Abou-Rebyeh H, Veltzke-Schlieker W, Radke C, Steinmüller T, Wiedenmann B, Hintze R E
Dept. of Internal Medicine, Division of Hepatology, Gastroenterology, Endocrinology, and Metabolism, University Hospital Charité, Virchow Hospital Campus, Humboldt University, Berlin, Germany.
Endoscopy. 2003 Jul;35(7):616-20. doi: 10.1055/s-2003-40242.
Ischemic-type biliary lesions (ITBLs) are the most frequent cause of nonanastomotic biliary strictures in liver grafts, affecting about 2-19 % of patients after liver transplantation. ITBL is characterized by bile duct destruction, subsequent stricture formation, and sequestration. We report here the case of a patient affected by extremely severe ITBL, with sequestration and disintegration of the entire bile duct system, in which it was possible to extract the complete biliary tree endoscopically in a single piece. Histological examination revealed that all cells of the bile duct wall had been destroyed within 3 months after liver transplantation and replaced by connective tissue. Subsequently, biliary stricture formation occurred at the hepatic hilum, as well as the adjacent large bile ducts. It may be hypothesized that cellular rejection of small bile ducts leads to the vanishing bile duct syndrome, whereas cellular rejection of large bile ducts results in ITBL. The strictures were repeatedly dilated by endoscopic means, allowing successful control of stricture formation, as well as maintenance of liver function. At the time of writing, the grafted organ and the patient had survived for more than 3 years in good health. This is the first detailed report on a sequestration of the entire bile duct system caused by ITBL, successfully treated for several years by endoscopic means.
缺血型胆管病变（ITBLs）是肝移植中非吻合口胆管狭窄最常见的原因，影响约2%-19%的肝移植患者。ITBL的特征是胆管破坏、随后的狭窄形成和胆汁隔离。我们在此报告一例受极其严重ITBL影响的患者，其整个胆管系统出现胆汁隔离和崩解，通过内镜能够完整取出整个胆管树。组织学检查显示，胆管壁的所有细胞在肝移植后3个月内被破坏，并被结缔组织取代。随后，肝门以及相邻的大胆管出现胆管狭窄。可以推测，小胆管的细胞排斥导致胆管消失综合征，而大胆管的细胞排斥则导致ITBL。通过内镜手段反复扩张狭窄部位，成功控制了狭窄的形成，并维持了肝功能。在撰写本文时，移植器官和患者已健康存活超过3年。这是关于由ITBL导致的整个胆管系统胆汁隔离的首例详细报告，通过内镜手段成功治疗了数年。
