Chapter 59: The Fetal Neural Axis

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1

acrania

condition associated with anencephaly in which there is complete or partial absence of the cranial bones

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alobar holoprosencephaly

most severe form of holoprosencephaly characterized by a single common ventricle and malformed brain; orbital anomalies range from fused orbits to hypotelorism, with frequent nasal anomalies and clefting of the lip and palate

3

anencephaly

neural tube defect characterized by lack of development of the cerebral and cerebellar hemispheres and cranial vault; this abnormality is incompatible with life

4

anomaly

an abnormality or congenital malformation

5

cebocephaly

form of holoprosencephaly characterized by a common ventricle, hypotelorism, and a nose with a single nostril

6

cyclopia

severe form of holoprosencephaly characterized by a common ventricle, fusion of the orbits with one or two eyes present, and a proboscis (maldeveloped cylindrical nose)

7

cystic hygroma

an increase in size of the jugular lymphatic sacs caused by abnormal development

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holoprosencephaly

a range of abnormalities from abnormal cleavage of the forebrain

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hydranencephaly

congenital absence of the cerebral hemispheres caused by an occlusion of the carotid arteries; midbrain structures are present, and fluid replaces cerebral tissue

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hydrocephalus

ventriculomegaly in the neonate; abnormal accumulation of cerebrospinal fluid within the cerebral ventricles, resulting in compression and frequently in destruction of brain tissue

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macrocephaly

enlargement of the fetal cranium as a result of ventriculomegaly

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meningocele

open spinal defect characterized by protrusion of the spinal meninges

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meningomyelocele

open spinal defect characterized by protrusion of meninges and spinal cord through the defect, usually within a meningeal sac

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spina bifida

neural tube defect of the spine in which the dorsal vertebrae (vertebral arches) fail to fuse together, allowing the protrusion of meninges and/or spinal cord through the defect; two types exist: spina bifida occulta (skin-covered defect of the spine without protrusion of meninges or cord) and spina bifida cystica (open spinal defect marked by sac containing protruding meninges and/or cord)

15

spina bifida occulta

closed defect of the spine without protrusion of meninges or spinal cord; alpha-fetoprotein analysis will not detect these lesions

16

ventriculomegaly

abnormal accumulation of cerebrospinal fluid within the cerebral ventricles resulting in dilatation of the ventricles; compression of developing brain tissue and brain damage may result; commonly associated with additional fetal anomalies