Abstract
Purpose: :
To establish patterns of presentation and the incidence of treatable sickle retinopathy in patients with sickle cell disease.
Methods: :
Patients aged above 16 years with sickle cell disease (genotypes SS, SC, Sbthal) attending the sickle clinic were included in the study. Patients were examined during their haematology visit in the period between October 2008 and August 2009. Patients with ocular symptoms referred to eye outpatients and eye casualty were also included. Examination included Snellen visual acuity, slit lamp examination of the anterior segment and dilated fundal examination.
Results: :
471 patients are registered with sickle haemoglobinopathy (genotypes SS, SC, Sbthal) in St Thomas’ hospital. Over a 10 month period 144 of these patients attended the haematology clinic and were reviewed prospectively. An additional 51 patients were included from retrospective review of notes of patients attending eye clinic prior to and during this 10 month period. Thus 193 cases were reviewed by an ophthalmologist. Age ranged 17-73 (Mean age 36). Male to female ratio was 1:1.5.23% (44/193) patients had no retinopathy, 27% (53/193) had non-proliferative sickle retinopathy, 28% (55/193) had stage 1-3 proliferative sickle retinopathy (Goldberg classification), and 17% (33/193) had stage 4-5 including resolved vitreous haemorrhage (VH) and stable or previously treated retinal detachment (RD).127 patients were asymptomatic and 66 patients were symptomatic. Symptoms included: reduced vision- 49 patients; distortion - 2 patients; floaters- 7 patients; headache and nausea- 1 patient (papilloedema due to benigh intracranial hypertension); epiphora/discharge-2. Causes of reduced vision included: acute loss of vision due to RD- 1, loss of vision following previously treated RD- 5, low grade VH- 2, previous incipient CRVO- 1, previous CRAO- 1, glaucoma-1, other- 38 patients.Only one patient required treatment for sickle retinopathy. She presented with sudden loss of vision and underwent surgery for tractional rhegmatogenous RD secondary to stage 5 PSR. One asymptomatic patient underwent prophylactic laser retinopexy for a small localised round hole RD not related to sickle retinopathy.
Conclusions: :
Our series shows that patient who required treatment presented with acute loss of sight and none of the asymptomatic patients required treatment for complications of sickle retinopathy.
Keywords: retina • clinical (human) or epidemiologic studies: treatment/prevention assessment/controlled clinical trials