Hepatic venocclusive disease developed in a 14-yr-old white girl after allogenic
bone marrow transplantation from an HLA-identical sibling donor. Clinical diagnosis of venocclusive disease was based on the development of
ascites,
hepatomegaly, and
jaundice 3 wk after
transplantation. Current treatment of hepatic venocclusive disease is ineffective. The pathophysiology of the hepatic lesion suggests that construction of a side-to-side
portacaval shunt should be beneficial in relieving the
ascites and preventing further hepatic damage. Because the
ascites was refractory to medical
therapy and she was clinically deteriorating, a side-to-side
portacaval shunt was performed. Histologic examination of a liver biopsy specimen obtained at surgery documented the presence of venocclusive disease. Postoperatively, the patient diuresed and returned to her baseline weight. One year after surgery the patient was doing well, her weight was stable, and she was being maintained on
salt restriction alone. While the resolution of
ascites and improvement of hepatic function in our patient after side-to-side
portacaval shunt does not guarantee that such an approach will be uniformly successful, it should serve to encourage others to consider such
therapy for this frequently devastating complication of chemoradiation
therapy.