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A Child With Severe Autism Spectrum Disorder With Bilateral Corneal Ulcers

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 6-year-old boy with severe autism spectrum disorder (ASD) was brought to the hospital by his mother for whitening in the right eye. The patient was nonverbal at baseline and exhibited restrictive and repetitive behaviors. His diet consisted exclusively of cookies, apples, potato chips, and Gatorade. His mother noticed him frequently rubbing his eyes for several weeks, which she initially attributed to new-onset allergies. She did not describe any reduction in visual function or ability to perform low-luminance tasks.

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Xerophthalmia and keratomalacia from vitamin A deficiency

B. Check serum vitamin A levels

The clinical examination revealed bilateral xerophthalmia, a manifestation of vitamin A deficiency. Thus, serum vitamin A levels should be checked (choice B). While vitamin A deficiency is one of the most common causes of malnutrition in low-income countries, it is rarely seen in high-income countries.

In 2009, the World Health Organization estimated that 5.2 million preschool-aged children had night blindness and 190 million had low serum retinol concentrations.1 Those with a serum retinol concentration of less than 20.06 μg/dL (to convert to micromoles per liter, multiply by 0.0349) are vitamin A deficient, and severe deficiency is indicated at levels below 10.03 μg/dL.1 Eye findings include hyperemia, conjunctival and corneal keratinization, and Bitot spots. Yellow or white punctate peripheral retinal lesions may be seen, indicating photoreceptor dysfunction. Pediatric idiopathic intracranial hypertension and nerve atrophy have also been associated with vitamin A deficiency.2,3 Children with ocular signs of vitamin A deficiency have reduced immunity and a higher mortality rate.3,4

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Article Information

Corresponding Author: Jennifer Park, MD, Department of Ophthalmology, SUNY Downstate Medical Center, SUNY Downstate Health Sciences University, 450 Clarkson Ave, New York, NY 11203 (jennifer.park@downstate.edu).

Published Online: April 21, 2022. doi:10.1001/jamaophthalmol.2022.0018

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s parents for granting permission to publish this information.

References
1.
World Health Organization.  Global Prevalence of Vitamin A Deficiency in Populations at Risk 1995-2005: WHO Global Database on Vitamin A Deficiency. World Health Organization; 2009.
2.
Dotan  G , Goldstein  M , Stolovitch  C , Kesler  A .  Pediatric pseudotumor cerebri associated with low serum levels of vitamin A.   J Child Neurol. 2013;28(11):1370-1377. doi:10.1177/0883073812474344PubMedGoogle ScholarCrossref
3.
Chiu  M , Watson  S .  Xerophthalmia and vitamin A deficiency in an autistic child with a restricted diet.   BMJ Case Rep. 2015;bcr2015209413. doi:10.1136/bcr-2015-209413PubMedGoogle ScholarCrossref
4.
Wadhwani  M , Singh  R .  Bilateral keratomalacia leading to blindness secondary to diet-induced vitamin A deficiency in infants.   J Pediatr Ophthalmol Strabismus. 2020;57:e12-e14. doi:10.3928/01913913-20191210-01PubMedGoogle ScholarCrossref
5.
Chan  E , Buzzard  J , Helms  R , Grigorian  A .  Evaluation and clinical course of keratomalacia with descemetocele in a child with autism and vitamin A deficiency.   J Pediatr Ophthalmol Strabismus. 2020;57:e1-e3. doi:10.3928/01913913-20190812-01Google ScholarCrossref
6.
Adachi  S , Torio  M , Okuzono  S ,  et al.  Vitamin A deficiency-associated corneal perforation in a boy with autism spectrum disorder: a case report and literature review.   Nutrition. 2021;90:111275. doi:10.1016/j.nut.2021.111275PubMedGoogle ScholarCrossref
7.
Ross  DA .  Recommendations for vitamin A supplementation.   J Nutr. 2002;132(9)(suppl):2902S-2906S. doi:10.1093/jn/132.9.2902SPubMedGoogle ScholarCrossref
8.
Bonini  S , Coassin  M , Aronni  S , Lambiase  A .  Vernal keratoconjunctivitis.   Eye (Lond). 2004;18(4):345-351. doi:10.1038/sj.eye.6700675PubMedGoogle ScholarCrossref
9.
Borkar  DS , Gonzales  JA , Tham  VM ,  et al.  Association between atopy and herpetic eye disease: results from the Pacific Ocular Inflammation study.   JAMA Ophthalmol. 2014;132(3):326-331. doi:10.1001/jamaophthalmol.2013.6277PubMedGoogle ScholarCrossref
10.
Vanathi  M , Panda  A , Vengayil  S , Chaudhuri  Z , Dada  T .  Pediatric keratoplasty.   Surv Ophthalmol. 2009;54(2):245-271. doi:10.1016/j.survophthal.2008.12.011PubMedGoogle ScholarCrossref
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