PERIPHERAL RETINOPATHY ASSOCIATED WITH APLASTIC ANEMIA

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Abstract

Purpose:

To report a case of severe, bilateral, rapidly progressing peripheral retinal nonperfusion associated with underlying aplastic anemia.

Methods:

An interventional case report.

Results:

A 4-year-old girl presented with decreased visual acuity. On clinical examination, she was found to have a RAPD, elevated intraocular pressure, 360° rubeosis, vitreous hemorrhage, severe exudative retinal detachment, and telangiectasia with severe peripheral retinal nonperfusion. Laboratory workup was significant for pancytopenia, and a bone marrow biopsy showed extreme hypocellularity with no malignant cells. The patient was diagnosed with primary aplastic anemia. She developed dramatic progression of retinal nonperfusion in the left eye, as well as in the fellow right eye. This bilateral retinopathy was poorly responsive to aggressive management, which included laser photocoagulation and intravitreal injections of anti–vascular endothelial growth factor medications.

Conclusion:

Asymmetric, bilateral quickly progressing peripheral retinal ischemia, in conjunction with pancytopenia and otherwise negative workup may be related to underlying bone marrow failure and aplastic anemia.

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