Retinal artery and vein occlusion in calciphylaxis

Area of Science:

• Ophthalmology
• Nephrology
• Vascular Medicine

Background:

• Calciphylaxis is a rare systemic disease characterized by medial vascular calcification, leading to ischemia.
• Ocular manifestations like anterior ischemic optic neuropathy and crystalline retinopathy are reported but uncommon.
• This study focuses on a unique presentation of multiple retinal vascular events in a calciphylaxis patient.

Observation:

• A 52-year-old female with a history of branch retinal artery occlusion (BRAO) presented with vision loss and a new scotoma.
• Imaging revealed extensive vascular calcifications and delayed retinal perfusion.
• The patient had end-stage renal disease, secondary hyperparathyroidism, and confirmed calciphylaxis.

Findings:

• The patient experienced sequential branch retinal artery occlusion (BRAO), branch retinal vein occlusion (BRVO), and paracentral acute middle maculopathy (PAMM).
• Optical coherence tomography (OCT) showed middle retinal hyperreflectivity, indicative of PAMM.
• Fluorescein angiography (FFA) confirmed delayed perfusion in the superior retinal arcade.

Implications:

• Calciphylaxis should be considered in the differential diagnosis for patients with end-stage renal disease presenting with diverse ophthalmic vascular events.
• This case expands the spectrum of known ocular manifestations of calciphylaxis.
• Early recognition and consideration of calciphylaxis may improve patient outcomes by enabling timely management.