Near-total splenectomy: a new technique for the management of hereditary spherocytosis.

Abstract

The authors used a new surgical technique of near-total splenectomy (NTS) and report their experience. Total splenectomy is indicated for the management of patients with hereditary spherocytosis but may be complicated by severe infections and thromboembolic events. Studies have shown that partial or subtotal parenchymal resections can lead to excessive regeneration of the residual parenchyma. The resulting onset of hemolysis requires total splenectomy in a significant portion of patients. Our hypothesis was that a more radical approach to open resection permanently decreases recurrent hemolysis while potentially ensuring immune function. This longitudinal cohort study included 42 patients with moderate to severe hereditary spherocytosis who underwent NTS according to an open procedure developed by the authors. The end criterion was to conserve a remnant spleen of 10 cm in size. Patient age ranged between 2 and 42 years. Mean resected spleen weight was 580 g; mean remnant volume was 10 cm (range, 8-11 cm). A surgical complication (loss of spleen) occurred in 1 patient. Six-month to 6-year follow-up data was available on 22 patients; 21 of 22 showed preserved phagocytosis and normal blood circulation of the remnant; 1 of 22 experienced secondary remnant necrosis. On average, the remnant spleen grew back to four and a half times its postoperative size. No patients required transfusions, developed gallstones, or symptomatic hemolysis. This new technique of NTS is safe, effective, and can minimize the late sequelae of secondary splenectomy.