小儿先天性胆总管囊肿并肝脏损害的临床及病理分析

Clinical and Pathological Analysis in Children with Congenital Choledochal Cyst Combined with Liver Damage

目的探讨小儿先天性胆总管囊肿(CCC)并肝脏损害的病理基础及临床意义。方法CCC患儿38例按年龄分为A、B二组,A组(6个月～3岁)24例,B组(4～14岁)14例。对二组临床特点、手术前后肝功能、术中情况及肝脏活组织病理检查等资料进行回顾性分析。结果临床表现:A组以黄疸、白陶土大便及腹部包块为主,B组以腹痛为主;A组肝功能损害、碱性磷酸酶增高、胆红素增高及凝血功能异常较B组严重(Pa<0.05);胆总管囊形扩张29例,A组24例,B组5例;梭形扩张9例,均为B组。术中发现:囊肿内有结石、蛋白栓11例,A组8例,B组3例;囊肿远端明显狭窄闭锁32例(84.2%),A组27例,B组5例;远端有明显开口6例,均为B组;肝大、胆汁淤积明显20例,A组15例,B组5例;术中胆总管下端狭窄及梗阻A组较B组明显。肝脏活检结果:肝硬化改变17例,A组12例,B组5例,主要表现为汇管区小胆管及间质纤维组织明显增生,小叶中央胆汁淤积,有大量胆色素沉积,肝细胞变性,正常肝小叶结构被破坏,部分肝细胞萎缩。肝脏活检肝硬化改变A组发生率较B组高。结论不同年龄组CCC有各自的临床特点,年龄越小,梗阻越重,肝脏损害越重,肝硬变机会越大;胆道梗阻胆汁淤积是肝脏损害的病理基础,早期诊断,早期手术治疗,胆道梗阻解除后肝脏损害可恢复。

Objective To explore the pathological basis and clinical characteristics of children with congenital choledochal cyst（ CCC ） combined with liver damage. Methods According to the age,38 patients with CCC were divided into 2 groups：group A had 24 cases（ ranged 6 months to 3 years old） ;group B had 14 cases（ ranged 4 to 14 years old）. A comparative analysis of them was conducted retrospectively in the clinical characteristics, hepatic pathological changes, perioperatively liver function and operation. Results Group A was obvious higher than group B in jaundice ,white bole stool and abdominal mass, but group B was more in stomachache. Group A was obvious higher than group B in liver function lesion, alkaline phosphatase, bilirubin increase and blood coagulation disfunction （ Pa 〈 0.05 ）. Twenty - nine cases had cystic biliary dilatation,9 cases had fusiform dilatation. Finding in operation, 11 cases had calculus and albumen epistom in the cyst. Thirty - two cases（84.2% ） had obvious stenosis and atresia in distal end. Twenty - seven cases were in group A and 5 cases were in group B.6 cases had obvious debouchement in the distal end and were all in Group B. Twenty cases had obvious cholestasis.15 cases were in group A and 5 cases were in B group. The result of liver biopsy was that. 17 cases had liver cirrhosis. 12 cases were in group A and 5 cases were in group B, and displayed that, the center of hepatic lobule had, generous cholestasis, cholochrome deposition and cell degeneration, the structure of normal hepatic lobule was destroyed and a part of liver cells were atrophic. Group A was obvious higher than group B in stenosis and atresia in the distal end and liver cirrhosis rate. Conclusions CCC lead to the injury of liver function in different age group. They had the self characteristics in infants and children. The little the age, the larger the cyst. the worse the obstruction, the worse the liver cirrhosis. Obstruction of the bile ducts is the main cause of liver iniurv. It may recover after the biliarv obstruction relieved by operation.