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Matthias C. Grieshaber, MD; Selim Orgul, MD; Elisabeth Bruder, MD; Faruk Hadziselimovic, MD; Josef Flammer, MD

Arch Ophthalmol. 2006;124:1495-1497.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Congenital iris ectropion (CIE) associated with glaucoma is a rare ocular manifestation of a neural crest syndrome. We describe a 5-year-old boy with unilateral congenital iris ectropion, glaucoma, and features of intestinal neuronal dysplasia (IND) on rectal mucosal biopsy. The affected eye was buphthalmic and displayed an iridotrabecular dysgenesis with advanced glaucomatous damage. The contralateral eye showed a mild trabeculodysgenesis without ectropion uveae, coloboma, or sector heterochromia of the iris. The association of iridotrabecular dysgenesis and IND is suggested to represent a manifestation, to our knowledge not previously described, of a new neural crest syndrome.

Congenital iris ectropion is a rare, usually nonprogressive anomaly characterized by the posterior iris pigment epithelium on the anterior surface of the iris stroma, anterior insertion of the iris root, and trabecular dysgenesis. It is frequently associated with developmental . . . [Full Text of this Article]

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