Zonular pulverulent cataract 1 is a form of cataracts that appears at birth or in early childhood. Cataracts is the clouding of the lens in the eye. The lens, which is usually clear, focuses light onto the eye. Zonular pulverulent cataract 1 is characterized by ‘puffy’ dark spots in the lens. These dark spots can cause problems with vision.
Zonular pulverulent cataract 1 is often linked with problems in the cornea (the outer layer of the eye). These problems include microcornea (abnormally small cornea) or sclerocornea (underdeveloped cornea). Problems in the cornea can lead to glaucoma, or damage to the optic (eye) nerve, which can cause blindness.
Zonular pulverulent cataract 1 is caused by a mutation (change) in the GJA8 gene. GJA8 is inherited in an autosomal dominant manner. Autosomal dominant means an individual only needs one copy of the changed gene or mutation that causes the condition. If one parent is a carrier for the mutated gene, children have a 50% chance of having the condition.
Treatment of zonular pulverulent cataract 1 may involve cataract surgery when loss of vision is severe. Usually, surgery becomes necessary in the first or second decade of life. If you or a family member has been diagnosed with zonular pulverulent cataract 1, talk to your doctor about the most current treatment options. Support groups are also a good source of information.
Description Last Updated: Aug 29, 2018