Satvik Jhamb1, Christopher Decker2, Ricardo Romero2, Ramon E Rivera2, W Charles Conway3. 1. Department of Surgery, Ochsner Clinic Foundation, New Orleans, LA. 2. Department of Gastroenterology, Ochsner Clinic Foundation, New Orleans, LA. 3. Department of Surgery, Ochsner Clinic Foundation, New Orleans, LA ; The University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA.
Abstract
BACKGROUND: Congenital or primary intrahepatic bile duct (IHBD) dilatation is a rare disorder with symptoms of abdominal pain and hepatomegaly that usually presents in childhood and adolescence. Recurrent cholangitis, liver abscesses, septicemia, and biliary cirrhosis may result secondary to biliary sludge and hepatolithiasis. CASE REPORT: We present a case of IHBD dilatation with hepatolithiasis cured with surgical resection and discuss the management of this disease. CONCLUSION: IHBD dilatation should be treated, as chronic biliary stasis and hepatolithiasis can lead to infection and recurrent cholangitis that can progress to cholangiocarcinoma. Treatment for IHBD dilatation usually involves multiple modalities including medical therapy, but ultimately resection of the diseased segments or lobes is required given the increased risk of malignancy.
BACKGROUND: Congenital or primary intrahepatic bile duct (IHBD) dilatation is a rare disorder with symptoms of abdominal pain and hepatomegaly that usually presents in childhood and adolescence. Recurrent cholangitis, liver abscesses, septicemia, and biliary cirrhosis may result secondary to biliary sludge and hepatolithiasis. CASE REPORT: We present a case of IHBD dilatation with hepatolithiasis cured with surgical resection and discuss the management of this disease. CONCLUSION: IHBD dilatation should be treated, as chronic biliary stasis and hepatolithiasis can lead to infection and recurrent cholangitis that can progress to cholangiocarcinoma. Treatment for IHBD dilatation usually involves multiple modalities including medical therapy, but ultimately resection of the diseased segments or lobes is required given the increased risk of malignancy.
Entities:
Keywords:
Bile ducts–intrahepatic; cholangiocarcinoma; cholangitis
Authors: Jean-Yves Mabrut; Christian Partensky; Daniel Jaeck; Elie Oussoultzoglou; Jacques Baulieux; Olivier Boillot; Jan Lerut; Jean de Ville de Goyet; Catherine Hubert; Jean-Bernard Otte; Maxime Audet; Christian Ducerf; Jean-François Gigot Journal: Ann Surg Date: 2007-08 Impact factor: 12.969