Photo Essay Profound Presentation of Retinopathy in a Patient with Sickle Cell Trait and Diabetes Mellitus Gautam Vangipuarm1, MD; Steven S. Saraf1, MD; Qinqin Zhang2, PhD; Ruikang Wang1,2, PhD; Kasra A Rezaei1, MD 1Department of Ophthalmology, University of Washington, Seattle, WA, USA 2Department of Bioengineering, University of Washington, Seattle, WA, USA ORCID: Kasra A Rezaei: https://orcid.org/0000-0003-4287-3187 J Ophthalmic Vis Res 2020; 15 (1): 116–117 Correspondence to: Kasra A Rezaei, MD. University of Washington, Depart- ment of Ophthalmology, Seattle, WA, 908 Jefferson St, Seattle, WA 98104. E-mail: krezaei@uw.edu Received: 01-01-2019 Accepted: 01-05-2019 Access this article online Website: https://knepublishing.com/index.php/JOVR DOI: 10.18502/jovr.v15i1.5962 PRESENTATION A 43-year-old functionally monocular African Amer- ican woman with longstanding type 2 diabetes mellitus presented for care of her better-seeing left eye. Originally suspected of having prolif- erative diabetic retinopathy (PDR) as the cause of her bilateral visual impairment, fluorescein angiography and optical coherence tomography angiography revealed a marked peripheral non- perfusion which was out of proportion for a typical diabetic retinopathy (Figure 1). A comprehensive uveitic and vasculopathic workup was therefore initiated. The workup was largely negative except for hemoglobin electrophoresis, which was consis- tent with the sickle cell trait (or hemoglobinopa- thy) (Table 1). The patient was counseled on her diagnosis and continues to be treated with laser photocoagulation for her peripheral neovascular- ization. Figure 1. Color fundus photo, left eye (A) early (B) and late (C) fluorescein angiography of the left eye showing marked peripheral ischemia and posterior pole neovascularization. OCT angiography (D) showing severely decreased vascular density. DISCUSSION This report strengthens the hypothesis that dia- betic retinopathy and coexisting vasculopathic This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. How to cite this article: Vangipuarm G, Saraf SS, Zhang Q, Wang R, Rezaei KA. Profound Presentation of Retinopathy in a Patient with Sickle Cell Trait and Diabetes Mellitus. J Ophthalmic Vis Res 2020;15:116–117. 116 © 2020 JOURNAL OF OPHTHALMIC AND VISION RESEARCH | PUBLISHED BY PUBLISHED BY KNOWLEDGE E http://crossmark.crossref.org/dialog/?doi=10.18502/jovr.v15i1.5962&domain=pdf&date_stamp=2019-07-17 https://knepublishing.com/index.php/JOVR Photo Essay; Rezaei et al Table 1. Laboratory assessment of other etiologies for extensive peripheral non-perfusion including pro-thrombotic and vasculitic causes Test ordered Result (normal range) Angiotensin converting enzyme (U/L) 26U/L (8–53 U/L) Anti-nuclear antibody Negative Cryoglobulin Negative Erythrocyte sedimentation rate (mm/H) 60 mm/H high (0–20 mm/H) HIV Ag and Ab Nonreactive Anti-myeloperoxidase Negative Anti PR3 Negative Rheumatoid factor < 10 Serologic syphilis panel Negative Anti-thrombin activity 123% (normal) C-reactive protein (mg/L) 24.9 mg/L high (0–10 mg/L) Activated protein S (%) 113% (65–150%) Activated protein C (%) 121% (55–150%) Factor V Leiden Negative Homocysteine Negative Prothrombin time (s) 14.1 s (10.7–15.6 s) INR (s) 1.1 s (0.8–1.3 s) CBC Normal CMP Glucose 353 mg/dL (62–125 mg/dL) Herpes type 1&2 serology Positive for HSV-1 and HSV-2 CMV (serum antibody) Positive Hemoglobin electrophoresis Consistent with HbS trait Quantiferon-TB Gold Negative CBC, complete blood count; CMP, comprehensive metabolic panel; CMV, antibodies to cytomegalovirus; HIV, human immunodeficiency virus; INR, international normalized ratio; mg/dL, milligrams per deciliter; mm/H, millimeter per hour U/L, Units Per Liter diseases, even sickle cell trait, may have a syner- gistic effect on the overall disease burden. A broad differential must be maintained in patients with presumed diabetic retinopathy, especially those with uncharacteristic imaging findings.[1–5] Financial Support and Sponsorship This study was supported by the Department of Ophthalmology, University of Washington. Conflicts of Interest There are no conflicts of interest. REFERENCES 1. Tsaras G, Owusu-Anash A, Boateng FO, Amoateng- Adjepong Y. Complications associated with SCT: A brief narrative review. Am J Med 2009;122:507–512. 2. Downes S, Hambleton I, Chuang EL, Lois N, Serjeant GR, Bird AC, et al. Incidence and natural history of proliferative sickle cell retinopathy. Ophthalmology 2005;112:1869– 1875 3. Jampol L, Goldbaum M. Peripheral proliferative retinopathies. Surv Ophthalmol 1980;25:1–14 4. Jackson H, Bentley CR, Hingorani M, Atkinson, P Acliman- dos WA, Thompson GM. Sickle retinopathy in patients with sickle trait. Eye 1995;9:589–593. 5. Nagpal KC, Asdourian GK, Patrianakos D, Goldberg MF, Rabb MF, Goldbaum M. Proliferative retinopathy in SCT. Report of seven cases. Arch Intern Med 1977;137:325–328. JOURNAL OF OPHTHALMIC AND VISION RESEARCH VOLUME 15, ISSUE 1, JANUARY-MARCH 2020 117