婴儿期外科相关性胆汁淤积症临床分析

Clinical analysis of obstructive infantile cholestasis

目的 总结外科相关性婴儿胆汁淤积症的病因,探讨其外科治疗经验. 方法 回顾性分析2009年4月至2014年4月上海交通大学附属儿童医院收治的108例外科相关性婴儿胆汁淤积症患儿的临床资料. 结果 本组108例外科相关性婴儿胆汁淤积症患儿经腹腔镜探查、胆道造影后明确诊断,其中不可吻合型胆道闭锁（biliary atresia,BA）81例（75.0％）,可吻合型BA5例（4.6％）,浓缩胆栓综合征8例（7.4％）,婴儿肝炎综合征6例（5.6％）,胆总管囊肿4例（3.7％）,胆道发育不良2例（1.9％）,胆总管自发破裂1例（0.9％）,肝门处淋巴结肿大压迫l例（0.9％）.对不可吻合型BA行开腹肝门空肠吻合术或腹腔镜肝门空肠吻合术,可吻合型BA及胆总管囊肿行腹腔镜囊肿切除肝总管空肠Roux-en-Y吻合术,浓缩胆栓综合征、肝炎综合征和胆道发育不良行腹腔镜胆囊造瘘、胆道冲洗术,胆总管白发破裂行胆总管T型管引流术,肝门处淋巴结肿大压迫行腹腔镜淋巴结切除术.随访3个月～5年,行Kasai术患儿术后黄疸消退率不尽相同,行非Kasai术患儿术后黄疸均消退,未见复发.结论 胆道闭锁、浓缩胆栓综合征及肝炎综合征是引起外科相关性婴儿胆汁淤积症的常见原因,肝门或肝管空肠吻合术、胆囊造瘘胆道冲洗术是治疗外科相关性婴儿胆汁淤积症的主要方法.

Objective To summarize the etiology and surgical treatment of obstructive infantile cholestasis.Methods Clinical data of 108 cases of obstructive infantile cholestasis was studied retrospectively from April 2009 to April 2014.Results Correct diagnosis was established in all 108 patients by laparoscopic biliary tract exploration and cholangiography.Among those,there were noncorrectable biliary atresia in 81 cases （75.0%）,correctable biliary atresia in 5 cases （4.6%）,inspissated bile syndrome in 8 cases （7.4%）,infantile hepatitis syndrome in 6 cases （5.6%）,choledochal cyst in 4 cases （3.7%）,biliary hypoplasia in 2 cases （1.9%）,1 case （0.9%） suffered from spontaneous bile duct perforation,1 case （0.9%） suffered from oppression of lymph nodes in hepatic portal.Patients of nocorrectable biliary atresia were treated with open Kasai portoenterostomy or laparoscopic Kasai portoenterostomy,correctable biliary atresia and choledochal cyst underwent laparoscopic cyst excision and Roux-Y hepaticojejunostomy,inspissated bile syndrome,infantile hepatitis syndrome and biliary hypoplasia were treated by laparoscopic cholecystostomy and biliary tract irrigation.The patient of spontaneous bile duct perforation was treated with laparoscopic common bile duct exploration and T-tube drainage,the lymph node was excised in patient with oppression of lymph nodes in hepatic portal.All infants were followed-up for 3 months to 48 months,the clearance of jaundice rate varied in patients with Kasai portoenterostomy,patients with non-Kasai portoenterostomy were all in good condition and there were no symptom recurrence.Conclusion Biliary atresia,inspissated bile syndrome,infantile hepatitis syndrome,choledochal cyst and biliary hypoplasia are the most common cause of surgery-related infantile cholestasis.Kasai portoenterostomy,hepaticojejunostomy and cholecystostomy and biliary tract irrigation are the main surgical method for surgery-related infantile cholestasis.