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

Characteristics and pathological study of intrahepatic biliary cysts after Kasai procedure

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。

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.