Friday, March 23, 2007

Doyne Honeycomb Choroiditis

Doyne Honeycomb Choroiditis (Dominant Orbruch Membrane Drusen; Hutchinson-Tays Central Guttate Choroiditis; Holthouse-Batten Superficial Choroiditis; Malattia-Leventinese Syndrome)

General:
Autosomal dominant; represents early manifestation of senile macular degeneration; both sexes affected; onset in advanced age; patients present with drusen at an early age (second to third year of life) with near-normal visual acuity in childhood.

Ocular: Drusen with multiple yellow lesions becoming calcified and presenting crystalline appearance.

Clinical: None.

Lewis H, et al. Retinal degeneration of pigment epithelium. Ophthalmology 1985; 92:1485-1495.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

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: http://www.ncbi.nlm.nih.gov/omim/.

Reichel E, Sandberg MA. Hereditary macular degeneration. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, vol. II. Philadelphia: WB Saunders, 1994: 1254.

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