Sickle cell retinopathy: Patient awareness, mode of presentation, and treatment modalities in Ibadan, South-West Nigeria

Abstract

Background: Sickle cell retinopathy is a recognized complication of sickle cell disease (SCD) which may lead to visual impairment or blindness. Despite this, many patients with SCD hemoglobinopathy SC and SS are unaware of their genotype, hence resulting in only occasional or no eye checks with possibilities of getting blind. Purpose: The purpose of this study was to describe the genotype awareness, pattern of presentation, and treatment of sickle cell retinopathy in Ibadan. Methods: This was a retrospective review of the case notes of 64 patients with the diagnosis of sickle cell retinopathy seen over two years (January 2018 to December 2019). Sociodemographic characteristics, clinical data, ophthalmic assessment, and treatment performed on patients were extracted onto pro forma. Information obtained included age, sex, sickle cell genotype, genotype awareness from their medical history, retinal findings using Goldberg classification, and treatment modalities for the patients. Data analysis was performed using the IBM SPSS software version 22. Analysis was done using proportions and percentages. Results: Medical records of 64 patients were reviewed. The mean age of the patients was 39.05 ± 10.48 (range: 20–65) years, with a male-to-female ratio of 1.8:1. Sixty (93.8%) patients had genotype SC. Forty-six (71.8%) patients were aware of their genotype. Fifty-six patients presented with Proliferative sickle cell retinopathy (PSR) in the right eye, while 55 had PSR in the left eye. These spanned all the different grades of PSR. Treatment offered at the first visit included laser photocoagulation, intravitreal anti-vascular endothelial growth factor (bevacizumab), vitrectomy, and scleral buckle. At subsequent follow-up visits, detailed ocular examination on patients was done to look out for new/active lesions. If any of these lesions were found, repeat or additional treatment was offered to help stabilize and/or improve the best-corrected visual acuity of patients. Conclusion: This study has demonstrated high genotype awareness among the studied patients. Despite this high awareness, majority of our patients presented with varying stages of proliferative sickle cell retinopathy. While our patients had more than one type of treatment, some defaulted due to lack of funds. Therefore, to improve the quality of life of SCD patients, it is essential for health-care providers and other stakeholders to design policies for sustainable and accessible eye care programs to avoiding needless blindness from sickle cell retinopathy.