Ocular findings in children with severe clinical symptoms of homozygous sickle cell anaemia in Kaduna, Nigeria.
BACKGROUND
Sight-threatening retinopathy in Sickle Cell Disease is thought to be due mainly to vasoocclusion. Yet it is reportedly rarely found in children with Haemoglobin SS, (who most often suffer from vasoocclusion). However, earlier reports included patients with a wide range of clinical severity.


OBJECTIVE
To document ocular pathology in children with Haemoglobin SS with severe clinical disease.


METHODS
Thirty-seven children with severe clinical disease (at least 3 vaso-occlusive episodes in one year) had detailed ocular examinations over a one-year period.


RESULTS
No child (aged 3 to 13 years) had ocular symptoms. Visual acuity was abnormal in one child. Retinal pathology was found only in patients over 8 years. Neovascularization was observed in 3 eyes of 2 patients both of whom had higher than average irreversibly sickled cell counts and haemoglobin levels. Retinal and choroidal infarcts were found in 11 and 2 eyes respectively; sunburst lesions and salmon patch haemorrhages in 5 eyes each. Changes observed over the one-year period in the 32 survivors, were photocoagulation scars in one eye of a child who had undergone laser therapy and resolution of the salmon patch haemorrhages.


CONCLUSIONS
Despite lack of visual symptoms, young children with haemoglobin SS with severe clinical symptoms can develop sight-threatening retinopathy. The possible role of autoinfarction in the causation of these lesions is discussed. Our study shows that routine yearly ophthalmological examinations are essential for children over 8 years.
Eruchalu
U V
UV
Department of Ophthalmology, Ahmadu Bello University Teaching Hospital, Kaduna, Nigeria.
Pam
V A
VA
Akuse
R M
RM
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005123:Eye / Q000098:blood supply; D005260:Female; D006801:Humans; D008028:Light Coagulation; D008297:Male; D009389:Neovascularization, Pathologic; D009549:Nigeria / Q000453:epidemiology; D011446:Prospective Studies; D012164:Retinal Diseases / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D012720:Severity of Illness Index