Bilateral paracentral acute middle maculopathy in a pediatric patient with sickle cell disease

To describe the clinical course of a pediatric patient with sickle cell disease (SCD) presenting with isolated bilateral paracentral acute middle maculopathy (PAMM) in the setting of influenza A.

Fundus photography, fundus autofluorescence, optical coherence tomography (OCT) and OCT-angiography (OCTA) were obtained.

An 8-year-old male with a history of HbSS SCD, ADHD and no prior sickle cell retinopathy presented with new onset blurred vision, headache, photopsia, and metamorphopsia along with symptoms of an upper respiratory infection. Fundus examination showed bilateral placoid whitening of the temporal macula and corresponding OCT imaging revealed hyperreflectivity and thickening of the inner nuclear layer (INL), consistent with bilateral PAMM lesions. Thought to be a harbinger of a sickle crisis, the patient received hydroxyurea 500 mg daily and demonstrated partial recovery of symptoms at 2-month follow-up.

The bilaterality and symmetry of this patient's presentation suggests a systemic vaso-occlusive state, whereby sickled red blood cells occluded the retinal capillaries as a function of the location and organization of the retinal capillary vasculature. The patient's risk factors for the development of a sickling event included recent viral infection and use of sympathomimetic medication. PAMM lesions can often be harbingers of larger ischemic events such as retinal artery or vein occlusions. Therefore, in SCD patients, presentation of PAMM warrants urgent evaluation of sickling triggers, treatment and prophylaxis.