Type IVa choledochal cysts with cylindrical dilatation of the intrahepatic ducts constitute a relatively less recognized variety of choledochal cysts, and differ from cystic dilatation of intrahepatic ducts in their clinical manifestations and response to treatment. Five patients with type IVa choledochal cysts and cylindrical dilatation of major intrahepatic ducts who underwent cyst excision and Roux-en-Y hepaticojejunostomy are reported. The duration of symptoms was less than 1 year in all patients. Palpable abdominal mass and abdominal pain were present in 3 patients. The traid of jaundice, abdominal pain and mass was present in only 1 patient. The intrahepatic dilatation regressed after excision of the extrahepatic cyst just below the hilum of the liver. The surgical technique is described and the need for excision of the cyst is emphasized.
We present a 64-year-old Malay lady who had undergone a choledochoduodenostomy (CDD) two years ago for obstructive jaundice. She was admitted with jaundice and underwent ultrasonography, percutaneous transhepatic cholangiography (PTC), endoscopic retrograde cholangio pancreatography (ERCP) and computed tomographic (CT) scanning of the liver and biliary tree. All the investigations confirmed a type IVa choledochal cyst. At operation, the grossly dilated biliary system was packed with a thick mucoid material and the mucosa of the bile ducts was visibly abnormal with scattered nodules. This mucoid material had caused occlusion of the entire biliary tree resulting in obstructive jaundice. To the best of our knowledge, this is probably the first report of obstructive jaundice caused by thick mucus. The peculiar management problems of this case and the risk of malignant change in choledochal cysts are discussed.