Fatal hemorrhage caused by disease progression after partial splenectomy for type III Gaucher's disease

An 18-month-old boy with Gaucher's disease, massive hepatosplenomegaly, and hypersplenism underwent partial (85%) splenectomy and recovered without complication. During the next 4 months, his residual splenic segment showed progressive enlargement from 2 to 6 cm below the left costal margin. By this time, genetic typing had indicated central nervous system involvement (type III). A compatible donor was not available for bone marrow transplantation. Complete splenectomy was not recommended despite continued enlargement of the organ into the pelvis and across the abdominal midline by 7 1/2 months postoperatively. The next week he presented to the emergency room in shock with an hematocrit of 6% and a platelet count of 18,000/mm3. During emergency surgery, massive intraabdominal hemorrhage was noted from a splenic rupture on the inferior pole that was opposite the site of prior division. He died in the operating room during attempted splenectomy. This is a report of fatal outcome after partial splenectomy for type III Gaucher's disease. Patients with subacute neuronopathic disease may not be candidates for subtotal splenectomy.