Vitamin a deficiency and xerophthalmic fundus in autoimmune hepatitis and cirrhosis.

[hirschsprung disease]

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 .