Liver Research Laboratory, Institute of Biomedical Research, Queen Elizabeth Hospital, Edgbaston, Birmingham, UK.
Eur J Gastroenterol Hepatol. 2009 Feb;21(2):206-13. doi: 10.1097/MEG.0b013e3283060ee8.
Biliary obstruction as a consequence of portal biliopathy, because of extrahepatic portal vein occlusion is an uncommon cause of biliary disease in the western world. We reviewed all patients presenting to the Regional Liver Transplant Unit in Birmingham, UK with symptomatic portal biliopathy between 1992 and 2005 and report the presentation, investigation, management and outcome of these complex patients.
Thirteen patients with symptomatic portal biliopathy were followed up for a median of 2 years (range 1-18 years). Jaundice was the presenting feature in all cases and was associated with bile duct stones or debris in 77% (10 of 13) of cases. Successful treatment of biliary problems was achieved by biliary decompression in six cases (metallic stent=three, plastic stent=one, combined procedure=one and sphincterectomy=one) and portal decompression in three cases (transjugular intrahepatic portosystemic shunt=two, meso-caval shunt=one). Successful biliary drainage could not be achieved endoscopically or by portal decompression in one case that was accepted for combined liver and small bowel transplantation. Three patients had spontaneous resolution without recurrence over the follow-up period. Ten patients (77%) experienced gastrointestinal bleeding. Two deaths over the follow-up period occurred; both were associated with portal hypertensive bleeding.
Endoscopic management (sphincterectomy and stone extraction or stent insertion) is effective initial therapy for patients with symptomatic portal biliopathy. In the case of persistent biliary obstruction porto-systemic shunting (transjugular intrahepatic portosystemic shunt or surgical) should be considered, however, the extent of vascular thrombosis precludes this in most cases.
由于肝外门静脉闭塞导致的门脉性胆病引起的胆道阻塞，在西方世界是一种不常见的胆道疾病病因。我们回顾了所有 1992 年至 2005 年间在英国伯明翰地区肝脏移植中心就诊的有症状的门脉性胆病患者，并报告了这些复杂患者的临床表现、检查、治疗和预后。
13 例有症状的门脉性胆病患者中位随访 2 年（范围 1-18 年）。所有病例均以黄疸为首发症状，77%（13 例中的 10 例）伴有胆管结石或胆管碎片。6 例通过胆道减压（金属支架=3 例，塑料支架=1 例，联合手术=1 例，括约肌切开术=1 例）和 3 例通过门脉减压（经颈静脉肝内门体分流术=2 例，肠系膜腔静脉分流术=1 例）成功治疗胆道问题。1 例因无法通过内镜或门脉减压成功引流而接受肝肠联合移植。3 例在随访期间自发性缓解且无复发。10 例（77%）患者发生胃肠道出血。随访期间发生 2 例死亡，均与门脉高压性出血有关。
对于有症状的门脉性胆病患者，内镜治疗（括约肌切开术和取石或支架置入）是有效的初始治疗方法。在持续胆道阻塞的情况下，应考虑门体分流术（经颈静脉肝内门体分流术或手术），但大多数情况下血管血栓形成会妨碍这种治疗。
