1. 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.

2. 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

3. 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

4. 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

5. 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

6. Outline Esophageal Peroration –Etiology of esophageal perforation –Manifestations/Presentation –Diagnosis –Management Chest Pain –Non-cardiac causes –Pericarditis –Mediastinitis

7. 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

8. Etiology Most cases are traumatic or iatrogenic Spontaneous –Boerhaave syndrome –Triad of emesis, chest pain, and subcutaneous emphysema Case reports Review of experience

10. 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

11. 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

12. Risk With Endoscopy Flexible: 0.03% Rigid: 0.11% Dilation: 0.09 to 5% Sclerotherapy:1 to 5%

13. Etiology: Children Children’s Mercy Hospital, Kansas City –1995 to 2010, retrospective chart review Etiology (n=8) –Stricture dilation4 –Foreign Body2 –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

15. Etiology: Children James Whitcomb Riley Hospital, Indiana –1975 to 1995, retrospective review Etiology (n=25) –Iatrogenic17 Dilation (8), operation (5), NG tube (2), endoscopy(2) –Traumatic 3 Gun shot wound (2), blunt (1) –Foreign Body 3 –Unknown 2 No cases of spontaneous Arch Surgery;131,611-618,1996

16. Etiology: Children James Whitcomb Riley Hospital, Indiana –1975 to 1995, retrospective review Etiology (n=25) –Iatrogenic17 Dilation (8), operation (5), NG tube (2), endoscopy(2) –Traumatic 3 Gun shot wound (2), blunt (1) –Foreign Body 3 –Unknown 2 No cases of spontaneous Arch Surgery;131,611-618,1996)

17. 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

19. 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

20. Cervical Subcutaneous emphysema most common –Found in 90% Spread to mediastinum is slower Dysphagia Dyspnea Neck pain Dysphonia Bloody regurgitation

21. 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

22. 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

23. Clinical Manifestations: Neonates History of difficult ET or NG tube placement Hypersalivation Coughing/choking/cyanosis with feedings Pneumothorax Fever Bloody drainage from gastric tube

24. Differential Diagnosis Peptic ulcer with perforation Acute pancreatitis Spontaneous pneumothorax and mediastinum Pneumonia Aortic aneurysm dissection Acute myocardial infarction

25. 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%

26. 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

28. 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

29. 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

30. 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

31. 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

32. Management: Conservative Works Best If: Perforation is instrumental Perforation is cervical in location Perforation is detected early (<24hrs) Perforation is well contained

33. 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?

34. 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

35. Chest Pain Chief complaint: chest pain Peds cardiology referral 2 nd 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

36. 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

37. 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

38. Chest Pain: Mediastinitis Character –Severe and substernal Worse with inspiration and coughing Radiates to neck or interscapular area

39. 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