婴幼儿胆道发育不良的胆道造影及病理特点和预后

The clinical analysis of infantile biliary hypoplasia diagnosed by laparoscopy cholangiography

目的探讨经腹腔镜胆道造影诊断的婴幼儿胆道发育不良病例的特点，总结其诊断标准。方法回顾性分析我院4年中通过腹腔镜胆道造影诊断的8例胆道发育不良患儿的病历资料，对其临床特点、影像学资料、病理特点及随访结果进行总结。结果术中见2例年长患儿肝脏增大，且有结节状改变；其余6例肝脏大小、质地均基本正常；7例胆囊发育偏小，充盈差。造影结果均示左右肝管、胆总管细小，肝内胆管无明显显影。胆囊造瘘术后引流出少量黄色稀薄胆汁，某些患儿口服熊去氧胆酸可以增加胆汁引流量。病理结果示所有肝组织标本均显示肝内胆汁淤积表现，所有标本汇管区及小叶间胆管减少或消失；均有不同程度的炎细胞浸润及肝细胞坏死；其中2例手术年龄较大者有假小叶形成趋势，即早期肝硬化表现。随访时间为术后6个月～4年。2例于6～8月龄死亡；存活至今的6例患儿，其黄疸缓慢加重或无明显变化，伴有生长发育不良、腹胀。其中1例再次行胆囊结肠吻合术，另1例术后2年行肝移植。结论婴幼儿胆道发育不良是一类在临床特点、影像学、病理学方面具有共性的疾病，其治疗方法及预后与其他婴幼儿梗阻性黄疸性疾病不同，对其进行早期确诊有重要临床意义。其诊断依据可能包括：①病史：出生后早期即出现梗阻性黄疸并持续加重；②胆道造影：肝外胆管纤细≤2mm，肠道有造影剂进人，肝内胆管可有细微显影但不清晰；③病理特点：肝内淤胆表现，伴有肝内小胆管减少或消失；④胆囊造瘘术后引流可有少量胆汁。

Objective To analyze the characteristics of cholangiography and pathology of biIiary hypoplasia diagnosed by laparoscopic cholangiography. Methods Medical records of 8 patients with biliary hypoplasia diagnosed by laparoscopic cholangiography during the last 4 years were reviewed. The clinical features, cholangiography, pathology and prognosis were analyzed. Results Two specimens from eider cases showed enlargement of liver with nodosity. The specimens from the other 6 cases showed almost normal liver. The gallbladders of 7 cases were poorly developed and smaller than normal. Cholangiography showed that in all cases the hepatic ducts and choledochus were extremely thin, the intrahepatic bile ducts were unwell developed. There was a little bile drainage from gallbladder fistulation, and in some eases the bile drainage increased after orally intaking ursodeoxycholic acid. Intrahepatic cholestasis and the paucity of interlobular bile duets were pathologically detected in all cases. The specimens of 2 elder eases showed pseudolohule, the rest of cases showed slight fibrosis. The patients were followed up from 6 months to 4 years. Two patients died at the age of 6-8 months, the other 6 patients survived with persistent cholestasis, retarded growth, abdominal distension. Conclusions Biliary hypoplasia is a type of disease that show common features in clinical features, cholangiography and pathology. But its treatment and prognosis are different from other obstructive jaundice diseases of infants. It is important to make an early and definite diagnosis, so that Kasai procedure would not be misused. We propose that the diagnostic criteria for biliary hypoplasia should include： Obstructive jaundice appears soon after birth andcontinue aggravating; Cholangiography shows the hepatic ducts and choledochus are extremely thin （≤2 mm）, the inlrahepatic bile ducts are not well developed; Pathology shows intrahepatic cholestasis and paucity of inlerlohular bile ducts; A little bile drainage can be seen through gallhladder fistulation.