Thoracoscopic Repair of Esophageal Atresia with

Tracheoesophageal Fistula

George W. Holcomb, III, M.D., MBAChildren’s Mercy Hospital

Kansas City, Missouri

EA/TEFHistory

Before 1670 Pre-recognition Era

1670 - 1939 Pre-survival Era

1939 Survival Era

1970 Salvage Era

EA/TEFHistory

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-AP E14 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 37:1065-1067, 2002J Pediatr Surg 37:1065-1067, 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

Waterston Spitz113 cases (1951-59) 357 Cases (1980-1992)

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, orsevere anomaly(6% survival) (71% survival)

EA/TEFNew 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, 2005American Surgical Association, 2005

Ann Surg 242:422-430, 2005Ann Surg 242:422-430, 2005

Thoracoscopic Repair EA/TEFInstitution Location Authors

Children’s Mercy Hospital Kansas City, MO Holcomb, Ostlie

Hospital for Infants and Children at Presbyterian-St. Luke’s Medical Center

Denver, CO Rothenberg

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, CA Albanese

Chinese University of Hong Kong

Hong Kong, China Yeung

Thoracoscopic Repair EA/TEF

• Retrospective study

• Six international centers

• 2000 – 2004

• 104 Pts

Thoracoscopic Repair EA/TEF104 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/TEFResults

(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/TEFAssociated 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/TEFResults

(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/TEFComplications (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/TEFRight 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/TEFStaged Operation

• 1 pt: long gap – thoracoscopic ligation

3 mos later – repair via thoracotomy (2 myotomies needed)

Thoracoscopic Repair EA/TEFConversion 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/TEF104 Patients

Waterston A: > 5.5 lb with no significant associated problemsWaterston B: 4-5.5 lbs. or higher weight with moderate pneumonia or congenital anomalyWaterston C: weight < 4 lb or higher weight with severe pneumonia or congenital anomaly

Waterston A 62 Patients

Waterston B 30 Patients

Waterston C 12 Patients

Operation converted 2 2 1

Operation staged 1 - -

Esophageal anastomotic leak 2 3 3

Stricture (on initial esophagram) 3 1 -

Patients needing only 1 dilation 7 5 -

Patients needing 2 dilations 9 1 2

Patients needing 3 dilations - 3 1

Patients needing >3 dilations 3 2 -

Recurrent tracheoesophageal fistula 1 1 -

Fundoplication 19 6 1

Imperforate anus operations 4 4 2

Duodenal atresia repairs - 2 2

Aortopexy 6 1 -

Death 1 - 2

Thoracoscopic Repair EA/TEF

N.R.: Not reportedA: 87% are Gross Type CB: Stricture is defined as a significant narrowing on the initial esophagramC: Stricture in this paper is defined as requiring > 4 dilationsD: Stricture in this paper is defined as requiring > 2 dilations

Current Engum, et al (1971-93)

Spitz, Kelly (1980-84)

Randolph, et al (1982-88)

Manning, et al (1977-85)

Number of Patients

104 174 148A

39 63

Mean length of hospitalization (days)

18.1

(6-120)

N.R. N.R. N.R. 24 (9-174)

Anastomotic leak 7.6% N.R. 21% 10.2% 17%

Anastomotic stricture

3.8%B

32.7%C

17.7% 33.3% 4.3%D

Patients requiring at least 1 dilation

31.7% 32.7% N.R. 33.3% N.R.

Anastomotic revision

1.9% 0.9% 2.7% 5.1% N.R.

Fundoplication 24.0% 25.2% 18% 15.3% 16.9%

Aortopexy 6.7% N.R. 16% N.R. 4.7%

Mortality Related EA/TEF Not Related

0.9% 1.9% 2.8%

4.5% (overall)

14.8% (overall)

0% 7.6% 7.6%

3.1% 11.1% 14.2%

Recurrent fistula 1.9% 2.2% 12% 5.1% 6.4%

Preoperative Bronchoscopy

Preoperative Bronchoscopy

Patient Position

Port/Instrument Positions

Impact Of Suture MaterialCMH

• 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, 2006AAP, 2006

Absorbable (N=62) Mean

+/- Standard Error

Non-Absorbable (N=32) Mean +/-

Standard Error

P-

Value Estimated Gestational Age at Birth (Weeks)

36.4 +/- 0.6 36.7 +/- 0.4 0.64

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 stricture)

3.4 +/- 1.0 2.4 +/- 0.3 0.21

Impact Of Suture MaterialCMH

AAP, 2006AAP, 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 MaterialCMH

AAP, 2006AAP, 2006

EA/TEFOperative Approach

Thoracoscopy Thoracotomy

• Transpleural • Extrapleural

• Longer operative time • Shorter operative time

• Better visualization • Adequate visualization

• Anesthesia important • Anesthesia 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 Jaureguizar E, et al: Morbid musculoskeletal sequelae of thoracotomy for tracheo-esophageal fistula. J Pediatr Surg 20: 511-514, 1985for 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/TEFAdvantages of Thoracoscopy

• Avoidance of musculoskeletal sequelae

• Superior visualization of anatomy

• Easy to identify fistula for ligation

Thoracoscopic Repair EA/TEFConcerns 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 17:380-382, 2007J LAST 17:380-382, 2007

How To Get StartedIdeal 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/TEFSummary

• 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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