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

Esophageal Atresia

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

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

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

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

American Surgical Association, 2005 Ann Surg 242: , 2005

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

Thoracoscopic Repair EA/TEF Retrospective study Six international centers 2000 – Pts

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

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

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

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)

Thoracoscopic Repair EA/TEF Associated Anomalies (104 Patients)

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)

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

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

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

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

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

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

Patient Position

Port/Instrument Positions

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

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

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

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

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: , 1985

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

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

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

Second TE Fistula

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

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

How To Get Started Not The Ideal Case kg Very high upper pouch Complex single ventricle physiology Prostaglandin dependent

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

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

QUESTIONS