This is a case presentation by Dr. Chang about a 9-year-old boy who ate only fries and had Vitamin A deficiency. Dr. Chang also discusses about the manifestations, clinical presentation and management.

Lecturer: Dr. Wesley Chang, Singapore National Eye Center, Singapore

Transcript

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DR CHANG: Hi. Morning, everyone. So I’ll be presenting an interesting case that we saw in my local hospital in Singapore. So it was this 9-year-old Malay boy, who first presented to our pediatric eye service in March, last year, complaining of blinking and rubbing of his eyes for a month. He had some eye redness in the right eye. And he was given some preservative-free lubricants, as well as antibiotics, by the emergency department. Otherwise there was no history of trauma or no history of any irritation or chemical injury. The child was a full term baby, normal vaginal delivery. Had a normal birth weight as well. He was a bit small. He was only at the 25th percentile for his height and weight. On examination, his visual acuity was 6/15 and 6/10. With pinhole in the right and left eye. There were lots of papillae, fine papillae in the lids. The pupils were — they reacted very quickly. Eye movements were full. The lids had some fine papillae. The eye was white, but he had a very dry ocular surface. And there were many punctal epithelial erosions. So on examination, we saw that he had a subepithelial haze on the right and left eye. The ocular surface was very dry. And he had some keratinization of his conjunctiva as well. So then they weren’t sure what was going on. And a provisional diagnosis of allergic eye disease was made. He was started on some steroids and antihistamine drops. And he was reviewed a week later, with no improvement. So he was subsequently referred to our tertiary eye center. And was seen by the cornea team. And on examination, they also noted his corneal haze with conjunctival xerosis, bilaterally. When they took a photohistory, they also found out that he had difficulty seeing in the dark. He had difficulty seeing in the dark. He couldn’t see in the dark. Yeah. And there was a grey patch over his eyes, that his parents noted for about the past two to three weeks. There was a patch on the eye. Which the parents noted for the past two to three weeks. So they took a further history. And found out that he was born and raised in Singapore. But he was a very picky eater. He stopped eating rice and porridge at the age of 1. And only ate French fries. And did not take any fruits or vegetables. So a diagnosis of vitamin A — likely a vitamin A deficiency was made. And ERG was done for him. Which showed a severe rod suppression, which was consistent with a vitamin A deficiency. He was subsequently referred to the pediatric medical team, who found out he had a vitamin A, vitamin D, B12, and folate deficiency. So he was started on supplementation. The visual acuity improved slightly, but his ocular surface was still very dry. So just a few slides on vitamin A deficiency. So vitamin A is usually found in leafy green vegetables. Orange vegetables, eggs. It’s essential for our visual function, as well as cellular proliferation. So you can get it from vegetable sources, animal sources, as well as from synthetic formulae. As it digests through our body, it gets stored in the liver, in our retina, rods, and cones. And our cellular transcription factors. So they can present… So the way they present depends on how long they have been deficient from vitamin A. From normal stores to subnormal stores to reduced serum levels to keratinization of our mucosa. Not just on the eye. But in our respiratory tract, as well as intestinal tract. And lastly, it can lead to blindness. So some of the common causes are due to poor dietary intake, poor food intake, poor absorption. This was due to parasites in the intestines. Commonly patients with vitamin A deficiency will complain of night blindness, as well as problems with poor ocular surface, such as photophobia, dry eyes, blurry vision, discomfort. Systemically, they can also have poor growth, diarrhea, reduced immunity, frequent infections, and this can even lead to death. So for the eye signs, commonly on the conjunctiva they get a very dry ocular surface. They can get some pigmentation, as well as wrinkling of the conjunctiva. And commonly you can get Bitot’s spots, which are these white frothy areas, due to bacteria growing on the surface. Like you saw in the photo just now, you can have corneal signs as well. You can have subepithelial haze. In bad cases, the cornea can even melt. And there’s a risk of secondary infection. In the fundus, you can get the steepling of the macula, RPE changes of the macula, and if you do OCT, you can see some material in the subretinal space as well. ERG will show a rod and cone dysfunction. And the optic nerve can become pale. So the WHO categorizes vitamin A deficiency according to different grades. You have the mild ones, starting from just night blindness, to the severe ones, with a corneal ulcer. Which tends to present earlier in children. 1 to 4 years old. And there’s a high risk of death as well. So for the management, you usually have to co-manage with the pediatric team. You have to find the underlying cause for the vitamin A deficiency. And you have to supplement either orally or with intramuscular injections. The prognosis is generally good. They respond within 24 to 48 hours of vitamin A supplementation. However, if there are any corneal ulcers or corneal melts, this can lead to corneal scarring. The retina usually improves about 2 to 4 months after supplementation, and the visual fields return to normal. All right. Thank you.

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June 6, 2017

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