Transcript Case Conference - Dayton Children`s Hospital

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