先天性食管闭锁若干复杂问题的外科处理对策

Strategies of managing the complications of esophageal atresia

目的回顾性总结我院治疗先天性食管闭锁复杂情况，如：特殊类型、低体重儿、合并畸形、严重肺炎等的经验。方法自1998年7月～2006年7月共收治先天性食管闭锁41例，男33例，女8例，出生体重1100～3900g。低体重儿（〈2500g）6例；手术的37例患儿按Gross分类：Ⅰ型2例，Ⅲa23例，Ⅲb11例，复发性气管食管瘘1例；合并先天性心脏病6例，无肛3例，环状胰腺1例。结果除2例弃婴，1例先天性气管狭窄及1例家属放弃手术外，余均作外科食管纠治手术。术后28例（75．68％）有单侧或双侧严重肺炎；近期吻合口狭窄21例（56．76％）；近期吻合口漏9例（24．32％）。37例手术患儿全部痊愈出院。术后常规3个月、6个月随访，随访率72．97％（27／37），进奶好，无呕吐，每个月增加体重350～550g。结论本组手术分离后治愈率100％。对特殊类型的Ⅰ型作胃管Ⅰ期代食管手术是一种有效的手术方式，而复杂的复发性气管食管瘘采用内窥镜辅助下分离结扎术成功，十分满意。本组合并畸形以先心病与消化道畸形为主，急诊手术解决消化道梗阻是治疗的一个组成部分。低体重儿食管闭锁治疗采用多种形式外科营养支持，是手术成功的保证。

Objective The management of congenital esophageal atresia （EA） in babies with low-birth-weight, associated anomalies and severe pneumonia is challenging. This study analyzes the experience from a single center. Methods Between July 1998 and July 2006, 41 patients with EA were admitted to the department of pediatric surgery, Shanghai Xinhua Hospital, Shanghai Children＇s Medical Center, Medical School of Shanghai Jiaotong University. There were 33 males （80.49%） and 8 females （19.51%） with the birth weight ranging from 1100 g to 3900 g. Results The parents of 4 patients refused further treatment. Thirty-seven patients underwent surgical correction of the esophagus and survived. Of those： Ⅰ. There were 6 neonates with low birth weight （〈2500 g, 16.22%）, who received special care regime for the low-birth-weight newborns. TPN and PICC were routinely administered and enterostomy catheters were placed in 2 patients for early enteral nutrition. They thrived and were discharged with weight over 3000 g. Ⅱ. According to Gross＇s classification, there were 23 type Ⅲ-a, 11 type Ⅲ-b EA, 2 type Ⅰ, and 1 patient with recurrent tracheo-esophageal fistula in the operative 37 cases. Nine patients with type Ⅲ-b EA underwent modified Livaditis procedure. One-staged gastric tube interposition was performed in both cases of type Ⅰ EA. Ligation of the fistula in the recurrent TEF patient was achieved with the help of endoscope. Ⅲ. The associated anomalies included 6 congenital cardiac anomalies, 3 anorectal malformation, and one case of annular pancreas. Colostomies were performed to all those 3 patients with anorectal malformation, and Pena＇s anoplasties were performed 3 to 6 months later. Ⅳ. Twenty-eight patients （75.68%） developed pneumonia post-operatively. Ⅴ. Twenty-one patients developed stoma stenosis postoperatively, and requiring pneumatic dilatation （1 to 4 times）. Ⅵ. Nine patients had signs of stoma leakage. Conclusions Nutrition support through PICC, enterotomy catheter feeding and early enteral nutrition improved the survival of the low-birth-weight patients with EA.