全前腦畸型：二病例報告

Holoprosencephaly: Two Cases Report

阮慶定(Ching-Ding Roan) ； 鄧木火(Michael Mu-Huo Teng) ； 何明德(Donald M. Ho) ； 黃棣棟(Tai-Tong Wong) ； 張遵(Tsuen Chang)

《中華放射線醫學雜誌》 13卷3期 (1988/09) Pp. 325-329

摘要

全前腦畸型是因胚胎在四、五週時，前腦從中線分裂不全所造成。依照其嚴重程度可分為大葉型、半葉型、及無葉型。本文提出二位半葉型全前腦畸型的病例。其中一例的母親在懷孕的前三個月有陰道出血現象，於39週出生，有染色體的不正常，出生後五個半月後死亡，在本院接受屍體解剖。另一例在懷孕34週時，因胎兒頭很大而剖腹生產，生產後因水腦症接受腦室一腹腔分流手術。

關鍵字

全前腦畸型 ； 雙眼間距較短 ； 背囊

並列摘要

Holoprosencephaly is a congenital malformation of the face and brain, which is caused by a defect of cleavage. It can be divided into alobar, semilobar, and lobar types according to the severity of its involvement.
In this paper, we report two cases of semilobar holoprosencephaly. One was born full term with chromosomal abnormality. His mother had vaginal bleeding during the first trimester. Patient died at the age of 5 months and autopsy was made. The other one was born at the age of 34 weeks by caesarean section because of hyclrocephalus. Thickening of the cerebral mantle was found after ventriculo-peritoneal shunt insertin.

並列關鍵字

holoprosencephaly ； hypotelorism ； dorsal cyst