Anencephaly

anencephaly /an·en·ceph·a·ly/ (an?en-sef´ah-le) congenital absence of the cranial vault, with the cerebral hemispheres completely missing or reduced to small masses.anencephal´ic

---

an·en·ceph·a·ly (nn-sf-l)

n.

Congenital absence of most of the brain and spinal cord.

---

anen·ce·phalic (-s-flk), anen·cepha·lous (-sf-ls) adj.

---

Anencephaly

A hereditary defect resulting in the partial to complete absence of a brain and spinal cord. It is fatal.

Mentioned in: Amniocentesis, Polyhydramnios and Oligohydramnios

---

anencephaly

congenital absence of the cranial vault, with the cerebral hemispheres completely missing or reduced to small masses.

---

anencephaly Neonatology A lethal malformation consisting of congenital partial or complete absence of the cranial vault accompanied by absence of overlying tissues, including the brain and cerebral hemispheres, skull and scalp; anencephaly develops in the 1^st month of gestation and affects 0.14-0.7/1000 live births; the 1º abnormality is failure of cranial neurulation, the embryologic process separating the forebrain precursors from the amniotic fluid; since neural tissue is exposed, cerebral tissue is hemorrhagic, fibrotic, gliotic without functional cortex Etiology Usually idiopathic, possibly multifactorial or polygenic in origin. See Uniform Determination of Death Act.