Ocular manifestations of primary thrombotic microangiopathies : a descriptive systematic review

Primary thrombotic microangiopathies (TMAs) include Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS), which may cause ocular signs that need to be detected and managed accordingly. Therefore, we aim to identify the ocular manifestations caused by the systemic effects of primary TMAs, including HUS and TTP.

We searched PubMed, Scopus, and Web of Science databases from inception until March 2024. We included case reports, case series, and retrospective reviews reporting ocular manifestations of primary HUS or TTP. We excluded experimental studies, papers written in a language other than English, studies that included patients with secondary TTP or HUS, pregnant patients, patients with ocular trauma or surgery, and patients with other comorbidities that impair the detection of ocular findings.

We included 37 studies comprising 58 patients (mean age 18.87 ± 12.4 years), with a mean age of 18.87 years. The most common ocular manifestations were retinal hemorrhages (25.86%, n = 15), Purtscher-like retinopathy (24.13%, n = 14), retinal detachment (20.68%, n = 12), optic disc changes (18.96%, n = 11), capillary non-perfusion on fundus fluorescein angiography (FFA) (13.79%, n = 8), and isolated cotton-wool spots (12.06%, n = 7). Rarer manifestations were capillary leakage on FFA (n = 5, 8.6%), vascular tortuosity (8.6%, n = 5), retinal ischemic changes (8.6%, n = 5), sluggish pupil reactivity (6.9%, n = 4), vitreous hemorrhage (6.9%, n = 4), early hypofluorescence (6.9%, n = 4), hypopigmented subretinal lesions (6.9%, n = 4), shifting subretinal fluids (5.2%, n = 3), and attenuated retinal arterioles (5.2%, n = 3).

We found that retinal hemorrhage, Purtscher-like retinopathy, retinal detachment, optic disc changes, capillary non-perfusion on FFA, and isolated cotton-wool spots were the most commonly reported ocular manifestations of primary TMAs. Identification of these manifestations will help to adequately detect and manage them in patients with primary TMAs. We recommend further investigations that provide reliable methods of prevention of those sequelae in patients with primary TMAs.

Not applicable.