Leber Congenital Amaurosis

Leber Congenital Amaurosis or LCA is a rare eye condition that involves retinal degeneration, it is an inherited condition that typically surfaces at birth or within the fiirst few month's of an infants life, the condition can be chronic and patients with LCA can have very little or no light perception. In some cases, patients with Leber Congenital Amaurosis (LCA) can go blind in infancy. Symptoms of Leber Congenital Amaurosis include involuntary rhythmic eye movement (nystagmus), slow pupillary light response, misalignment of the eyes (strabismus), cataracts, keratoconus (a thin and cone-shaped cornea) and sensitivity to light (photophobia). An infant with very little vision as a result of Leber Congenital Amaurosis may rub and press their eyes, this is known as Franceshetti's oculo-digital sign and can result in damage to the lens of the eye as well as loss of the fatty tissue that surrounds the eyes, this can cause the eyes to look deep set.