Corneal dystrophy, hereditary polymorphous posterior (pcd)  298

General: Autosomal dominant.

Ocular: Clouding of posterior cornea; reduced number of endothelial cells; thickening and opacities of Descemet membrane; associated with keratoconus; iridocorneal adhesions; glassy membranes; pupillary ectropion (rare).

Clinical: None.

Driver PJ, et al. Familial cases of keratoconus associated with posterior polymorphous dystrophy. Am J Ophthalmol 1994; 118:256-257.

Laganowski HC, et al. Distinguishing features of the iridocorneal endothelial syndrome and posterior polymorphous dystrophy: value of endothelial specular microscopy. Br J Ophthalmol 1991; 75:212-216.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: https://www.ncbi.nlm.nih.gov/omim/.

Rodrigues MM, et al. Posterior polymorphous corneal dystrophy: recent developments. Birth Defects 1982; 18: 479-491.

Sekundo W, et al. An ultrastructural investigation of an early manifestation of the posterior polymorphous dystrophy of the cornea. Ophthalmology 1994; 101:1422-l431.