J Korean Soc Neonatol.  2009 May;16(1):81-84.

A Neonatal Case of Septo-Optic Dysplasia with Schizencephaly

Affiliations
  • 1Department of Pediatrics, Collage of Medicine Chosun University, Gwangju, Korea. skpark@chosun.ac.kr

Abstract

Septo-optic dysplasia is a rare congenital disorder characterized by the absence of the septum pallucidum, hypoplasia of the optic chiasma and nerves, and various types of hypothalamic-pituitary dysfunction. Schizencephaly is an abnormal cleft in the brain, lined with gray matter which may communicate with the ventricular system. Septo-optic dysplasia with schizencephaly is associated with endocrinologic disorders, visual impairment, mental retardation, and seizures. We report a case of septo-optic dysplasia with schizencephaly which was diagnosed in the early neonatal period.

Keyword

Septo-optic dysplasia; Schizencephaly

MeSH Terms

Brain
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Intellectual Disability
Malformations of Cortical Development
Optic Chiasm
Seizures
Septo-Optic Dysplasia
Vision Disorders
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