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目的 讨论儿童胆总管囊肿导致肝损害的主要原因、儿童胆汁性肝脏纤维化分级及临床转归。方法 收集 88例儿童胆总管囊肿手术时肝活检的病理结果进行临床分析、肝纤维化分级 ,其中 11例因分期手术曾有二次肝活检结果。根据随访及二次肝活检结果对儿童胆汁性肝纤维化的转归进行讨论。结果 88例中术后 3例死于肝衰竭 ,5 9例获长期随访 ,恢复好。88例肝纤维化分级为 :0级 10例、1级 4 2例、2级 2 1例、3级 10例、4级 5例。其中 11例二次肝活检的病例在胆总管囊肿外引流 3～ 8个月后 8例肝纤维化程度分别下降 1～ 2级 ,3例无变化 (其中 1例为 0级 )。结论 1继发性胆道梗阻是儿童胆总管囊肿导致肝损害的主要原因。 2建立统一的肝纤维化分级标准有助于临床评判。 3儿童胆汁性肝纤维化在病因解除后在一定程度上可得到逆转 ,即使是假小叶形成后仍可能存在一定机会。
Objective To discuss the main causes of hepatic damage caused by choledochal cyst in children and the classification and clinical outcome of biliary liver fibrosis in children. Methods Eighty-eight children with cholangiocarcinoma were enrolled in this study. The pathological results of liver biopsy were collected for clinical analysis and grading of liver fibrosis. Eleven of them had secondary liver biopsy results due to staging surgery. The outcome of follow-up and secondary liver biopsy in children with biliary cirrhosis is discussed. Results In 88 cases, 3 patients died of liver failure after operation, and 59 patients were long-term follow-up and recovered well. The grade of liver fibrosis in 88 cases was grade 0 in 10 cases, grade 1 in 42, grade 2 in 21, grade 3 in 10 and grade 4 in 5. Of the 11 cases with secondary liver biopsy, 8 cases of hepatic fibrosis were reduced by 1 ~ 2 grades after 3 ~ 8 months of drainage and 3 cases had no change (1 case was grade 0). Conclusion 1 Secondary biliary obstruction is the leading cause of hepatic impairment in children with choledochal cyst. 2 Establish a uniform classification of liver fibrosis contribute to clinical judgment. 3 Children with biliary cirrhosis can be reversed to some extent after the cause has been relieved, and there may still be some chance even after the formation of pseudolobules.