Operative technique and results of subtotal splenectomy for Gaucher disease.

Patients with type 1 (adult form) Gaucher disease complicated by massive splenomegaly and hypersplenism have previously been treated by total splenectomy. Subtotal splenectomy in Gaucher disease will retain splenic tissue which may protect the patient from the risk of overwhelming sepsis. Removal of at least 85 per cent of the splenic mass is necessary to eliminate hypersplenism and to decrease splenic size. The surgical technique and outcome of four patients undergoing subtotal splenectomy for massive splenomegaly due to type 1 Gaucher disease are discussed.