Abstract:

Objective To study the pathomorphological characteristics of extrahepatic portal vein wallthickening in children with biliary atresia, and to investigate the clinical significance and risk factors of the degree of extrahepatic portal vein wall thickening. Methods The clinicopathological data of 60 pediatric liver transplant recipients with biliary atresia from Children's Organ Transplantation Department of Tianjin First Central Hospital in June 2022 to December 2022 were analyzed. The pathomorphological changes of extrahepatic portal vein wall thickening in children with biliary atresia were observed. According to the median thickness of the total wall of extrahepatic portal vein, children with biliary atresia were divided into mild portal vein thickening group and severe portal vein thickeninggroup. The survival time of native liver after Kasai operation was compared between the two groups. Univariate and multivariate logistic regression were used to analyze the influencing factors of the degree of extrahepatic portal vein wallthickening. Results The wall of extrahepatic portal vein in children with biliary atresia was thickened to varyingdegrees， and the main pathological changes were interstitial edema under vascular endothelial cells， proliferation of fibers and fibroblasts， and a small amount of inflammatory cell infiltration. The intimal thickness of extrahepatic portal vein was 110（30 ～ 640）μm. Total wall thickness was 373（160~1320）μm. The ratio of portal vein intima thickness to total portal vein wall thickness was 0.341（0.105 ~ 0.636）. Biliary atresia patients were furhter grouped, total wall hickness ≤ 373 μm was defined as portal vein mild thickening group, total wall thickness ＞ 373 μm was defined as severe portal vein thickening group. The survival time of native liver in severe portal vein thickening group was significantly lowerthan that in mild portal vein thickening group （P ＜ 0.05）. Univariate analysis showed that Kasai operation history and cholangitis history were related factors affecting the degree of extrahepatic portal vein wall thickening in biliary atresia （P ＜ 0.05）. Multivariate analysis showed that the history of cholangitis was an independent risk factor for the degree ofextrahepatic portal vein wall thickening in biliary atresia （odds ratio ＝ 4.000, 95% confidence interval as 1.272 ~ 12.578, P ＜ 0.05）.Conclusion Extrahepatic portal vein wall thickening in children with biliary atresia was mainly characterized by interstitial edema under vascular endothelial cells, proliferation of fibers and fibroblasts, and a small amount of inflammatory cell infiltration. The degree of extrahepatic portal vein wall thickening in biliary atresia may affect the survival time of native liver after Kasai operation. Prevention and treatment of cholangitis could help to reduce the degree of portal vein wall thickening. 

Key words:

Biliary atresia , Portal vein , Pathological characteristics , Native liver survival , Risk factors, Liver transplantation

摘要：

目的 研究胆道闭锁患儿肝外门静脉管壁增厚的病理形态学特征，同时探讨肝外门静脉管壁增厚程度的临床意义及危险因素。方法 分析 2022 年 6 月至 2022 年 12 月天津市第一中心医院儿童器官移植科 60 例行肝移植治疗的胆道闭锁患儿的临床病理资料，观察胆道闭锁患儿肝外门静脉管壁增厚的病理形态学改变。根据肝外门静脉总管壁厚度的中位数，将胆道闭锁患儿分为门静脉轻度增厚组及门静脉重度增厚组。比较两组间 Kasai 术后自体肝生存时间。单因素和多因素 Logistic 回归分析肝外门静脉管壁增厚程度的影响因素。结果 胆道闭锁患儿肝外门静脉管壁出现不同程度增厚，以血管内皮细胞下间质水肿、纤维和成纤维细胞增生及少量炎细胞浸润为主要病理学改变。肝外门静脉管壁内膜厚度为 110（30 ~ 640）μm，总管壁厚度为373（160 ~ 1320）μm，门静脉内膜厚度 / 门静脉总管壁厚度的比值 0.341（0.105 ~ 0.636）。对胆道闭锁进行分组，总管壁厚度≤ 373 μm 定义为门静脉轻度增厚组，总管壁厚度＞ 373 μm 定义为门静脉重度增厚组。门静脉重度增厚组自体肝生存时间显著低于门静脉轻度增厚组，差异有统计学意义（P ＜ 0.05）。单因素分析结果显示：Kasai 手术史和胆管炎病史是影响胆道闭锁肝外门静脉管壁增厚程度的相关因素（P ＜ 0.05）。多因素分析结果显示 ：胆管炎病史是影响胆道闭锁肝外门静脉管壁增厚程度的独立危险因素（优势比＝ 4.000，95% 可信区间＝ 1.272 ~ 12.578，P ＜ 0.05）。结论 胆道闭锁患儿肝外门静脉管壁增厚以血管内皮细胞下间质水肿、纤维和成纤维细胞增生及少量炎细胞浸润为主要特征。胆道闭锁肝外门静脉管壁增厚程度可能影响 Kasai 术后自体肝生存时间。预防并治疗胆管炎，有助于减轻门静脉管壁增厚的程度。 

关键词:

胆道闭锁 , 门静脉 , 病理特征 , 自体肝生存 , 危险因素 , 肝移植