Paracentral acute middle maculopathy associated with peripheral retinal capillary hemangioma.

Abstract

Background: Paracentral acute middle maculopathy (PAMM) has not previously been reported in association with peripheral retinal capillary hemangioma (RCH). We report a case of PAMM associated with a peripheral RCH, with multimodal imaging findings compatible with a localized venous congestion-related ischemic mechanism.

Case presentation: A 36-year-old woman presented with a superior paracentral visual field defect in the right eye with 20/20 vision. Fundus examination revealed multifocal parafoveal whitish lesions and an orange-red capillary hemangioma with surrounding hemorrhages in the inferotemporal peripheral retina, accompanied by venous dilation and tortuosity. Spectral-domain optical coherence tomography (OCT) demonstrated a hyperreflective band involving the inner plexiform, inner nuclear, and outer plexiform layers, consistent with PAMM. OCT angiography showed focal hypoperfusion at the deep capillary plexus with preserved superficial plexus. Ultra-widefield fluorescein angiography revealed early arterial filling, delayed venous drainage, and late peritumoral leakage, suggesting localized venous outflow impairment.

Conclusions: This case broadens the clinical spectrum of RCH-related complications by illustrating a possible association between peripheral RCH, localized venous congestion, and PAMM, highlighting the importance of careful vascular assessment and longitudinal surveillance.