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Starr, Matthew R., MD; Softing Hataye, Alaina L., OD; Bakri, Sophie J., MD

doi: 10.1097/ICB.0000000000000849
Case Report: PDF Only

Purpose: To describe a unique presentation of multifocal paracentral acute middle maculopathy in a young patient with juvenile dermatomyositis including optical coherence tomography angiography findings.

Methods: Retrospective single case report.

Patient: Seventeen-year-old male patient with a history of juvenile dermatomyositis presented for routine hydroxychloroquine screening.

Results: Optical coherence tomography revealed undulations of the outer retinal layers and retinal thinning near the fovea in both eyes. Optical coherence tomography angiography revealed loss of flow void densities in the superficial and deep capillary plexi in both eyes. These findings were consistent with a diagnosis of paracentral acute middle maculopathy.

Conclusion: To the authors' knowledge, this is the first report of the optical coherence tomography angiography findings in paracentral acute middle maculopathy associated with juvenile dermatomyositis. The defects were located in the superficial and deep capillary plexi and likely due to the known occlusive retinopathy associated with juvenile dermatomyositis.

A 17-year-old, asymptomatic male patient with a history of juvenile dermatomyosis presented for a routine hydroxychloroquine screening. Optical coherence tomography revealed multifocal areas of outer retinal layer irregularities. Optical coherence tomography angiography revealed multiple flow density voids in the deep and superficial capillary plexi. The patient was diagnosed with paracentral acute middle maculopathy.

Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota.

Reprint requests: Sophie J. Bakri, MD, Department of Ophthalmology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905; e-mail:

Supported by Research to Prevent Blindness, New York. The funding source had no involvement in study design; collection, analysis, or interpretation of the data; writing the report; or the decision to submit for publication.

None of the authors have any proprietary interests to disclose.

© 2019 by Ophthalmic Communications Society, Inc.