Severe Vaso-Occlusive and Ocular Decompression Retinopathy Revealing a Sickle Cell Trait in a Patient with Herpetic Uveitis.

PURPOSE: To describe a patient with hypertensive herpetic uveitis complicated by arterial retinal occlusions and a decompression retinopathy revealing a sickle cell trait.

STUDY DESIGN: Case report.

RESULTS: A 24-year-old African man presented with a hypertensive herpetic keratouveitis. A brutal lowering of the intraocular pressure (IOP) by systemic acetazolamide resulted in a ocular decompression retinopathy and multiple arterial occlusions involving the macular and the mid-periphery retina. A hemoglobin electrophoresis revealed a sickle cell trait.

CONCLUSION: Under rare circumstances, vaso occlusive events can occur in patients with a sickle cell trait. We identified high IOP and acetazolamide to be responsible of an increased blood viscosity and a reduction of the vessels' caliber, resulting in sickling and arterial retinal occlusions. We recommend a thorough anamnesis and a sickle cell screening for patients of African or Mediterranean descent with acute elevated IOP, especially if they have to be treated with carbonic anhydrase inhibitors. Abbreviations: HbA: Hemoglobin A; HbS: Hemoglobin S; HSV1: Herpes Simplex Virus - 1; IOP: IntraOcular Pressure; OCT-A: OCT-Angiography; SD-OCT: Spectral Domain Optical Coherence Tomography.