A 27-year experience with splenectomy for Gaucher's disease

Gaucher's disease is an inherited metabolic condition that causes massive enlargement of the spleen. The accepted treatment is surgical removal of the entire spleen. Because of the risk of fatal infection and the potential for bone and liver disease after splenectomy, partial splenectomy is considered preferable by some surgeons. A retrospective study was undertaken of patients undergoing splenectomy for Gaucher's disease over the past 27 years. The records of 48 patients were reviewed to determine both the short and long-term results of treatment. Total splenectomy was performed on 35 patients (73 percent), and partial splenectomy on 13 patients (27 percent). There was one death following surgery; this patient had undergone total splenectomy. Thirteen patients had postoperative complications. Within an average follow-up period of 25 months, 11 patients (23 percent) who had undergone total splenectomy showed accelerated bone disease. None of the partial splenectomy patients had evidence of accelerated bone disease. Since surgery, eight patients have died; four deaths were from malignancies that occurred after total splenectomy. These findings indicate that both total and partial splenectomy can be performed safely with minimal increased illness. Total splenectomy is associated with more aggressive bone disease and a tendency to develop malignancy. (Consumer Summary produced by Reliance Medical Information, Inc.)