Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula George W. Holcomb, III, M.D., MBA Children’s Mercy Hospital Kansas City, Missouri

EA/TEF History Before 1670Pre-recognition Era Pre-survival Era 1939Survival Era 1970Salvage Era

EA/TEF History 1941 Haight, Ann Arbor: March 15 Left extrapleural approach Single layer anastomosis Leak/stricture/single dilation

Esophageal Atresia

Rat Model of Esophageal Atresia/ Tracheoesophageal Fistula E14 TEF-APE14 TEF-Lateral

Fistula originates as a bud from the lung as a trifurcation Fistula E12 Trifurcation

Neonatal fistula tract expresses a respiratory lineage molecule E13 TEF whole mount for TTF1 TTF1 in e19 TEF J Pediatr Surg 37: , 2002

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

EA/TEF WaterstonSpitz 113 cases ( ) 357 Cases ( ) Grp A > 5-1/2 lb., healthy (95% survival)(99% survival) Grp B – 4-5 ½ lb., well, or wt, moderate pneumonia or congenital anomaly (68% survival)(95% survival) Grp C - < 4 lb., well, or wt, several pneumonia, or severe anomaly (6% survival)(71% survival)

EA/TEF New Risk Classification (1994) Spitz Grp I – Wt > 1500 gm, no major cardiac anomaly (97% survival) Grp II – Wt < 1500 gm or major cardiac anomaly (59% survival) Grp III – Wt < 1500 gm plus major cardiac anomaly (22% survival)

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

Thoracoscopic Repair EA/TEF

Thoracoscopic Repair of Esophageal Atresia and Tracheoesophageal Fistula: A Multi-Institutional Analysis George W. Holcomb III, Steven S. Rothenberg, Klaas MA Bax, Marcelo Martinez-Ferro, Craig T. Albanese, Daniel J. Ostlie, David C. van der Zee, C K Yeung 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 104 Patients Waterston A: > 5.5 lb with no significant associated problems Waterston B: lbs. or higher weight with moderate pneumonia or congenital anomaly Waterston C: weight < 4 lb or higher weight with severe pneumonia or congenital anomaly

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

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. AAP, 2006

Impact Of Suture Material CMH AAP, 2006

There is no difference in leak rates based on suture material or size Suture material or type has no effect on stricture formation Impact Of Suture Material CMH AAP, 2006

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

EA/TEF Evolution of technology? Shorter operative time? Reduced hospitalization? Reduced short term morbidity? Reduced long term morbidity? Why Thoracoscopy?

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 Concerns With Thoracoscopy Clip ligation/migration recurrent TEF Transpleural route Anesthesia issues

Thoracoscopic Repair EA/TEF Surgisis placed b/w esophagus & tracheal suture line to help prevent recurrent TEF J LAST 17: , 2007

How To Get Started Ideal Case Baby > 3 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