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7 affected and 4 normal individuals from three consecutive generations of a family with central areolar pigment epithelial dystrophy were examined. The pedigree was indicative of an autosomal dominant disorder with variable expression. The macular changes varied from numerous small drusen-like defects to large colobomatous appearing lesions while the visual acuities ranged from 6/4 to 3/120. Some of those with excellent visual acuity had small central scotomata. Urinary metabolic screening, plasma amino acid levels and 24-hour urinary amino acid levels were normal.

A four-year-old girl sustained facial injuries from multiple dog bites. Although the globe exhibited slight adduction saccades the medial rectus muscle appeared to be intact when examined by computer tomography. However, on surgical exploration of the orbit the muscle parenchyma was found to have been completely severed.

SUMMARY A family with au t oso mal dominant megalocornea was studied clinically and echographically. The anterior chamber angles, intraocular pressures and axial lengths were all normal. These findings strongly support the dualist proposition which contends that e ti o logic ally, megalocornea and congenital glaucoma are distinct.