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Vitamin A deficiency and the retinal “double carrot” sign with optical coherence tomography

Abstract

Background

Spectral-domain optical coherence tomography (SD-OCT) and full-field electroretinography (ERG) allow retinal assessment with vitamin A deficiency (VAD). Using SD-OCT, this study aimed to characterize and follow a novel retinal abnormality in patients with VAD and intramuscular supplementation.

Methods

Patients with VAD were retrospectively reviewed, including SD-OCT and electroretinography.

Results

Three patients had VAD following bariatric or colon surgery and varying supplementation. All had nyctalopia, extinguished scotopic rod-specific function with ERG, and decreased serum vitamin A. None demonstrated surface abnormalities. All received intramuscular vitamin A with subjective resolution of symptoms. On SD-OCT, four of six eyes exhibited homogenous foveal hyperreflectivity anterior to retinal pigment epithelium-Bruch complex, reminiscent of a “double carrot”, which improved following supplementation. ERG findings demonstrated improved scotopic rod-specific function in all cases; however, photopic function remained diminished in two cases.

Conclusions

Structural improvement of the proposed “double carrot” sign occurs soon after vitamin A supplementation. While scotopic function improves rapidly following supplementation, cone function recovers more slowly. Therefore, foveal changes such as the “double carrot” sign suggest that structural recovery of cones precedes functional recovery.

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Fig. 1: Spectral-domain optical coherence tomography (SD-OCT) demonstrates abnormal, outer retinal hyperrflectivitiy that improved after vitamin A supplementation (OD—right eye, OS—left eye).
Fig. 2: Fundus autofluorescence (FAF) before and after intramuscular supplementation of vitamin A.
Fig. 3: Scotopic electroretinogram (ERG) recovery following vitamin A supplementation in three patients.
Fig. 4: Schematic cross-sectional illustration of proposed “double carrot” sign relative to macular anatomy.

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Authors can confirm that all relevant data are included in the article and/or its supplementary information files.

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Funding

Jonas Children’s Vision Care is supported by the National Institute of Health 5P30CA013696, U01EY030580, U54OD020351, R24EY028758, R24EY027285, 5P30EY019007, R01EY018213, R01EY024698, R01EY026682, R21AG050437, R01EY024091, the Schneeweiss Stem Cell Fund, New York State [SDHDOH01-C32590GG-3450000], the Foundation Fighting Blindness New York Regional Research Center Grant [PPA-1218-0751-COLU], Nancy & Kobi Karp, the Crowley Family Funds, The Rosenbaum Family Foundation, Alcon Research Institute, the Gebroe Family Foundation, the Research to Prevent Blindness (RPB) Physician-Scientist Award, and unrestricted funds from RPB, New York, NY, USA, and the Kogod Family (MPB), none of which had any role in study design, collection, analysis and interpretation of data, writing the report, and the decision to submit the report for publication.

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Contributions

Each author contributed to the manuscript as follows: design and conduct of the study (M.P.B., J.K.O., S.H.T.) collection, management, analysis, and interpretation of the data (M.P.B., J.K.O., J.R.S., S.F., S.H.T.); preparation, review, or approval of the manuscript and decision to submit for publication (M.P.B., J.K.O., S.A.B., J.A.K., R.K., C.M.R. J.R.S., S.F., S.H.T.). At least two authors (M.P.B., J.K.O.) had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Corresponding author

Correspondence to Stephen H. Tsang.

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The authors declare no competing interests.

Ethics approval and consent to participate

The need for patient consent was waived due to minimal risk offered to patients and the retrospective nature of this study design as per Columbia University Institutional Review Board approved protocol AAAR8743. All procedures were reviewed and in accordance with the tenets of the Declaration of Helsinki.

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Breazzano, M.P., Oh, J.K., Batson, S.A. et al. Vitamin A deficiency and the retinal “double carrot” sign with optical coherence tomography. Eye 37, 1489–1495 (2023). https://doi.org/10.1038/s41433-022-02137-9

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