A Case of Alobar Holoprosencephaly with Cyclopia and Proboscis in Prematurity.

Park,  Seok Woo; Kim,  Yun Hee; Sung,  Tae Jeoung; Kwon,  Young Se; Jun,  Yong Hoon; Kim,  Lucia

Case Report

Journal of the Korean Society of Neonatology 2004;11(2):247-251.
Published online November 1, 2004.

A Case of Alobar Holoprosencephaly with Cyclopia and Proboscis in Prematurity.

Seok Woo Park, Yun Hee Kim, Tae Jeoung Sung, Young Se Kwon, Yong Hoon Jun, Lucia Kim

¹Department of Pediatrics, College of Medicine, Inha University, Incheon, Korea. neojun@inha.ac.kr
²Department of Pediatrics, College of Medicine, Hanllym University, Seoul, Korea.
³Department of Pathology, College of Medicine, Inha University, Incheon, Korea.

양안 유합증과 안구위 관상돌기물 안면 기형이 관찰된 Alobar형의 전전뇌증 미숙아 1례

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Abstract

Holoprosencephaly is a developmental malformation complex of forebrain and midface which arises from incomplete cleavage of the embryonic forebrain. It is subdivided into alobar, semilobar and lobar types based on the degree of growth disturbance within the anterior wall of the telencephalon, particularly in the midline. Cyclopia is the most severe form of alobar holoprosencephaly presenting a single median eye and a blind-ending proboscis usually located above the eye. We report a case of alobar holoprosencephaly with cyclopia and proboscis in premature infant.

Key Words: Alobar holoprosencephaly