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Small Case Series |

Ocular Surface Disease Secondary to Vitamin A Deficiency in the Developed World: It Still Exists FREE

Phoebe Lin, MD, PhD; Robert E. Fintelmann, MD; Yousuf M. Khalifa, MD; Mohammed Rami Bailony, BS; Bennie H. Jeng, MD
[+] Author Affiliations

Author Affiliations: Department of Ophthalmology, University of California, San Francisco.


Arch Ophthalmol. 2011;129(6):798-804. doi:10.1001/archophthalmol.2011.126.
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Published online

Vitamin A deficiency remains a leading cause of preventable blindness worldwide but has only rarely been reported within the past 2 decades in the United States where nutritional deficiencies are not largely endemic. We describe 4 patients in the United States who had ocular surface disease as a result of vitamin A deficiency (Table).

Table Graphic Jump LocationTable. Patients With Vitamin A–Induced Ocular Surface Disease
Case 1

A 79-year-old woman with a 15-year history of primary biliary cirrhosis and secondary Sjögren syndrome presented with a 1-year history of chronic conjunctivitis refractory to treatment with topical antibiotics. Three months prior, the patient underwent a tectonic penetrating keratoplasty in her right eye because of a perforated corneal ulcer. She had nyctalopia for 2 years. Her visual acuity was 20/200 OD and counting fingers at 3 ft OS. In addition to diffuse punctate epithelial erosions in both eyes, she had a peripheral corneal ulcer in the left eye, which was treated with topical antibiotics. Five days later, the ulcer had progressed to a small perforation. This was managed with cyanoacrylate glue and a bandage contact lens. Her vitamin A level was 20 μg/dL (to convert to micromoles per liter, multiply by 0.0349) (reference range, 38-98 μg/dL). After intramuscular vitamin A administration, the patient's ocular condition stabilized, and she was discharged to follow up with her local ophthalmologist.

Case 2

A 54-year-old woman with a 10-year history of primary biliary cirrhosis was referred for severe dry eyes starting 6 months prior, which were unsuccessfully treated with cyclosporine A, 0.05%, and frequent lubrication. On examination, her visual acuity was 20/25 OD and 20/30 OS. Schirmer I test results were abnormal in both eyes. She had diffuse punctate epithelial erosions with filaments in both eyes and interpalpebral lissamine green staining. Her vitamin A level was 11 μg/dL, and she started treatment with vitamin A ointment, 0.01%, once daily at bedtime and oral vitamin A supplementation. Her symptoms and staining pattern improved significantly over 1 month.

Case 3

A 9-year-old autistic boy was referred for visual loss, in addition to red eyes and decreased night vision, over the past 4 months as noted by his mother. He had been diagnosed as having chronic conjunctivitis, which was unsuccessfully treated with a corticosteroid-antibiotic combination. On examination under anesthesia, he had chronic bilateral nonhealing epithelial defects, a relative afferent pupillary defect in the left eye, and profound optic atrophy in the left eye greater than in the right eye. His visual acuity was not attainable because of lack of cooperation. It was later discovered that his diet consisted exclusively of french fries. His vitamin A level was 3 μg/dL. His epithelial defects healed with frequent lubrication and remained healed with oral vitamin A supplementation.

Case 4

A 26-year-old homeless and severely depressed woman presented with recurrent corneal ulcers and corneal perforation necessitating penetrating keratoplasty in the right eye. Her ocular condition was thought to be secondary to severe atopic disease. On examination, she had hand motions vision OU, a failed corneal graft with corneal thinning and an epithelial defect in the right eye, and severe corneal thinning with a corneal perforation in the left eye. The corneal perforation in the left eye was managed with cyanoacrylate glue and a bandage contact lens. The patient was malnourished because of her social situation and psychiatric deterioration, with a vitamin A level of 5 μg/dL. After improving nutritional intake, both eyes improved, with resolution of the epithelial defect in the right eye and stabilization of the perforation in the left eye. She was discharged with recommendations to continue oral vitamin A supplementation.

The World Health Organization lists vitamin A deficiency as one of the most important causes of preventable childhood blindness.1 Dysfunction of corneal and conjunctival epithelial cell differentiation governed by vitamin A results in graded abnormalities in the ocular surface ranging from punctate epithelial erosions to corneal perforation.2,3 It can be initially misdiagnosed since the signs are easily mistaken for epithelial erosions seen in keratoconjunctivitis sicca or exposure keratopathy. Without treating the underlying vitamin A deficiency, these ocular surface conditions are progressive and refractory to lubricating therapy or even surgical intervention.

In the developed world where vitamin A deficiency is thought to be nearly eradicated, severe ocular surface disease due to vitamin A deficiency has been isolated to cases of malnutrition in patients with psychiatric conditions or malabsorption syndromes due to chronic liver or gastrointestinal disease.2,4 Cooney and associates4 described a malnourished psychiatric patient who, like case 4, had bilateral corneal perforations secondary to vitamin A deficiency before she was diagnosed and treated with the appropriate supplementation. Lewis and colleagues,5 similar to case 3, described an autistic child with a diet restricted mainly to carbohydrates who developed xerophthalmia due to profound vitamin A deficiency that improved only with vitamin A supplementation. The association between xerophthalmia and iatrogenically induced malabsorption syndromes in obese patients who have undergone bariatric surgery is also noteworthy given the increasing rate of obesity in the United States.6

Primary biliary cirrhosis is an autoimmune condition characterized by the progressive destruction of intrahepatic biliary canaliculi, ultimately resulting in malabsorption of fat-soluble vitamins. A significant proportion of patients with primary biliary cirrhosis also have secondary Sjögren syndrome. In the patients who have developed vitamin A deficiency, mucin deficiency occurs as a result of abnormal terminal differentiation of the conjunctival goblet cells in which vitamin A plays a crucial role, and aqueous tear deficiency from secondary Sjögren syndrome compounds the effect of mucin deficiency on corneal health. In cases 2 and 3, although anti-Ro and anti-La antibody tests were not performed, given that clinical improvement was only seen after initiation of vitamin A therapy, we believe that the vitamin A deficiency played a more significant role than the secondary Sjögren syndrome in the development of the clinical findings.

In all 4 cases, the underlying vitamin A deficiency was not recognized until severe ocular surface disease was seen, in many cases requiring surgical intervention. This may be because of the perception that vitamin A deficiency does not occur in developed nations. However, our cases remind us that in refractory cases of ocular surface disease not amenable to standard treatments, especially in the setting of psychiatric conditions, autistic children, and malabsorption syndromes, one should consider vitamin A deficiency. Complications of this condition are preventable with vitamin A supplementation by the appropriate route.

Correspondence: Dr Jeng, Department of Ophthalmology, University of California, San Francisco, 10 Koret Way, K-304, San Francisco, CA 94143-0730 (jengb@vision.ucsf.edu).

Financial Disclosure: None reported.

Funding/Support: This work was supported in part by an unrestricted grant from Research to Prevent Blindness and a That Man May See grant to the Department of Ophthalmology, University of California, San Francisco.

Previous Presentation: This study was presented in part at the Association for Research in Vision and Ophthalmology annual meeting; May 3, 2010; Fort Lauderdale, Florida.

World Health Organization, Global Prevalence of Vitamin A Deficiency in Populations at Risk 1995–2005. WHO Global Database on Vitamin A Deficiency.  Geneva, Switzerland World Health Organization2009;
Heinz  CSteuhl  KPMeller  D Corneal perforation associated with vitamin-A-deficiency [in German]. Ophthalmologe 2004;101 (6) 614- 617
PubMed
Smith  JSteinemann  TL Vitamin A deficiency and the eye. Int Ophthalmol Clin 2000;40 (4) 83- 91
PubMed
Cooney  TMJohnson  CSElner  VM Keratomalacia caused by psychiatric-induced dietary restrictions. Cornea 2007;26 (8) 995- 997
PubMed
Lewis  CDTraboulsi  EIRothner  ADJeng  BH Xerophthalmia and intracranial hypertension in an autistic child with vitamin A deficiency [published online March 26, 2010]. J Pediatr Ophthalmol Strabismus 2010;1- 3
PubMed10.3928/01913913-20100318-04
Lee  WBHamilton  SMHarris  JPSchwab  IR Ocular complications of hypovitaminosis a after bariatric surgery. Ophthalmology 2005;112 (6) 1031- 1034
PubMed

Figures

Tables

Table Graphic Jump LocationTable. Patients With Vitamin A–Induced Ocular Surface Disease

References

World Health Organization, Global Prevalence of Vitamin A Deficiency in Populations at Risk 1995–2005. WHO Global Database on Vitamin A Deficiency.  Geneva, Switzerland World Health Organization2009;
Heinz  CSteuhl  KPMeller  D Corneal perforation associated with vitamin-A-deficiency [in German]. Ophthalmologe 2004;101 (6) 614- 617
PubMed
Smith  JSteinemann  TL Vitamin A deficiency and the eye. Int Ophthalmol Clin 2000;40 (4) 83- 91
PubMed
Cooney  TMJohnson  CSElner  VM Keratomalacia caused by psychiatric-induced dietary restrictions. Cornea 2007;26 (8) 995- 997
PubMed
Lewis  CDTraboulsi  EIRothner  ADJeng  BH Xerophthalmia and intracranial hypertension in an autistic child with vitamin A deficiency [published online March 26, 2010]. J Pediatr Ophthalmol Strabismus 2010;1- 3
PubMed10.3928/01913913-20100318-04
Lee  WBHamilton  SMHarris  JPSchwab  IR Ocular complications of hypovitaminosis a after bariatric surgery. Ophthalmology 2005;112 (6) 1031- 1034
PubMed

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