Strokes in children with sickle cell disease at the National Hospital Abuja Nigeria

Abstract

Abstract Background: Strokes occur in sickle cell disease (SCD), and are associated with significant morbidity and mortality.

Objectives: To determine the prevalence of strokes amongst children with SCD, and document the major clinical features, complications, effect of treatment with chronic transfusion therapy (CTT) and outcome.

Methods: A descriptive retrospective study of SCD children with strokes seen at the National Hospital Abuja, Nigeria over a 2.5 year period from January 2009 – June 2012. Data was collected by scrutinizing case files obtained from the hospital medical records unit. Information obtained included demographic data, clinical features, packed cell volume (PCV), brain imaging, long term neurologic deficits, effect of CTT, stroke recurrence and outcome.

Results: There were 31 children with strokes among 596 children with SCD documented in the register, giving a prevalence of 5.2%. Twenty six (26) case notes were retrieved. There were 12 males and 14 females, M: F ratio of 0.9:1; mean age was 6.4 years (SD 3.4) range: 1 year 7 months – 14 years; mean PCV at the time of strokes was 21.1% (SD 3.9) range 14 – 29%. All (100%) had Haemoglobin SS on electrophoresis. Presentations were convulsions 18, inability to use limbs 11, weakness of limbs 10; long term neurological deficits were hemiplegia 11, cognition loss 11. Three (3) children had no deficits. Brain imaging (Computed Tomography Scan and Magnetic Resonance Imaging) done in 16 (61.5%) children showed cerebral atrophy in 10, acute cerebral infarcts in 9, chronic cerebral infarcts in 6, acute intra cranial haemorrhage in 1 and normal imagings in 4 children. Twelve (12) children (46.2%) children had recurrences of stroke ranging in number from 1 to 4, which occurred 6 months to 3 years after the initial stroke. There were no statistical significant differences between the children with recurrences of stroke compared to those
without regarding the age, sex, weight or PCVs p > 0.05. Fifteen (15) children (57.7%) were enrolled in CTT. Two (2) out of 7children (28.6%) that had reglar CTT had stroke recurrence; compared to 5 out of 11 children (45.4%) with no CTT (p > 0.05). Four (4) out of 6 (66.7%) children with irregular CTT and 1 of 2 children who stopped CTT had stroke recurrence.

Outcome: 17 children were alive, 7 were lost to follow up, 1 died and 1 was referred to another center.

Conclusion: Strokes were an important cause of morbidity in Nigerian children with SCD, with major long term neurologic deficits. CTT appeared beneficial in preventing stroke recurrences. Primary prevention strategy by Trans Cranial Doppler ultrasound studies of the cerebral arteries, with the aim of promptly initiating appropriate preventive therapy for stroke is strongly  advocated.

Keywords: Sickle cell disease, Stroke, Children, Chronic Transfusion Therapy