Journal of Pediatric Ophthalmology and Strabismus

Short Subjects 

Evaluation and Clinical Course of Keratomalacia With Descemetocele in a Child With Autism and Vitamin A Deficiency

Eric Chan, MD; Joshua Buzzard, BS; Richard Helms, PhD; A. Paula Grigorian, MD

Abstract

Autistic children with selective diets have an elevated risk for vitamin A deficiency. The authors present the case of a 7-year-old boy with keratomalacia resulting from dietary vitamin A deficiency. Optical coherence tomography and ultrasound biomicroscopy can provide useful details of the cornea and underlying structures. Vitamin A supplementation can result in significant resolution, obviating the need for surgical intervention. [J Pediatr Ophthalmol Strabismus. 2020;57:e1–e3.]

Abstract

Autistic children with selective diets have an elevated risk for vitamin A deficiency. The authors present the case of a 7-year-old boy with keratomalacia resulting from dietary vitamin A deficiency. Optical coherence tomography and ultrasound biomicroscopy can provide useful details of the cornea and underlying structures. Vitamin A supplementation can result in significant resolution, obviating the need for surgical intervention. [J Pediatr Ophthalmol Strabismus. 2020;57:e1–e3.]

Introduction

Autistic children with selective diets have an elevated risk for vitamin A deficiency. The authors describe a 7-year-old boy with keratomalacia resulting from dietary vitamin A deficiency.

Case Report

A 7-year-old boy with autism presented to the emergency department with red swollen eyes of 1-week duration despite a trial of topical olopatadine 0.1% twice a day for 5 days. He was non-verbal and communicated by pointing and moving objects. His parents noted that he was photophobic and had kept his hands over his eyes since onset. He had a selective diet of dry cereal, soda, and juice, which was reflected in his thin body habitus. His vision could not be assessed due to significant photophobia.

An examination under anesthesia was performed due to lack of cooperation in the clinic. The periocular skin was dry and scaly, and the conjunctival epithelium was keratinized with lost luster bilaterally. His right eye demonstrated a 4-mm inferior corneal ulcer with a large fibrin clot in the superior anterior chamber. His left eye demonstrated a superior corneal ulcer and descemetocele with a fibrin plaque in the superior anterior chamber, which was also significantly shallow and confirmed by anterior segment optical coherence tomography (Figures 12). There was no view of the retina.

Images obtained during initial examination under anesthesia, with fluorescein used to aid visualization of corneal defects. The right eye (OD) shows a 4-mm corneal ulcer with a large fibrin clot in the superior anterior chamber. The left eye (OS) shows a shallow superior corneal ulcer and descemetocele with fibrin plaque.

Figure 1.

Images obtained during initial examination under anesthesia, with fluorescein used to aid visualization of corneal defects. The right eye (OD) shows a 4-mm corneal ulcer with a large fibrin clot in the superior anterior chamber. The left eye (OS) shows a shallow superior corneal ulcer and descemetocele with fibrin plaque.

Anterior segment optical coherence tomography of both eyes during initial examination performed at the level of the corneal ulceration in the right eye and peak of the descemetocele in the left eye to assess the corneal thickness.

Figure 2.

Anterior segment optical coherence tomography of both eyes during initial examination performed at the level of the corneal ulceration in the right eye and peak of the descemetocele in the left eye to assess the corneal thickness.

Because keratomalacia was suspected as a cause of the corneal ulcerations, a serum of vitamin A and bacterial cultures from both corneal ulcers was collected. The patient began treatment with topical moxifloxacin and bacitracin and a single intramuscular dose of vitamin A followed by daily oral supplements. The initial serum vitamin A level was undetectable (normal: 17 to 56 µg/dL), and the bacterial cultures showed no growth. No other nutritional deficiencies were investigated, but he was referred to a nutritionist due to his thin body habitus.

Repeat examination under anesthesia after 1 week showed reduction in the size of the corneal ulcers and resolution of the bilateral fibrin plaques in the anterior chamber. However, the left eye was notable for an irregular pupil with iris strands tracking to the descemetocele. Ultrasound biomicroscopy was used to define the extent and location of iris stranding, which likely sealed an underlying perforation (Figure 3). Repeat examination under anesthesia was performed 1 month later, demonstrating resolved bilateral xerophthalmia with corneal scarring at the previous corneal ulcer sites (Figure 4). The left pupil remained irregular, with diffuse anterior and posterior synechiae. Dilated fundus examination results were normal. The patient was seen 1 month later in the office with improved interaction and excellent visual behavior in both eyes despite the small corneal scars.

(A) Ultrasound biomicroscopy (50-MHz) at the level of the descemetocele in the left eye to evaluate structures behind the cornea, obstructed by ulcerated tissue and (B) scarring at 1 week after initial treatment, showing iris stranding contacting the descemetocele.

Figure 3.

(A) Ultrasound biomicroscopy (50-MHz) at the level of the descemetocele in the left eye to evaluate structures behind the cornea, obstructed by ulcerated tissue and (B) scarring at 1 week after initial treatment, showing iris stranding contacting the descemetocele.

Images of the right (OD) and left (OS) eyes obtained during examination under anesthesia 1 month following the initial visit and treatment. Corneal scarring is seen in both eyes at the site of previous ulceration.

Figure 4.

Images of the right (OD) and left (OS) eyes obtained during examination under anesthesia 1 month following the initial visit and treatment. Corneal scarring is seen in both eyes at the site of previous ulceration.

Discussion

Vitamin A is an important nutrient in the retina, conjunctiva, and cornea. In the retina, vitamin A acts as the precursor to photoreceptor pigment. Lack of vitamin A will manifest clinically as nyctalopia due to dysfunction of the rod photoreceptors and sometimes as focal retinal pigment epithelium defects (xerophthalmic fundus). The full-field electroretinogram has decreased amplitudes, especially with dark adaptation. In the conjunctiva and cornea, vitamin A is needed for cellular metabolism and replication. Deficiency will manifest initially as conjunctival and corneal xerosis. The conjunctiva will demonstrate dry, granular patches with areas of keratinization including the characteristic Bitot spot, which is a composition of metaplastic conjunctival epithelium, keratin, and Corynebacterium species. The cornea will initially appear with findings of dry eyes with a poor tear film and punctate epithelial erosions, but it can progress to corneal edema and epithelial defects with subsequent thinning and ulceration.

Although vitamin A deficiency in children is uncommon in the United States, autistic children with selective diets are at risk. Prior cases of vitamin A deficiency were reported with poor visual outcome, severe scarring of the cornea, or needing surgical treatment.1–4 Our case demonstrates that timely vitamin A supplementation can reverse advanced keratomalacia with perforation. Additionally, we emphasize the importance of anterior segment imaging (eg, ultrasound biomicroscopy) in evaluating the anterior chamber structures obscured by the corneal opacities. Although imaging studies may not be widely available, they helped us assess the extent of the lesions and guide the treatment accordingly, demonstrating closed perforation and ultimately avoiding surgical intervention.

References

  1. Duignan E, Kenna P, Watson R, Fitzsimon S, Brosnahan D. Ophthalmic manifestations of vitamin A and D deficiency in two autistic teenagers: case reports and a review of the literature. Case Rep Ophthalmol. 2015;6:24–29. doi:10.1159/000373921 [CrossRef]
  2. Lin P, Fintelmann R, Khalifa Y, Bailony M, Jeng B. Ocular surface disease secondary to vitamin A deficiency in the developed world: it still exists. Arch Ophthalmol. 2011;129:798–799. doi:10.1001/archophthalmol.2011.126 [CrossRef]
  3. Steinemann T, Christiansen S. Vitamin A deficiency and xerophthalmia in an autistic child. Arch Ophthalomol. 1998;116:392–393. doi:10.1001/archopht.116.3.392 [CrossRef]
  4. Uyanik O, Dogangun B, Kayaalp L, Korkmaz B, Dervent A. Food faddism causing vision loss in an autistic child. Child Care Health Dev. 2006;32:601–602. doi:10.1111/j.1365-2214.2006.00586.x [CrossRef]
Authors

From the University Hospitals/Case Western Reserve School of Medicine, Cleveland, Ohio (EC, JB, RH, APG); and the Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas (APG).

The authors have no financial or proprietary interest in the materials presented herein.

Correspondence: A. Paula Grigorian, MD, Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, AR 72205. E-mail: Grigorian_paula@yahoo.com

Received: May 24, 2019
Accepted: July 09, 2019
Posted Online: January 24, 2020

10.3928/01913913-20190812-01

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