Grand Rounds
May 2011
James Rick, MD
Pediatric Gastroenterology
The opinions expressed during this
presentation are my own and do
not reflect those of the USG, DOD
or USAF. Also, I have no financial
relationships to disclose and do not
intend to discuss non FDA
approved uses of drugs or medical
equipment.
Now the rest of the story
• Admitted to CCHMC 5/25/09 to 5/29/09
– CT with contrast: pneumomediastinum
– ENT, GI, PULM, RHEUM
– Stopped antibiotics/discharged home
• Outpatient procedure 6/3/09
– Nl laryngoscopy and bronchoscopy
– EGD: Esophageal and gastric ulcers with air
bubbling out esophageal lesion with
purulent discharge
– BAL showed acute inflammatory exudate
Now the rest of the story #2
• Further Diagnosis
– Esophagram:
• esophageal ulcer right anterolateral aspect 3 cm
above LES
• Communicated with right infrahilar retrocardiac
region
• Treatment
– NJ feeds, IV PPI, NPO for 14 days, unasyn
14 days
– Repeat esophagram 6/25/09, no leak
– Advanced to regular diet
Follow Up
• Chest CT 6/26/09
• EGD/Bronch 7/28/09
– Healing of previous esophageal ulcer
– Gastric ulcer, mild gastritis
– Negative bronchoalveolar lavage
• EGD 11/24/09
– Gastric ulcer, healed esophageal ulcer
– Mild distal esophagitis and gastritis
• EGD 6/28/10
Objectives
• Understand the high morbidity and
mortality for esophageal perforation
in children and the need for a high
index of suspicion to ensure a timely
diagnosis
• Illustrate the diagnostic approach for
the evaluation of esophageal
perforation in children
Outline
• Esophageal Peroration
– Etiology of esophageal perforation
– Manifestations/Presentation
– Diagnosis
– Management
• Chest Pain
– Non-cardiac causes
– Pericarditis
– Mediastinitis
Quick Look at the Objectives
• Morbidity and Mortality
– Morbidity: roughly 1/3
• Prolonged mechanical ventilation or persistent
leak
– Mortality:
• Wide range: 4 to 44%
• Usually from sepsis or multi-organ failure
• Increased with delay in dx and tx
• Diagnostic Approach
– Plain radiographs: may be normal
– Contrast esophagography: study of choice
with controversary over ideal agent
Etiology
• Most cases are traumatic or
iatrogenic
• Spontaneous
– Boerhaave syndrome
– Triad of emesis, chest pain, and
subcutaneous emphysema
• Case reports
• Review of experience
Historical Background
• 1723: Herman Boerhaave described
barogenic esophageal rupture
• 1947: first surgical repair of esophageal
perforation
• 1952: first esophagectomy after perforation
and infant with spontaneous perforation
related to esophageal web
• 1961: first report in newborn after respiratory
suctioning
• 1960’s to 1970’s: improved M&M
• 1980’s +: change in etiology
Etiology: Adult
• Adult review published in 2004
(N=559)
• Instrumentation (59%)
• Spontaneous (15%)
• Foreign Body (12%)
• Trauma (9%)
• Operative Injury (2%)
• Other (2%)
Ann Thorac Surg
2004;77:1475-1483
Risk With Endoscopy
•
•
•
•
Flexible: 0.03%
Rigid: 0.11%
Dilation: 0.09 to 5%
Sclerotherapy:1 to 5%
Etiology: Children
• Children’s Mercy Hospital, Kansas City
– 1995 to 2010, retrospective chart review
• Etiology (n=8)
– Stricture dilation
4
– Foreign Body
2
– NG tube and stricture resection 1 each
• 75% of esophageal perforations are
iatrogenic
• All were managed conservatively
Journal of Surgical Research:
164, 13-7, 2010
Etiology: Children
• James Whitcomb Riley Hospital, Indiana
– 1975 to 1995, retrospective review
• Etiology (n=25)
– Iatrogenic
17
• Dilation (8), operation (5), NG tube (2), endoscopy(2)
– Traumatic
3
• Gun shot wound (2), blunt (1)
– Foreign Body
– Unknown
• No cases of spontaneous
Arch Surgery;131,611-618,1996
3
2
Etiology: Children
• James Whitcomb Riley Hospital, Indiana
– 1975 to 1995, retrospective review
• Etiology (n=25)
– Iatrogenic
17
• Dilation (8), operation (5), NG tube (2), endoscopy(2)
– Traumatic
3
• Gun shot wound (2), blunt (1)
– Foreign Body
– Unknown
3
2
• No cases of spontaneous
Arch Surgery;131,611-618,1996)
Etiology: Case Reports
• Infectious
•
•
•
•
•
•
•
– HSV esophagitis
– Candida esophagitis
– Tuberculosis
Eosinophillic esophagitis
Pill induced esophagitis
Reflux esophagitis/ulceration
Barrett esophagus/ulcers
Zollinger Ellison Syndrome
Behcet’s disease
Interesting lack of crohn dz and NSAIDs
Clinical Presentation
• Spontaneous esophageal perforation
– Middle age man
– Dietary over indulgence and alcohol
consumption
– Mackler’s triad: Chest pain, subcutaneous
emphysema, and recent vomiting/retching
• Will depend on:
– Location of perforation
– Etiology of the perforation
Cervical
• Subcutaneous emphysema most
common
– Found in 90%
•
•
•
•
•
•
Spread to mediastinum is slower
Dysphagia
Dyspnea
Neck pain
Dysphonia
Bloody regurgitation
Thoracic
•
•
•
•
•
Rapidly contaminate the mediastinum
May spread to pleural cavity as well, L>R
Mediastinal and subcutaneous emphysema
Involvement of pericardium has been reported
Inflammatory response
– Chest pain: Retrosternal, can spread to arms, back,
and shoulders
– Tachycardia, tachypnea, grunting, dyspnea, resp
distress
– Hypovolumia
– Leukocytosis
• Systemic sepsis and shock with in hours
Abdominal
• Uncontained and results in
contamination of the peritoneal cavity
• Back pain and difficulty lying supine
• Epigastric abdominal pain and
dysphagia
– Maybe referred to the shoulder
• Fever, tachycardia, tachypnea,
• Rapid deterioration to sepsis and
shock
Clinical Manifestations: Neonates
• History of difficult ET or NG tube
placement
• Hypersalivation
• Coughing/choking/cyanosis with
feedings
• Pneumothorax
• Fever
• Bloody drainage from gastric tube
Differential Diagnosis
• Peptic ulcer with perforation
• Acute pancreatitis
• Spontaneous pneumothorax and
mediastinum
• Pneumonia
• Aortic aneurysm dissection
• Acute myocardial infarction
Diagnosis
• History of esophageal manipulation or
trauma
• Otherwise requires high index of
suspicion
• Initial tests: AP and lateral CXR
– May show
• Pleural effusion, pneumothorax, subcutaneous
emphysema, pneumomediastinum,
pneumopericardium, subdiaphargmatic air
– May also be normal in 12-33%
Contrast Radiography
• Establishes diagnosis/localizes injury
• Water soluble contrast
– Most recommend this first
• Limitations
• Thin barium (greater density)
– Improves sensitivity to 60% in cervical and 90% in
thoracic perforations
– May cause inflammatory reaction in pleural space
• Three injury patterns
– Retropharyngeal collection
– Contrast tracking parallel and posterior to the
esophagus
– Free perforation into pleural space
Could this be congenital H type
TEF?
• No
– Bronch showed normal trachea
– Symptom free till recently
– Esophageal and gastric ulcers
• Yes: never say never
– Case report dx H type in 10 yr girl dx by
esophagoscopy and bronchoscopy
– Their literature review noted 3 pts over
age 10 yrs dx with H type fistula
• CLIN PEDIATR February 1996 vol. 35 no. 2 103-104
Diagnosis
Computed Tomography
• Some institutions use CT
with oral contrast as
primary modality after
plain films
• Trend in US is for
contrast esophagram
• Some advocate use of
both in all patients with
suspected esophageal
perforation
Endoscopy
• Various
recommendations
• Pros
– May better localize size
and location
– May aid in diagnosis of
cause if unknown
• Cons
– May worsen injury
– Inferior to contrast study
Management
• Historically based on adult reports
– Adult surgeons favored direct surgical repair
• Kids esophagi are not little adult esophagi
– Adult perfs have more underlying pathology
– Kids have increased propensity to heal and
often difficult to localize leak
• Case series in 1988 (N=12)
– All patients treated conservatively
– All but 1 healed without need of surgery
J of Thoracic Cardiovasc Surgery 1998:95:692
Management: Conservative
• Basic Tenant: promote spontaneous healing
– Minimize proximal flow, prevent contamination, maintain
downstream flow, support nutrition
• Broad spectrum antibiotics for 7-14 days
– Cultures usually grow polymicrobial organisms
• NPO and gastric drainage
• Nutrition
– TPN if unable to secure enteral access
– Enteral
• Tube placement: endoscopy, fluoroscopy, place tube in perforation
• Resuming oral feeds
– When repeat esophagram shows no leak
– Average time to esophagram 7 day, restarting feeds 11
days
• Thoracostomy tube
Management: Conservative
Works Best If:
•
•
•
•
Perforation is instrumental
Perforation is cervical in location
Perforation is detected early (<24hrs)
Perforation is well contained
Back to the Case
• Esophageal perforation, etiology
unknown led to mediastinitis
– Suspect reflux related
– Possibly iatrogenic or spontaneous
• Previous pericarditis
– Could this have been related to
esophageal perforation and
esophagopericardial fistula?
Esophagopericardial fistula
• Case review (n=49) AJR 141:171-173;July 1983
• Etiology
– #1 etiology was esophagitis/esophageal
ulcer (75%). Many of these patients had
previous reflux or hiatal hernia surgery
• Radiographs
– Pneumopericardium in 50%
– Pneumomediastinum in 17%
• Recent case report in a 1yr old
Chest Pain
• Chief complaint: chest pain
• Peds cardiology referral 2nd to murmur
• Musculoskeletal most common
– 15-30% prevalence
• Non-cardiac 98% of the time
• More likely cardiac if
– Increases with exertion, s/s myocardial
ischemia and abnormal cardiac exam
– Pediatr. Rev. 2010;31;e1-e9
Chest Pain: Gastrointestinal
• Gastrointestinal 8% of the time
– GERD and PUD
• epigastric, burning, regurg, related to
eating, respond to acid blockade
– Esophageal spasm or inflammation
– Atypical
• cholecystitis
• esophageal foreign body, strictures,
ingestions
Chest Pain: Pericarditis
• With or without effusion
• Usually infectious in nature
• Character
– Sharp, retrosternal, radiates to left shoulder
• Worse with supine position and
inspiration
• Improves with bending forward
Chest Pain: Mediastinitis
• Character
– Severe and substernal
• Worse with inspiration and coughing
• Radiates to neck or interscapular
area
Summary
• Esophageal perforation is rare in
children and most commonly iatrogenic
• Mediastinits/pneumomediastinum
• Diagnosis is based on contrast
esophagram with +/- CT and
endoscopy
• Early diagnosis and treatment helps
limit morbidity and mortality
• Trend toward conservative treatment