Successful treatment of hepatic venocclusive disease in a bone marrow transplant patient with side-to-side portacaval shunt.
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.
D000293:Adolescent; D001706:Biopsy; D016026:Bone Marrow Transplantation; D003131:Combined Modality Therapy; D005260:Female; D006503:Hepatic Veins / Q000473:pathology; D006504:Hepatic Veno-Occlusive Disease / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007951:Leukemia, Myeloid / Q000150:complications / Q000628:therapy; D011167:Portacaval Shunt, Surgical