SEPTO-OPTIC DYSPLASIA ASSOCIATED WITH BILATERAL COMPLEX MICROPHTHALMOS

An 8-month-old girl was examined because of corneal clouding and micro phthalmos. The fundi of both eyes could not be visualized because of c orneal clouding. Orbital and cranial computerized tomographic scanning and magnetic resonance imaging demonstrated bilateral microphthalmos and presumed retinal dysplasia, hypoplasia of the optic nerves and chi asm, agenesis of the septum pellucidum, thinning of corpus callosum, a nd a normal pituitary infundibulum. Cerebral cortex and white matter w ere unremarkable. Other ocular malformations were anterior segment dys genesis in the right eye and congenital cataract or lens abnormality i n the left eye. Endocrine studies revealed normal serum hormone levels . There were no colobomatous lesions and systemic anomalies suggestive of a coloboma syndrome. This case represents the rare association of septo-optic dysplasia with complex microphthalmos.