Hepatobiliary and Pancreatic: Liver transplantation for massive hepatic lymphangiomatosis

摘要

A young male, aged 14, was investigated because of anorexia and progressive abdominal distension over the preceding 12 months. At the age of 5 years, he was diagnosed with ileal lymphangiomatosis and was treated with a small bowel resection. On examination of his abdomen, there was asymmetrical abdominal distension (more pronounced on the right side) and some blue discoloration of the skin over the right upper quadrant. His liver was large and irregular and extended to the pelvic brim. He also had splenomegaly, 4 cm below the left costal margin. Blood tests revealed a mild elevation of bilirubin (27 mumol/L, 1.6 mg/dL) and gamma glutamyl transpeptidase (47 U/L). He also had a normo-cytic anemia (hemoglobin 104 g/L), a low white cell count (2.9 x 109/L), a low platelet count (116 x 109/L) and a prolonged INR (1.4) and APTT (44 seconds). A magnetic resonance imaging scan (T2 coronal, fat-suppressed, Figure 1 left and Tl coronal, post-contrast, Figure 1 right) showed a large and irregular liver that was largely replaced by multiple large cystic structures.