Congenital ectropion uveae and glaucoma.

Congenital ectropion uveae (CEU) is a rare, nonprogressive condition scarcely mentioned in the ophthalmic literature. According to its etiology, ectropion is classified into 2 groups: acquired and congenital. It is believed that the cause of acquired ectropion is membranous traction of the iris from secondary causes such as inflammation and ischemia. According to Dowling et al, the term congenital ectropion was introduced in 1869 by Colsman; however, it was later revealed that what was really described were flocculi. The first authors to actually describe congenital ectropion were Wicherkiewicz in 1891 and Spiro in 1896. Congenital ectropion uveae consists of iris pigment epithelium on the anterior surface of the iris, anterior insertion of the iris, dysgenesis of the drainage angle, and glaucoma. Although the actual anomaly is nonprogressive, multiple studies have linked it with the appearance of progressive open-angle glaucoma due to angle dysgenesis. Most descriptions of CEU have shown a relationship between the presence of ectropion and the eventual development of glaucoma. Some studies have also associated ectropion uveae with congenital defects and genetic diseases, but researchers have not yet verified whether the disease is genetically predetermined or whether it is clearly associated with any of these genetic defects. In this article, a case of CEU in a 3-year-old girl is reported. Histopathologic examination of an iris tissue specimen obtained during surgical management of glaucoma demonstrated iris neovascularization. Thorough clinical examination revealed no neoplasia. Implications of neovascularization in the pathogenesis of CEU are discussed.