LCA Eye Disease

Leber congenital amaurosis (LCA) is an inherited eye disease characterized by very reduced vision at birth. The retinas appear normal during physical examination, but tests measuring visual function detect little activity in the retina.

First Signs

• The first signs of LCA eye disease typically become noticeable to the parents within the child's first few months of life. He shows lack of visual responsiveness, aimless roving eye movements and nystagmus, which looks like the eyes jerking back and forth.

Other Effects

• Children with LCA often have deep-set eyes and may rub at them with a fist or fingers. These children show sensitivity to bright light and poor tracking ability.

Acuity

• Vision usually does not deteriorate much further. Acuity typically is limited to the ability to count fingers, detect some motion and see bright lights.

Accompanying Disorders

• Some children with LCA also have central nervous system disorders, including epilepsy and motor skill impairment. Other disorders associated with LCA eye disease include skeletal, muscular, heart, ear and kidney problems. However, most children with LCA have normal intelligence.

Frequency

• LCA occurs in about 3 out of 100,000 births, according to the American Association for Pediatric Ophthalmology and Strabismus (AAPOS). LCA accounts for about 10 percent of cases of congenital blindness or severely-reduced vision.