Congenital Esophageal Stenosis

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=Abstract=

Congenital Esophageal Stenosis

Seong-Cheol Lee, M.D., Won-Shik Han, M.D., Ki-Hong Kim, M.D., Sung-Eun Jung, M.D., Kwi-Won Park, M.D., and Woo-Ki Kim, M.D.

Department of Surgery, Seoul National University College of Medicine Seoul, Korea

Congenital esophageal stenosis (CES) is a narrowing of the esophageal lumen from birth. Three types of CES have been described; tracheobronchial remnants (TBR), membranous web (MW), and fibromuscular stenosis (FMS). We reviewed the clinical features and the surgical outcome of 14 patients, pathologically confirmed as CES. Nine patients had TBR, 3 FMS, and 2 MVV. The mean age at operation was 3.8 years. Five patients were boys and 9 girls. Four patients had other congenital anomalies. Segmental resection of the lesion and end to end anastomosis was utilized in all cases except one who underwent myotomy. The stenotic segment was located at the distal esophagus in all patients. There were 8 complications in 6 patients, but no mortality. The mean follow-up period was 68 months. There were no feeding problems but 3 patients had minor gastroesophageal reflux. Our result indicates that segmental resection and anastomosis is a satisfactory surgical procedure in the management of CES.

Index Words: Stenosis, Web, Esophaf{us, Conf{enital, Trachea-bronchial remnants

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Correspondence: Seong-Cheol Lee, M.D., Department oj Surgery, Seoul National University College of Medicine, 28 Younkundong, Chongnoku, Seoul 110-744, Korea Tel:

02-760-3636, FAX: 02-766-3975,. E-mail: leesc@p/aza.snu.ac.kr

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Table 1. Summary of 14 Patients with Congenital Esophageal Stenosis

Age at Diagnostic Length of Operative Associated

Case Pathology Sex Approach

Op Sx tool stenosis method anomaly

1 TBR F 6yr 9m

xis

A R&A

2 TBR F 7yr 2yr X 1 A R&A

3 TBR M 3yr 8m X 2 A R&A

4 TBR ^M 5yr 8m X 2 A R&A

5 TBR+ F 2yr 6m X T R&A

6 TBR F 6yr 1yr

xis

1 A R&A Down.MD.OC

7 TBR M 2yr 1d

xis

2 T R&A TEF.VSD.ASD

8 TBR F 4yr 1d

xis

2 A R&A Down

9 'FBR M 2yr 5m X 4 T R&A

10 FMS F 4yr 1m X 1 & 1 * T Myotomy

11 FMS+ F 6m 20d X 2 T R&A

12 FMS F 8yr 1yr

xis

1 A R&A Down

13 MW M 2yr 1yr S 2 A R & Ai

14 MW+ F 1m 2d X 1 TA R&A

Abbreviations: Op; operation. Sx; symptom onset. TBR ;tracheobronchial remnants. FMS;

fibromuscular stenosis. MW;membraneous web. X; esophagography. S; esophagoscopy. A;

transabdominal. T; transthoracic. TA; transabdominothoracic. R & A; resection and anastomosis. Down; Downs syndrome. MD; Meckel's diverticulum. OC; omental cyst. TEl"; tracheoesophageal fistula. VSD; ventricular septal defect. ASD; atrial septal defect

* This patient has two stenotic segments.

-I Pyloroplasty was simultaneously done due to vagus nerve injury.

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Table 2. Clinical Manifestations of 14 CES Symptom

Vomiting solid liquid

Recurrent respiratory infection Foreign body impaction

No_ of Cases (%)

14 (100 %) 4 ( 29 %) 5 ( 35 %) 1 ( 7 %)

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Congenital Esophageal Stenosis