Until recently congenital cystic dilatation of the intrahepatic biliary ducts has been infrequently recognized. It is usually manifested by cholelithiasis, cholangitis, upper abdominal and Rt. upper quadrant discomfortness but in severe cases, symptoms of liver abscess or sepsis may developed.
Among diseases to be differentiated are congenital hepatic fibrosis, polycystic liver and obstructive bile duct dilatation. Operative cholangiogram is most valuable test for diagnosis and extent of the disease. Although hepatic lobectomy or segmental resection yields the best result when the disease is confined to a lobe or a segment internal drainage procedures are widely used. Authors recently, experienced 3 patients with this anomaly, 2 of these were, undertaken surgical intervention for two times respectively owing to recurrent cholangitis and eholelithiasis. The other one had left lobectomy with good result upto 3 years postoperatively authors also reviewed references.
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