Effective early treatment of hepatic venoocclusive disease with a central splenorenal shunt in an infant.
Venoocclusive disease of the liver (VOD) is a well-described complication following chemotherapy. It is manifested by jaundice and signs of portal hypertension and carries a mortality rate approaching 50%. There is no known treatment for the disease itself, although several recent reports suggest portacaval diversion may be effective in treating its sequelae. A 6.75-kg 8-month-old boy with VOD following bone marrow ablation and bone marrow transplantation (BMT) for juvenile chronic myelogenous leukemia (JCML) is presented. Over a 6-week period following bone marrow ablation he developed ascites refractory to diuretics, jaundice, and hematemasis with normal hepatocellular function. Splenectomy with a central splenorenal shunt was performed, which resulted in a significant reduction in portal pressures and complete resolution of his ascites and hematemasis without resultant encephalopathy. We propose that central end-to-side splenorenal shunting is an acceptable treatment for portal hypertension due to VOD and can be successfully performed in infants.
Jacobson
B K
BK
Department of Surgery, University of Wisconsin School of Medicine, Madison.
Kalayoglu
M
M
D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D006504:Hepatic Veno-Occlusive Disease / Q000150:complications / Q000209:etiology / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery*; D007223:Infant; D008297:Male; D011168:Portal System / Q000000981:diagnostic imaging; D011859:Radiography; D013164:Splenorenal Shunt, Surgical