Cited by 6Retinal Development and DisordersRetinal Diseases and TreatmentsRetinal and Optic Conditions

DISRUPTION OF THE OUTER SEGMENTS OF THE PHOTORECEPTORS ON OPTICAL COHERENCE TOMOGRAPHY AS A FEATURE OF VITAMIN A DEFICIENCY

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Jevnikar, Kristina, Šuštar, Maja, Kozjek, Nada Rotovnik, Štrucl, Ana M., Markelj, Špela, Hawlina, Marko, Fakin, Ana·2022·RETINAL Cases & Brief Reports

Abstract

Purpose: To describe the optical coherence tomography features of vitamin A deficiency. Methods: Case series includes three male patients aged 50 to 66 years with vitamin A deficiency and visual symptoms ranging from 2 to 8 months. Examination included optical coherence tomography (OCT), fundus autofluorescence imaging, full-field electroretinography6 and laboratory work-up. Results: Patient 1 had inoperable pancreatic neuroendocrine tumor and presented with worsening nyctalopia. The electroretinography showed absent rod function 2 months after the onset of symptoms, followed by a decrease of the cone function eight months after the onset. Optical coherence tomography showed poorly distinguishable outer segments of the photoreceptors with the disappearance of the interdigitation zone. At that time, vitamin A deficiency along with several other deficiencies was confirmed. After the initiation of parenteral nutrition, a substantial improvement of the patient's overall well-being was noted and the OCT showed normalization of the retinal structure. Two other patients were diagnosed with vitamin A deficiency based on similar OCT features. Conclusion: Disruption of the outer segments of the photoreceptors and the disappearance of the interdigitation zone on OCT may be helpful in recognition of vitamin A deficiency. Early detection and malnutrition evaluation are especially important in patients with a history of gastrointestinal disorders who may have several other underlying deficiencies. Treatment with either enteral or parenteral nutrition not only leads to resolution of visual symptoms but vastly improves their general condition and quality of life.

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  • Vitamin A replacement results in improvement of retinal function as well as structure, but long-term deficiency can lead to permanent degeneration of photoreceptors.

    “Vitamin A replacement results in improvement of retinal function as well as structure, however long-term deficiency can lead to permanent degeneration of photoreceptors [31,80,81,83,84,85,86,87,88,90].”

    From The Role of Vitamin A in Retinal Diseases (2022) · cites this paper as [83]

    The Case Reports figure captions report complete recovery of rod and cone function and improved photoreceptor outer-segment appearance after treatment in Patient 1. The Discussion also describes normalization of OCT features after treatment. Our copy does not say that long-term vitamin A deficiency causes permanent photoreceptor degeneration. (LLM, conf=0.88)

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  • Optical coherence tomography has been shown to be a useful diagnostic method for vitamin A deficiency, showing abnormalities at the level of photoreceptor outer segments.

    “Recently, optical coherence tomography (OCT) has been shown as a useful diagnostic method, which shows abnormalities at the level of photoreceptor outer segments [83] (Figure 2).”

    From The Role of Vitamin A in Retinal Diseases (2022) · cites this paper as [83]

  • Vitamin A is needed for normal visual function, and vitamin A deficiency (hypovitaminosis A) results in visual dysfunction that is reversible with vitamin A supplementation.

    “Vitamin A is needed for normal visual function and vitamin A deficiency (hypovitaminosis A) results in visual dysfunction that is reversible with vitamin A supplementation.”

    From The Role of Vitamin A in Retinal Diseases (2022) · cites this paper as [83]

  • Hypovitaminosis A has been associated with reversible ganglion cell thinning.

    “Interestingly, hypovitaminosis A has also been associated with reversible ganglion cell thinning [83], the reason for which is not understood but may reflect other vitamin A roles in the retina.”

    From The Role of Vitamin A in Retinal Diseases (2022) · cites this paper as [83]

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Research works printed but not linked: 6

  1. [2]Kontos A Kayhanian H El-Khouly F Gillmore R. Night blindness due to vitamin A deficiency associated with resected adenocarcinoma of the pancreas. Int J Ophthalmol 2015; 8: 206– 207. 25709934 PMC4325268
  2. [3]Singer JR Bakall B Gordon GM Reddy RK. Treatment of vitamin A deficiency retinopathy with sublingual vitamin A palmitate. Doc Ophthalmol 2016; 132: 137– 145. 26980447
  3. [4]Hansen BA Mendoza-Santiesteban CE Hedges TR III. Reversible nyctalopia associated with vitamin a deficiency after resected malignant ileal carcinoid and pancreatic adenocarcinoma. Retin Cases Brief Rep 2018; 12: 127– 130. 29554055
  4. [5]Saenz-de-Viteri M Sadaba LM. Optical coherence tomography assessment before and after vitamin supplementation in a patient with vitamin A deficiency: a case report and literature review. Medicine 2016; 95: e2680. 26871796 PMC4753891
  5. [9]Chow CC Mieler WF. Vitamin A deficiency and xerophthalmic fundus in autoimmune hepatitis and cirrhosis. Retin Cases Brief Rep 2014; 8: 164– 166. 25372429
  6. [10]Herron WL Jr Riegel BW. Production rate and removal of rod outer segment material in vitamin A deficiency. Invest Ophthalmol 1974; 13: 46– 53. 4809509

Cited by

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  • The Role of Vitamin A in Retinal Diseases

    Sajovic, J., Meglič, A., Glavač, D., Markelj, Š., Hawlina, M., & Fakin, A. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23031014 · Cited by 109 · see citing works

    “Interestingly, hypovitaminosis A has also been associated with reversible ganglion cell thinning [83], the reason for which is not understood but may reflect other vitamin A roles in the retina.”
    BackgroundInfluentialSelf-citation

Authors
Jevnikar, K., Šuštar, M., Kozjek, N. R., Štrucl, A. M., Markelj, Š., Hawlina, M., & Fakin, A.
Affiliations
  • Ljubljana University Medical Centre (SI)
  • Institute of Oncology Ljubljana (SI)
Corresponding author
  • Jevnikar, Kristina
  • Šuštar, Maja
  • Markelj, Špela
  • Hawlina, Marko
  • Fakin, Ana
Venue
RETINAL Cases & Brief Reports
Published
2020-11-06
Volume
16
Issue
5
Pages
658-662
ISSN
1935-1089 (ISSN-L)
OpenAlex
W3102030185
Cited by
6
References
4
Citations by year
2024: 1 · 2023: 3 · 2022: 2
FWCI
0.41
Type
journal article
Language
en
Open access
Locations
  • Retinal Cases & Brief Reports (publishedVersion, cc-by-nc-nd)
  • PubMed Central (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinal Development and Disorders
  • Health Sciences › Medicine › Ophthalmology › Retinal Diseases and Treatments
  • Health Sciences › Medicine › Ophthalmology › Retinal and Optic Conditions
Keywords
vitamin A deficiency, photoreceptor outer segments, interdigitation zone, optical coherence tomography findings, night blindness, rod and cone function, electroretinogram, malnutrition, pancreatic neuroendocrine tumors, parenteral nutrition, enteral nutrition, gastrointestinal diseases
MeSH terms
Electroretinography, Fluorescein Angiography, Humans, Male, Quality of Life, Visual Acuity, Vitamin A Deficiency, Tomography, Optical Coherence

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