TY - JOUR
T1 - Association of Acute Macular Neuroretinopathy or Paracentral Acute Middle Maculopathy with Sickle Cell Disease
AU - Ong, Sally
AU - Ahmed, Ishrat
AU - Scott, Adrienne W.
N1 - Funding Information:
Supported by the Johns Hopkins University School of Medicine, Wilmer Eye Institute Retina Division research fund, Baltimore, Maryland; and private philanthropy from Gail C. and Howard Woolley, Baltimore, Maryland. Optovue, Inc., generously provided the on-loan instrument used in this study. The funding organization or companies had no role in the design or conduct of this research.
Publisher Copyright:
© 2021 American Academy of Ophthalmology
PY - 2021/11
Y1 - 2021/11
N2 - Purpose: To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) demonstrating acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including OCT and OCT angiography (OCTA). Design: Retrospective case series. Participants: Four hemoglobin SS (HbSS) or hemoglobin SC (HbSC) patients who demonstrated 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 2 patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In 1 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 1-year and 4-month follow-up examinations, the hyperreflectivities improved with no ensuing retinal atrophy. Deep capillary plexus and choriocapillaris flow also improved. Of the 2 patients with PAMM, 1 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 1-month and 1-year follow-up examinations, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCT angiography demonstrated worsening of flow deficits in the corresponding SCP and DCP. Conclusions: To the best of our knowledge, this is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. Paracentral acute middle maculopathy may precede macular thinning commonly observed in SCD. OCT angiography is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.
AB - Purpose: To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) demonstrating acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including OCT and OCT angiography (OCTA). Design: Retrospective case series. Participants: Four hemoglobin SS (HbSS) or hemoglobin SC (HbSC) patients who demonstrated 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 2 patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In 1 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 1-year and 4-month follow-up examinations, the hyperreflectivities improved with no ensuing retinal atrophy. Deep capillary plexus and choriocapillaris flow also improved. Of the 2 patients with PAMM, 1 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 1-month and 1-year follow-up examinations, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCT angiography demonstrated worsening of flow deficits in the corresponding SCP and DCP. Conclusions: To the best of our knowledge, this is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. Paracentral acute middle maculopathy may precede macular thinning commonly observed in SCD. OCT angiography is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.
KW - Acute macular neuroretinopathy
KW - OCT
KW - OCT angiography
KW - Paracentral acute middle maculopathy
KW - Sickle cell disease
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U2 - 10.1016/j.oret.2021.01.003
DO - 10.1016/j.oret.2021.01.003
M3 - Article
C2 - 33476854
AN - SCOPUS:85101866346
SN - 2468-7219
VL - 5
SP - 1146
EP - 1155
JO - Ophthalmology Retina
JF - Ophthalmology Retina
IS - 11
ER -