先天性胆总管囊肿合并门脉高压症  

Choledochal Cyst with Portal Hypertension.

作　　者：陈新英[1] 于增文[1] 李振东[1] 

机构地区：[1]河北医科大学第二医院小儿外科,石家庄050000

出　　处：《中华小儿外科杂志》1999年第5期296-298,共3页Chinese Journal of Pediatric Surgery

摘　　要：目的　探讨先天性胆总管囊肿（ＣＣ） 合并门脉高压的原因、类型及转归。方法　将１７例ＣＣ合并门脉高压与１３ 例ＣＣ不合并门脉高压者进行比较，项目包括病程、胆源性发热、胆总管囊肿的最大前后径、胆道压力、肝组织病理变化、病理图像分析，对门脉高压患儿进行随访。结果　门脉高压组（ＰＨ）胆源性发热发生率高，胆道压力高于非门脉高压组（ＮＰＨ），门脉高压组肝组织病理观察发现：１４ 例肝小叶完整，门静脉及肝静脉分支走行正常；３ 例纤维组织分割肝小叶，肝窦受压变形，门静脉及肝静脉分支走行异常。门脉高压组汇管区与肝组织、小胆管与汇管区、小动脉与肝组织之间面积比大于非门脉高压组；小静脉与小胆管面积比小于非门脉高压组，两组之间肝血窦与肝小叶的面积比、病程及胆总管囊肿最大前后径均无显著性差异。门脉高压患儿术后随访时体征均恢复正常，Ｂ超检查显示：脾脏厚度、门静脉及脾静脉宽度、门静脉及脾静脉血流速度、肝动脉阻力指数均恢复或接近正常。结论　ＣＣ合并门脉高压的主要原因为窦前门静脉受阻，即由于胆管扩张、增生首先压迫位于同一个Ｇｌｉｓｓｏｎ 鞘内压力较低的门静脉分支，汇管区的纤维组织压迫扭曲其内的门静脉分支，使门静脉压力增高，而肝窦无明显受压。Objective To review the progress of patients suffering from choledochal cysts(CC) with portal hypertension(PH). Methods Clinical progress, cholangitis, maximal anterio posterior diameter of the choledochal cyst, bile duct pressure, and liver histological imaging studies were compared between 17 CC patients with PH and 13 CC patients without PH. Results The CC patients with portal hypertension had higher rate of cholangitis and higher bile duct pressure. Liver histology of 14 CC patients with PH was normal whereas the remaining 3 showed fibrosis and compression of the sinusoids. The ratio of areas between portal area and liver tissue, bile ductules and portal area, arterioles and liver tissue in the CC patients with PH were higher than those patients without PH. Postoperatively, the patients with PH were followed up with ultrasound. The parameters including size of spleen, diameters of portal and splenic veins, the velocities of portal and splenic blood flow and the hepatic artery resistance were close to normal. Conclusions The cause of CC with PH is pre sinusoidal portal vein obstruction. PH reverses after relieving of bile duct obstruction. PH becomes irreversible once hepatic lobules are damaged.

关 键 词：胆总管囊肿 肝硬变 门脉高压症 儿童 

分 类 号：R726.574[医药卫生—儿科] R726.573.4[医药卫生—临床医学]