulmonary
aspiration secondary to esophageal obstruction. Large tumors
originating at the level of the tracheal bifurcation can produce
symptoms of airway obstruction and syncope by direct compression of the tracheobronchial tree and heart (Fig. 25-71).
The duration of dysphagia and age of the patients affected
with these tumors are similar to those with carcinoma of the
esophagus.
A barium swallow usually shows a large polypoid intraluminal esophageal mass, causing partial obstruction and dilatation of the esophagus proximal to the tumor (Fig. 25-72). The
smooth polypoid nature of the lesion, although not diagnostic,
is distinctive enough to suggest the presence of a sarcoma rather
than the more common ulcerating, stenosing carcinoma.
Esophagoscopy commonly shows an intraluminal necrotic
mass. When biopsy is attempted, it is important to remove the
necrotic tissue until bleeding is seen on the tumor’s surface.
When this is not done, the biopsy specimen will show only tissue necrosis. Even when viable tumor is obtained on biopsy,
it has been these authors’ experience that it cannot be definitively identified as carcinoma, sarcoma, or carcinosarcoma on
the basis of the histology of the portion biopsied. Biopsy results
cannot be totally relied on to identify the presence of sarcoma,
and it is often the polypoid nature of the lesion that arouses suspicion that it may be something other than carcinoma.
Polypoid sarcomas of the esophagus, in contrast to infiltrating carcinomas, remain superficial to the muscularis propria
and are less likely to metastasize to regional LNs. In one series
of 14 patients, local extension or tumor metastasis would have
prevented a potentially curative resection in only five. Thus, the
presence of a large polypoid tumor should not deter the surgeon
from resecting the lesion.
Sarcomatous lesions of the esophagus can be divided into
epidermoid carcinomas with spindle cell features, such as carcinosarcoma, and true sarcomas that arise from mesenchymal
tissue, such as leiomyosarcoma, fibrosarcoma, and rhabdomyosarcoma. Based on current histologic criteria for diagnosis, fibrosarcoma and rhabdomyosarcoma of the esophagus are
extremely rare lesions.
Surgical resection of polypoid sarcoma of the esophagus
is the treatment of choice because radiation therapy has little
1079
Table 25-13
Randomized trials of neoadjuvant chemoradiotherapy vs. surgery, or neoadjuvant chemotherapy vs. surgery
YEAR
TREATMENT SCHEDULE TREATMENT SCHEDULE
ACTIVATED (RADIOTHERAPY)
(CHEMOTHERAPY)
Two cycles: cisplatin 20 mg/m2 d 1–5; Sequential
bleomycin 5 mg/m2 d 1–5
Concurrent
Two cycles: cisplatin 100 mg/m2 d 1;
5-fluorouracil 1000 mg/m2 d 1–4
Two cycles: cisplatin 100 mg/m2 d 1;
Sequential
5-fluorouracil 600 mg/m2 d 2–5,
22–25
1989
45 Gy, 1.5 Gy/fraction Two cycles: cisplatin 20 mg/m2 d 1–5; Concurrent
over 3 wk
5-fluorouracil 300 mg/m2 d 1–21;
vinblastine 1 mg/m2 d 1–4
1989
37 Gy, 3.7 Gy/fraction Two cycles: cisplatin 80 mg/m2 d 0–2 Sequential
over 2 wk
1990
40 Gy, 2.7 Gy/fraction Two cycles: cisplatin 75 mg/m2 d 7;
Concurrent
over 3 wk
5-fluorouracil 15 mg/kg d 1–5
1990
40 Gy, 2.7 Gy/fraction Two cycles: cisplatin 75 mg/m2 d 7;
Concurrent
over 3 wk
5-fluorouracil 15 mg/kg d 1–5
Concurrent
1994
35 Gy, 2.3 Gy/fraction One cycle: cisplatin 80 mg/m2 d 1;
over 3 wk
5-fluorouracil 800 mg/m2 d 2–5
2006
50.4 Gy, 1.8 Gy/
Two cycles: cisplatin 60 mg/m2 d 1;
Concurrent
fraction over 5.6 wk
5-fluorouracil 1000 mg/m2 d 3–5
Concurrent
1999
45.6 Gy, 1.2 Gy/
Two cycles: cisplatin 60 mg/m2 d 1;
fraction over 28 d
5-fluorouracil 1000 mg/m2 d 3–5
Chemotherapy
1982
—
Two cycles: cisplatin 120 mg/m2 d 1;
—
vindesine 3 mg/m2 d 1, 8; bleomycin
10 U/m2 d 3–6
1983
—
Two cycles: cisplatin 20 mg/m2 d 1–5; —
bleomycin 5 mg/m2 d 1–5
c
1988
—
Three cycles: cisplatin 20 mg/m2 d 1–5; —
5-fluorouracil 1000 mg/m2 d 1–5
1988
—
Two cycles: cisplatin 100 mg/m2 d 1;
—
bleomycin 10 mg/m2 d 3–8;
vinblastine 3 mg/m2 d 1, 8
—
1989
—
Two cycles: cisplatin 100 mg/m2 d 1;
5-fluorouracil 1000 mg/m2 d 1–5
1990
—
Two cycles: cisplatin 80 mg/m2 d 1;
—
etoposide 200 mg/m2 d 1–5
—
1990
—
Three cycles: cisplatin 100 mg/m2 1;
5-fluorouracil 1000 mg/m2 days 1–5
1992
—
Two cycles: cisplatin 100 mg/m2 d 1;
—
5-fluorouracil 1000 mg/m2 d 1–5
—
1992
—
Two cycles: cisplatin 80 mg/m2 d 1;
5-fluorouracil 1000 mg/m2 d 1–4
MEDIAN
SAMPLE FOLLOWSIZE
UP (MO)
SCC
78
18a
SCC
69
12a
SCC
86
12a
SCC and
100
adenocarcinoma
98
SCC
293
55
Adenocarcinoma
113
24
SCC
61
10
SCC and
256
adenocarcinoma
SCC and
56
adenocarcinoma
SCC
101
65
60
SCC
39
20
SCC
106
18a
SCC
46
75
SCC
46
17a
SCC
147
17
SCC
160
19a
SCC and
467
adeno-carcinoma
SCC
96
56
SCC and
802
adeno-carcinoma
37
25
24
Estimated as median survival.
Unpublished thesis.
c
Year of activation not reported, but imputed.
d
Only available as an abstract.
SCC = squamous cell carcinoma.
Reproduced with permission from Gebski V, Burmeister B, Smithers BM, et al: Survival benefits from neoadjuvant chemoradiotherapy or chemotherapy
in oesophageal carcinoma: a meta-analysis, Lancet Oncol. 2007 Mar;8(3):226-234.
a
b
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Chemoradiotherapy
1983
35 Gy, 1.75 Gy/
fraction over 4 wk
1986
40 Gy, 2 Gy/fraction
over 4 wk
1988
20 Gy, 2 Gy/fraction
over 12 d
CONCURRENT
OR
SEQUENTIAL TUMOR TYPE
1080
Table 25-14
Results of the meta-analysis applied to effects of
preoperative chemoradiotherapy and chemotherapy on
2-y survival for patients with various levels of risk
RISK
GROUP
2-Y
SURVIVAL
RATE (%)
EXPECTED 2-Y MORTALITY
CONTROL
(%)
TREATEDa
(%)
ARR
(%)
NNT
PART II
Chemoradiotherapy
High
20
80
64.8
15.2
7
Medium
35
65
52.7
12.3
8
Low
50
50
40.5
9.5
10
SPECIFIC CONSIDERATIONS
Chemotherapy
High
20
80
72.0
12.0
8
Medium
35
65
58.5
6.5
15
Low
50
50
45.0
5.0
20
Based on a 19% relative mortality reduction for those receiving
concurrent chemoradiotherapy and a 10% relative mortality reduction
for those receiving chemotherapy.
ARR = absolute risk reduction; NNT = number needed to treat to
prevent one death.
Reproduced with permission from Gebski V, Burmeister B, Smithers
BM, et al: Survival benefits from neoadjuvant chemoradiotherapy or
chemotherapy in oesophageal carcinoma: a meta-analysis, Lancet Oncol.
2007 Mar;8(3):226-234.
a
success and the tumors remain superficial, with local invasion
or distant metastases occurring late in the course of the disease.
As with carcinoma, the absence of both wall penetration and
LN metastases is necessary for curative treatment, and surgical resection is consequently responsible for the majority of the
reported 5-year survivals. Resection also provides an excellent
means of palliating the patient’s symptoms. The surgical technique for resection and the subsequent restoration of the GI continuity is similar to that described for carcinoma.
In these authors’ experience, four of the eight patients with
carcinosarcoma survived for 5 years or longer. Even though
this number is small, it suggests that resection produces better
results in epithelial carcinoma with spindle cell features than
in squamous cell carcinoma of the esophagus. Similarly, with
leiomyosarcoma of the esophagus, the same scattered reports
exist with little information on survival. Of seven patients with
leiomyosarcoma, two died from their disease—one in 3 months
and the other 4 years and 7 months after resection. The other
five patients were reported to have survived more than 5 years.
It is difficult to evaluate the benefits of resection for leiomyoblastoma of the esophagus because of the small number of
reported patients with tumors in this location. Most leiomyoblastomas occur in the stomach, and 38% of these patients succumb to the cancer in 3 years. Fifty-five percent of patients
with extragastric leiomyoblastoma also die from the disease,
within an average of 3 years. Consequently, leiomyoblastoma
should be considered a malignant lesion and apt to behave like
a leiomyosarcoma. The presence of nuclear hyperchromatism,
increased mitotic figures (more than one per high-power field),
tumor size larger than 10 cm, and clinical symptoms of longer
than 6 months’ duration are associated with a poor prognosis.
BENIGN TUMORS AND CYSTS
Benign tumors and cysts of the esophagus are relatively uncommon. From the perspectives of both the clinician and the pathologist, benign tumors may be divided into those that are within the
muscular wall and those that are within the lumen of the esophagus.
Intramural lesions are either solid tumors or cysts, and the
vast majority are leiomyomas. They are made up of varying portions of smooth muscle and fibrous tissue. Fibromas, myomas,
fibromyomas, and lipomyomas are closely related and occur
rarely. Other histologic types of solid intramural tumors have
been described, such as lipomas, neurofibromas, hemangiomas,
osteochondromas, granular cell myoblastomas, and glomus
tumors, but they are medical curiosities.
Intraluminal lesions are polypoid or pedunculated growths
that usually originate in the submucosa, develop mainly into the
lumen, and are covered with normal stratified squamous epithelium. The majority of these tumors are composed of fibrous
tissue of varying degrees of compactness with a rich vascular
supply. Some are loose and myxoid (e.g., myxoma and myxofibroma), some are more collagenous (e.g., fibroma), and some
contain adipose tissue (e.g., fibrolipoma). These different types
of tumor are frequently collectively designated fibrovascular
Table 25-15
Results of neoadjuvant therapy in adenocarcinoma of the esophagus
INSTITUTION
YEAR
NO. OF PATIENTS
REGIMEN
COMPLETE PATHOLOGIC
RESPONSE (%)
SURVIVAL
MD Anderson
1990
35
P, E, 5-FU
3
42% at 3 y
SLMC
1992
18
P, 5-FU, RT
17
40% at 3 y
Vanderbilt
1993
39
P, E, 5-FU, RT
19
47% at 4 y
Michigan
1993
21
P, VBL, 5-FU, RT
24
34% at 5 y
MGH
1994
16
P, 5-FU
0
42% at 4 y
MGH
1994
22
E, A, P
5
58% at 2 y
A = doxorubicin; E = etoposide; 5-FU = 5-fluorouracil; MGH = Massachusetts General Hospital; P = cisplatin; RT = radiation therapy; SLMC = St. Louis
University Medical Center; VBL = vinblastine.
Reproduced with permission from Wright CD, Mathisen DJ, Wain JC, et al: Evolution of treatment strategies for adenocarcinoma of the esophagus and
gastroesophageal junction, Ann Thorac Surg. 1994 Dec;58(6):1574-1578.
1081
Barium swallow, endoscopy
Clinical evaluation
Late disease or
significant comorbidity
Early disease
Advanced disease
- Distant organ metastasis
- Imminent cardiac pulmonary
or hepatic failure
Tumor suspected not
to be through the wall and/or less than
8 lymph nodes involved
Through the wall and multiple
lymph node metastasis
Chemoradiation
Curative
en bloc resection
Preoperative chemoradiation
followed by en bloc resection
Treatment failure or
recurrence
Severe debility
Advanced disease
Local recurrence
No metastases
Complete excision
possible
Unresectable proximal
or bleeding tumor
Airway fistula or
unresectable primary
tumor or local
recurrence
Distant metastasis
No local recurrence
Supportive care
Palliative surgery
Laser ablative therapy
Stent
Chemotherapy
Figure 25-70. Suggested global algorithm for the management of carcinoma of the esophagus. CT = computed tomography.
polyps, or simply as polyps. Pedunculated intraluminal tumors
should be removed. If the lesion is not too large, endoscopic
removal with a snare is feasible.
Leiomyoma
Leiomyomas constitute more than 50% of benign esophageal
tumors. The average age at presentation is 38, which is in sharp
contrast to that seen with esophageal carcinoma. Leiomyomas
are twice as common in males. Because they originate in
smooth muscle, 90% are located in the lower two-thirds of the
esophagus. They are usually solitary, but multiple tumors have
been found on occasion. They vary greatly in size and shape.
Actually, tumors as small as 1 cm in diameter and as large as
10 lb have been removed.
Typically, leiomyomas are oval. During their growth, they
remain intramural, having the bulk of their mass protruding
toward the outer wall of the esophagus. The overlying mucosa is
freely movable and normal in appearance. Dysphagia and pain
are the most common complaints, the two symptoms occurring
more frequently together than separately. Bleeding directly
related to the tumor is rare, and when hematemesis or melena
occur in a patient with an esophageal leiomyoma, other causes
should be investigated.
A barium swallow is the most useful method to demonstrate a leiomyoma of the esophagus (Fig. 25-73). In profile, the
tumor appears as a smooth, semilunar, or crescent-shaped filling
defect that moves with swallowing, is sharply demarcated, and
is covered and surrounded by normal mucosa. Esophagoscopy
should be performed to exclude the reported observation of a
coexistence with carcinoma. The freely movable mass, which
bulges into the lumen, should not be biopsied because of an
increased chance of mucosal perforation at the time of surgical
enucleation. Endoscopic ultrasound is also a useful adjunct in
the workup of leiomyoma and provides detail related to the anatomic extent and relationship to surrounding structures.
Despite their slow growth and limited potential for malignant degeneration, leiomyomas should be removed unless there
are specific contraindications. The majority can be removed by
simple enucleation. If, during removal, the mucosa is inadvertently entered, the defect can be repaired primarily. After tumor
removal, the outer esophageal wall should be reconstructed by
closure of the muscle layer. The location of the lesion and the
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Tumor staging
(CT chest and abdomen,
endoscopic ultrasonography)
1082
PART II
SPECIFIC CONSIDERATIONS
B
A
Figure 25-71. A. Computed tomographic scan of a leiomyosarcoma (black arrow) that caused compression of the heart and symptoms of
syncope. B. Surgical specimen of leiomyosarcoma shown in A with a pedunculated luminal lesion (white arrow) and a large extraesophageal
component (black arrow). There was no evidence of lymph node metastasis at the time of operation.
A
B
Figure 25-72. A. Barium swallow showing a large polypoid intraluminal esophageal mass causing partial obstruction and dilation of the
proximal esophagus. B. Operative specimen showing 9-cm polypoid leiomyoblastoma.
ESOPHAGEAL PERFORATION
extent of surgery required will dictate the approach. Lesions of
the proximal and middle esophagus require a right thoracotomy,
whereas distal esophageal lesions require a left thoracotomy. Videothoracoscopic and laparoscopic approaches are now frequently
used. The mortality rate associated with enucleation is low, and
success in relieving the dysphagia is near 100%. Large lesions or
those involving the GEJ may require esophageal resection.
Esophageal Cyst
Cysts may be congenital or acquired. Congenital cysts are
lined wholly or partly by columnar ciliated epithelium of the
respiratory type, by glandular epithelium of the gastric type, by
squamous epithelium, or by transitional epithelium. In some,
epithelial lining cells may be absent. Confusion over the embryologic origin of congenital cysts has led to a variety of names,
such as enteric, bronchogenic, duplication, and mediastinal
cysts. Acquired retention cysts also occur, probably as a result
of obstruction of the excretory ducts of the esophageal glands.
Enteric and bronchogenic cysts are the most common,
and they arise as a result of developmental abnormalities during the formation and differentiation of the lower respiratory
tract, esophagus, and stomach from the foregut. During its
embryologic development, the esophagus is lined successively
with simple columnar, pseudostratified ciliated columnar, and,
finally, stratified squamous epithelium. This sequence probably
accounts for the fact that the lining epithelium may be any or a
combination of these; the presence of cilia does not necessarily
indicate a respiratory origin.
Cysts vary in size from small to very large, and they are
usually located intramurally in the middle- to lower-third of
the esophagus. Their symptoms are similar to those of a leiomyoma. The diagnosis similarly depends on radiographic,
endoscopic, and endosonographic findings. Surgical excision
by enucleation is the preferred treatment. During removal, a
fistulous tract connecting the cysts to the airways should be
sought, particularly in patients who have had repetitive bronchopulmonary infections.
Diagnosis
Abnormalities on the chest radiogram can be variable and
should not be depended upon to make the diagnosis. This is
because the abnormalities are dependent on three factors: (a)
the time interval between the perforation and the radiographic
examination, (b) the site of perforation, and (c) the integrity of
the mediastinal pleura. Mediastinal emphysema, a strong indicator of perforation, takes at least 1 hour to be demonstrated and is
present in only 40% of patients. Mediastinal widening secondary to edema may not occur for several hours. The site of perforation also can influence the radiographic findings. In cervical
perforation, cervical emphysema is common and mediastinal
emphysema rare; the converse is true for thoracic perforations.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Figure 25-73. Barium esophagogram showing a classical, smooth,
contoured, punched-out defect of a leiomyoma.
Perforation of the esophagus constitutes a true emergency. It
most commonly occurs following diagnostic or therapeutic procedures. Spontaneous perforation, referred to as Boerhaave’s
syndrome, accounts for only 15% of cases of esophageal perforation, foreign bodies for 14%, and trauma for 10%. Pain is a
striking and consistent symptom and strongly suggests that an
esophageal rupture has occurred, particularly if located in the
cervical area following instrumentation of the esophagus, or substernally in a patient with a history of resisting vomiting. If subcutaneous emphysema is present, the diagnosis is almost certain.
Spontaneous rupture of the esophagus is associated with a
high mortality rate because of the delay in recognition and treatment. Although there usually is a history of resisting vomiting,
in a small number of patients, the injury occurs silently, without
any antecedent history. When the chest radiogram of a patient
with an esophageal perforation shows air or an effusion in the
pleural space, the condition is often misdiagnosed as a pneumothorax or pancreatitis. An elevated pleural amylase caused by
the extrusion of saliva through the perforation may fix the diagnosis of pancreatitis in the mind of an unwary physician. If the
chest radiogram is normal, a mistaken diagnosis of myocardial
infarction or dissecting aneurysm is often made.
Spontaneous rupture usually occurs into the left pleural
cavity or just above the GEJ. About 50% of patients have
concomitant GERD, suggesting that minimal resistance to the
transmission of abdominal pressure into the thoracic esophagus
is a factor in the pathophysiology of the lesion. During vomiting,
high peaks of intragastric pressure can be recorded, frequently
exceeding 200 mmHg, but because extragastric pressure
remains almost equal to intragastric pressure, stretching of the
gastric wall is minimal. The amount of pressure transmitted to
the esophagus varies considerably, depending on the position
of the GEJ. When it is in the abdomen and exposed to intraabdominal pressure, the pressure transmitted to the esophagus
is much less than when it is exposed to the negative thoracic
pressure. In the latter situation, the pressure in the lower
esophagus will frequently equal intragastric pressure if the
glottis remains closed. Cadaver studies have shown that when
this pressure exceeds 150 mmHg, rupture of the esophagus is
apt to occur. When a hiatal hernia is present and the sphincter
remains exposed to abdominal pressure, the lesion produced is
usually a Mallory-Weiss mucosal tear, and bleeding rather than
perforation is the problem. This is due to the stretching of the
supradiaphragmatic portion of the gastric wall. In this situation,
the hernia sac represents an extension of the abdominal cavity,
and the GEJ remains exposed to abdominal pressure.
1083
1084
PART II
SPECIFIC CONSIDERATIONS
Figure 25-75. Radiographic study of a patient with a perforation
of the esophagus using water-soluble contrast material. The patient
is placed in the lateral decubitus position with the left side up to
allow complete filling of the esophagus and demonstration of the
defect.
Figure 25-74. Chest radiogram showing air in the deep muscles
of the neck following perforation of the esophagus (arrow). This
is often the earliest sign of perforation and can be present without
evidence of air in the mediastinum.
Frequently, air will be visible in the erector spinae muscles on
a neck radiogram before it can be palpated or seen on a chest
radiogram (Fig. 25-74). The integrity of the mediastinal pleura
influences the radiographic abnormality in that rupture of the
pleura results in a pneumothorax, a finding that is seen in 77%
of patients. In two-thirds of patients, the perforation is on the left
side; in one-fifth, it is on the right side; and in one-tenth, it is
bilateral. If pleural integrity is maintained, mediastinal emphysema (rather than a pneumothorax) appears rapidly. A pleural
effusion secondary to inflammation of the mediastinum occurs
late. In 9% of patients, the chest radiogram is normal.
The diagnosis is confirmed with a contrast esophagram,
which will demonstrate extravasation in 90% of patients. The
use of a water-soluble medium such as Gastrografin is preferred.
Of concern is that there is a 10% false-negative rate. This may
be due to obtaining the radiographic study with the patient in
the upright position. When the patient is upright, the passage of
water-soluble contrast material can be too rapid to demonstrate
a small perforation. The studies should be done with the patient
in the right lateral decubitus position (Fig. 25-75). In this, the
contrast material fills the entire length of the esophagus, allowing the actual site of perforation and its interconnecting cavities
to be visualized in almost all patients.
To get adequate exposure of the injury, a dissection similar to
that described for esophageal myotomy is performed. A flap
of stomach is pulled up and the soiled fat pad at the GEJ is
removed. The edges of the injury are trimmed and closed primarily (Fig. 25-77). The closure is reinforced with the use of a
pleural patch or construction of a Nissen fundoplication.
Mortality associated with immediate closure varies between
8% and 20%. After 24 hours, survival decreases to <50%, and
is not influenced by the type of operative therapy (i.e., drainage
alone or drainage plus closure of the perforation). If the time delay
before closing a perforation approaches 24 hours and the tissues
are inflamed, division of the cardia and resection of the diseased
portion of the esophagus are recommended. The remainder of the
esophagus is mobilized, and as much normal esophagus as possible is saved and brought out as an end cervical esophagostomy.
In some situations, the retained esophagus may be so long that
Management
The key to optimum management is early diagnosis. The most
favorable outcome is obtained following primary closure of the
perforation within 24 hours, resulting in 80% to 90% survival.
Figure 25-76 is an operative photograph taken through a left
thoracotomy of an esophageal rupture following a pneumatic
dilation for achalasia. The most common location for the injury
is the left lateral wall of the esophagus, just above the GEJ.
Figure 25-76. Left thoracotomy in a patient with an esophageal
rupture at the gastroesophageal junction following forceful dilation of the lower esophagus for achalasia (the surgical clamp is on
the stomach, and the Penrose drain encircles the esophagus). The
injury consists of a mucosal perforation and extensive splitting of
the esophageal muscle from just below the Penrose drain to the
stomach.
1085
conditions are met, it is reasonable to treat the patient with hyperalimentation, antibiotics, and cimetidine to decrease acid secretion and diminish pepsin activity. Oral intake is resumed in 7 to
14 days, dependent on subsequent radiographic examinations.
MALLORY-WEISS SYNDROME
Figure 25-77. The technique of closure of an esophageal perforation through a left thoracotomy. A. A tongue of stomach is pulled
up through the esophageal hiatus, and the gastroesophageal fat pad
is removed; the edges of the mucosal injury are trimmed and closed
using interrupted modified Gambee stitches. B. Reinforcement of
the closure with a parietal pleural patch.
it loops down into the chest. The contaminated mediastinum is
drained and a feeding jejunostomy tube is inserted. The recovery from sepsis is often immediate, dramatic, and reflected by a
marked improvement in the patient’s condition over a 24-hour
period. On recovery from the sepsis, the patient is discharged and
returns on a subsequent date for reconstruction with a substernal
colon interposition. Failure to apply this aggressive therapy can
result in a mortality rate in excess of 50% in patients in whom the
diagnosis has been delayed.
Nonoperative management of esophageal perforation has
been advocated in select situations. The choice of conservative therapy requires skillful judgment and necessitates careful radiographic examination of the esophagus. This course of
management usually follows an injury occurring during dilation of esophageal strictures or pneumatic dilations of achalasia.
Conservative management should not be used in patients who
have free perforations into the pleural space. Cameron proposed
three criteria for the nonoperative management of esophageal
perforation: (a) the esophagram must show the perforation to
be contained within the mediastinum and drain well back into
the esophagus (Fig. 25-78), (b) symptoms should be mild, and
(c) there should be minimal evidence of clinical sepsis. If these
In 1929, Mallory and Weiss described four patients with acute
upper GI bleeding who were found at autopsy to have mucosal
tears at the GEJ. This syndrome, characterized by acute upper
GI bleeding following vomiting, is considered to be the cause
of up to 15% of all severe upper GI bleeds. The mechanism is
similar to spontaneous esophageal perforation: an acute increase
in intra-abdominal pressure against a closed glottis in a patient
with a hiatal hernia.
Mallory-Weiss tears are characterized by arterial bleeding,
which may be massive. Vomiting is not an obligatory factor,
as there may be other causes of an acute increase in intraabdominal pressure, such as paroxysmal coughing, seizures, and
retching. The diagnosis requires a high index of suspicion, particularly in the patient who develops upper GI bleeding following prolonged vomiting or retching. Upper endoscopy confirms
the suspicion by identifying one or more longitudinal fissures in
the mucosa of the herniated stomach as the source of bleeding.
In the majority of patients, the bleeding will stop spontaneously with nonoperative management. In addition to blood
replacement, the stomach should be decompressed and antiemetics administered, as a distended stomach and continued
vomiting aggravate further bleeding. A Sengstaken-Blakemore
tube will not stop the bleeding, as the pressure in the balloon is
not sufficient to overcome arterial pressure. Endoscopic injection of epinephrine may be therapeutic if bleeding does not
stop spontaneously. Only occasionally will surgery be required
to stop blood loss. The procedure consists of laparotomy and
high gastrotomy with oversewing of the linear tear. Mortality is
uncommon, and recurrence is rare.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Figure 25-78. Barium esophagogram showing a stricture and a
contained perforation following dilation. The injury meets Cameron
criteria: It is contained within the mediastinum and drawn back into
the esophagus, the patient had mild symptoms, and there was no
evidence of clinical sepsis. Nonoperative management was successful.
1086
CAUSTIC INJURY
Accidental caustic lesions occur mainly in children, and, in general,
rather small quantities of caustics are taken. In adults or teenagers,
the swallowing of caustic liquids is usually deliberate, during a
suicide attempt, and greater quantities are swallowed. Alkalis are
more frequently swallowed accidentally than acids, because strong
acids cause an immediate burning pain in the mouth.
Pathology
PART II
SPECIFIC CONSIDERATIONS
The swallowing of caustic substances causes an acute and a
chronic injury. During the acute phase, care focuses on controlling the immediate tissue injury and the potential for perforation. During the chronic phase, the focus is on treatment
of strictures and disturbances in pharyngeal swallowing. In the
acute phase, the degree and extent of the lesion are dependent
on several factors: the nature of the caustic substance, its concentration, the quantity swallowed, and the time the substance
is in contact with the tissues.
Acids and alkalis affect tissue in different ways. Alkalis
dissolve tissue, and therefore penetrate more deeply, while acids
cause a coagulative necrosis that limits their penetration. Animal
experiments have shown that there is a correlation between the
depth of the lesion and the concentration of sodium hydroxide
solution. When a solution of 3.8% comes into contact with the
esophagus for 10 seconds, it causes necrosis of the mucosa and
the submucosa but spares the muscular layer. A concentration
of 22.5% penetrates the whole esophageal wall and into the
periesophageal tissues. Cleansing products can contain up to
90% sodium hydroxide. The strength of esophageal contractions
varies according to the level of the esophagus, being weakest
at the striated muscle–smooth muscle interface. Consequently,
clearance from this area may be somewhat slower, allowing
caustic substances to remain in contact with the mucosa longer.
This explains why the esophagus is preferentially and more
severely affected at this level than in the lower portions.
The lesions caused by lye injury occur in three phases. First
is the acute necrotic phase, lasting 1 to 4 days after injury. During
this period, coagulation of intracellular proteins results in cell
necrosis, and the living tissue surrounding the area of necrosis
develops an intense inflammatory reaction. Second is the ulceration and granulation phase, starting 3 to 5 days after injury.
During this period, the superficial necrotic tissue sloughs, leaving an ulcerated, acutely inflamed base, and granulation tissue
fills the defect left by the sloughed mucosa. This phase lasts 10
to 12 days, and it is during this period that the esophagus is the
weakest. Third is the phase of cicatrization and scarring, which
begins the third week following injury. During this period, the
previously formed connective tissue begins to contract, resulting in narrowing of the esophagus. Adhesions between granulating areas occur, resulting in pockets and bands. It is during this
period that efforts must be made to reduce stricture formation.
Clinical Manifestations
The clinical picture of an esophageal burn is determined by the
degree and extent of the lesion. In the initial phase, complaints
consist of pain in the mouth and substernal region, hypersalivation, pain on swallowing, and dysphagia. The presence of
fever is strongly correlated with the presence of an esophageal lesion. Bleeding can occur, and, frequently, the patient
vomits. These initial complaints disappear during the quiescent
period of ulceration and granulation. During the cicatrization
and scarring phase, the complaint of dysphagia reappears and
is due to fibrosis and retraction, resulting in narrowing of the
Table 25-16
Endoscopic grading of corrosive esophageal and
gastric burns
First degree: Mucosal hyperemia and edema
Second degree: Limited hemorrhage, exudate ulceration, and
pseudomembrane formation
Third degree: Sloughing of mucosa, deep ulcers, massive
hemorrhage, complete obstruction of lumen by edema,
charring, and perforation
esophagus. Of the patients who develop strictures, 60%
do so within 1 month, and 80% within 2 months. If dysphagia
does not develop within 8 months, it is unlikely that a stricture
will occur. Serious systemic reactions such as hypovolemia and
acidosis resulting in renal damage can occur in cases in which
the burns have been caused by strong acids. Respiratory complications such as laryngospasm, laryngoedema, and occasionally pulmonary edema can occur, especially when strong acids
are aspirated.
Inspection of the oral cavity and pharynx can indicate that
caustic substances were swallowed, but does not reveal that the
esophagus has been burned. Conversely, esophageal burns can
be present without apparent oral injuries. Because of this poor
correlation, early esophagoscopy is advocated to establish the
presence of an esophageal injury. To lessen the chance of perforation, the scope should not be introduced beyond the proximal
esophageal lesion. The degree of injury can be graded according
to the criteria listed in Table 25-16. Even if the esophagoscopy
is normal, strictures may appear later. Radiographic examination is not a reliable means to identify the presence of early
esophageal injury, but it is important in later follow-up to identify strictures. The most common locations of caustic injuries
are shown in Table 25-17.
Treatment
Treatment of a caustic lesion of the esophagus is directed toward
management of both the immediate and late consequences of the
injury. The immediate treatment consists of limiting the burn by
administering neutralizing agents. To be effective, this must be
done within the first hour. Lye or other alkali can be neutralized
with half-strength vinegar, lemon juice, or orange juice. Acid
can be neutralized with milk, egg white, or antacids. Sodium
bicarbonate is not used because it generates carbon dioxide,
Table 25-17
Location of caustic injury (n = 62)
Pharynx
10%
Esophagus
70%
Upper
15%
Middle
65%
Lower
2%
Whole
18%
Stomach
20%
Antral
91%
Whole
9%
Both stomach and esophagus
14%
1087
Ingestion of caustic agent
Esophagoscopy
(Within 12 hours)
1° burn
2° & 3° burn
Observation
24–48 hours
Exploratory
laparotomy
Viable
esophagus
and
stomach
Questionable
esophagus
and
stomach
Full thickness
necrosis
of esophagus
and stomach
- Intraluminal
esophageal
stent
- Second look
at 36 hours
- Esophagogastric
resection
- Posterior
gastric
wall biopsy
- Jejunostomy
- Cervical
esophagostomy
- Jejunostomy
- Resection of
adjacent
involved
organs
Figure 25-79. Algorithm summarizing the management of acute
caustic injury.
Figure 25-80. The use of an esophageal stent to prevent stricture.
The stent is constructed from a chest tube and placed in the esophagus at the time of an exploratory laparotomy. A Penrose drain is
placed over the distal end as a flap valve to prevent reflux. The stent
is supported at its upper end by attaching it to a suction catheter
that is secured to the nares. Continuous suction removes saliva and
mucus trapped in the pharynx and upper esophagus.
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
which might increase the danger of perforation. Emetics are
contraindicated because vomiting renews the contact of the
caustic substance with the esophagus and can contribute to
perforation if too forceful. Hypovolemia is corrected, and
broad-spectrum antibiotics are administered to lessen the
inflammatory reaction and prevent infectious complications.
If necessary, a feeding jejunostomy tube is inserted to provide
nutrition. Oral feeding can be started when the dysphagia of the
initial phase has regressed.
In the past, surgeons waited until the appearance of a stricture before starting treatment. Currently, dilations are started
the first day after the injury, with the aim of preserving the
esophageal lumen by removing the adhesions that occurred in
the injured segments. However, this approach is controversial
in that dilations can traumatize the esophagus, causing bleeding, and perforation, and there are data indicating that excessive dilations cause increased fibrosis secondary to the added
trauma. The use of steroids to limit fibrosis has been shown to
be effective in animals, but their effectiveness in human beings
has not been established.
Extensive necrosis of the esophagus frequently leads to
perforation, and it is best managed by resection. When there is
extensive gastric involvement, the esophagus is nearly always
necrotic or severely burned, and total gastrectomy and near-total
esophagectomy are necessary. The presence of air in the esophageal wall is a sign of muscle necrosis and impending perforation
and is a strong indication for esophagectomy.
Management of acute injury is summarized in the algorithm in Fig. 25-79. Some authors have advocated the use of
an intraluminal esophageal stent (Fig. 25-80) in patients who
are operated on and found to have no evidence of extensive
esophagogastric necrosis. In these patients, a biopsy of the
posterior gastric wall should be performed to exclude occult
injury. If, histologically, there is a question of viability, a
second-look operation should be done within 36 hours. If a
stent is inserted, it should be kept in position for 21 days, and
removed after a satisfactory barium esophagogram. Esophagoscopy should be done, and if strictures are present, dilations
initiated.
Once the acute phase has passed, attention is turned to
the prevention and management of strictures. Both antegrade
dilation with a Hurst or Maloney bougie and retrograde dilation with a Tucker bougie have been satisfactory. In a series of
1079 patients, early dilations started during the acute phase gave
excellent results in 78%, good results in 13%, and poor results
in 2%. During the treatment, 55 patients died. In contrast, of
333 patients whose strictures were dilated when they became
symptomatic, only 21% had excellent results, 46% good, and
6% poor, with three dying during the process. The length of time
the surgeon should persist with dilation before consideration of
esophageal resection is problematic. An adequate lumen should
be re-established within 6 months to 1 year, with progressively
longer intervals between dilations. If, during the course of treatment, an adequate lumen cannot be established or maintained
(i.e., smaller bougies must be used), operative intervention
should be considered. Surgical intervention is indicated when
there is (a) complete stenosis in which all attempts from above
and below have failed to establish a lumen, (b) marked irregularity and pocketing on barium swallow, (c) the development of
a severe periesophageal reaction or mediastinitis with dilatation,
(d) a fistula, (e) the inability to dilate or maintain the lumen
above a 40F bougie, or (f) a patient who is unwilling or unable
to undergo prolonged periods of dilation.
1088
PART II
SPECIFIC CONSIDERATIONS
The variety of abnormalities seen requires that creativity
be used when considering esophageal reconstruction. Skin tube
esophagoplasties are now used much less frequently than they
were in the past, and are mainly of historical interest. Currently,
the stomach, jejunum, and colon are the organs used to replace
the esophagus, through either the posterior mediastinum or the
retrosternal route. A retrosternal route is chosen when there has
been a previous esophagectomy or there is extensive fibrosis in
the posterior mediastinum. When all factors are considered, the
order of preference for an esophageal substitute is (a) colon, (b)
stomach, and (c) jejunum. Free jejunal grafts based on the superior thyroid artery have provided excellent results. Whatever
method is selected, it must be emphasized that these procedures
cannot be taken lightly; minor errors of judgment or technique
may lead to serious or even fatal complications.
Critical in the planning of the operation is the selection of
cervical esophagus, pyriform sinus, or posterior pharynx as the
site for proximal anastomosis. The site of the upper anastomosis
depends on the extent of the pharyngeal and cervical esophageal
damage encountered. When the cervical esophagus is destroyed
and a pyriform sinus remains open the anastomosis can be made
to the hypopharynx (Fig. 25-81). When the pyriform sinuses are
completely stenosed, a transglottic approach is used to perform
an anastomosis to the posterior oropharyngeal wall (Fig. 25-82).
This allows excision of supraglottic strictures and elevation and
anterior tilting of the larynx. In both of these situations, the
patient must relearn to swallow. Recovery is long and difficult
and may require several endoscopic dilations—and often reoperations. Sleeve resections of short strictures are not successful
because the extent of damage to the wall of the esophagus can
be greater than realized, and almost invariably the anastomosis
is carried out in a diseased area.
The management of a bypassed damaged esophagus after
injury is problematic. If the esophagus is left in place, ulceration from gastroesophageal reflux or the development of
carcinoma must be considered. The extensive dissection necessary to remove the esophagus, particularly in the presence of
marked periesophagitis, is associated with significant morbidity.
Leaving the esophagus in place preserves the function of the
Figure 25-81. Anastomosis of the bowel to a preserved pyriform
sinus. To identify the site, a finger is inserted into the free pyriform
sinus through a suprahyoid incision (dotted line). This requires
removing the lateral inferior portion of the thyroid cartilage as
shown in cross-section.
Figure 25-82. Anastomosis of the bowel to the posterior oropharynx. The anastomosis is done through an inverted trapezoid incision
above the thyroid cartilage (dotted line). A triangle-shaped piece of
the upper half of the cartilage is resected. Closure of the oropharynx
is done so that the larynx is pulled up (sagittal section).
vagus nerves, and, in turn, the function of the stomach. On the
other hand, leaving a damaged esophagus in place can result
in multiple blind sacs and subsequent development of mediastinal abscesses years later. Most experienced surgeons recommend that the esophagus be removed unless the operative risk
is unduly high.
ACQUIRED FISTULA
The esophagus lies in close contact with the membranous portion of the trachea and left bronchus, predisposing to the formation of fistula to these structures. Most acquired esophageal
fistulas are to the tracheobronchial tree and secondary to either
esophageal or pulmonary malignancy. Traumatic fistulas and
those associated with esophageal diverticula account for the
remainder. Fistulas associated with traction diverticula are usually due to mediastinal inflammatory disease, and traumatic
fistulas usually occur secondary to penetrating wounds, lye
ingestion, or iatrogenic injury.
These fistulas are characterized by paroxysmal coughing following the ingestion of liquids, and by recurrent or
chronic pulmonary infections. The onset of cough immediately
after swallowing suggests aspiration, whereas a brief delay
(30–60 seconds) suggests a fistula.
Spontaneous closure is rare, owing to the presence of
malignancy or a recurrent infectious process. Surgical treatment of benign fistulas consists of division of the fistulous tract,
resection of irreversibly damaged lung tissue, and closure of the
esophageal defect. To prevent recurrence, a pleural flap should
be interposed. Treatment of malignant fistulas is difficult, particularly in the presence of prior irradiation. Generally, only
palliative treatment is indicated. This can best be done by using
a specially designed esophageal endoprosthesis that bridges and
occludes the fistula, allowing the patient to eat. A salivary tube
is also a good option for proximal esophageal fistulas. This tube
has a proximal “lip” that rests on the cricopharyngeal muscle
and thereby directs the saliva into the tube and past the fistula. Rarely, esophageal diversion, coupled with placement of
a feeding jejunostomy, can be used as a last resort.
TECHNIQUES OF ESOPHAGEAL RECONSTRUCTION
Partial Esophageal Resection
Distal benign lesions, with preserved proximal esophageal function, are best treated with the interposition of a segment of proximal jejunum into the chest and primary anastomosis. A jejunal
interposition can reach to the inferior border of the pulmonary
hilum with ease, but the architecture of its blood supply rarely
allows the use of the jejunum proximal to this point. Because
the anastomosis is within the chest, a thoracotomy is necessary.
The jejunum is a dynamic graft and contributes to bolus
transport, whereas the stomach and colon function more as
a conduit. The stomach is a poor choice in this circumstance
because of the propensity for the reflux of gastric contents
into the proximal remaining esophagus following an intrathoracic esophagogastrostomy. It is now well recognized that this
occurs and can lead to incapacitating symptoms and esophageal
destruction in some patients. Short segments of colon, on the
other hand, lack significant motility and have a propensity for
the development of esophagitis proximal to the anastomosis.
Replacement of the cervical portion of the esophagus,
while preserving the distal portion, is occasionally indicated in
cervical esophageal or head and neck malignancy, and following the ingestion of lye. Free transfer of a portion of jejunum
to the neck has become a viable option and is successful in
the majority of cases. Revascularization is achieved via use
1089
Reconstruction After Total Esophagectomy
Neither the intrathoracic stomach nor the intrathoracic colon
functions as well as the native esophagus after an esophagogastrectomy. The choice between these organs will be influenced
by several factors, such as the adequacy of their blood supply
and the length of resected esophagus that they are capable of
bridging. If the stomach shows evidence of disease, or has been
contracted or reduced by previous gastric surgery, the length
available for esophageal replacement may not be adequate. The
presence of diverticular disease, unrecognized carcinoma, or
colitis prohibits the use of the colon. The blood supply of the
colon is more affected by vascular disease than the blood supply
of the stomach, which may prevent its use. Of the two, the colon
provides the longest graft. The stomach can usually reach to the
neck if the amount of lesser curvature resected does not interfere
with the blood supply to the fundus. Gastric interposition has the
advantage that only one anastomosis is required. On the other
hand, there is greater potential for aspiration of gastric juice or
stricture of the cervical anastomosis from chronic reflux when
stomach is used for replacement.
Following an esophagogastrectomy, patients may have
discomfort during or shortly after eating. The most common
symptom is a postprandial pressure sensation or a feeling of
being full, which probably results from the loss of the gastric
reservoir. This symptom is less common when the colon is used
as an esophageal substitute, probably because the distal third of
the stomach is retained in the abdomen and the interposed colon
provides an additional reservoir function.
King and Hölscher have reported a 40% and 50% incidence of dysphagia after reestablishing GI continuity with the
stomach following esophagogastrectomy. This incidence is
similar to Orringer’s results after using the stomach to replace
the esophagus in patients with benign disease. More than onehalf of the patients experienced dysphagia postoperatively;
Figure 25-83. A. The portion of the thoracic inlet to be resected to provide space for a free jejunal graft and access to the internal mammary
artery (shaded area). B. Cross-section showing the space available after resection of the sternoclavicular joint and one-half of the manubrium.
(Reproduced with permission from Shields TW: General Thoracic Surgery, 3rd ed. Philadelphia, PA: Lea & Febiger; 1989.)
CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Options for esophageal substitution include gastric advancement, colonic interposition, and either jejunal free transfer or
advancement into the chest. Rarely, combinations of these grafts
will be the only possible option. The indications for esophageal resection and substitution include malignant and end-stage
benign disease. The latter includes reflux- or drug-induced
stricture formation that cannot be dilated without damage to the
esophagus, a dilated and tortuous esophagus secondary to severe
motility disorders, lye-induced strictures, and multiple previous
antireflux procedures. The choice of esophageal substitution has
significant impact upon the technical difficulty of the procedure
and influences the long-term outcome.
of the internal mammary artery and the internal mammary or
innominate vein. Removal of the sternoclavicular joint aids
in performing the vascular and distal esophageal anastomosis
(Fig. 25-83).
1090
PART II
SPECIFIC CONSIDERATIONS
two-thirds of this group required postoperative dilation, and
one-fourth had persistent dysphagia and required home dilation.
In contrast, dysphagia is uncommon, and the need for dilation is
rare following a colonic interposition. Isolauri reported on 248
patients with colonic interpositions and noted a 24% incidence
of dysphagia 12 months after the operation. When it occurred,
the most common cause was recurrent mediastinal tumor. The
high incidence of dysphagia with the use of the stomach is probably related to the esophagogastric anastomosis in the neck and
the resulting difficulty of passing a swallowed bolus.
Another consequence of the transposition of the
stomach into the chest is the development of postoperative
duodenogastric reflux, probably due to pyloric denervation,
and adding a pyloroplasty may worsen this problem. Following
gastric advancement, the pylorus lies at the level of the
esophageal hiatus, and a distinct pressure differential develops
between the intrathoracic gastric and intra-abdominal duodenal
lumina. Unless the pyloric valve is extremely efficient, the
pressure differential will encourage reflux of duodenal contents
into the stomach. Duodenogastric reflux is less likely to occur
following colonic interposition because there is sufficient intraabdominal colon to be compressed by the abdominal pressure
and the pylorus and duodenum remain in their normal intraabdominal position.
Although there is general acceptance of the concept that
an esophagogastric anastomosis in the neck results in less postoperative esophagitis and stricture than one at a lower level,
reflux esophagitis following a cervical anastomosis does occur,
albeit at a lower rate than when the anastomosis is at a lower
level. Most patients undergo cervical esophagogastrostomy for
malignancy; thus, the long-term sequelae of an esophagogastric
anastomosis in the neck are not of concern. However, patients
who have had a cervical esophagogastrostomy for benign disease may develop problems associated with the anastomosis
in the fourth or fifth postoperative year that are severe enough
to require anastomotic revision. This is less likely in patients
who have had a colonic interposition for esophageal replacement. Consequently, in patients who have a benign process or
a potentially curable carcinoma of the esophagus or cardia, a
colonic interposition is used to obviate the late problems associated with a cervical esophagogastrostomy. Colonic interposition
for esophageal substitution is a more complex procedure than
gastric advancement, with the potential for greater perioperative
morbidity, particularly in inexperienced hands.
Composite Reconstruction
Occasionally, a combination of colon, jejunum, and stomach
is the only reconstructive option available. This situation may
arise when there has been previous gastric or colonic resection,
when dysphagia has recurred after a previous esophageal resection, or following postoperative complications such as ischemia
of an esophageal substitute. Although not ideal, combinations
of colon, jejunum, and stomach used to restore GI continuity
function surprisingly well and allow alimentary reconstruction
in an otherwise impossible situation.
Vagal Sparing Esophagectomy With Colon
Interposition
Traditional esophagectomy typically results in bilateral vagotomy and its attendant consequences. It is likely that symptoms
such as dumping, diarrhea, early satiety, and weight loss seen in
15% to 20% of patients postesophagectomy are at least in part,
if not completely, due to vagal interruption. The technique of
vagal sparing esophagectomy with colon interposition has been
described in an effort to avoid the morbidities associated with
standard esophagectomy.
Through an upper midline abdominal incision, the right
and left vagal nerves are identified, circled with a tape, and
retracted to the right. A limited, highly selective proximal gastric vagotomy is performed along the cephalad 4 cm of the lesser
curvature. The stomach is divided with an Endo-GIA stapler just
below the GEJ. The colon is prepared to provide an interposed
segment as previously described. A neck incision is made along
the anterior border of the left sternocleidomastoid muscle, and
the strap muscles are exposed. The omohyoid muscle is divided
at its pulley, and the sternohyoid and sternothyroid muscles are
divided at their manubrial insertion. The left carotid sheath is
retracted laterally and the thyroid and trachea medially. The left
inferior thyroid artery is ligated laterally as it passes under the
left common carotid artery. The left recurrent laryngeal nerve is
identified and protected. The esophagus is dissected circumferentially in an inferior direction, from the left neck to the apex
of the right chest, to avoid injury to the right recurrent laryngeal
nerve. The esophagus is divided at the level of the thoracic inlet,
leaving about 3 to 4 cm of cervical esophagus. The proximal
esophagus is retracted anteriorly and to the right with the use of
two sutures to keep saliva and oral contents from contaminating
the neck wound.
Returning to the abdomen, the proximal staple line of
the gastric division is opened, and the esophagus is flushed
with povidone-iodine solution. A vein stripper is passed up
the esophagus into the neck wound. The distal portion of the
esophagus in the neck is secured tightly around the stripping
cable with “endoloops” and an umbilical tape for a trailer. The
tip of the stripper is exchanged for a mushroom head, and the
stripper is pulled back into the abdomen, inverting the esophagus as it transverses the posterior mediastinum. This maneuver
strips the branches of the esophageal plexus off the longitudinal muscle of the esophagus, preserving the esophageal plexus
along with the proximal vagal nerves and the distal vagal
nerve trunks. In patients with end-stage achalasia, only the
mucosa is secured around the stripping cable, so that it alone
is stripped and the dilated muscular wall of the esophagus,
with its enriched blood supply, remains. The resulting mediastinal tunnel, or in the case of achalasia the muscular tube,
is dilated with a Foley catheter containing 90 mL of fluid in
the balloon. The previously prepared interposed portion of the
transverse colon is passed behind the stomach and up through
the mediastinal tunnel into the neck. An end-to-end anastomosis is performed to the cervical esophagus using a single layer
technique. The colon is pulled taut and secured to the left crus
with four or five interrupted sutures. Five centimeters below
the crura an opening is made in the mesentery adjacent to the
colon along its mesenteric border, through which an EndoGIA stapler is passed and the colon is divided. The proximal
end, which is the distal end of the interposed colon, is anastomosed high on the posterior fundic wall of the stomach, using
a triangular stapling anastomotic technique. This is done by
stapling longitudinally the stomach and colon together with a
75-mm Endo-GIA stapler, spreading the base of the incision
apart, and closing it with a T-55 stapler. Colonic continuity is
reestablished by bringing the proximal right colon to the distal staple line in the left colon and performing an end-to-end
anastomosis using a double-layer technique.
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CHAPTER 25 ESOPHAGUS AND DIAPHRAGMATIC HERNIA
Although conceptually appealing, preservation of vagal
nerve integrity or the gastric reservoir function after vagal sparing esophagectomy only recently has been validated. Banki and
associates compared patients undergoing vagal sparing esophagectomy to those with conventional esophagectomy and colon
or gastric interposition. This study showed that vagal sparing
esophagectomy preserved gastric secretion, gastric emptying,
meal capacity, and body mass index, compared to esophagogastrectomy with colon interposition or standard esophagectomy
with gastric pull-up. Vagal sparing esophagectomy patients
functioned, for the most part, similarly to normal subjects,
allowing them to eat a normal meal, free of dumping or diarrhea.
These results indicate that the vagal-sparing esophagectomy
procedure does indeed preserve the vagal nerves, and it may
be considered in the treatment of benign and early malignant
lesions requiring esophagectomy.