Peters anomaly (306229) usually occurs as an isolated ocular malformation and is often unilateral. However, in some patients with bilateral involvement it is part of a systemic syndrome or other congenital conditions such as chromosomal deletions and the fetal alcohol syndrome. It is called Peters Plus syndrome in the condition described here because of the association of a specific combination of systemic features.
The ocular features are consistent with dysgenesis of the anterior chamber. The clinical picture is highly variable but generally consists of iris adhesions to the cornea centrally (classical Peters anomaly), occasionally lenticular adhesions as well, and thinning of the central corneal stroma. As a result, the cornea may become edematous, cataracts may develop, and glaucoma is common.