胆道闭锁Kasai术后肝内胆管囊性扩张的形成特点与病理研究

目的分析胆道闭锁(biliary atresia,BA)患儿Kasai术后肝内胆管囊性扩张(intrahepatic biliary cysts,IBC)的形成特点及病理学表现,探索其与预后之间的关系。方法选取2017年1月至2018年12月天津市儿童医院Kasai术后行肝移植手术的BA患儿179例(男75例,女104例),收集患儿的临床资料和影像学资料,用以判断是否存在IBC,其中36例CT检查结果提示存在肝内胆管扩张,作为IBC(+)组,143例未检测到肝内胆管扩张,作为IBC(-)组,比较两组的自体肝生存状况及肝功能指标。同时收集到上述患儿中50例患儿的肝移植时的病肝组织,其中IBC(+)组18例,IBC(-)组32例,进行HE染色,观察肝脏纤维化程度、胆管增生程度、淤胆以及胆管板畸形的发生情况。结果①IBC(+)组女患儿有27例,占75.0%(27/36),IBC(-)组女患儿有77例,占53.8%(77/143),两组中女患儿占比的差异具有统计学意义(P=0.021),两组患儿在行Kasai术时的日龄及术后胆管炎的发生率的比较中,差异无统计学意义(P>0.05);②IBC(+)组患儿的中位生存期为11.5个月,明显高于IBC(-)组患儿的9.0个月。Log-Rank检测发现,IBC(+)组术后自体肝生存状况优于IBC(-)组患儿,且差异具有统计学意义(P=0.038);③肝功能的比较结果发现IBC(+)组除胆汁酸(total bile acids,TBA)外,均低于IBC(-)组患儿,其中总胆红素(total bilirubin,TBI)、结合胆红素(direct bilirubin,DBI)水平在两组的差异具有统计学意义(P<0.05);④IBC(+)组患儿的肝纤维化程度、胆管增生程度及胆管板畸形发生率均较IBC(-)组略低,差异无统计学意义;但IBC(+)组淤胆程度明显低于IBC(-)组,差异具有统计学意义(P=0.013)。结论BA患儿Kasai术后发生IBC可能是肝脏代偿的积极表现,短期内不会导致胆汁淤积的加重,相反扩张的胆管可以减轻胆汁淤积的程度,改善肝功能及病理分级,从而延长自体肝生存时间。

Characteristics and pathological study of intrahepatic biliary cysts after Kasai procedure

Objective To explore the characteristics and pathological manifestations of intrahepatic biliary cysts(IBC)after Kasai operation for biliary atresia(BA)in children and examine their relationship with prognosis.Methods A total of 179 BA patients after liver transplantation(LT)were selected and their clinical data were collected.Based upon the findings of computed tomography(CT),they were divided into IBC(+)group with bile duct expansion(n=36)and IBC(-)group without bile duct expansion(n=143).Native liver survival status and liver function were compared between IBC(+)and IBC(-)groups.And 50/179 samples[including 18 cases in IBC(+)group and 32 cases in IBC(-)group]were randomly selected for HE staining to compare the pathological characteristic of degree of liver fibrosis,bile duct proliferation,portal bile plug and occurrence of bile duct plate deformity.Results Twenty-seven girls(27/36,75.0%)in IBC(+)group were greater than 77 girls(77/143,53.8%)in IBC(-)group and the difference was statistically significant(P=0.021).Comparing average age of Kasai procedure and occurrence of postoperative cholangitis,inter-group difference was not statistically significant(P>0.05).Furthermore,median time of liver failure was longer in IBC(+)group than that in IBC(-)group(11.5 vs 9.0 months).Log-Rank test revealed that IBC(+)group was better than IBC(-)group and the difference was statistically significant(P=0.038).Comparing liver function of two groups,except for TBA,other parameters of IBC(+)group were lower than those of IBC(-)group and inter-group differences in TB/DB level were statistically significant(P<0.05).Degree of liver fibrosis,bile duct proliferation and bile duct plate deformity were slightly lower in IBC(+)group than those in IBC(-)group and the difference was not statistically significant.However,degree of portal bile plug was significantly lower in IBC(+)group than that in IBC(-)group and the difference was statistically significant(P=0.013).Conclusions Intrahepatic biliary cyst after Kasai procedure may be a positive manifestation of liver compensation in BA children.In a short term,it will not increase cholestasis.On the contrary,IBC reduces cholestasis and liver function and extends the survival time of native liver.