was given antihistamine eyedrops, was 3 pg/mL (7.8 pmol/L) (refer- Blindness in an American Boy and was discharged home with a di- ence range, 8.9-47.0 pg/mL [23.1- Caused by Unrecognized agnosis of an allergic reaction. 122.2 pmol/L]). He received 5 days Deficiency The parents subsequently took of high-dose vitamin A supplemen- the child to a Chinese herbalist who tation and 12 days of zinc supple- is an enor- gave the patient unknown eyedrops mentation, as well as high doses of mous problem in the developing for the next 2 days, with improve- cholecalciferol. The boy also had world, where UNICEF and other or- ment of the child’s redness and swell- macrocystic anemia on admission, ganizations now distribute more than ing. The parents thought no further and therefore cyanocobalamin and 400 million high-dose, biannual medical advice was necessary. Three iron supplementation was started. supplements every year. In devel- weeks afterward, however, they no- He also received treatment for oped countries vitamin A deficiency ticed that the boy did not want to get thrombophlebitis of his right foot. is rarely found. Xerophthalmia out of bed because of He was discharged after 3 weeks of caused by vitamin deficiency has been and decreased vision. The parents hospitalization. reported in food faddists and psychi- brought him to another medical in- On ophthalmic evaluation at atric patients.1 However, most of the stitution in March 2002, where the Massachusetts Eye and Ear Infir- cases observed in developed coun- physicians discovered that the pa- mary in December 2002, the pa- tries are due to alcoholism and con- tient was on a strict vegetarian diet tient had a visual acuity of no light ditions causing malabsorption.2 Since also followed by the parents (no meat, perception in both eyes, extraocu- better nutritional standards in afflu- no eggs, no dairy, and no fish). The lar motility was full in both eyes, and ent societies make vitamin A defi- boy had narrowed his diet to potato the eyes were soft by palpation. Slit- ciency with ocular complications chips, rice, soy milk, and tofu. lamp examination showed areas of rare, early diagnosis of such defi- On examination, the child was corneal thinning and ectasia, se- ciency may be overlooked. We re- not able to identify objects, and oph- verely vascularized and conjuncti- port a case of bilateral keratomala- thalmologic examination under an- valized , and no view of the cia caused by vitamin A deficiency, esthesia showed bilateral corneal anterior chamber. The patient was leading to bilateral irreversible blind- perforation and conjunctival kera- sent to the Service for con- ness in a 6-year-old Asian child liv- tinization with aqueous leaking from sultation. Ultrasonography showed ing in New York, NY. the left eye. The patient was taken a total cicatricial retinal detach- to the operating room for bilateral ment in the left eye, but only a trac- Report of a Case. A 6-year-old Asian debridement of the corneal ulcers tion detachment in the right eye that, boy was referred to the Ser- and corneal transplants. The post- to one examiner, appeared to have vice of the Massachusetts Eye and operative note reported total cor- some light perception (Figure). Ear Infirmary, Boston, in Decem- neal melts, epithelial ingrowth, ex- The patient came for follow-up ber 2002 with a 10-month history of trusion of vitreous, and choroidal 6 months later, and light perception bilateral corneal ulceration, perfo- and retinal detachments in both eyes. in the right eye was confirmed. B- ration, keratoplasty, and, finally, ir- The patient received 0.3% tobramy- scan ultrasonography was identical reparable retinal detachments. cin and 0.1% dexamethasone so- to that from his previous visit. The The parents reported that 3 dium phosphate postoperatively. patient underwent an attempt at sur- months before their visit to an out- The patient was found to be se- gical rehabilitation of the right eye in- side institution, the boy appeared to verely vitamin A and zinc deficient, cluding removal of the conjunctival have “swollen” eyes and gradual vi- as well as mildly protein deficient. flap, penetrating keratoplasty (8.5 sion loss after a classmate “splashed Vitamin A level was undetectable, mm/8.0 mm), intracapsular chocolate” in his face. He was zinc level was 45 µg/dL (6.9 µmol/L) extraction, and anterior vitrectomy. brought to the emergency depart- (reference range, 57-113 µg/dL [8.7- One month after surgery, visual ment of that institution, where he 17.3 µmol/L), and vitamin D level acuity remained light perception in

A B

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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©2004 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 the right eye and no light perception 1. Buchanan NM, Atta HR, Crean GP, McColl KE. ity was 20/30 in the right eye, and A case of due to dietary vitamin A de- in the left eye. Slitlamp examination ficiency in Glasgow. Scott Med J. 1987;32:52-53. hand motions close to his face with showed an edematous graft in the 2. Raynor RJ, Tyrrell JC, Hiller EJ, et al. Night blind- accurate projection of rays in the left ness and conjunctival xerosis caused by vita- eye. Intraocular pressure in the right right eye and an opacified, vascular- min A deficiency in patients with cystic fibro- ized cornea in the left eye. Renewed sis. Arch Dis Child. 1989;64:1151-1156. and left eyes was 14 and 12 mm Hg, B-scan ultrasonography in the right 3. al-Husainy S, Deane J. Bilateral in respectively. The left eye revealed a a cachectic scleroderma patient. Eye. 1999;13: eye showed that the patient now had 586-588. thin moderate-sized bleb and a quiet total in a funnel 4. Slansky HH, Dohlman CH. Collagenase and the anterior chamber with normal depth. configuration. The left eye showed a cornea. Surv Ophthalmol. 1970;14:402-415. Results of a dilated examination re- 5. Sommer A. Xerophthalmia, keratomalacia and tight funnel retina as previously seen. nutritional blindness. Int Ophthalmol. 1990;14: vealed a posterior at the pu- This configuration was considered in- 195-199. pillary margin at the 1 o’clock po- operable, and given the extent of the sition and a peripheral iridectomy in patient’s anterior segment problems, the same meridian. The crystalline further surgery was deferred. Capsular Bag Hematoma appeared to have a brownish Following Trabeculectomy hue throughout, with a bright red Comment. Xerophthalmia (xero- collection in the anterior subcapsu- sis, dry; ophthalmia, inflamed eye) is Since its introduction in 1967, tra- lar area just behind the area of pos- a term that includes all ocular mani- beculectomy has become the stan- terior synechia (Figure, arrow). festations of vitamin A deficiency dard surgical treatment modality for There was no view of the posterior (night blindness to keratomalacia) most forms of . The early segment. Findings from a B-scan ul- and has been categorized by the postoperative complications re- trasonogram revealed a normal pos- World Health Organization. ported are hypotony, shallow or flat terior segment in the left eye. The The differential diagnosis of anterior chamber, , cho- patient was posted for phacoemul- keratomalacia includes severe sicca roidal detachment, uveal effusion, sification and aspiration of the blood syndrome, , or wound leak, malignant glaucoma, along with intraocular lens implan- (infectious, neuro- suprachoroidal hemorrhage, and tation. Capsular staining with trypan pathic, or autoimmune).3 Xeroph- .1,2 We describe an blue failed to provide adequate con- thalmic ulceration ranges from small, interesting case of a capsular bag trast in view of the dark reflex of characteristically sharp-margined ul- hematoma following trabeculec- intralenticular contents. Capsu- cers located in the periphery of the tomy, a hitherto unreported com- lorhexis was then achieved from the cornea, to full-thickness, nearly lim- plication. anterior capsule reflex under high bus-to-limbus melting. The mecha- magnification. Phacoemulsifica- nism of corneal necrosis remains un- Report of a Case. A 57-year-old man tion power was totally ineffective in clear, but it has been postulated that was initially examined at our ter- removing the blood-impregnated inflammatory cells releasing prote- tiary care institute and had a his- epinuclear shell, and it had to be ases such as collagenases may be re- tory of total visual loss in his left eye manually stuffed into the port of the sponsible for the corneal necrosis.4,5 following surgery for glaucoma else- phaco tip with a chopper. A nor- When keratomalacia has pro- where 3 weeks earlier. On exami- mal red reflex was achieved as soon gressed to almost total melt involv- nation, his best-corrected visual acu- as this blood clot was removed, and ing the entire cornea, vitamin A treat- ment 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 di- agnosed late and led to blindness de- spite the best medical effort. This case reminds us once again that social cus- toms, cultural differences, and life- style matter in making an accurate and prompt diagnosis.

Margarita I. Rodrigues, MD Claes H. Dohlman, MD, PhD

The authors have no relevant finan- cial interest in this article. Correspondence: Dr Dohlman, De- partment of , Massa- chusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114 (claes Left eye of the patient showing a brownish hue of intralenticular contents. Note the area of bright red [email protected]). anterior subcapsular blood collection (arrow).

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