SECTION EDITOR: ELISABETH J. COHEN, MD Vitamin A Deficiency and Xerophthalmia in an Autistic Child A 5-YEAR-OLD boy with severe autism was referred to the cor- neal and external disease service with a history of bilateral corneal ulcer- ation. The referring ophthalmolo- gist had examined the child under anesthesia and noted an active cor- neal ulcer in the right eye and a healed ulcer in the left eye. The pa- tient’s mother stated that his eyes had appeared red and that he was light sensitive for several weeks, rub- bing both eyes frequently during this time. She also noted that the child appeared to be clumsy, bumping into and falling over furniture inside the house. A second examination under anesthesia at our institution re- vealed generalized hyperkeratosis and lash hypertrichosis (Figure 1). The right eye was moderately vaso- dilated. The ocular surface was dry and lusterless, and there was dif- fuse rose bengal staining bilaterally (Figure 2 and Figure 3). Bitot spots were seen at the superior lim- bus of the left eye (Figure 3). A cen- tral ulcer was cultured in the right eye (Figure 2) and yielded Strepto- coccus pneumoniae. The dilated reti- nal evaluation disclosed numerous yellow flecks in the peripheral retina at the level of the retinal pigment epi- thelium (Figure 4). A conjuncti- val biopsy specimen displayed keratinized conjunctival epithe- lium (Figure 5). Intensive antibi- otic drop therapy (hourly cefazolin sodium [50 mg/mL] and 0.3% ofloxacin) was started. Another ex- amination under anesthesia was per- formed 5 days later and disclosed a corneal descemetocele in the right eye (Figure 6). Further questioning of the pa- tient’s mother revealed that the pa- tient had a very poor diet. She stated that he was a picky eater who would eat only bacon and an occasional blueberry muffin and would drink only Kool Aid. The child had repeat- edly refused vitamin supplementa- tion. A serum vitamin A level mea- sured less than 50 μg/L (normal range, 360-1200 μg/L [SI units equivalent is measured in micro- moles per liter]). The following day, a penetrat- ing keratoplasty and tarsorrhaphy were performed on the right side, as well as 4-eyelid punctal occlusion. Vitamin A palmitate, 100 000 USP units, was given intramuscularly. The vitamin A injection was re- peated (50 000 USP units intramus- cularly) 2 months later. The patient returned for an evaluation under anesthesia 3 months after the penetrating kera- toplasty. The ocular surface ap- peared normal in each eye. The cor- neal graft was clear and lustrous. A scotopic and photopic electroreti- nogram was performed and results were normal for each eye. A Teller binocular acuity was 6.5 cycles/cm, measured at 84 cm. COMMENT Vitamin A deficiency with second- ary xerophthalmia is uncommon in developed countries. 1 When a sys- temic (absorptive or hepatic) ori- gin has been ruled out, dietary de- ficiency must be considered, and it has been reported in patients with mental illness, autism, and fad diet- ing. 2 This patient displayed classic features of ocular complications re- lated to vitamin A deficiency, in- cluding conjunctival and corneal xe- rosis, Bitot spots, keratomalacia, and xerophthalmic fundus lesions. Thomas L. Steinemann, MD Stephen P. Christiansen, MD Little Rock, Ark This study was supported in part by an unrestricted grant from Research to Prevent Blindness Inc, New York, NY. Reprints: Thomas L. Steine- mann, MD, Department of Ophthal- mology, Jones Eye Institute, Univer- sity of Arkansas, 4301 W Markham St, Mail Slot 523, Little Rock, AR 72205. REFERENCES 1. Sommer A. Nutritional blindness: xerophthalmia and keratomalacia. In: Duane TD, ed. Clinical Oph- thalmology. Vol 5. Philadelphia, Pa: JB Lippin- cott; 1988:chap 6. 2. Pyott AAE, Kirkness CM. Nutritional Disorders. In: Krachmer JH, Mannis MJ, Holland EF, eds. Cor- nea . Vol 2. St Louis, Mo: Mosby–Year Book Inc; 1997:955-966. PHOTO ESSAY ARCH OPHTHALMOL / VOL 116, MAR 1998 392 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ MetroHealth Medical Center by Thomas Steinemann on 10/29/2023