What is Peters Anomaly?
To see a picture showing Peters Anomaly http://newborns.stanford.edu/PhotoGallery/Peters1.html
Peters anomaly is a rare genetic disorder characterized by hazy corneas. The cornea is the clear front window of the eye, and the sclera is the tough ‘white’ of the eye. A child with the disorder is born with the central part of the cornea hazy, like the sclera. Glare occurs when light is scattered by the cloudy cornea, so the vision is worse in bright light . Abnormal development of structures in front of the vitreous homour (cornea, iris, ciliary body, and lens) of the eyes is found in Peters anomaly .
There are two types of Peters anomaly, depending on the lens’ condition. In type I, the lens does not adhere to the cornea. In type II, the lens is cataractous and adheres to the cornea. Familial isolated Peter anomaly is an autosomal recessive trait in most cases, but autosomal dominant inheritance pattern has been observed .
The majority of cases is sporadic, bilateral , and associated with other systemic malformations. Bilateral Peters anomaly has a stronger association (71.8%) with systemic malformation compared to unilateral Peters anomaly (36.8%) .
Peters anomaly is a common feature of Peters-plus syndrome, which is also known as Krause-Kivilin syndrome. Prenatal developmental delay can be observed in most cases, and postnatal clinical features include short stature and short-limb dwarfism. Affected individuals have a thin cupid-bow-shaped upper lip, and some may have a cleft lip and/or a cleft palate. 83% of those affected also exhibit mental delay .
Peters-plus syndrome is an autosomal recessive disorder. The causative mutations are in the B3GALTL gene, which is on the long arm of chromosome 13 .
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