A rare neural tube defect that combines extreme retroflexion (backward bending) of the head with severe defects of the spine. The affected infant tends to be short, with a disproportionately large head. The diagnosis can be made immediately after birth because the head is so severely retroflexed (bent back) 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, cephalocele (a disorder in which part of the cranial contents protrudes from the skull), hydrocephalus, cyclopia, absence of the mandible (the lower jaw bone), cleft lip and cleft palate, cardiovascular disorders, diaphragmatic hernia, and gastrointestinal malformation. The disorder is more common among females. The prognosis (outlook) 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.
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Malformation consisting of a cranial defect at the occiput, with the brain exposed; often in combination with a cervical rachischisis and retroflexion. [G. inion, back of the head, + enkephalos, brain]

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in·i·en·ceph·a·ly -'sef-ə-lē n, pl -lies the condition of being an iniencephalus

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in·i·en·ceph·a·ly (in″e-ən-sefґə-le) [ini- + Gr. enkephalos brain] a developmental anomaly characterized by enlargement of the foramen magnum and absence of the laminae and spinous processes of the cervical, dorsal, and sometimes lumbar vertebrae, with vertebrae reduced in number and irregularly fused, the brain and much of the spinal cord occupying a single cavity.

Medical dictionary. 2011.

Look at other dictionaries:

  • Iniencephaly — DiseaseDisorder infobox Name = PAGENAME ICD10 = ICD10|Q|00|2|q|00 ICD9 = ICD9|740.2 ICDO = Caption = OMIM = OMIM mult = MedlinePlus = eMedicineSubj = eMedicineTopic = DiseasesDB = Iniencephaly is a type of cephalic disorder. It is a rare neural… …   Wikipedia

  • iniencephaly — noun A cephalic disorder characterized by spina bifida and spinal retroflexion …   Wiktionary

  • Neural tube defect — Classification and external resources ICD 10 Q00, Q01, Q05 …   Wikipedia

  • Syringomyelia — Classification and external resources An idiopathic syrinx. See the thin light grey shape inside the spinal cord, placed at centre in the bottom half of the above image. ICD 10 G …   Wikipedia

  • Hydrocephalus — For other uses, see Hydrocephalus (trilobite). Not to be confused with Hypocephalus. Hydrocephalus Classification and external resources Hydrocephalus seen on a CT scan of the brain. ICD …   Wikipedia

  • Spina bifida — Classification and external resources ICD 10 Q05, Q76.0 …   Wikipedia

  • Cephalic disorder — Classification and external resources ICD 10 Q00 Q07, Q67, Q75 ICD 9 740 742 Cephalic disorders (from the …   Wikipedia

  • Holoprosencephaly — Classification and external resources Diagram depicting the main subdivisions of the embryonic vertebrate brain. ICD 10 Q …   Wikipedia

  • Megalencephaly — Classification and external resources ICD 10 Q04.5 ICD 9 742.4 …   Wikipedia

  • Microcephaly — Classification and external resources ICD 10 Q02 ICD 9 …   Wikipedia

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