Iniencephaly

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Iniencephaly Classification and external resources
ICD-10	Q00.2
ICD-9	740.2

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.

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 disorders, diaphragmatic 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

• TheFetus.Net

v • d • e Congenital malformations and deformations of nervous system (Q00-Q07, 740-742)
Brain	Anencephaly (Acephaly, Acrania, Iniencephaly) - Encephalocele - Microcephaly - Congenital hydrocephalus (Dandy-Walker syndrome) - other reduction deformities (Holoprosencephaly, Lissencephaly, Pachygyria, Hydranencephaly) - Septo-optic dysplasia - Megalencephaly - Congenital cerebral cysts (Porencephaly, Schizencephaly) - Congenital brain tumors
Brain stem	Arnold-Chiari malformation
Spinal cord	Spina bifida - Currarino syndrome - Sacrococcygeal teratoma - Diastematomyelia - Syringomyelia
see also non-congenital CNS and PNS (G, 320-359)

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .