BILATERAL PARACENTRAL ACUTE MIDDLE MACULOPATHY IN A PEDIATRIC PATIENT WITH SICKLE CELL DISEASE.
Charu Vyas, Sitara H Hirji, Meera S Ramakrishnan et al.
✦ AI-curated · Sources linked
Case in brief
An 8-year-old boy with HbSS sickle cell disease and no previous sickle cell retinopathy developed blurred vision, headache, photopsia, and metamorphopsia during an influenza A-associated upper respiratory illness. Examination showed bilateral temporal macular whitening; optical coherence tomography demonstrated inner nuclear layer hyperreflectivity and thickening, consistent with bilateral paracentral acute middle maculopathy (PAMM). The symmetric bilateral lesions were considered suggestive of systemic vaso-occlusion. He received hydroxyurea 500 mg daily and had partial symptom recovery at 2-month follow-up.
What made this case unusual
PAMM occurred bilaterally and symmetrically in a child with HbSS disease, without prior sickle cell retinopathy, during an influenza A-associated illness.
Diagnostic / clinical pearl
In sickle cell disease, bilateral PAMM may signal systemic vaso-occlusion and a potential sickling crisis; assess for relevant triggers and ischemic complications.
Why it matters
PAMM lesions may precede larger ischemic events, including retinal artery or vein occlusion, making their recognition clinically important in this setting.