LA00.1
枕骨裂脑露畸形
Iniencephaly
- 上级条目
- LA00 无脑畸形或类似异常
- 层级
- 5 级
定义
露脑畸形是一种罕见的神经管缺陷,其中颈颅交界畸形与中枢神经系统畸形有关。其主要特征是枕骨缺损,颈胸段椎骨部分或全部缺失,胎儿头部后屈和颈部特征性缺失。它与中枢神经系统(脊柱裂和/或无脑畸形)、胃肠系统(脐膨出)和心血管系统畸形有关。
Iniencephaly is a rare form of neural tube defect in which a malformation of the cervico-occipital junction is associated with a malformation of the central nervous system. The cardinal features are occipital bone defect, partial or total absence of cervicothoracic vertebrae, fetal retroflexion of the head and characteristic absence of the neck. It is associated with malformations of the central nervous (spina bifida and/or anencephaly), gastrointestinal (omphalocele) and cardiovascular systems.
索引词 可用于检索该条目的同义词与别名
- 枕骨裂脑露畸形
- 枕骨裂脑露畸胎
- 露脑畸形
- 开放性露脑
- 开放性枕骨裂脑露畸胎
- 闭合性露脑
- 闭合性枕骨裂脑露畸胎
英文词条(6)
- Iniencephaly
- Iniencephalus
- Open iniencephaly
- Open iniencephalus
- Closed iniencephaly
- Close iniencephalus
后组配轴 Postcoordination
该条目未定义后组配轴,直接使用上面的编码即可。后组配是在主干编码之后追加侧别、具体解剖部位、严重程度、病因等细节的可选组合方式,不是必填项。