Location
Philadelphia, PA
Start Date
17-4-2026 1:30 PM
End Date
17-4-2026 2:30 PM
Description
Introduction: Hypertensive retinopathy is a relatively common pathological finding in individuals with elevated blood pressure. Acute spikes in blood pressure may cause pathophysiologic changes that may trigger rapid changes in the posterior pole of the eye. On the other hand, thrombotic microangiopathy (TMA) is rare with a multitude of causes ranging from complement-mediated thrombotic microangiopathy TMA, also known as atypical hemolytic uremic syndrome (aHUS), to severe hypertension. This case exemplifies an episode of malignant hypertension with acute-on-chronic TMA causing retinal ischemia. Initial visual assessment noted scotomas nasally and temporally on central visual field testing, along with uniocular reduced color vision of the right eye. Fundoscopic examination revealed peripapillary cotton wool spots (CWS) 360 degrees around the optic nerve. The inciting pathophysiology in this unique case of a young adult female is unknown; severe hypertension may have caused acute TMA or vice versa.
Methods: Sequential ocular examination and appropriate testing
Results: At two-week follow up, the patient’s visual scotomas were symptomatically improved, distance visual acuity improved to 20/20, and color deficits resolved. On repeat dilated fundus examination, bilateral CWS were significantly reduced. Optical coherence tomography revealed decreasing bilateral optic nerve edema. Blood pressure was better controlled with medications. Empiric eculizumab was ultimately started as an outpatient for acute-on-chronic TMA diagnosed by renal biopsy.
Discussion: Case reports and literature review were performed.
This 31-year-old female may have been predisposed to the development of TMA given her race and past history of gestational hypertension, both of which are known risk factors for acute TMA. Chronic TMA results in sustained endothelial injury, fibrinoid necrosis, and occlusive vasculopathy, which may have contributed to this patient’s solely peripapillary vascular instability and hypertensive damage. The presence of peripapillary CWS suggests retinal ischemia consistent with both TMA-related microvascular occlusion and malignant hypertension, although TMA may have influenced the disease progression due to its propensity for microvascular occlusion. Following treatment, repeat fundus examination showed marked resolution of CWS, indicating recovery of retinal perfusion and confirming effective systemic disease management. Sequential digital photography of the optic disc and macula showed large arterioles and small arteries around the optic disc and a central foveal avascular zone with small arterioles around the macula. Further research on how these structures are differentially impacted by hypertension in the setting of acute on chronic TMA may prove advantageous in understanding ocular vascular disease progression. The pathophysiologic linkage between acute on chronic TMA and malignant hypertension should also be studied further for a better understanding of vascular disease in the eye. This is a unique case of hypertensive retinopathy in the setting of underlying systemic disease that presented with vague visual changes consisting of multiple transient scotomas. Consideration should be given in the differential diagnosis for new onset visual changes.
Embargo Period
6-4-2026
Included in
Hypertensive Scotomas in the Setting of Thrombotic Microangiopathy
Philadelphia, PA
Introduction: Hypertensive retinopathy is a relatively common pathological finding in individuals with elevated blood pressure. Acute spikes in blood pressure may cause pathophysiologic changes that may trigger rapid changes in the posterior pole of the eye. On the other hand, thrombotic microangiopathy (TMA) is rare with a multitude of causes ranging from complement-mediated thrombotic microangiopathy TMA, also known as atypical hemolytic uremic syndrome (aHUS), to severe hypertension. This case exemplifies an episode of malignant hypertension with acute-on-chronic TMA causing retinal ischemia. Initial visual assessment noted scotomas nasally and temporally on central visual field testing, along with uniocular reduced color vision of the right eye. Fundoscopic examination revealed peripapillary cotton wool spots (CWS) 360 degrees around the optic nerve. The inciting pathophysiology in this unique case of a young adult female is unknown; severe hypertension may have caused acute TMA or vice versa.
Methods: Sequential ocular examination and appropriate testing
Results: At two-week follow up, the patient’s visual scotomas were symptomatically improved, distance visual acuity improved to 20/20, and color deficits resolved. On repeat dilated fundus examination, bilateral CWS were significantly reduced. Optical coherence tomography revealed decreasing bilateral optic nerve edema. Blood pressure was better controlled with medications. Empiric eculizumab was ultimately started as an outpatient for acute-on-chronic TMA diagnosed by renal biopsy.
Discussion: Case reports and literature review were performed.
This 31-year-old female may have been predisposed to the development of TMA given her race and past history of gestational hypertension, both of which are known risk factors for acute TMA. Chronic TMA results in sustained endothelial injury, fibrinoid necrosis, and occlusive vasculopathy, which may have contributed to this patient’s solely peripapillary vascular instability and hypertensive damage. The presence of peripapillary CWS suggests retinal ischemia consistent with both TMA-related microvascular occlusion and malignant hypertension, although TMA may have influenced the disease progression due to its propensity for microvascular occlusion. Following treatment, repeat fundus examination showed marked resolution of CWS, indicating recovery of retinal perfusion and confirming effective systemic disease management. Sequential digital photography of the optic disc and macula showed large arterioles and small arteries around the optic disc and a central foveal avascular zone with small arterioles around the macula. Further research on how these structures are differentially impacted by hypertension in the setting of acute on chronic TMA may prove advantageous in understanding ocular vascular disease progression. The pathophysiologic linkage between acute on chronic TMA and malignant hypertension should also be studied further for a better understanding of vascular disease in the eye. This is a unique case of hypertensive retinopathy in the setting of underlying systemic disease that presented with vague visual changes consisting of multiple transient scotomas. Consideration should be given in the differential diagnosis for new onset visual changes.