Two Cases of Holoprosencephaly.

Chae, Ill Hyoun; Kim, Myoung Chan; Shin, Se Won; Lee, Seong Lim; Kim, In Sun; Kim, In Bae; Jo, Bong Choon; Choi, In Cheol; Park, Jong Gak

Case Report

Korean Journal of Obstetrics & Gynecology 1999;42(8):1869-1876.
Published online January 1, 2001.

Two Cases of Holoprosencephaly.

Ill Hyoun Chae, Myoung Chan Kim, Se Won Shin, Seong Lim Lee, In Sun Kim, In Bae Kim, Bong Choon Jo, In Cheol Choi, Jong Gak Park

Abstract

Holoprosencephaly is a rare malformation complex or development defect including different degrees of incomplete cleavages of the embryonic prosencephalon and varying degrees of the midface defects, resulting from the defect of prechordal mesoderm, migrating forward into the area anterior to the notochord during the third week of fetal development. Early antenatal diagnosis of holoprosencephaly is important to find out its severity, to predict its prognosis, and to determine proper management according to its prognosis and severity. The possibility of early antenatal diagnosis of holoprosencephaly by ultrasound has been suggested, but occasionally missed and rarely confirmed. We present one case of lobar holoprosencephaly, diagnosed postnatally and one case of alobar holoprosencephaly, diagnosed antenatally in our hospitals.

Key Words: Holoprosencephaly, Prechordal mesoderm, Antenatal diagnosis