Caroli disease presenting with recurrent cholangitis and secondary small bowel obstruction: a case report
DOI:
https://doi.org/10.18203/2349-3933.ijam20261091Keywords:
Caroli disease, Recurrent cholangitis, Small bowel obstructionAbstract
Caroli disease is a rare congenital hepatobiliary disorder characterized by non-obstructive dilatation of the intrahepatic bile ducts and classified as Todani type V choledochal cyst. Although congenital in origin, the disease is often diagnosed in adulthood due to its indolent course and nonspecific symptoms, frequently after complications such as recurrent cholangitis have occurred. We report a case of a 46-year-old male presenting with recurrent epigastric pain, abdominal distension, fever, jaundice, nausea, vomiting, and bowel movement disturbance. Laboratory findings revealed leukocytosis, direct hyperbilirubinemia, and cholestatic liver enzyme elevation consistent with acute cholangitis. Abdominal radiography demonstrated features of partial small bowel obstruction. Magnetic resonance cholangiopancreatography (MRCP) with contrast revealed multifocal cystic dilatation of the intrahepatic bile ducts without extrahepatic obstruction, consistent with Caroli disease. The patient was managed conservatively with intravenous antibiotics, ursodeoxycholic acid, bowel decompression, and supportive care, resulting in gradual clinical improvement. This case highlights the diagnostic value of MRCP and demonstrates that conservative management can be effective in adult Caroli disease complicated by recurrent cholangitis and secondary small bowel obstruction.
References
Lasagni A, Cadamuro M, Morana G, Fabris L, Strazzabosco M. Fibrocystic liver disease: novel concepts and translational perspectives. Transl Gastroenterol Hepatol. 2021;6:26.
Beuers U, Trauner M, Jansen P, Poupon R. New paradigms in the treatment of hepatic cholestasis: from UDCA to FXR, PXR and beyond. J Hepatol. 2015;62(1):S25-37.
Bourabaa S, Laalou T, Mansouri A, Hamid M, Settaf A. Surgical management of Caroli disease in a low-mid income country: a single-center study and review of literature. BMC Surg. 2025;25(1):214.
Mayumi T, Okamoto K, Takada T, Strasberg SM, Solomkin JS, Schlossberg D, et al. Tokyo Guidelines 2018: management bundles for acute cholangitis and cholecystitis. J Hepatobiliary Pancreat Sci. 2018;25(1):96-100.
Lewin M, Desterke C, Guettier C, Valette PJ, Agostini H, Franchi-Abella S, et al. Diffuse Versus Localized Caroli Disease: A Comparative MRCP Study. AJR Am J Roentgenol. 2021;216(6):1530-8.
Deardo Purba Girsang A, Agung Fattahillah RT, Nazilinly F, Nurmilatun Kamilah N, Nengah Andra Putra Pramudya I, Nabilah Mario A, et al. Choledochal Cyst: Case Variations and Management. J Biol Trop. 2024;24(1b):139-47.
Khot R, Ganeshan D, Sundaram KM, Depetris JN, Ludwig DR. Cystic lesions and their mimics involving the intrahepatic bile ducts and peribiliary space: diagnosis, complications, and management. Abdom Radiol. 2024;50(7):2969-88.
Ulrich F, Pratschke J, Pascher A, Neumann UP, Lopez-Hänninen E, Jonas S, et al. Long-Term Outcome of Liver Resection and Transplantation for Caroli Disease and Syndrome. Ann Surg. 2008;247(2):357-64.
Dumonceau JM, Kapral C, Aabakken L, Papanikolaou IS, Tringali A, Vanbiervliet G, et al. ERCP-related adverse events: European Society of Gastrointestinal Endoscopy (ESGE) Guideline. Endoscopy. 2020;52(2):127-49.
Zielinski MD, Bannon MP. Current Management of Small Bowel Obstruction. Adv Surg. 2011;45(1):1-29.
Schick MA, Kashyap S, Collier SA, Meseeha M. Small Bowel Obstruction. StatPearls. 2025.
Karimzadeh‐Soureshjani E, Pourhasan F, Simab PA, Shojaei S, Mohammadian Y. A Rare Diagnosis of Caroli Syndrome in a Young Patient. Clin Case Rep. 2025;13(6).
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