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

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.