Choledochal cyst causes liver fibrosis, the extent of which varies with each case. Liver damage seldom progresses to cirrhosis, but when it does, it is generally irreversible. We report an infantile case of liver cirrhosis associated with choledochal cyst in which complete clinical resolution was achieved by surgery. Pancytopenia caused by splenomegaly, massive ascites, hypoalbuminemia, and coagulation abnormality that were observed during the early postoperative period had disappeared within 4 months after surgery. Needle liver biopsy performed at 1 year after surgery revealed marked improvement in liver fibrosis.
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