Iniencephaly
232047
221478086
2008-06-24T17:56:46Z
Ufinne
4476154
fi:
{{DiseaseDisorder infobox |
Name = {{PAGENAME}} |
ICD10 = {{ICD10|Q|00|2|q|00}} |
ICD9 = {{ICD9|740.2}} |
ICDO = |
Image = |
Caption = |
OMIM = |
OMIM_mult = |
MedlinePlus = |
eMedicineSubj = |
eMedicineTopic = |
DiseasesDB = |
}}
'''Iniencephaly''' is a type of [[cephalic disorder]]. It is a rare neural tube defect that combines extreme retroflexion (backward bending) of the head with severe defects of the [[spine (anatomy)|spine]].
==Presentation==
The affected infant tends to be short, with a disproportionately large head. Diagnosis can be made immediately after birth because the head is so severely retroflexed that the face looks upward. The [[skin]] of the face is connected directly to the skin of the [[chest]] and the scalp is directly connected to the skin of the [[back]]. Generally, the [[neck]] is absent.
Most individuals with iniencephaly have other associated anomalies such as [[anencephaly]], [[encephalocele]] (a disorder in which part of the cranial contents protrudes from the [[skull]]), [[hydrocephalus]], [[cyclopia]], absence of the mandible (lower jaw bone), cleft lip and palate, [[cardiovascular]] [[Disease|disorders]], [[diaphragm (anatomy)|diaphragm]]atic hernia, and [[gastrointestinal]] malformation. The disorder is more common among [[females]].
==Prognosis==
The prognosis for those with iniencephaly is extremely poor. Newborns with iniencephaly seldom live more than a few hours. The distortion of the fetal body may also pose a danger to the mother's life.
==External links==
* [http://www.thefetus.net/page.php?id=120 TheFetus.Net]
{{Congenital malformations and deformations of nervous system}}
[[Category:Congenital disorders]]
[[es:Iniencefalia]]
[[pt:Iniencefalia]]
[[fi:Takaraivohalkio]]