De Morsier`s Syndrome Expressed as Congenital Exotropia.

Han, Jung Won; Lee, Young Chun

Original Article

Journal of the Korean Ophthalmological Society 1999;40(3):869-874.
Published online March 1, 1999.

De Morsier`s Syndrome Expressed as Congenital Exotropia.

Jung Won Han, Young Chun Lee

Department of Ophthalmology, Catholic University Medical College, Uijongbu St. Marys Hospital.

선천성 외사시로 표현된 De Morsier 증후군 1예

한정원(Jung Won Han),이영춘(Young Chun Lee)

Abstract

De Morsier syndrome, or septo-optic dysplasia is a developmental anomaly characterized by unilateral or bilaterral optic nerve hypoplasia, agenesis of septum pellucidum, visual disturbance, and a variable spectrum of disturbed hypothalamic-pituitary function. We experienced a 7-month-old female with de Morsier syndrome whose presenting manifestation was congenital exotropia of right eye, and subsequent evaluation revealed optic nerve hypoplasia of right eye, absence of septum pellucidum, and hypothyroidism.

Key Words: Congenital exotropia;De Morsier`s syndrome