Landen S, Bardaxoglou E, Maddern G J, Delugeau V, Gosselin M, Launois B
Department of Digestive Surgery and Transplantation, C.H.U. Pontchaillou, Rennes, France.
Acta Chir Belg. 1993 Sep-Oct;93(5):224-6.
We report the case of a 31-year-old male patient with a diffuse form of Caroli's disease presenting as recurring bouts of biliary pancreatitis. Following sphincterotomy, the patient remained asymptomatic for 5 years. He then developed acute cholangitis and, at laparotomy, all superficial liver cysts were fenestrated in order to remove intrahepatic bile duct calculi. A right hepatectomy, removing the most severely affected liver parenchyma was not considered feasible because of the small size of the left lobe and the existence of an associated congenital hepatic fibrosis. In diffuse forms of Caroli's disease biliary drainage techniques have often proved ineffective in preventing recurring bouts of cholangitis. Moreover, liver resection is seldom feasible because of associated congenital hepatic fibrosis. In this setting, liver transplantation may represent the only effective and durable form of treatment.
我们报告了一例31岁男性患者，其患有弥漫型卡罗里病，表现为复发性胆源性胰腺炎。行括约肌切开术后，患者5年无症状。随后他发生了急性胆管炎，在剖腹手术中，所有表浅肝囊肿均行开窗术以清除肝内胆管结石。由于左叶体积小且存在相关的先天性肝纤维化，切除受影响最严重的肝实质的右肝切除术被认为不可行。在弥漫型卡罗里病中，胆道引流技术常常被证明在预防复发性胆管炎发作方面无效。此外，由于相关的先天性肝纤维化，肝切除术很少可行。在这种情况下，肝移植可能是唯一有效且持久的治疗方式。
