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Incidence and natural history of proliferative sickle cell retinopathy: observations from a cohort study
Susan M Downes1, Ian R Hambleton, Elaine L Chuang
1MRC Laboratories, Kingston, Jamaica.
Insights
Proliferative sickle cell retinopathy (PSR) is more common and severe in sickle cell C (SC) disease than sickle cell SS disease. Spontaneous regression of PSR occurs in 32% of affected eyes, with permanent vision loss being rare.
Area of Science:
- Ophthalmology
- Hematology
- Genetics
Background:
- Sickle cell retinopathy (SCR) is a vision-threatening complication of sickle cell disease.
- Proliferative sickle cell retinopathy (PSR) represents an advanced stage of SCR.
- Understanding the natural history of PSR is crucial for patient management.
Purpose of the Study:
- To determine the incidence, prevalence, and natural history of proliferative sickle cell retinopathy (PSR).
- To compare the occurrence and progression of PSR in patients with sickle cell SS (SS) disease and sickle cell C (SC) disease.
Main Methods:
- Prospective longitudinal study over 20 years.
- Newborn screening identified children with SS and SC disease.
- Annual ophthalmic examinations included angiography and angioscopy to document retinal vascular changes.
Main Results:
- PSR developed in 14% of SS and 43% of SC disease patients by ages 24-26.
- Annual incidence rates were 0.5% for SS and 2.5% for SC disease.
- Spontaneous regression of PSR occurred in 32% of affected eyes; permanent visual loss was uncommon.
Conclusions:
- SC disease exhibits a higher incidence and severity of PSR compared to SS disease.
- A significant proportion of PSR cases undergo spontaneous regression.
- Long-term follow-up indicates a low risk of permanent vision loss from PSR in young adulthood.
Objective:
To describe the incidence, prevalence, and natural history of proliferative sickle cell retinopathy (PSR).
Design:
Prospective longitudinal study over 20 years.
Participants:
Newborn screening of 100000 consecutive deliveries from 1973 to 1981 identified 315 children with homozygous sickle cell (SS) disease and 201 with SS-hemoglobin C (SC) disease. By the age of 5 years, 307 SS patients and 166 SC patients were alive and living in Jamaica and were recruited for this ophthalmic study.
Methods:
Description of retinal vascular changes on annual angiography and angioscopy.
Main Outcome Measures:
Incidence and prevalence of PSR and its behavior on follow-up. Progression of PSR was investigated using the number of eyes affected (none, one, both) and the interval until PSR onset.
Results:
At last review in January 2000, PSR had developed in 59 patients (14 SS, 45 SC), unilaterally in 36 patients and bilaterally in 23. Incidence increased with age in both genotypes, with crude annual incidence rates of 0.5 cases (95% confidence interval [CI], 0.3-0.8) per 100 SS subjects and 2.5 cases (95% CI, 1.9-3.3) per 100 SC subjects. Prevalence was greater in SC disease, and by the ages of 24 to 26 years, PSR had occurred in 43% subjects with SC disease and in 14% subjects with SS disease. Patients with unilateral PSR had a 16% (11% SS, 17% SC) probability of regressing to no PSR and a 14% (16% SS, 13% SC) probability of progressing to bilateral PSR. Those with bilateral PSR had an 8% (8% SS, 8% SC) probability of regressing to unilateral PSR and a 1% (0 SS, 2% SC) probability of regressing to a PSR-free state. Irretrievable visual loss occurred in only 1 of 82 PSR-affected eyes, and 1 required detachment surgery and recovered normal visual acuity.
Conclusions:
Longitudinal observations over 20 years in a cohort of patients followed from birth confirms a greater incidence and severity of PSR in SC disease, and shows that spontaneous regression occurred in 32% of PSR-affected eyes. Permanent visual loss was uncommon in subjects observed up to the age of 26 years.
