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 novel case of PAMM associated with a peripheral RCH, illustrating a venous congestion–driven 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 demonstrating an association between peripheral RCH, localized venous congestion, and PAMM, highlighting the importance of careful vascular assessment and longitudinal surveillance.