Paracentral Acute Middle Maculopathy in IRVAN Syndrome: A Case Report Highlighting Vascular Mechanisms
Ana Paula Couto1, Giovanna Provenzano1, Bruno F Fernandes2
1Department of Ophthalmology, Hospital Universitario Antonio Pedro, Fluminense Federal University (UFF), Niteroi, Brazil.
Purpose:
To describe a rare case of paracentral acute middle maculopathy (PAMM) in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome, highlighting potential vascular mechanisms underlying this association.
Methods:
Observational Case report.
Results:
An 18-year-old male previously diagnosed with bilateral IRVAN syndrome presented with a sudden paracentral scotoma in the left eye. Multimodal imaging showed multiple macroaneurysms, retinal hemorrhages, focal arteriolar vasculitis, and lipid exudation in both eyes. Swept-source optical coherence tomography (SS-OCT) of the left eye revealed a hyperreflective band in the inner nuclear layer in the paracentral temporal superior macular area, consistent with PAMM, without clear evidence of arterial compression. A conservative approach was chosen, and after three years of follow-up, retinal features and visual acuity remained stable.
Conclusion:
PAMM may manifest in IRVAN independent of detectable localized aneurysm compression of the deep capillary plexus. The findings underscore the importance of multimodal imaging and long-term monitoring to assess ischemic complications. Further investigations are warranted to clarify the pathophysiological relationship between these conditions.
Insights
This case report describes paracentral acute middle maculopathy (PAMM) in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Findings suggest PAMM can occur in IRVAN syndrome without direct arterial compression, highlighting vascular mechanisms.
Area of Science:
- Ophthalmology
- Retinal Vascular Diseases
- Macular Disorders
Background:
- Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare condition characterized by inflammation and vascular abnormalities in the retina.
- Paracentral acute middle maculopathy (PAMM) is an ischemic condition affecting the inner nuclear layer of the retina.
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