Association of acute macular neuroretinopathy or paracentral acute middle maculopathy with sickle cell disease

Abstract Purpose To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) presenting with acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including optical coherence tomography (OCT) and OCT angiography (OCTA). Design Retrospective case series Subjects These were HbSS or HbSC patients who presented with vision loss attributed to AMN (n=2) or PAMM (n=2). Methods Clinical characteristics including best-corrected visual acuity, and multimodal imaging features from fundus photography, fluorescein angiography, OCT and OCTA were analyzed. Main Outcome Measures Longitudinal changes in the structure and microvasculature of the retina and choriocapillaris were examined. Results In two patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In one patient, the lesion was located in the foveal avascular zone and only flow deficits in the choriocapillaris were noted. In the second patient, no flow loss was observed in the superficial capillary plexus (SCP) corresponding to the lesion, but flow loss was seen in the deep capillary plexus (DCP) and choriocapillaris. At the respective one-year and four-month follow up, the hyperreflectivities improved with no ensuing retinal atrophy. DCP and choriocapillaris flow also improved. In two patients with PAMM, one had PAMM in association with branch retinal arteriolar occlusions. In both patients, a hyperreflective band involving the inner plexiform, inner nuclear and outer plexiform layers was seen in or adjacent to areas of prior macular thinning. These areas corresponded to flow deficits in both the SCP and DCP. At the respective one-month and one-year follow up, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCTA demonstrated worsening of flow deficits in the corresponding SCP and DCP. Conclusions This is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. PAMM may precede macular thinning commonly observed in SCD. OCTA is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.