Abstract

Splenomegaly is common in sickle cell disease (SCD) and can lead to complications. In order to evaluate its prevalence and evolution in Senegalese children with SCD, we analysed the records of 698 patients (94.6% SS, 4.4% SC and 1% S-beta-thalassemia) followed up at the Albert Royer Children’s Hospital in Dakar from January 1991 to December 2005.

Age, clinical course specifying the size of the spleen beyond the costal margin and its evolution were the main data recorded.

The average age of subjects when included in the cohort was 7 years. Splenomegaly was observed in 122 patients (17.5%), measuring 1–17 cm beyond the costal margin (average 4.7 cm). It was more frequent in infants aged 0–12 months (25.6%) and in SC patients (32.3%) compared with SS (16.5%), p = 0.025. The evolution of splenomegaly was marked by a subsequent reduction in 17.3% of cases, a total reversal in 43.4% and no size variation in 39.3%. Acute splenic sequestration occurred in two SS cases aged 29 and 32 months and was managed with blood transfusion. Seven sicklers (six SS and one SC) aged 4–13 years presented with hypersplenism. Splenectomy was performed in four cases, whereas the complication reversed after two to three blood transfusions in the three remaining cases.

The prevalence of splenomegaly is relatively low in Senegalese children with SCD. The risk of complications necessitates monitoring of the spleen and to teach parents palpation of the baby’s abdomen for the early management of splenic sequestration.