Caroli’s disease: An unusually early presentation

• Author Details:   
• Geetanjali Srivastava
• Uma Raju
• Poonam Sherwani
• Manish Kumar
• Dheeraj Gupta

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Caroli’s disease is a rare congenital disorder characterized by cystic dilatation of large intra- hepatic bile ducts. The affected patient presents with recurrent episodes of cholangitis usually manifesting in adulthood. Imaging studies and liver biopsy help establish a diagnosis. We describe a case of Caroli’s disease in an infant aged 5 months who presented with recurrent episodes of high grade fever, lethargy and refusal to feed since neonatal period necessitating hospitalisation and antibiotic therapy.  A sibling aged 5 years had died due to polycystic kidney disease. In the current hospitalisation, laboratory parameters were suggestive of sepsis. USG abdomen was suggestive of central intra hepatic bile duct dilatation .Diagnosis was established on MRCP which revealed multifocal dilatation of main hepatic ducts with patent biliary confluence. However intrahepatic biliary radicles appeared normal. The liver biopsy showed mild proliferation of irregular ductal structures with normal appearing parenchyma. Thus the clinical, laboratory and imaging studies were pathognomic of Caroli’s disease. The patient was provided IV antibiotics with supportive care and discharged home on nutritional supplements and ursodeoxycholic acid. The patient was advised subsequent liver transplantation. The uncommon feature of this case of Caroli’s disease was its presentation in infancy as opposed to the hitherto reported cases which are in the second or third decade of life. This case highlights the need to nurse a high index of suspicion when dealing with an infant/child who has repeated hospitalisations with features of sepsis to look for an underlying anomaly.

Keywords: Caroli’s Disease, Cholangitis, Intrahepatic Biliary Dilatation, Magnetic Resonance Cholangiopancreaticography (MRCP)