Cataract, congenital total with posterior sutural opacities    206

General: Sex-linked; initial lens changes occur in both men and women with continuation of process in men; women show progression at much later age; it has been suggested that several X-linked cataract syndromes are due to deletions of different sizes in the X chromosome.

Ocular: Y-shaped sutural cataracts; congenital cataracts; nuclear cataract; cortical cataract; posterior subcapsular cataract; asymptomatic posterior y-sutural cataracts; severe visual impairment; bilateral pendular nystagmus; bilateral microcornea; exotropia; keratoconus.

Clinical: Mental retardation.

Crews SJ, Bundey SE. Is there an X-linked form of congenital cataracts? Clin Genet 1982; 21:351-353.

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/.

Waarburg M. X-linked cataract and X-linked microphthalmos: how many deletion families? [Letter] Am J Med Genet 1989; 34:451-453.