Cirurgias conservadoras do baço para tratamento da doença de Gaucher

Abstract

Complications of splenomegaly in Gaucher's disease, such as hypersplenism and mechanical compressions, have been treated by partial splenectomy. However, the splenic remnant, vascularized by the hilar vessels, may enlarge leading to recurrent splenomegaly and all its adverse effects. More than eighteen years of experience treating portal hypertension, splenic trauma, myeloid splenomegaly, splenomegalic somatic and sexual hypodevelopment, chronic lymphocytic lymphoma and cystadenoma of pancreatic tail by subtotal splenectomy, maintaining the upper pole supplied only by the splenogastric vessels showed that the splenic remnant does not increase its size. Based on evidence that the hilar vessels may contribute to the increase of the splenic remnant, we performed in five cases with Gaucher's disease subtotal splenectomy, maintaining the upper pole supplied only by the splenogastric vessels. The splenic remnant did not modify in size during a postoperative follow-up of more than twelve years, and the hematological parameters remained normal. Another patient whose splenogastric vessels were insufficient to maintain the upper splenic pole, was submitted to total splenectomy and autologous splenic tissue transplantation on the greater omentum. The autotransplant survived and presented with function. All six patients had uneventful postoperative follow-up in relation to the splenic conservative procedure. In conclusion, in presence of giant splenomegaly with severe hematologic and clinical symptoms the spleen must be resected in a conservative fashion such as subtotal splenectomy or total splenectomy followed by autotransplants of splenic tissue.