El-Matary Wael, Roberts Eve A, Kim Peter, Temple Michael, Cutz Ernest, Ling Simon C
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada.
Eur J Pediatr. 2008 Nov;167(11):1339-42. doi: 10.1007/s00431-008-0675-4. Epub 2008 Feb 13.
Portal hypertensive biliopathy (PHB) is defined as abnormal biliary changes that take place most likely secondary to extrahepatic portal vein obstruction (EHPVO) with portal hypertension. This condition may be asymptomatic or could lead to a cholestatic state, which is not well-described in children. We report a child who developed a cholestatic nature with portal hypertension some time after having neonatal surgery for duodenal atresia. We discuss the differential diagnosis and management of this rare condition. Symptomatic PHB has been only rarely reported in children. It should be suspected in patients with portal hypertension and having features of biliary obstruction. Hepaticojejunostomy may have a therapeutic role in selected patients in whom endoscopic or percutaneous manipulation of the biliary tree is unsuccessful and who have not responded to a surgical portal-systemic shunt procedure.
门静脉高压性胆汁病（PHB）的定义为，最有可能继发于肝外门静脉阻塞（EHPVO）伴门静脉高压的异常胆汁变化。这种情况可能无症状，也可能导致胆汁淤积状态，而儿童中的相关描述并不充分。我们报告了一名儿童，其在因十二指肠闭锁接受新生儿手术后一段时间出现了伴有门静脉高压的胆汁淤积情况。我们讨论了这种罕见病症的鉴别诊断和管理。有症状的PHB在儿童中仅有极少的报道。门静脉高压且有胆道梗阻特征的患者应怀疑患有此病。对于内镜或经皮胆道操作失败且对手术门静脉-体循环分流术无反应的特定患者，肝空肠吻合术可能具有治疗作用。
