Brian K Do1, Damien C Rodger. 1. aUSC Roski Eye Institute, Keck School of Medicine bUSC Institute for Biomedical Therapeutics, University of Southern California, Los Angeles, California, USA.
Abstract
PURPOSE OF REVIEW: To review recent literature pertaining to sickle cell retinopathy (SCR) and, in particular, sickle cell maculopathy. RECENT FINDINGS: Several recent studies suggest that macular perfusion abnormalities seen in patients with sickle cell disease of various genotypes may affect both the superficial and deep capillary plexi, with a predilection for the deep capillary plexus. Further, these changes may be associated with areas of macular thinning, as well as with peripheral retinal ischemia, even in individuals without visual symptoms, contrary to what has previously been described in both diabetic retinopathy and retinal vein occlusion. Several cases also suggest that paracentral acute middle maculopathy may be the pathophysiologic mechanism by which microvascular occlusion leads to macular thinning. SUMMARY: Sickle cell disease can manifest in a number of ways within the orbit as well as intraocularly because of its nonspecific vasoocclusive episodes. However, SCR is the most common ophthalmic manifestation of this disease. Historically, SCR has been considered a peripheral retinopathy, but the development and use of spectral-domain optical coherence tomography and optical coherence tomography angiography suggest that significant macular vascular changes occur early in this disease, even in asymptomatic individuals.
PURPOSE OF REVIEW: To review recent literature pertaining to sickle cell retinopathy (SCR) and, in particular, sickle cell maculopathy. RECENT FINDINGS: Several recent studies suggest that macular perfusion abnormalities seen in patients with sickle cell disease of various genotypes may affect both the superficial and deep capillary plexi, with a predilection for the deep capillary plexus. Further, these changes may be associated with areas of macular thinning, as well as with peripheral retinal ischemia, even in individuals without visual symptoms, contrary to what has previously been described in both diabetic retinopathy and retinal vein occlusion. Several cases also suggest that paracentral acute middle maculopathy may be the pathophysiologic mechanism by which microvascular occlusion leads to macular thinning. SUMMARY: Sickle cell disease can manifest in a number of ways within the orbit as well as intraocularly because of its nonspecific vasoocclusive episodes. However, SCR is the most common ophthalmic manifestation of this disease. Historically, SCR has been considered a peripheral retinopathy, but the development and use of spectral-domain optical coherence tomography and optical coherence tomography angiography suggest that significant macular vascular changes occur early in this disease, even in asymptomatic individuals.
Authors: Eid Ayed Almasoudi; Sultan Fahad Magliah; Abubakr Salem Alzwaihri; Abdullah Omar Aljuwaybiri; Abdullah Saleh Alqahtani Journal: Ann Med Surg (Lond) Date: 2022-06-21
Authors: Ashraf Abdelkader; Mohamed Shaaban; Mahmoud M Zahran; Mostafa F Mohammed; Anas M Ebrahim; Ahmed I Galhoom Journal: Int J Clin Pract Date: 2022-08-29 Impact factor: 3.149