Basal cell nevus syndrome (nevoid basal cell carcinoma syndrome; nevoid basalioma syndrome; gorlin syndrome; gorlin-goltz syndrome; multiple basal cell nevi syndrome)  124

General: Autosomal dominant; onset of 858g64i skin lesions in childhood, usually at puberty.

Ocular: Basal cell carcinomas of eyelids; strabismus; hypertelorism; congenital cataracts; choroidal colobomas; glaucoma; medullated nerve fibers; prominence of supraorbital ridges; corneal leukoma; basalioma of the skin; coloboma of the choroid and optic nerve.

Clinical: Basal cell tumors with facial involvement; shallow pits of the skin of the hands and feet; jaw cysts; rib anomalies; kyphoscoliosis and fusion of vertebrae; medulloblastoma; frontal and temporoparietal bossing; broad nasal root.

Collins JF. Handbook of Clinical Ophthalmology. New York: Masson, 1982:263-264.

Dejong PT, et al. Medullated nerve fibers: a sign of multiple basal cell nevi (Gorlin's) syndrome. Arch Ophthalmol 1985; 103: 1833-l836.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Gorlin RJ. Goltz RW Multiple nevoid basal cell epithelioma, jaw cysts and bifid rib: a syndrome. N Engl J Med 262:908.

Manzi G, et al. The Gorlin-Goltz syndrome: case report. Ophthalmologica 1990; 200:104-l06.