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Editor,—Vitamin A deficiency is a major cause of ocular morbidity especially in underdeveloped countries. In the West sporadic cases have been reported usually associated with malabsorption syndromes, small intestinal bypass surgery, or abnormal metabolism. We report a case of an ophthalmological diagnosis of vitamin A deficiency secondary to coeliac disease. To our knowledge keratomalacia in association with coeliac disease has not been described previously.
A 64 year old man presented with a 6 week history of sudden progressive redness of his right eye associated with blurring of vision. His left eye was asymptomatic and he had no ocular history of note. He had a history of diet controlled coeliac disease proved by jejunal biopsy. Several weeks earlier he had complained of persistent diarrhoea despite adherence to his gluten-free diet.
On examination visual acuity was counting fingers on the right and 6/6 on the left. The left eye was normal except for multiple fluorescein staining scattered fine superficial punctate erosions. The right eye was diffusely injected and the cornea showed filamentary changes with multiple underlying punctate staining epithelial defects. There were no other pathological features.
A provisional diagnosis of filamentary keratitis was made and he was treated with topical acetylcysteine to the right eye and topical lubricants to the left eye. Two weeks later he reported a marked deterioration in symptoms. Visual acuity remained unchanged. Corneal examination revealed a large central full thickness epithelial defect. Regular topical lubricants were applied and a botulinum toxin injection was administered. Full physical examination at this stage revealed a thin man but no other relevant findings.
After 1 week there was complete failure of re-epithelialisation (Fig1). The suspicion of a keratomalacia secondary to vitamin A deficiency was raised and an intramuscular injection of 100 000 units of vitamin A was immediately administered. Subsequent serum vitamin A levels were 13 (normal 330–1100). Six days later there was a dramatic symptomatic improvement. Visual acuity had improved to 6/18. The cornea was almost completely healed with only a few areas of superficial epithelial deficit remaining. The patient was referred back to the gastroenterologists who investigated his continuing malabsorptive symptoms and administered standard dietary supplementation including regular vitamin A injections.
Ophthalmology review occurred 1 month later when full corneal healing was noted. The visual acuity had improved to 6/12 and since then he has had no further ocular problems.
Vitamin A deficiency and xerophthalmia are generally considered to be problems of developing countries. Vitamin A (retinol) is obtained from the diet and is required in the cornea for maintenance of epithelial surface integrity and immune competence. It is also required for normal retinal function.
Although dietary insufficiency is the leading cause of vitamin A deficiency in the developing world, liver disease and chronic malabsorption are the foremost aetiological factors in the West.1 Xerophthalmia secondary to avitaminosis A has been described extensively in the literature related to a wide variety of disorders such as cystic fibrosis,2 primary sclerosing cholangitis,3 primary biliary cirrhosis,3gross dietary malnutrition associated with chronic alcoholism4 5 or autism,6 7 and iatrogenically after small bowel resections and jejunoileal bypass surgery.8 Treatment consists of remedying the underlying vitamin deficiency with supplementation via the oral or intramuscular route, each of which have been demonstrated to be equally efficacious.9
In our case we made the diagnosis of hypovitaminosis A from the corneal pathology and rapid healing was seen once vitamin A supplementation was instituted. Although rare, the ophthalmologist must be alert to the potential causes of severe vitamin A deficiency and consider this diagnosis in unexplained keratomalacia, since early intervention will significantly minimise morbidity and prevent long term sequelae.