J Korean Soc Neonatol.  2004 Nov;11(2):247-251.

A Case of Alobar Holoprosencephaly with Cyclopia and Proboscis in Prematurity

Affiliations
  • 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.

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.

Keyword

Alobar holoprosencephaly

MeSH Terms

Holoprosencephaly*
Humans
Infant, Newborn
Infant, Premature
Prosencephalon
Telencephalon
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