胆道闭锁Kasai术后迟发性胆漏合并肝外胆汁湖的诊疗分析

Clinical analysis of delayed bile leakage with extrahepatic bile lake after Kasai operation in infants with biliary atresia

目的探讨胆道闭锁Kasai术后迟发性胆漏合并肝外胆汁湖的临床特点及诊治经验。方法回顾性分析2005年1月至2021年4月山西省儿童医院收治的4例胆道闭锁Kasai术后迟发性胆漏合并肝外胆汁湖患儿的临床资料,其中男2例,女2例;行Kasai手术的中位年龄为44 d,范围在37~84 d;比较患儿胆汁湖形成前后血液白细胞计数、丙氨酸转氨酶、天冬氨酸转氨酶、γ-谷氨酰转移酶、总胆红素、直接胆红素浓度,总结其临床特点和诊疗方法并随访。结果4例患儿入院时白细胞计数、丙氨酸转氨酶、天冬氨酸转氨酶、γ-谷氨酰转移酶较胆汁湖形成前均有不同程度升高,但总胆红素、直接胆红素均下降。4例患儿予以积极抗感染、经皮肝外囊肿引流术(percutaneous transcystic drainage,PTD)治疗。其中3例采取超声引导下PTD术,3例患儿中2例引流4~5周后拔除引流管,1例因胆汁性腹膜炎再行腹腔镜下PTD;另1例行开腹PTD,2周后移除引流管。4例患儿均治愈,随访6个月胆漏及胆汁湖均无复发,再行腹腔镜下PTD的1例患儿Kasai术后10个月因胆管炎、肝功能衰竭家属放弃治疗而死亡,其余3例携自体肝持续生存,目前仍在随访中。结论胆道闭锁Kasai术后迟发性胆漏合并肝外胆汁湖是胆道闭锁术后少见并发症,术中精准操作可预防发生,结合彩色多普勒超声等辅助检查可明确诊断,PTD是安全、有效的治疗方法。

Objective To explore the diagnosis and treatment of delayed bile leakage with extrahepatic bile lake after Kasai operation in infants with biliary atresia(BA).Methods From January 2005 to April 2021,the relevant clinical data were retrospectively reviewed for 4 BA infants of delayed bile leakage with extrahepatic bile lake after Kasai operation.There were 2 boys and 2 girls.The median age at Kasai operation was 44(37-84)day.The levels of leucocytes,alanine aminotransferase,aspartate aminotransferase,γ-glutamyl transferase,total bilirubin and direct bilirubin were compared before and after the formation of extrahepatic bile lake.And clinical characteristics,diagnosis and treatment were summarized.Results Leucocytes,alanine aminotransferase,aspartate aminotransferase andγ-glutamyl transferase spiked at admission.Yet total bilirubin and direct bilirubin declined.All of them received antibiotics and underwent percutaneous transcystic drainage(PTD).PTD was ultrasound-guided(n=3)and one case underwent laparoscopic PTD due to biliary peritonitis.Drainage tube was removed after 4-5 weeks(n=2).All of them were cured.Neither bile leakage nor bile lake recurred during a follow-up period of 6 months.One case of laparoscopic PTD died at Month 10 after Kasai surgery due to cholangitis and liver failure.The remainders survived with native liver and were still under follow-ups.Conclusions Delayed bile leakage with extrahepatic bile lake after Kasai operation is rare in BA infants.It may be prevented by accurate operations.A definite diagnosis is based upon color Doppler ultrasound and other auxiliary examinations.PTD is both safe and effective for delayed biliary leakage after Kasai.