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Blindness in an American Boy Caused by Unrecognized Vitamin A Deficiency
Arch Ophthalmol. 2004;122:1228-1229.
Vitamin A deficiency is an enormous problem in the developing world, where UNICEF and other organizations now distribute more than 400 million high-dose, biannual supplements every year. In developed countries vitamin A deficiency is rarely found. Xerophthalmia caused by vitamin deficiency has been reported in food faddists and psychiatric patients.1 However, most of the cases observed in developed countries are due to alcoholism and conditions causing malabsorption.2 Since better nutritional standards in affluent societies make vitamin A deficiency with ocular complications rare, early diagnosis of such deficiency may be overlooked. We report a case of bilateral keratomalacia caused by vitamin A deficiency, leading to bilateral irreversible blindness in a 6-year-old Asian child living in New York, NY.
Report of a Case
A 6-year-old Asian boy was referred to the Cornea Service of the Massachusetts Eye and Ear Infirmary, Boston, in December 2002 with a 10-month history of bilateral corneal ulceration, perforation, keratoplasty, and, finally, irreparable retinal detachments.
The parents reported that 3 months before their visit to an outside institution, the boy appeared to have "swollen" eyes and gradual vision loss after a classmate "splashed chocolate" in his face. He was brought to the emergency department of that institution, where he was given antihistamine eyedrops, and was discharged home with a diagnosis of an allergic reaction.
The parents subsequently took the child to a Chinese herbalist who gave the patient unknown eyedrops for the next 2 days, with improvement of the child's redness and swelling. The parents thought no further medical advice was necessary. Three weeks afterward, however, they noticed that the boy did not want to get out of bed because of photophobia and decreased vision. The parents brought him to another medical institution in March 2002, where the physicians discovered that the patient was on a strict vegetarian diet also followed by the parents (no meat, no eggs, no dairy, and no fish). The boy had narrowed his diet to potato chips, rice, soy milk, and tofu.
On examination, the child was not able to identify objects, and ophthalmologic examination under anesthesia showed bilateral corneal perforation and conjunctival keratinization with aqueous leaking from the left eye. The patient was taken to the operating room for bilateral debridement of the corneal ulcers and corneal transplants. The postoperative note reported total corneal melts, epithelial ingrowth, extrusion of vitreous, and choroidal and retinal detachments in both eyes. The patient received 0.3% tobramycin and 0.1% dexamethasone sodium phosphate postoperatively.
The patient was found to be severely vitamin A and zinc deficient, as well as mildly protein deficient. Vitamin A level was undetectable, zinc level was 45 µg/dL (6.9 µmol/L) (reference range, 57-113 µg/dL [8.7-17.3 µmol/L), and vitamin D level was 3 pg/mL (7.8 pmol/L) (reference range, 8.9-47.0 pg/mL [23.1-122.2 pmol/L]). He received 5 days of high-dose vitamin A supplementation and 12 days of zinc supplementation, as well as high doses of cholecalciferol. The boy also had macrocystic anemia on admission, and therefore cyanocobalamin and iron supplementation was started. He also received treatment for thrombophlebitis of his right foot. He was discharged after 3 weeks of hospitalization.
On ophthalmic evaluation at Massachusetts Eye and Ear Infirmary in December 2002, the patient had a visual acuity of no light perception in both eyes, extraocular motility was full in both eyes, and the eyes were soft by palpation. Slitlamp examination showed areas of corneal thinning and ectasia, severely vascularized and conjunctivalized corneas, and no view of the anterior chamber. The patient was sent to the Retina Service for consultation. Ultrasonography showed a total cicatricial retinal detachment in the left eye, but only a traction detachment in the right eye that, to one examiner, appeared to have some light perception (Figure 1).
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External appearance of right eye (A) and left eye (B) 1 year after bilateral corneal ulceration caused by vitamin A deficiency.
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The patient came for follow-up 6 months later, and light perception in the right eye was confirmed. B-scan ultrasonography was identical to that from his previous visit. The patient underwent an attempt at surgical rehabilitation of the right eye including removal of the conjunctival flap, penetrating keratoplasty (8.5 mm/8.0 mm), intracapsular cataract extraction, and anterior vitrectomy.
One month after surgery, visual acuity remained light perception in the right eye and no light perception in the left eye. Slitlamp examination showed an edematous graft in the right eye and an opacified, vascularized cornea in the left eye. Renewed B-scan ultrasonography in the right eye showed that the patient now had total retinal detachment in a funnel configuration. The left eye showed a tight funnel retina as previously seen. This configuration was considered inoperable, and given the extent of the patient's anterior segment problems, further surgery was deferred.
Comment
Xerophthalmia (xerosis, dry; ophthalmia, inflamed eye) is a term that includes all ocular manifestations of vitamin A deficiency (night blindness to keratomalacia) and has been categorized by the World Health Organization.
The differential diagnosis of keratomalacia includes severe sicca syndrome, exposure keratopathy, or corneal ulcer (infectious, neuropathic, or autoimmune).3 Xerophthalmic ulceration ranges from small, characteristically sharp-margined ulcers located in the periphery of the cornea, to full-thickness, nearly limbus-to-limbus melting. The mechanism of corneal necrosis remains unclear, but it has been postulated that inflammatory cells releasing proteases such as collagenases may be responsible for the corneal necrosis.4-5
When keratomalacia has progressed to almost total melt involving the entire cornea, vitamin A treatment has virtually no effect. Herein we have described the unexpected tragic evolution of a case of vitamin A deficiency in a boy from a highly educated, affluent family that was diagnosed late and led to blindness despite the best medical effort. This case reminds us once again that social customs, cultural differences, and lifestyle matter in making an accurate and prompt diagnosis.
The authors have no relevant financial interest in this article.
AUTHOR INFORMATION
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Margarita I. Rodrigues, MD;
Claes H. Dohlman, MD, PhD
Correspondence: Dr Dohlman, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114 (claes_dohlman{at}meei.harvard.edu).
REFERENCES
1. Buchanan NM, Atta HR, Crean GP, McColl KE. A case of eye disease due to dietary vitamin A deficiency in Glasgow. Scott Med J. 1987;32:52-53.
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2. Raynor RJ, Tyrrell JC, Hiller EJ, et al. Night blindness and conjunctival xerosis caused by vitamin A deficiency in patients with cystic fibrosis. Arch Dis Child. 1989;64:1151-1156.
ABSTRACT
3. al-Husainy S, Deane J. Bilateral keratomalacia in a cachectic scleroderma patient. Eye. 1999;13:586-588.
4. Slansky HH, Dohlman CH. Collagenase and the cornea. Surv Ophthalmol. 1970;14:402-415.
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5. Sommer A. Xerophthalmia, keratomalacia and nutritional blindness. Int Ophthalmol. 1990;14:195-199.
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SECTION EDITOR: W. RICHARD GREEN, MD
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