1. Current Management of Esophageal Atresia and Tracheoesophageal Fistula George W. Holcomb, III, M.D., MBA Surgeon-in-Chief Children’s Mercy Hospital Kansas City, MO

2. Esophageal Atresia

3. EA/TEF 1 per 2500 – 3500 live births Sporadic, non-syndromal Dysmotile distal esophagus Deficiency of tracheal cartilage 50% have 1 or more associated anomalies: cardiac, anorectal, GU, vertebral/skeletal, others

4. Postoperative Problems GER:40% (20% require fundoplication) Mgmt:treat aggressively postoperatively ? partial vs complete fundoplication Tracheomalacia: 10% symptomatic (<5% require aortopexy)

5. EA/TEF Preoperative Evaluation Echocardiogram – assess cardiac anomalies Renal US – assess kidneys CXR/spine films – assess vertebral anomalies PE – assess limb, anorectal anomalies US great vessels – assess location of aortic arch

6. Thoracoscopic Repair EA/TEF Please use this link if you experience problems viewing the video above.this link

7. American Surgical Association, 2005 Ann Surg 242:422-430, 2005

8. Thoracoscopic Repair EA/TEF InstitutionLocationAuthors Children’s Mercy HospitalKansas City, MOHolcomb, Ostlie Hospital for Infants and Children at Presbyterian-St. Luke’s Medical Center Denver, CORothenberg Wilhelmina Children’s Hospital Utrecht, The Netherlands Bax, van der Zee J.P. Garrahan National Children’s Hospital Buenos Aires, Argentina Martinez-Ferro Lucille Packard Children’s Hospital Palo Alto, CAAlbanese Chinese University of Hong Kong Hong Kong, ChinaYeung

9. Thoracoscopic Repair EA/TEF Retrospective study Six international centers 2000 – 2004 104 Pts

10. Thoracoscopic Repair EA/TEF (104 Patients) Tracheal intubation 30 - 45º prone position 3 ports (99 pts) 4 ports (5 pts) CO 2 insufflation used

11. Thoracoscopic Repair EA/TEF (104 Patients) Fistula Ligation 37 pts: suture ligation 67 pts: clip ligation

12. Thoracoscopic Repair EA/TEF (104 Patients) Anastomosis – Suture 46 pts: Vicryl 40 pts: PDS 11 pts: Silk 7 pts: “Other” Anastomosis – Technique 42 pts: extracorporeal 62 pts: intracorporeal

13. Thoracoscopic Repair EA/TEF Results (104 Patients) Mean Age (days)1.2 (± 1.1) Mean Wt (kg)2.6 (± 0.5) Mean Operative Time (min)129.9 (± 55.5) Mean Days Ventilation3.6 (± 5.8) Mean Hospitalization (days) 18.1 (± 18.6)

14. Thoracoscopic Repair EA/TEF Associated Anomalies (104 Patients)

15. Thoracoscopic Repair EA/TEF Results (104 Patients) Fundoplication26 (22 Nissen, 4 Thal) Aortopexy7 ( 6 thoracoscopic) Duodenal atresia4 (4 laparoscopic) Imperforate anus10 (7 high, 3 low) Cardiac operations5 ( other than VSD/ASD)

16. Thoracoscopic Repair EA/TEF Complications (104 Patients) Recurrent fistula2 ( 3 mos, 8 mos) Mortality 3 7 mo old - NEC 10 day old – CHD 21 day old with esophageal disruption at intubation

17. Thoracoscopic Repair EA/TEF Right Aortic Arch 6 Pts Conversion from R thoracoscopy 3 to L thoracoscopy Conversion from R thoracoscopy 1 to L open Left thoracoscopy2

18. Thoracoscopic Repair EA/TEF Staged Operation 1 pt: long gap – thoracoscopic ligation 3 mos later – repair via thoracotomy (2 myotomies needed)

19. Thoracoscopic Repair EA/TEF Conversion to Open 5 Pts 1 Pt:R aortic arch (despite negative ECHO) 3 Pts:Intraoperative desaturation, relatively long gap 1 Pt:1.2 kg baby – only 1 port placed – too small

20. Thoracoscopic Repair EA/TEF N.R.:Not reported A:87% are Gross Type C B:Stricture is defined as a significant narrowing on the initial esophagram C:Stricture in this paper is defined as requiring > 4 dilations D:Stricture in this paper is defined as requiring > 2 dilations

21. Preoperative Bronchoscopy Please use this link if you experience problems viewing the video above.this link

22. Patient Position

23. Port/Instrument Positions

24. Impact Of Suture Material CMH 99 patients Absorbable suture used in 32 patients Permanent suture in 62 patients Combination used in 5 patients No difference in weight at operation, EGA, age at repair, or mean number of associated anomalies between the groups. Ann Pediatr Surg 3:78-82, 2007

25. Impact Of Suture Material CMH Ann Pediatr Surg 3:78-82, 2007

26. There is no difference in leak rates based on suture material or size Suture material or size has no effect on stricture formation Impact Of Suture Material CMH Ann Pediatr Surg 3:78-82, 2007

27. EA/TEF Operative Approach ThoracoscopyThoracotomy TranspleuralExtrapleural/Transpleural Longer operative timeShorter operative time Better visualizationAdequate visualization Anesthesia importantAnesthesia standard

28. EA/TEF 89 pts/16 yrs shoulder elevation: 24% chest deformity: 20% abduction limited:100% spine deformities: 18% breast deformities: 27% (3/11) Why Thoracoscopy? Jaureguizar E, et al: Morbid musculoskeletal sequelae of thoracotomy for tracheo-esophageal fistula. J Pediatr Surg 20: 511-514, 1985

29. Musculoskeletal Morbidity Following Thoracotomy for EA/TEF 1.Durning RP, et al: J Bone Joint Surg AM 62:1156, 1980 2.Gilsanz V, et al: Am J Roentgenol 141:457, 1983 3.Chetcuti P, et al: J Pediatr Surg 24: 244, 1989 4.Goodman P, et al: J Comput Assist Tomogr 17:63, 1993 5.Frola C, et al: Am J Roentgenol 164: 599, 1995 6.Bianchi A, et al: J Pediatr Surg 33: 1798, 1998

30. Thoracoscopic Repair EA/TEF Advantages of Thoracoscopy Avoidance of musculoskeletal sequelae Superior visualization of anatomy Easy to identify fistula for ligation

31. Thoracoscopic Repair EA/TEF Fistula Ligation Metal clip Weck clip Tie (x2 ?) Suture ligature (x2 ?) Suture closure – tracheal side

32. Second TE Fistula

33. Tips/Tricks Oscillating ventilator U-clips anterior anastomosis Please use this link if you experience problems viewing the video above.this link

34. 2007 – 2010 17 neonates - 12 EA/TEF - 5 CDH - Mean age - 4 days - Mean wt - 2.9 ±1.0 kg - Median vent changes – 3/pt J Laparoendosc Surg 21:877-879, 2011

35. How To Get Started Not The Ideal Case 2 - 2.5 kg Very high upper pouch Complex single ventricle physiology Prostaglandin dependent

36. How To Get Started Ideal Case Baby – 2.5-3 kg; no other anomalies Esophageal segments close together (CXR, Bronchoscopy) Start thoracoscopically – Go as far as comfortable Try it again

37. Thoracoscopic Repair EA/TEF Summary Thoracoscopic repair of EA/TEF can be performed safely and effectively The thoracoscopic approach may be advantageous by reducing the musculoskeletal sequelae seen following thoracotomy

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