Global InformationIniencephaly information
| Iniencephaly | |
|---|---|
| Other names | Iniencephaly clausus |
 | |
| Iniencephaly showing a stargazing head and an enlarged skull | |
| Symptoms | Neural malformations |
| Usual onset | Congenital |
| Duration | Long term |
| Types | Iniencephaly apertus, Iniencephaly clausus |
| Causes | Unknown |
| Diagnostic method | Prenatal screening |
| Differential diagnosis | Klippel–Feil syndrome |
| Prevention | Prenatal screening |
| Treatment | None |
| Medication | None |
| Prognosis | Invariably fatal |
| Frequency | Rare |
Iniencephaly is a rare type of cephalic disorder[1][2] characterised by three common characteristics: a defect to the occipital bone, spina bifida of the cervical vertebrae and retroflexion (backward bending) of the head on the cervical spine.[3] Stillbirth is the most common outcome, with a few rare examples of live birth, after which death invariably occurs within a short time.
The disorder was first described by Étienne Geoffroy Saint-Hilaire in 1836. The name is derived from the Ancient Greek word ἰνίον inion, for the occipital bone/nape of the neck.
- ^ Kulkarni, PR; Rao, RV; Alur, MB; Joshi, SK (July 2011). "Iniencephaly clausus: A case report with review of literature". Journal of Pediatric Neurosciences. 6 (2): 121–3. doi:10.4103/1817-1745.92831. PMC 3296405. PMID 22408660.
- ^ Hemal U, Solanki RS, Varsheney A, Baliga S (2004). "Prenatal diagnosis of iniencephaly on ultrasound". Indian J Radiol Imaging. 14: 265–6. Archived from the original on 2020-09-23. Retrieved 2014-07-02.
- ^ Erdinçler Pamir; Kaynar Mehmet Y.; et al. (1998). "Iniencephaly: Neuroradiological and Surgical Features". Journal of Neurosurgery. 89 (2): 317–20. doi:10.3171/jns.1998.89.2.0317. PMID 9688130.