Greig syndrome (ocular hypertelorism syndrome; hypertelorism; primary embryonic hypertelorism; hypertelorism ocularis)

General: Condition is rare; sporadic or hereditary; autosomal dominant or sex linked; if not associated with mental deficiency, then adequate mental and physical development is found.

Ocular: Hypertelorism (wide spacing of orbits); enophthalmos; epicanthus; deformities of eyelids and brows; defects of the palpebral fissure; bilateral sixth nerve paralysis; esotropia; astigmatism; optic atrophy by tension on the optic nerve; strabismus.

Clinical: Skull may show mild malformations, including bitemporal eminences and decreased anteroposterior diameter; harelip; high-arched palate; cleft palate; broad and flat nasal root; mental impairment.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Fuller RW, Weber AN. Hypertelorism in association with sex chromatin abnormality in two siblings. Am J Ment Defic 1961-l962; 66:844.

Greig DM. Hypertelorism. A hitherto undifferentiated congenital-craniofacial deformity. Edinburgh Med J 1924; 31: 560-593.