A case of paracentral acute middle maculopathy and retinal vein occlusion: idiopathic occlusive retinal vasculitis or incomplete Behçet's disease?

Background Retinal vascular occlusion in young adults warrants systematic evaluation for systemic causes. Paracentral acute middle maculopathy (PAMM) and retinal vein occlusion are uncommon concurrent findings that can signal occlusive retinal vasculitis even when the work-up is unrevealing. This rare case presentation underscores the importance to assess for systemic vasculitis and to maintain high suspicion for incomplete Behçet disease (BD) during early or atypical presentations. Case presentation A 36-year-old man presented with blurred inferior-temporal vision in the left eye after three days of fever, cough, and fatigue. Past history was notable for Mediterranean origins and infrequent oral aphthosis. Visual acuity was 20/20 in both eyes. The anterior segments showed conjunctival hyperemia without cells or flare. The left fundus exhibited superior venous tortuosity and retinal hemorrhages compatible with incomplete branch retinal vein occlusion; the right eye was normal. Optical coherence tomography demonstrated PAMM, and fluorescein angiography showed delayed filling of the superior temporal vein, suggesting mixed arteriolar and venous occlusion. Visual field testing confirmed an inferior-temporal scotoma.Systemic work-up revealed elevated inflammatory markers and abnormal liver enzymes; infectious, hypercoagulability, autoimmune, cardiovascular, and neurovascular assessments were otherwise unrevealing. Prednisone 40 mg/day was initiated for presumed inflammatory vasculitis. Ten days later, two PEDs developed in the right eye, one associated with new PAMM suggestive of uncontrolled inflammatory occlusive vasculitis. Therapy was escalated to intravenous methylprednisolone (250 mg/day for three days) and initiation of Adalimumab. High-dose intravenous methylprednisolone followed by adalimumab 40 mg every two weeks led to regression of PEDs and resolution of PAMM, with no further vascular occlusion during corticosteroid taper.ConclusionsThis is a rare presentation of mixed retinal vascular occlusion manifesting as PAMM and incomplete retinal vein occlusion in a young male adult. In such cases, clinicians should perform extended work-up for systemic vasculitis and keep a high index of suspicion for early manifestations of BD. Systemic corticosteroids and prompt immunosuppression should be considered to avoid progression to visual loss.