Proliferative Retinopathy Associated with Repeated High-Altitude Exposure in a Patient with Sickle Cell Trait

Abstract

Broadhead Geoffrey K., Henry E. Wiley, David Peprah, Kenneth Olumba, and Alisa T. Thavikulwat. Proliferative retinopathy associated with repeated high-altitude exposure in a patient with sickle cell trait. High Alt Med Biol. 23:369-371, 2022.-Sickle cell trait (SCT), a carrier state characterized by one normal copy of the beta-globin gene (producing hemoglobin A) and one abnormal variant (producing hemoglobin S), is typically asymptomatic and very low risk for manifestations of hemoglobinopathy, including development of retinopathy. Reported cases of proliferative retinopathy in patients with SCT have occurred in the context of concurrent ocular or systemic disease. We report a case of an otherwise healthy patient with SCT who developed proliferative retinopathy requiring surgical intervention in the setting of significant exposure to high altitude through increased work hours as a flight attendant in the month leading to her presentation. Significant high-altitude exposure may contribute to development of retinopathy in patients with sickle trait. Practitioners should consider the possibility of sickle cell retinopathy in patients with sickle trait in these circumstances.

Keywords: high altitude; proliferative retinopathy; sickle cell disease; sickle cell retinopathy; sickle cell trait.

FIG. 1.

Ultra-widefield retinal imaging of proliferative retinopathy in both eyes. From top left: (A) Color image of the right retina centered on the posterior pole, showing evidence of prior laser photocoagulation temporally and nasally. (B) Color image of the left retina centered on the posterior pole demonstrating temporal lattice with an atrophic retinal hole. (C) Color image of the left retina steered inferiorly showing inferonasal areas of NV and inferotemporal areas of regressed NV. (D) Mid-phase FA image of the right retina showing inferior retinal nonperfusion (arrows) and leakage from regressed NV (star). (E) Mid-phase FA image of the left retina showing inferior leakage (star) from areas of active NV. (F) Steered inferior late phase FA image of the left retina showing leakage from areas of NV. FA, fluorescein angiogram; NV, neovascularization.