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. AIM: 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. CONCLUSION: 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.

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. Aim: 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. Conclusion: 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

A preliminary review of extracapsular cataract extraction with posterior chamber intraocular lens implantation in Kaduna, Nigeria.

BACKGROUND: Extracapsular cataract extraction with posterior chamber intraocular lens implantation came into vogue recently in many ophthalmic centres in Nigeria for the management of cataract and its accompanying aphakia. Evaluation of this procedure in the hands of surgeons who converted newly to extracapsular cataract extraction with intraocular lens implant microsurgery was reviewed. This is with the view to assess and improve on their surgical skill considering their delayed take off due to lack of facility in the centre. METHOD: A retrospective study of the first 48 patients (50 eyes) who had extracapsular cataract extraction with posterior chamber intraocular lens implantation between September 1999 and December 2000 was carried out. RESULT: Forty-six patients had extracapsular cataract extraction with posterior chamber intraocular lens implant in one eye only, while 2 had the procedure in both eyes. Fifty-six (56%) percent of the patients were above 55 years old. Male to female ratio was 1.8:1.0. The preoperative visual acuity in 92% of eyes was equal to or less than 3/60. Six (6%) percent of the eyes had good visual outcome (6/6-6/18), 70% had borderline (6/24-6/60) on the 1st postoperative day. Visual outcome improved steadily with the passage of time as the immediate postoperative complications resolved. The main intra-operative complications were large anterior capsular tags (35.7%) and cortical lens remnants (50%); while striae keratopathy and corneal oedema (54.5%) constituted the main postoperative complications. CONCLUSION: Good visual outcome of greater than 80% with available correction is possible in the early postoperative period. Improved surgeons skill through re-training and refresher courses will guarantee a good outcome. Finally, microsurgical facilities must be put in place before a conversion course or training.