Screening for opthalmic manifestations of sickle cell disease in the United Kingdom

ABSTRACT

There are marked variations in the manifestations of sickle cell disease in different populations. The occular complications of this condition amongst the Afro-Caribbeans living in the United Kingdom have not previously been reported. We present the preliminary results of an opthalmic screening programme at King's College Hospital, London. One hundred eyes of 50 patients with sickle cell disease were assessed. Full ocular examination was performed including fundus fluorescein angiography. We have looked at the haemotological and clinical profile of the patients involved as well as the number of days spent in the hospital during the year preceding the examination. The incidence of Grade 11 retinopathy was found to be significantly higher than grade 1 in SC disease. This concurs with the results of the Jamaican screening and confirms that the patients are at higher risk of visual impairment than those wuth the SS disease. Our results also agrees with the Jamaican experience which suggests that visual morbidity is mostly due to complications of proliferative sickle cell retinopathy (PSR). However, the findings in patients without proliferative changes are different; in particular, angloid streaks leading to disciforms are an important cause of visual loss in Jamaica, but were not seen in any of the 98 eyes examined in this study. No correlation was found between the grade of retinopathy and age, sex, systemic complications and various haematological parameters except for the percentage of haemoglobin F, which was significantly higher in patients with grade I (7.6) compared with grade II (4.2) retinopathy (p=0.0127)(AU)