小儿自发性胆道穿孔25例治疗经验

Therapeutic experiences of 25 patients with pediatric spontaneous biliary duct perforation

目的总结小儿自发性胆道穿孔治疗经验。方法回顾性分析首都医科大学附属北京儿童医院2008年1月1日至2014年12月31日收治的25例自发性胆道穿孔患儿的临床资料，总结治疗经验。结果患儿男性7例，女性18例，男女比例1：2．57，平均年龄2．41岁，最大10岁，最小11个月。术前彩超诊断21例，阳性率84．0％，准确率100％。25例患儿中2例保守治疗，好转后出院；23例急诊手术治疗。其中9例患儿采用胆囊造瘘＋肝门部置管引流术（胆囊造瘘组）；14例患儿采用胆总管置管＋肝门部置管引流术（胆总管置管组）。胆囊造瘘组患者平均住院时间25．2d，发生术后并发症3例（33．3％）；胆总管置管组患者平均住院时间16．1d，发生术后并发症2例（14．2％）。24例患儿术中或术后明确诊断为“先天性胆总管囊肿”或“胰胆管合流异常”。23例患儿在病情稳定后择期接受胆道重建术，无严重术后并发症。结论（1）儿童自发性胆道穿孔与先天性胆总管囊肿关系密切，胰胆管合流异常是其发病基础；（2）胆道弹力纤维发育不完善及胆道特有的血供特点是自发性胆道穿孔的重要发病因素；（3）应重视彩超检查诊断该病的重要作用；（4）对于病灶没有局限、有明显腹膜刺激征的患儿应积极手术治疗，胆总管置管＋肝门部置管引流是其理想术式；（5）所有术中或术后诊断先天性胆总管囊肿或胰胆管合流异常的患儿均应择期行胆总管（囊肿）切除＋肝管空肠Roux-Y吻合术。

Objective To summarize our therapeutic experiences on patients with pediatric sponta- neous biliary duct perforation. Methods We retrospectively analyzed the clinical data of patients diagnosed as spontaneous biliary duct perforation who were admitted into the Department of Pediatric General Surgery, the Beijing Children Hospital from January 2008 to December 2014, and summarized the therapeutic experi- ences. Results There were 7 boys and 18 girls, with a average age of 2.4 years （ range 11 months to 10 years）. Twenty-one patients （84.0%） were diagnosed by ultrasonography. Two patients were treated with conservative therapy and were discharged home. The remaining 23 patients were treated with emergent sur- gery. Of these patients, 9 were treated with cholecystostomy and abdominal drainage （ the cholecystostomy group）, and the remaining 14 were treated with choledochal drainage and abdominal drainage （the chole- dochal drainage group）. The mean hospitalization stay for the cholecystostomy group was 25.2 days, and 3 patients developed complications （33.3%）. The mean hospitalization stay for thecholedochal drainage group was 16.1 day, and 2 patients developed complications （ 14.2% ）. Twenty-four patients were diagnosed to suffer from congenital choledochal cysts or pancreaticobiliary maljunction by imaging studies during or after surgery. Elective choledochal cystectomy with hepaticojejunostomy were performed on 23 stable patients who developed no severe complications. Conclusions Pediatric spontaneous bile duct perforation is closely re- lated with congenital eholedochal cysts, and the pathological basis in diagnosis is pancreaticobiliary maljunc- tion. Bile duct elastic fiber hypogenesis and specific blood supply are important to the onset of perforation. Abdominal ultrasonography plays an important role in the diagnosis. Patients with peritoneal irritation and non-localized perforation should be operated in time, and choledochal drainage with abdominal drainage is a good treatment choice. All patients diagnosed as congenital eholedochal cysts or pancreaticobiliary maljunc- tion should undergo elective choledochal cystectomy with hepaticojejunostomy.