[Corneal Perforation Associated with Vitamin-A-deficiency]

Purpose: Vitamin A has been identified as a substantial substrate for ocular surface integrity. Vitamin A deficiency, which can be caused by different etiologies, may lead to severe complications including corneal perforation.

Patients: Case reports of three patients with vitamin A deficiency. The observed etiologies leading to vitamin A deficiency were: (1) primary biliary cirrhosis, (2) malnutrition induced by alcohol abuse, and (3) malabsorption syndrome caused by a graft-versus-host disease of the intestine after bone marrow transplantation.

Results: In all three cases vitamin A serum levels were below normal values. In two patients retinol binding protein was also reduced. Two of three had corneal perforation, which required penetrating keratoplasty (pKP). In one of these cases, pKP was performed three times on both eyes due to a rapid corneal stromal melting leading always to corneal perforation. The third case developed severe corneal thinning of both eyes with the formation of a descemetocele. Vitamin A substitution improved symptoms and ocular surface integrity.

Conclusion: Vitamin A in combination with zinc is a crucial factor for maintaining conjunctival and corneal ocular surface integrity. Moreover, vitamin A regulates mucin production of the ocular surface epithelium, which is known to be an important part of the ocular surface defense. Several different primary diseases might cause vitamin A deficiency. Especially patients with rapid progressive corneal ulceration should be examined for vitamin A deficiency. Substitution of vitamin A should be initiated as early as possible to prevent serious disease progression.