분열뇌증을 동반한 중격 시신경 형성장애 신생아 1례

A Neonatal Case of Septo-Optic Dysplasia with Schizencephaly

  • 이원희 (조선대학교 의과대학 소아과학교실) ;
  • 김은영 (조선대학교 의과대학 소아과학교실) ;
  • 박상기 (조선대학교 의과대학 소아과학교실)
  • Lee, Won-Hee (Department of Pediatrics, Collage of Medicine Chosun University) ;
  • Kim, Eun-Young (Department of Pediatrics, Collage of Medicine Chosun University) ;
  • Park, Sang-Kee (Department of Pediatrics, Collage of Medicine Chosun University)
  • 발행 : 2009.05.31

초록

저자들은 산전과 출생 초기에 시행한 두부 초음파에서 뇌실 확장과 뇌량 무형성증을 보여 조기에 진단된 분열뇌증을 동반한 중격 시신경 형성장애 신생아 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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.

키워드

참고문헌

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