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
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American Surgical Association, 2005
Ann Surg 242:422-430, 2005
Thoracoscopic Repair EA/TEF
Institution Location Authors
Children’s Mercy Hospital Kansas City, MO Holcomb, Ostlie
Hospital for Infants and Denver, CO Rothenberg
Children at Presbyterian-St.
Luke’s Medical Center
Wilhelmina Children’s Utrecht, The Bax, van der Zee
Hospital Netherlands
J.P. Garrahan National Buenos Aires, Martinez-Ferro
Children’s Hospital Argentina
Lucille Packard Children’s Palo Alto, CA Albanese
Hospital
Chinese University of Hong Hong Kong, China Yeung
Kong
Thoracoscopic Repair EA/TEF
• Retrospective study
• Six international centers
• 2000 – 2004
• 104 Pts
Thoracoscopic Repair EA/TEF
(104 Patients)
• Tracheal intubation
• 30 - 45º prone position
• 3 ports (99 pts)
• 4 ports (5 pts)
• CO2 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 Ventilation 3.6 (± 5.8)
Mean Hospitalization (days) 18.1 (± 18.6)
Thoracoscopic Repair EA/TEF
Associated Anomalies
(104 Patients)
Cardiac Renal
ASD/VSD 15 Horseshoe kidney 3
Right aortic arch 6 Unilateral agenesis 2
Tetralogy of Fallot 3 Crossed fused ectopia 1
Dextrocardia 3 VUR > Grade 3 1
PDA (ligation) 2 Duplex kidney 1
DORV 1 Ectopic kidney 1
Tricuspid atresia 1
Gastrointestinal Other
High imperforate anus 7 Vertebral anomalies 6
Duodenal atresia 4 Radial aplasia 3
Low imperforate anus 3 Tethered cord 1
Cloaca 1 Hydromyelia 1
Choanal atresia 1
Syndromes
VACTERL (>2 anomalies) 10
CHARGE 3
Down 3
Thoracoscopic Repair EA/TEF
Results
(104 Patients)
• Fundoplication 26
(22 Nissen, 4 Thal)
• Aortopexy 7
( 6 thoracoscopic)
• Duodenal atresia 4
(4 laparoscopic)
• Imperforate anus 10
(7 high, 3 low)
• Cardiac operations 5
( other than VSD/ASD)
Thoracoscopic Repair EA/TEF
Complications
(104 Patients)
• Recurrent fistula 2
( 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 thoracoscopy 2
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
Current Engum, et al Spitz, Kelly Randolph, et al Manning, et al
(1971-93) (1980-84) (1982-88) (1977-85)
Number of 104 174 A 39 63
148
Patients
Mean length of 18.1 N.R. N.R. N.R. 24
hospitalization (6-120) (9-174)
(days)
Anastomotic leak 7.6% N.R. 21% 10.2% 17%
Anastomotic B C 17.7% 33.3% D
3.8% 32.7% 4.3%
stricture
Patients requiring 31.7% 32.7% N.R. 33.3% N.R.
at least 1 dilation
Anastomotic 1.9% 0.9% 2.7% 5.1% N.R.
revision
Fundoplication 24.0% 25.2% 18% 15.3% 16.9%
Aortopexy 6.7% N.R. 16% N.R. 4.7%
Mortality Related
EA/TEF 0.9% 4.5% 14.8% 0% 3.1%
Not Related 1.9% (overall) (overall) 7.6% 11.1%
2.8% 7.6% 14.2%
Recurrent fistula 1.9% 2.2% 12% 5.1% 6.4%
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
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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
Absorbable Non-Absorbable
(N=62) Mean (N=32) Mean +/- P-
+/- Standard Error Standard Error Value
Estimated Gestational Age at Birth 36.4 +/- 0.6 36.7 +/- 0.4 0.64
(Weeks)
Weight at Repair (kg) 2.50 +/- 0.13 2.63 +/- 0.09 0.87
Age at Repair (days) 5.3 +/- 2.0 3.2 +/- 0.6 0.21
Congenital anomaly 53% 48% 0.43
Gender (% Male) 59% 61% 0.51
Suture Size 5.66 +/- 0.09 5.20 +/- 0.10 0.003
Leak (%) 3.1% 4.8% 0.82
Sticture (%) 37.5% 45.2% 0.47
Number of dilations (per patient with 3.4 +/- 1.0 2.4 +/- 0.3 0.21
stricture)
Ann Pediatr Surg 3:78-82, 2007
Impact Of Suture Material
CMH
• There is no difference in leak rates based
on suture material or size
• Suture material or size has no effect on
stricture formation
Ann Pediatr Surg 3:78-82, 2007
EA/TEF
Operative Approach
Thoracoscopy Thoracotomy
• Transpleural • Extrapleural/Transpleural
• Longer operative time • Shorter operative time
• Better visualization • Adequate visualization
• Anesthesia important • Anesthesia standard
EA/TEF
Why Thoracoscopy?
89 pts/16 yrs
• shoulder elevation: 24%
• chest deformity: 20%
• abduction limited: 100%
• spine deformities: 18%
• breast deformities: 27%
(3/11)
Jaureguizar E, et al: Morbid musculoskeletal sequelae of thoracotomy for
tracheo-esophageal fistula. J Pediatr Surg 20: 511-514, 1985
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
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
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• 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
How To Get Started
Not The Ideal Case
• 2 - 2.5 kg
• Very high upper pouch
• Complex single ventricle
physiology
• Prostaglandin dependent
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
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
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