ICD-11 definition : 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.;
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.