Combined CRVO with CRAO in a patient with protein C deficiency

To study the underlying factors in a young patient having central retinal venous obstruction with central retinal arterial obstruction and its effects on visual outcome.

A 33-year-old man presented with a complaint of sudden loss of vision in his right eye. A complete ophthalmic evaluation with fundus angiography showed combined central retinal venous obstruction with central retinal arterial obstruction. Detailed investigations revealed reduced (20%) functional assay of protein C suggestive of the diagnosis of severe Type II protein C deficiency with Factor V Leiden mutation. This ocular event was the first sign of the underlying disorder.

The ophthalmic event rapidly progressed to no perception of light. Anticoagulant therapy was started to prevent life-threatening systemic complications. Despite antiglaucoma medications, intraocular pressure was high and cyclocryotherapy was advised.

Retinal vascular disorders in the younger population have different underlying risk factors than in the older age group. Clinical presentation, severity, and management also depend on causative factors. Combined retinal artery and vein occlusion is very uncommon. Combined cases have mostly been attributed to rheological causes like thrombophilia, vessel wall inflammation, and mechanical compression. Protein C deficiency has mainly been linked to vein occlusions and there is no reference to protein C deficiency as a cause of combined central retinal venous obstruction and central retinal arterial obstruction. Combined cases usually present with severe visual loss and have rapid progression. Young patients having such presentation should be thoroughly evaluated to diagnose underlying factors and initiate appropriate management at the earliest.