Bayraktar Yusuf, Harmanci Ozgür, Gedikoğlu Gökhan, Balkanci Ferhun, Ersoy Osman, Aydinli Musa, Abbasoğlu Osman
Department of Gastroenterology, Hacettepe University, School of Medicine, Sihhiye, Ankara, Turkey.
Turk J Gastroenterol. 2010 Mar;21(1):63-7. doi: 10.4318/tjg.2010.0052.
Caroli syndrome is a rare condition and is composed of congenital cystic dilatation of the biliary system and congenital hepatic fibrosis. Although many associated conditions are defined and hypothesized to occur concomitantly, due to the rarity of this syndrome, none has proven to be an essential component of this syndrome. In order to investigate a patient presenting with a cholestatic clinical picture, ultrasound, endoscopic retrograde cholangiopancreatography, abdominal computed tomography, liver biopsy, splenoportal venous angiography, and all available liver tests were performed. Upon typical findings, a diagnosis of Caroli syndrome was made and an orthotopic liver transplantation was performed. Investigation of the patient demonstrated multiple intracystic stones mimicking hemangiomatosis in the ultrasound; severe irregularity and narrowing in the main bile duct mimicking sclerosing cholangitis in the endoscopic retrograde cholangiopancreatography; partial portal vein thrombosis with irregularity in the portography; and a unilobar cirrhosis of the left liver lobe while the right lobe demonstrated only congenital hepatic fibrosis in the explanted liver. Caroli syndrome may be associated with main bile duct and portal vein abnormalities. Although the syndrome can be monolobar in nature, a cirrhotic left lobe sparing the right lobe, partially affected by the cirrhotic process, has never been defined. Here, we report a case of Caroli syndrome who had liver transplantation, with very rare and interesting findings of the explanted liver, such as tapering cirrhosis from the left lobe to the right lobe and countless stones in biliary cysts mimicking hemangiomas.
卡罗里综合征是一种罕见疾病，由胆道系统先天性囊性扩张和先天性肝纤维化组成。尽管定义了许多与之相关的病症，并假设它们会同时出现，但由于该综合征罕见，尚无一种病症被证明是该综合征的必要组成部分。为了对一名出现胆汁淤积临床表现的患者进行检查，进行了超声检查、内镜逆行胰胆管造影、腹部计算机断层扫描、肝活检、脾门静脉血管造影以及所有可用的肝脏检查。根据典型表现，诊断为卡罗里综合征并进行了原位肝移植。对该患者的检查显示，超声检查中有多个类似血管瘤病的囊内结石；内镜逆行胰胆管造影中主胆管严重不规则和狭窄，类似硬化性胆管炎；门静脉造影中有部分门静脉血栓形成且不规则；在切除的肝脏中，左肝叶为单叶肝硬化，而右叶仅表现为先天性肝纤维化。卡罗里综合征可能与主胆管和门静脉异常有关。尽管该综合征本质上可能是单叶的，但从未定义过一种肝硬化左叶不累及右叶，而右叶仅部分受肝硬化过程影响的情况。在此，我们报告一例接受肝移植的卡罗里综合征病例，其切除的肝脏有非常罕见且有趣的发现，如从左叶到右叶逐渐变细的肝硬化以及胆管囊肿内无数类似血管瘤的结石。
