Role of splenectomy in homozygous sickle cell disease in childhood.

60 Jamaican children with homozygous sickle cell (SS) disease underwent splenectomy, 14 for prophylaxis against recurrent acute splenic sequestration and 46 for treatment of sustained hypersplenism. Age at operation varied from 9 months to 16 years. Patients were followed up for 1 month to 27 years (median 6 years), with a total of 369 years of patient-observation. None of the 3 patients who died, at ages 2 1/2, 6 1/2, and 21 years, had received prophylaxis against infection. Overwhelming sepsis was possible but not confirmed in the first two deaths which occurred 11 months and 2 1/2 years after operation; the third died from chronic renal failure 11 years after splenectomy. After operation, there were no confirmed cases of pneumococcal septicaemia or meningitis, and the commonest clinical event was the acute chest syndrome.