Last update 23 Jan 2025

Corneal Dystrophy, Posterior Amorphous

Basic Info

Synonyms
CHROMOSOME 12q21.33 DELETION SYNDROME, CORNEAL DYSTROPHY, POSTERIOR AMORPHOUS, Corneal Dystrophy, Posterior Amorphous
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Introduction
A very rare form of stromal corneal dystrophy with characteristics of irregular amorphous sheet-like opacities in the posterior corneal stroma and in the Descemet membrane along with mildly impaired vision. Prevalence of this form of corneal dystrophy is not known. To date cases have been reported primarily in the USA. Patients usually develop corneal abnormalities in infancy or childhood. The condition is non-progressive or slowly progressive. Unlike other corneal dystrophies, non-corneal manifestations have been observed and include abnormalities of the iris including iridocorneal adhesions, corectopia, and pseudopolycoria. An autosomal dominant pattern of inheritance has been reported.

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