The role of splenectomy in homozygous sickle cell disease in childhood - abstract

ABSTRACT

The persistence of splenomegaly in children with homozygous sickle cell (SS) disease can pose difficult management problems for the clinician. In the young child the prognosis of acute spenic sequestration (ASS) is worse if superimposed on hypersplenism and in the older child the development of chronic hypersplenism may have a wide range of detrimental effects on the child's health. Elective splenectomy abolishes the risk of further acute, or chronic, sequestration but the increased risk of fulminant sepsis following splenectomy and the known propensity to spontaneous atrophy counsels for conservative mangement. To understand better the role of splenectomy in SS disease the outcome of elective splenectomy in 60 Jamaican children is reviewed. The age at time of operation ranged from 9 months to 17 years, with a mean of 6 years and the period of follow up ranged from 1 month to 19 years. The principal indications were recurrent ASS under the age of 2 years and persistent hypersplenism in those over this age. There was no mortality or significant morbidity during the operative period. Two deaths occurred, one from pneumonia 11 months post operatively and one from unknown causes 30 months post operatively. The haematological response to splenectomy was striking, with significant sustained increases in haemoglobin, platelets and red cell survival times, and fall in reticulocyte count. In the last five years prophylaxis with penicillin and/or pneumococcal and Haemophilus influenzae vaccine has been given to most patients and may have improved the prognosis, but in 54 patients splenectomy precede this period. Elective splenectomy with modern surgical and anaesthetic techniques carries minimal morbidity and with careful patient selection (two episodes of ASS, or sustained hypersplenism) the procedure may confer considerable benefit on selected children with SS disease (AU)