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Neurology
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INIENCEPHALY
is 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. 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, cephalocele
(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.
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.
The following information is updated
information and (credit for this
information goes to
Neurology Channel)
Additional
information
The fetus.net
American Academy of Neurology
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