Tallón Aguilar L, Sánchez Moreno L, Barrera Pulido L, Pareja Ciuró F, Suárez Artacho G, Alamo Matinez J M, Bernal Bellido C, Garía González I, Serrano Díaz-Canedo J, Gómez Bravo M A, Bernardos Rodríguez A
Liver Transplant and Hepatobiliopancreatic Surgery Unit, General and Gastrointestinal Surgery Department, Hospitales Universitarios Virgen del Rocío, Seville, Spain.
Transplant Proc. 2008 Nov;40(9):3121-2. doi: 10.1016/j.transproceed.2008.08.104.
Caroli's disease is a rare condition that includes fibrocystic malformations of the bile duct. It consists of multifocal congenital dilatations of the intrahepatic bile ducts, which may be diffuse or limited, presenting in sack form that produces cystic structures which communicate with the biliary tree. Herein we have presented the case of a 44-year-old woman with recurrent cholangitis consequential to Caroli's syndrome. The distinctive feature of this case was that it was the first and only liver transplantation performed to date for this cause at our center among 700 procedures that had been performed over 19 years. The hepatectomy sample from the liver transplantation showed large cystic dilatations at the level of segments VII and VIII. The pathological study reported congenital dilatation of the intrahepatic bile ducts, associated with congenital hepatic fibrosis (Caroli's syndrome). Caroli's syndrome is a complex association of conditions which usually presents together with polycystic kidney lesions. Orthotopic liver transplantation is still the only therapeutic option for diffuse, uncontrollable cases or those with significant portal hypertension, as well as being the final option in the other cases in the event of a lack of response to other therapeutic options or as an alternative to them.
卡罗里病是一种罕见疾病，包括胆管的纤维囊性畸形。它由肝内胆管的多灶性先天性扩张组成，这些扩张可能是弥漫性的或局限性的，呈囊状，形成与胆管树相通的囊性结构。在此，我们报告了一例44岁女性因卡罗里综合征继发复发性胆管炎的病例。该病例的独特之处在于，在我们中心19年里进行的700例手术中，这是迄今为止因该病因进行的首例也是唯一一例肝移植手术。肝移植的肝切除样本显示在肝段VII和VIII水平有大的囊性扩张。病理研究报告肝内胆管先天性扩张，伴有先天性肝纤维化（卡罗里综合征）。卡罗里综合征是一组通常与多囊肾病变同时出现的复杂病症。原位肝移植仍然是弥漫性、无法控制的病例或伴有严重门静脉高压病例的唯一治疗选择，也是其他病例在对其他治疗选择无反应或作为替代方案时的最终选择。
