6

±o}5'1 JI} : All 9 l'1. All 1 J!:. 2003\1 Vol. 9. No. 1. June 2003

Clinical Experience of Esophageal Atresia

Seong-Chul Kim, M.D., Dae-Yeon Kim, M.D., Ellen Ai-Rhan Kim*, M.D.,

Ki-Soo Kim*, M.D., Soo-Young Pi*, M.D., and In-Koo Kim, M.D.

Division of Pediatric Surgery and Neonatology'"

University of Ulsan College of Medicine and Asan Medical Center

Seoul, Korea

This study reviews 14 years' experience treating esophageal atresia with special emphasis on the clinical profile and outcome. From May 1989 to February 2003, 65 cases of esophageal atresia (EA) were treated at Asan Medical Center. Boys outnumbered girls 2.4 to 1.

Prematutity and low birth weight were 27.7 % and 38.5 %. Esophageal atresia with distal tracheoesophageal fistula (TEF) was the most common type (87.7 %), followed by pure EA and H type fistula. Forty-six patients (70.8 %) had one or more associated anomalies, cardiac

malformations were the most common. Duodenal atresia was found in 7 cases. There were 6 patients (9.2 %) with VATER cluster. VACTERL cluster was present in 18 patients (27.7 %), one of who fulfilled the complete syndrome. Waterston group A, Band C made up 21.5 %, 40.0%

and 38.5% of the total group. Surgical treatment was attempted in 63 patients and deferred in 2 who had severe associated malformations. For EA with distal TEF, primary esophago esophagostomy was carried out in 51 cases, and division of TEF and gastrostomy in 4 cases

and no operation in 2 cases. For pure EA, colonic graft was done in 2 after gastrostomy and esophagostomy, and esophago esophagostomy was performed in 2 after gastrostomy. Two patients with pure EA are waiting for the second operation after gastrostomy. Division of

TEF was carried out in 2 cases with H type TEF. The overall survival rate was 76.9 %, and survival by Waterston classification was 100 % in group A, 80.8% in Band 60.0 % in C. Thorough workup for associated anomalies, interdepartmental approach and more careful

surgical decision and technique are required to improve the outcome of EA. (J Kor Assoc Pediatr Surg 9(1):6-11), 2003.

Index Words: Esophageal atresia

Correspondence : In-Koo Kim, M.D. Division of Pediatric

Surgery University of Ulsan College of Medicine and Asan

Medical Center. 388-1, Poongnap-Dong, Songpa-Ku, Seoul

138-736, Korea

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A-It:I~ 02

Table 1. Birth Weight

Birth weight(g)

1001-1500

1501-2000

2001-2500

2501-3000

3001-

Total

No. (%)

3 ( 4.6)

8 ( 12.3)

14 ( 21.5)

22 ( 33.9)

18 ( 27.7)

65 (100.0)

7

Table 2. Prevalence of Associated Anomalies

System AMC (n=65) KAPS (n=148) 1989 - 2002 1992 - 1994

Cardiac 39 (60.0) 46 (31.1)

Gastrointestinal 21 (32.8) 26 (17.6)

Anorectal malformation 8 (12.3) 19 (12.8)

Duodenal atresia 7 (10.8) 1 ( 0.7)

Genitourinary 15 (23.1) 10 ( 6.8)

Musculoskeletal 11 (16.9) 15 (10.1)

Others 19 (29.2) 19 (12.8)

AMC; Asan Medical Center, KAPS; Korean Association of Pediatric Surgeons, ( );%

Fig. 1. Radial limb deformity in a newborn with VACTE­RL cluster. This 2.1 kg baby had hemivertebrae, high type of Anorectal malformation, atrial and ventricular sep­tal defects, esophageal atresia with distal tracheoeso­phageal fistula, midline solitary kidney, radial limb de­formity, and lipomeningomyelcele.

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8

Table 3. Surgical Procedures and Complications

Type Procedures Leakage Mortality

EA + Esophagoesophagostomy (51) + (14) Sepsis (B2C2)* : 2m, 2m

distal TEF (57) Gastrostomy (2) DiGeorge syndrome (C2)* : 4y

- (37) Congestive heart failure (C2)* : 2w

Ventriculitis (C2)* : 1m

Intracranial hemorrhage (B2)* : 6m

Liver failure (C2)* : 10m

TEF division & gastrostomy (4) Prematurity (C2)* : 2d

~Esophagostomy (1) Respiratory failure (C2)* : 3d

Sepsis (C2)* : 2w

Endocarditis (B2)* : 8m

No operation (2) (C2,C2)*

Pure EA (6) Gastrostomy & esophagostomy (2) + (1)

,Colon graft (2) - (1) Meningitis (B1)* : 5d

Gastrostomy (4) + (2)

,Esphagoesophagostomy (2)

,Waiting (2)

H-type TEF (2) TEF division (2) Bowel adhesion (B2)* : 3w

EA;Esophageal atresia, TEF;Tracheoesophageal fistula, ( );Number of cases, ( )*;Waterston group

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9

Table 4. Waterston Classification

Group Case No.(%) % Survival

AMC 1989 - 2002 KAPS' 1992 - 1994 AMC 1989 - 2002 KAPS* 1992 - 1994

A 14 (21.5) 51 (40.5) 100.0 90.2

61 6 ( 9.2) 14 (11.1) 83.3 71.4

62 20 (30.8) 32 (25.4) 80.0 75.0

C1 1 ( 15) 4 ( 3.2) 100.0 75.0

C2 24 (36.9) 25 (19.8) 58.3 28.0

Total 65 (100) 126 (100) 76.9 71.4

AMC;Asan Medical Center, KAPS;Korean Association of Pediatric Surgeons

* Only operative cases were included.

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