Delezoide A L, Narcy F, Larroche J C
Laboratoire d'Histo-Cyto-Embryologie, Hôpital Necker - Enfants Malades, Paris.
Genet Couns. 1990;1(3-4):197-210.
Cerebral midline anomalies are defects of anatomical relationships between the two hemispheres. They include holoprosencephalies, septal and commissural agenesis. Agenesis of the olfactory tract (arhinencephalies) are often included in the spectrum of holoprosencephalies and the facial phenotype is thought to be affected and characteristic in the midline development abnormalities. This work concerns a review of the literature and personal experience in two units of Fetopathology in Paris. This study confirms the relationships between various cerebral malformations and their frequent association. However, arhinencephaly and moreover agenesis of corpus callosum should be considered as heterogeneous entities, often totally distinct and independent from the malformative process of the holoprosencephaly. In addition, if major facial anomalies such as cyclopia are almost pathognomonic for holoprosencephaly, minor malformations such as lateral facial clefts of cleft palates result from a great variety of malformative processes.
大脑中线异常是两个半球之间解剖关系的缺陷。它们包括全前脑畸形、中隔和连合发育不全。嗅束发育不全（无脑回畸形）通常包含在全前脑畸形范围内，并且面部表型被认为在中线发育异常中受到影响且具有特征性。这项工作涉及对巴黎两个胎儿病理学单位的文献回顾和个人经验。本研究证实了各种脑畸形之间的关系及其频繁的关联。然而，无脑回畸形以及胼胝体发育不全应被视为异质性实体，通常与全前脑畸形的形成过程完全不同且独立。此外，如果诸如独眼畸形等主要面部异常几乎是全前脑畸形的特征性表现，那么诸如腭裂等轻微畸形则由多种形成过程导致。
