title: Fatal hemorrhage caused by disease progression after partial splenectomy for type III Gaucher's disease.
abstract: 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.
authors.affiliation: Department of Pediatric Surgery, Vanderbilt University Medical Center, Nashville, TN.
authors.forename: G W
authors.initials: GW
authors.lastname: Holcomb
authors.forename: H L
authors.initials: HL
authors.lastname: Greene
mesh_terms: D017809:Fatal Outcome; D005776:Gaucher Disease / Q000145:classification / Q000601:surgery*; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D013156:Splenectomy / Q000379:methods*; D013161:Splenic Rupture / Q000209:etiology*; D013163:Splenomegaly / Q000209:etiology