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ABSTRACT: Clinical manifestations of hereditary spherocytosis, the most common red blood cell membrane disorder, can be controlled by splenectomy. However, concerns regarding exposure of patients to a life long risk for overwhelming infections have restricted its use, especially în children. Subtotal splenectomy, as long as 80% to 90% of the enlarged spleen is removed, is a logical alternative. Subtotal splenectomy was effective în decreasing the hemolytic rate, while maintaining the phagocytic and immune function of the spleen. This surgical procedure should be considered în transfusion-dependent infants and children whit hereditary spherocytosis and în older patients whit erythrocyte membrane defects. Based on our experience în laparoscopy (120 laparoscopic splenectomies) and open subtotal splenectomy (5 cases) we performed 2 laparoscopic subtotal splenectomies in patients with hereditary microspherocytosis with good short term results. We have had no problem with blood loss and no transfusions were needed. The procedure can be performed safely and easily with all the traditional advantages of a minimally invasive approach. In order to evaluate the long term clinical benefit a minimal follow-up of 5 years is needed.
Chirurgia (Bucharest, Romania: 1990) 08/2013; 98(6):571-6. · 0.78 Impact Factor