International Surgery Journal | November 2018 | Vol 5 | Issue 11 Page 3750 International Surgery Journal Yadav P et al. Int Surg J. 2018 Nov;5(11):3750-3753 http://www.ijsurgery.com pISSN 2349-3305 | eISSN 2349-2902 Case Report Caroli’s disease: a diagnostic challenge Padmini Yadav*, Shailesh Adhikari, Narendra Pandit, Lalijen Awale, Keerthi Vasan, Sarada Khadka INTRODUCTION Caroli's disease is a rare congenital malformation of the intrahepatic bile ducts characterized by duct ectasia and dilation, which may involve the biliary tract in a focal or multifocal manner. Caroli’s disease is less common than Caroli’s syndrome, and both are extremely rare with an approximate prevalence of less than one in 1,000,000 inhabitants. 1 Caroli’s disease combined with congenital hepatic fibrosis and/or renal cystic disease is referred to - Caroli’s syndrome. 2 It is currently included in group V of the Todani classification of biliary tract cystic diseases and was first described by the French gastroenterologists, Jacques Caroli et al., in 1958. 3 The clinical course can be asymptomatic in the first two decades from birth, in fact, typical symptoms may scarcely arise throughout the patients’ life. 4 Caroli’s disease may diffusely affected the liver or be localized to one lobe or segment. Caroli’s disease predispose to biliary stasis and intrahepatic stones formation leading to cholangitis, liver abscesses, septicemia, and ultimately to secondary biliary cirrhosis. 5 Most used diagnosis methods are ultrasound, computed tomography (CT), magnetic resonance cholangiopancreatography (MRPC). Endoscopic cholangiopancreatography (ERCP) can be used both as diagnostic and or therapeutic tool; a liver biopsy is rarely needed. 6 We describe a case of Caroli’s disease presenting with right upper abdominal pain. We believe the present reports are of medical significance since they relate to patients with atypical presentations and diagnosed at an unusually late age. It serves also as a ABSTRACT Caroli’s disease is a rare congenital hepatobiliary disease characterized by multifocal segmental dilatation of intrahepatic bile ducts affecting all or parts of the liver. It predisposes to biliary stasis and consequent lithiasis, cholangitis, abscesses, and septicemia. Sometimes it is difficult to diagnose and differentiate it from other similar disease conditions. 60-year-old female presented with features of recurrent cholangitis with hepatolithiasis and multiple cyst in liver, cholelithiasis was planned for cholecystectomy and drainage procedure. Patient underwent open cholecystectomy with common bile duct (CBD) exploration with T-tube placement due to intraoperative instability. Post-operative T tube cholangiogram was done. Post-operative T tube cholangiogram showed bilobar major duct cystic dilatation with predominant left lobe involvement, with few cysts containing calculi. Patient was planned for left hepatectomy with bile duct excision but patient refused to undergo aforementioned surgery. Therefore ERCP and sphincterotomy was done in view of poor performance status.Caroli’s disease being a rare disease is sometimes difficult to diagnose and treat in an old age patient with bilobar involvement and poor general conditions. Keywords: Caroli’s disease, Diagnostic challenge, Old age, Right upper abdominal pain Department of Surgery, B.P. Koirala Institute of Health Sciences, Dharan, Sunsari, Nepal Received: 30 August 2018 Accepted: 28 September 2018 *Correspondence: Dr. Padmini Yadav, E-mail: padmeeneyadav@gmail.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: http://dx.doi.org/10.18203/2349-2902.isj20184657