Vitamin A deficiency and xerophthalmic fundus in autoimmune hepatitis and cirrhosis

To report a patient with autoimmune hepatitis and cirrhosis who was diagnosed with severe vitamin A deficiency based on the findings of xerophthalmic fundus.

Retrospective chart review.

A 36-year-old African American woman who had a history of autoimmune hepatitis and cirrhosis presents with peripheral retinal white spots, which led to the diagnosis of vitamin A deficiency.

The patient presented to the eye clinic for intermittent blurry vision 3 months after the diagnosis of liver cirrhosis secondary to autoimmune hepatitis since age 15. She also underwent partial large and small intestine resection as an infant for Hirschsprung disease. Anterior segment examination was significant for severe punctate epithelial erosions and posterior segment examination was significant for extensive white spots in the peripheral retina in both eyes. Workup showed near nondetectable levels of vitamin A (<0.06 mg/L, normal 0.3-1.2 mg/L) and retinyl palmitate (<0.02 mg/L, normal > 0.2 mg/L). After treatment with 200,000 IU of vitamin A orally, the white spots slowly resolved in ∼6 months. She never developed nyctalopia and her dry eye symptoms completely resolved.

Although rare, vitamin A deficiency should be considered in patients with liver cirrhosis especially in those with a history of intestinal surgeries. Early diagnosis and treatment with vitamin A supplementation can reverse the signs and symptoms of xerophthalmia.