Hepatomegaly and multiple liver lesions.

Accepted 21 January 1998 A 33-year-old man was referred for possible liver transplantation. The patient was initially diagnosed at birth when he presented with an enlarged liver and episodes of hypoglycaemia. A liver biopsy at the time showed pale hepatic cells by virtue of cytoplasmic granularity and periportal nuclear ballooning (figure 1). He was treated initially with dietary modifications but subsequently required night time dextrose and corn starch. Failed medical therapy prompted referral for liver transplant evaluation. Physical examination showed massive hepatomegaly. Liver function tests were abnormal with a significantly raised alkaline phosphatase and transaminase. Sonography showed hepatomegaly with multiple focal lesions unchanged in size since ultrasound 3 years earlier. Computed tomography (CT) showed multiple well-defined low-attenuation lesions throughout the liver. The largest of these measured 8 x 8 x 4 cm and contained foci of coarse calcification (figure 2).