Early splenectomy in a large cohort of children with sickle cell anemia: risks and consequences

Abstract

In children with sickle cell anemia (SCA), early splenic complications can mandate splenectomy but the benefit-to-risk ratio and the age at which splenectomy may be safely performed remain unclear. To address this question, we analyzed the rate of post-splenectomy events in children with SCA splenectomized between 2000-2018 at Robert Debré University Hospital (Paris, France). A total of 188 children underwent splenectomy, including 101 (11.9%) from our newborn cohort and 87 referred to our center. Median (Q1-Q3) age at splenectomy was 4.1 yrs (2.5-7.3), with 123 (65.4%) and 65 (34.6%) children splenectomized 77or < 3 years of age, respectively. Median post-splenectomy follow-up was 5.9 yrs (2.7-9.2) yielding 1192.6 patient-years (PY) of observation. Indications for splenectomy were mainly acute splenic sequestration (101 (53.7%)) and hypersplenism 75 (39.9%)). All patients received penicillin prophylaxis, 98.3% received PP23 immunization and 91.9% a median number of 4 (3-4) PCV shots prior splenectomy. Overall incidence of invasive bacterial infection and thrombo-embolic events were 0.005/PY (no pneumococcal infections) and 0.003/PY, respectively, and did not differ according to age at splenectomy. There was an increased proportion of children with cerebral vasculopathy in children splenectomized < 3 years (0.037/PY vs 0.011/PY, p

Stroke without cerebral arteriopathy in sickle cell disease children: causes and treatment

“…The only criterion of severity we found surprisingly high was the rate of history of acute splenic sequestration (44% in the whole cohort of patients, 58% in the group with "atypical" vasculopathy). Recently, our group published the consequence of early splenectomy in a large cohort of children with SCA and showed an increased proportion of cerebral vasculopathy in children with early splenic dysfunction and splenectomized before 3 years old 19 . This relation between splenic dysfunction and neurological complications in children should probably be further investigated.…”