Journal of the Korean Society of Neonatology  2004;11(2):247-251

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

Seok Woo PARK 1 ; Yun Hee KIM ; Tae Jeoung SUNG ; Young Se KWON ; Yong Hoon JUN ; Lucia KIM

Affiliations

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Keywords

Alobar holoprosencephaly

Country

Republic of Korea

Language

Korean

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.