门静脉海绵体瘤胆管病变(PCC)是指慢性门静脉血栓形成导致的门静脉海绵体瘤患者出现典型的胆管造影改变。在没有其他胆道疾病的情况下。可能是由于与海绵状瘤有关的胆汁淤滞,PCC中胆泥和结石的发病率很高,通过适当的干预措施引发症状。持续和麻烦的症状通常是由于胆道狭窄或狭窄,可能有或没有胆道结石,可能是短或长,单发或多灶性,肝外或肝内。在过去的二十年中,在PCC中进行内窥镜干预的经验表明,这是胆管结石的首选方法。塑料支架重复,及时,支架交换是由于胆道狭窄引起的黄疸或胆管炎的一线干预措施。如果胆道梗阻没有解决,进行门体分流术(PSS)或经颈静脉肝内门体支架分流术(TIPS)以减压门静脉海绵体瘤。然而,对于静脉不可分流或分流阻塞的患者,重复更换塑料支架是唯一的选择,尽管有报道称在这种情况下使用胆道自膨式金属支架。如果PSS或TIPS成功后症状性胆道梗阻持续存在,第二阶段胆道手术可能是必要的。最近的经验表明,在术后良性胆管狭窄的情况下,通过球囊扩张和反复更换塑料支架束治疗PCC的胆管狭窄可能是有效的治疗方法。内镜治疗似乎与胆道出血频率增加有关,通常响应标准管理。复发性胆管炎形成污泥和结石可能是反复更换支架的问题。特别是如果患者依从性差。总之,目前的理解是,有症状的PCC最好由内窥镜医师和外科医生联合管理,并采用最初旨在建立和维持胆道引流的序贯干预措施。然后对门海绵体瘤进行减压,最后,如果需要,胆道狭窄的第二阶段胆道手术或内治疗。内镜治疗在以前的管理中占据着核心作用,在手术治疗期间和之后。随着对发病机理和自然史的认识的提高以及新的方法和技术的应用,内窥镜治疗的范式继续发展。
Portal cavernoma cholangiopathy (PCC) is the presence of typical cholangiographic changes in patients with a portal cavernoma due to chronic portal vein thrombosis, in the absence of other biliary tract diseases. Probably due to biliary stasis related to the cavernoma, there is a high incidence of biliary sludge and calculi in PCC, which trigger symptoms that resolve with appropriate interventions. Persistent and troublesome symptoms are usually due to biliary stenoses or strictures, which may occur with or without biliary calculi and may be short or long, solitary or multifocal, extrahepatic or intrahepatic. Experience with endoscopic interventions in PCC over the last twenty years has shown that it is the procedure of choice for bile duct calculi. Plastic stenting with repeated, timely, stent exchanges is the first line intervention for jaundice or cholangitis due to biliary strictures. If biliary obstruction does not resolve, portosystemic shunt surgery (PSS) or transjugular intrahepatic portosystemic stent shunt (TIPS) is performed to decompress the portal cavernoma. However, for patients with non-shuntable veins or blocked shunts, repeated plastic stent exchanges are the only option though there are reports of the use of biliary self-expandable metal stents in this situation. If symptomatic biliary obstruction persists after successful PSS or TIPS, second stage biliary surgery may be necessary. Recent experience suggests that treating biliary strictures in PCC on the lines of postoperative benign biliary strictures with balloon dilatation and repeated exchanges of plastic stent bundles may be effective therapy. Endoscopic management appears to be associated with an increased frequency of hemobilia, which usually responds to standard management. Recurrent cholangitis with formation of sludge and concretions may be a problem with repeated stent exchanges, especially if patient compliance is poor. In conclusion, the current understanding is that symptomatic PCC is best managed jointly by the endoscopist and surgeon with sequential interventions designed initially to establish and maintain biliary drainage, then to decompress the portal cavernoma and, finally, if required, second stage biliary surgery or endotherapy for biliary strictures. Endoscopic therapy occupies a central role in management before, during and after surgical therapy. Paradigms of endoscopic therapy continue to evolve as knowledge of pathogenesis and natural history improves and newer approaches and techniques are applied.