Oesophageal motility disorders
Key facts
 A spectrum of diseases involving failure of coordination
or contraction of the oesophagus and its related muscular
structures.
Pathological features
 In some cases degeneration of the inner and outer
myenteric plexuses can be demonstrated but often no
structural abnormality is seen.
Clinical features
 Achalasia
 Peak ages of incidence in young adulthood (idiopathic)
and old age (mostly degenerational).
 Slowly progressive dysphagia: initially worse for fluids
than solids.
 Frequent regurgitation of undigested food common late in
the disease.
 Secondary recurrent respiratory infections due to
aspiration.
 Diffuse oesophageal spasm
 Commonest in young adults;
 Characterized by acute pain along the length of the
oesophagus induced by ingestion, especially of hot or cold
substances (odynophagia).
Diagnosis and investigations
 Achalasia
 Video barium swallow. A characteristic failure of
relaxation of the lower oesophagus with a smooth
outline ˜rat's tail or bird beak.
 Oesophageal manometry. Hypertonic lower oesophageal
high pressure zone with failure of relaxation normally
induced by swallowing. In chronic cases the proximal
oesophagus may be adynamic.
 Oesophagoscopy. To exclude benign and malignant
strictures.
Diagnosis and investigations
 Diffuse oesophageal spasm
 Video barium swallow. ˜Corkscrew appearance of the
oesophagus caused by dyscoordinated diffuse
contractions.
 Oesophageal manometry. Diffuse hypertonicity and failure
of relaxation. Little or no evidence of coordinated
progressive peristalsis during epsiodes but normal
peristalsis when asymptomatic.
 Oesophagoscopy. Required to exclude underlying
associated malignancy.
Achalasia
Diffuse oesophageal spasm
Treatment
 Achalasia
 Endoscopically guided controlled balloon dilatation (fixed
pressure) successful in up to 80% of patients. Low
complications rate (perforation). May need multiple
procedures over time.
 Botulinum toxin injections: success in some patients
failing dilatation.
 Surgical myotomy (Heller's cardiomyotomy). Open or
thoracoscopically performed division of the lower
oesophageal muscle fibres. Highly successful in resistant
cases. Most applicable to young patients.
 Specific complications include reflux, obstruction of gastro-
oesophageal junction, oesophageal perforation.
Diffuse oesophageal spasm
 Oral calcium channel blockers, or relaxants, e.g.
benzodiazepines.
 Long-acting nitric oxide donors (smooth muscle relaxant).
 Widespread oesophageal pneumatic dilatations (often
repeated).
 Long surgical open myotomy rarely undertaken.
Key revision points anatomy and physiology of the
oesophagus
 Upper 2/3. Stratified squamous epithelial-lined (develops
squamous carcinoma), striated skeletal muscle, lymphatic
drainage to neck and mediastinal nodes, somatic
innervation of sensation (e.g. moderately accurate location
of level of pathology).
 Lower 1/3. Transition to columnar epithelium (develops
adenocarcinoma), transition to smooth muscle, lymphatic
drainage to gastric and para-aortic nodes, visceral
innervation (poor localization of pathology).
Key revision points anatomy and physiology of the
oesophagus
 Gastro-oesophageal junction is site of porto-systemic
anastomosis (between left gastric and (hemi)azygous
veins) may develop gastric or oesophageal varices.
 Upper oesophageal sphincter (UOS) = cricopharyngeus.
 Lower oesophageal sphincter (LOS) = functional zone of
high pressure above the gastro-oesophageal junction.
Relaxants include alcohol.
 Swallowing requires intact and coordinated innervation
from vagus (UOS, oesophagus, LOS) and intramural
myenteric plexus.
Pharyngeal pouch –
Zenker's diverticulum
Key facts
 An acquired pulsion diverticulum arising in the relatively
fibrous tissue between the inferior constrictor and
cricopharyngeus muscle: ˜Killian's dehiscence.
 Arises primarily as a result of failure of appropriate
coordinated relaxation of the cricopharyngeus causing
increased pressure on the tissues directly above during
swallowing.
 Typically occurs in the elderly.
Zenker's diverticulum
Key facts
 Associated with lower cranial nerve dysfunction (e.g.
motor neuron disease, previous CVA).
Pathological features
 Acquired diverticulum: fibrous tissue and serosa without
muscle fibres in most of the wall.
 Tends to lie to one side of the midline due to the cervical
spine directly behind.
Zenker's diverticulum
Clinical features
 Upper cervical dysphagia.
 Intermittent ˜lump appearing to the side of the neck on
swallowing.
 Regurgitation of food undigested.
Zenker's diverticulum
Zenker´s diverticulum
Diagnosis and investigations
 Diagnosis may be made on observed swallowing with a
transient neck swelling appearing.
 Video barium swallow will show filling of pouch.
 Gastroscopy should be avoided unless there is a question
of associated pathology since the pouch is easily missed
and easily damaged or perforated by inadvertent
intubation.
Zenker´s diverticulum
Treatment
 Endoscopic stapled pharyngoplasty: side to side stapling
of pouch to the upper oesophagus, which also divides the
cricopharyngeus muscle
Zenker´s diverticulum
Hiatus hernia
Key facts
 The presence of part or all of the stomach within the
thoracic cavity, usually by protrusion through the
oesophageal hiatus in the diaphragm
 Very common; majority are asymptomatic.
 May or may not be associated with gastro-oesophageal
reflux disease.
 Predisposing factors: obesity, previous surgery.
Hiatus hernia
Clinico-pathological features
 Sliding hernia
 Results from axial displacement of upper stomach through
the oesophageal hiatus, usually with stretching of the
phrenico-oesophageal membrane.
 By far the commonest form. May result in GORD.
Hiatus hernia
Clinico-pathological features
 Rolling (paraoesophageal) hernia
 Results from the displacement of part or all of the fundus
and body of the stomach through a defect in the phrenicooesophageal membrane such that it comes to lie alongside
the normal oesophagus.
 Much less common.
 Symptoms include hiccough, ˜pressure in the chest,
odynophagia.
 May result in volvulus or become incarcerated and cause
obstruction.
Diagnosis and investigations
 Video barium swallow usually identifies the type and
extent.
 CT scanning of the thorax is the investigation of choice in
acute presentations.
Treatment
 Medical (mainly for GORD symptoms)
 Reduce acid production. Stop smoking, lose weight,
reduce alcohol consumption.
 Counteract acid secretion: proton pump inhibitors,
symptomatic relief with antacids.
 Promote oesophageal emptying : promotilants, e.g.
metoclopramide.
 Surgical
 Rarely required. Indicated for:
 persistent symptoms despite maximal medical therapy;
 established complications of rolling hernia such as
volvulus or obstruction.
Elective procedure of choice is open or laparoscopic
reduction of the hernia and fixation (gastropexy), usually
with plication of the oesophageal opening (cural
plication), occasionally with a fundoplication (e.g.
Nissen's operation) if GORD symptoms predominate.
Acute presentations may require a partial gastrectomy.
Gastro-oesophageal reflux
disease GORD
Key facts
 Pathologically excessive entry of gastric contents into the
oesophagus.
 Reflux occurs in ˜normals up to 5% of the time.
 Commonest in middle-aged adults.
 Usually due to gastric acid but also due to bile reflux.
 Contributory factors include:
 reduced tone in the lower oesophageal sphincter: idiopathic,
alcohol, drugs, previous surgery, secondary to existing peptic
stricture.
 increased intragastric pressure: coughing, delayed gastric
emptying, large meal.
Gastro-oesophageal reflux
disease GORD
Pathological features
 Oesophagitis
 Results in inflammatory changes in the squamous lined
oesophagus.
 Varies in severity from minor mucosal erythema and
erosions to extensive circumferential ulceration and
stricturing. (graded I to IV).
Gastro-oesophageal reflux
disease GORD
Pathological features
 Stricture
 Chronic fibrosis and epithelial destruction may result in
stricturing.
 Eventually shortening and narrowing of the lower
oesophagus.
 May lead to fixation and susceptibility to further reflux.
Gastro-oesophageal reflux
disease GORD
Clinical features
 Dyspepsia may be the only feature; may radiate to back
and left neck.
 True reflux may occur with acid in the pharynx.
 Commonly worse at night, after large meals, and when
recumbent.
 Dysphagia may occur if there is associated ulceration or a
stricture.
Gastro-oesophageal reflux
disease GORD
Pathological features
 Oesophageal metaplasia ˜Barrett's oesophagus
 May develop as a result of gastro-oesophageal reflux;
possibly more commonly in biliary reflux.
 Normal squamous epithelium is replaced by columnar
epithelium.
 Dysplasia and premalignant change (metaplasia) may
occur in the columnar epithelium.
Gastro-oesophageal reflux
disease GORD
Diagnosis and investigations
 Under the age of 45
 Symptoms are relatively common and can be treated
empirically. Investigation is only required if symptoms
fail to respond to treatment.
 Over the age of 45
 Reflux can be confirmed by 24h continuous pH
monitoring. Peaks of pH change must correspond to
symptoms.
 Endoscopy should be performed in all new cases over the
age of 45 to exclude oesophageal malignancy.
Gastro-oesophageal reflux
disease GORD
Treatment
 Medical
 Reduce acid production: smoking, weight, alcohol
consumption.
 Counteract acid secretion: proton pump inhibitors (e.g.
omeprazole 20mg od), symptomatic relief with antacids
(e.g. Gaviscon 10mL PO od).
 oesophageal emptying: promotilants, e.g. metoclopramide
10mg tds PO.
Gastro-oesophageal reflux
disease GORD
 Surgical
 Procedure of choice is laparoscopic
fundoplication, ˜Nissen's operation (wrapping fundus of the
stomach around the intraabdominal oesophagus to
augment high pressure zone).
Gastro-oesophageal reflux
disease GORD
 Surgical
 Rarely required. Indicated for:
 persistent symptoms despite maximal medical therapy;
 large volume reflux with risk of aspiration pneumonia;
 complications of reflux including stricture and severe
ulceration.
 Uncertain role in the prevention of progressive dysplasia
in Barrett's oesophageal metaplasia in the absence of
symptoms.
Nissen's operation
Oesophageal tumours
Key facts and pathological features
 There are several types of oesophageal tumours.
 Adenocarcinoma
 Rapidly increasing incidence in Western world: 5:1 (M:F)
 Commonest in Japan, northern China, and South Africa,
 Associated with dietary nitrosamines, GORD, and
Barrett's metaplasia.
 Typically occurs in the lower half of the oesophagus.
Oesophageal tumours
Key facts and pathological features
 Squamous carcinoma
 Incidence slightly reducing in Western world: 3:1 (M:F)
 Associated with smoking, alcohol intake, diet poor in
fresh fruit and vegetables, chronic achalasia, chronic
caustic strictures.
 May occur anywhere in the oesophagus.
Oesophageal tumours
Key facts and pathological features
 Rhabdomyo(sarco)ma
 Malignant tumour of skeletal muscle wall of the
oesophagus. Very rare.
 Lipoma and gastrointestinal stromal tumours
 GIST are rare.
 GIST (gastrointestinal stromal tumours)
 10% of small bowel tumours.
 Arise from the mesenchymal tissues of the bowel wall and
mesentery (smooth muscle cells, fibroblasts, lipocytes).
 Previously called variously leiomyo(sarc)oma,
lipo(sarco)ma.
 Tumours of myenteric plexus tissues are a variant called
GANT (gastrointestinal autonomic nerve tumours).
Oesophageal tumours
Clinical features
 Dysphagia. Any new symptoms of dysphagia, especially
over the age of 45, should be assumed to be due to tumour
until proven otherwise.
 Haematemesis. Rarely the presenting symptom.
 Incidental/screening. Occasionally identified as a result of
follow-up/screening for Barrett's metaplasia, achalasia, or
reflux disease. Presence of high grade dysplasia in
Barrett's is associated with the presence of an occult
adenocarcinoma in 30%.
Oesophageal tumours
Clinical features
 Symptoms of disseminated disease. Cervical
lymphadenopathy, hepatomegaly due to metastases,
epigastric mass due to para-aortic lymphadenopathy.
 Symptoms of local invasion. Dysphonia in recurrent
laryngeal nerve palsy, cough and haemoptysis in tracheal
invasion, neck swelling in SVC obstruction, Horner's
syndrome in sympathetic chain invasion.
Oesophageal tumours
Diagnosis and investigations
 Diagnosis usually by flexible oesophagoscopy and biopsy.
 Barium swallow only indicated for failed intubation or
suspected post-cricoid carcinoma (often missed by
endoscopy).
Oesophageal tumours
Staging investigations
 Local staging: endoluminal ultrasound scan to assess
depth of invasion.
 Regional staging: CT scanning to evaluate local invasion,
locoregional lymphadenopathy, liver disease.
 Disseminated disease. PET scanning may be used to
exclude occult disseminated disease in patients otherwise
considered for potentially curative surgery.
Oesophageal tumours
Treatment
 Palliative
 Most patients present with incurable disease and require
palliation.
 Dysphagia can be treated by endoluminal self-expanding
metal stenting (SEMS), external beam radiotherapy.
Surgery is very rarely indicated for palliation.
 Metastases: systemic chemotherapy if symptomatic.
Oesophageal tumours
 Potentially curative
 Squamous carcinoma: radical external beam radiotherapy
followed by surgery (radical resection).
 Adenocarcinoma (large): neoadjuvant chemoradiotherapy
followed by surgery (radical resection).
 Adenocarcinoma (small) or high grade dysplasia in
Barrett's: surgical resection.
Oesophageal cancer
Oesophageal cancer
Stent
Self-expandable Esophageal
Stent