Fatal overwhelming postsplenectomy sepsis following autologous splenic transplantation in severe congenital osteopetrosis.

We report a boy with severe congenital osteopetrosis who underwent splenectomy at age 4, with simultaneous transplantation of portions of the spleen into the anterior rectus compartments. Despite demonstrated survival and partial functioning of the splenic implants, the patient died 9 years later from an overwhelming postsplenectomy infection (OPSI). This report suggests that the presence of the amount of retained splenic tissue transplanted in this case after splenectomy ultimately provides relatively ineffective protection against OPSI in congenital osteopetrosis.