Chapter 59

Question 1

Anencephaly is also know as …

Answer 1

aprosencephaly or atelacephaly

Question 2

The most common neural tube defect

Answer 2

anencephaly

Question 3

Anencephaly which means absence of the _____, is caused by failure of closure of the neural tube at the cranial end,

Answer 3

brain

Question 4

In anencephaly the reminate brain is covered by a thick membrane called _________ or _________.

Answer 4

angiomatous stroma, cerebrovasculosa

Question 5

Anencephaly is a lethal disorder, with up to ____ of cases resulting in fetal demise.

Answer 5

50%

Question 6

Acrania, also known as _________, is a lethal anomaly that manifests as absence of the cranial bones with the presence of complete, although abnormal, development of the cranial hemispheres.

Answer 6

exencephaly

Question 7

A neural tube defect in which the meninges alone or the meninges and the brain herniate through a defect in the calvarium.

Answer 7

cephalocele

Question 8

A term used to describe herniation of the meninges AND brain through the defect.

Answer 8

enecphalocle

Question 9

Describes the herniation of only the meninges

Answer 9

cranial meningocele

Question 10

Cepholoecles occur at a rate of 1-3 in _______ live births.

Answer 10

10,000

Question 11

In ____ of cases cephaloceles are located at the midline and involve the occipital bone.

Answer 11

75%

Question 12

Chromosomal anomalies and syndromes have been identified with cephoceles, including trisomy ___ and ________.

Answer 12

13, Meckel-Gruber syndrome

Question 13

Meckel-Gruber syndrome is an autosomal-recessive disorder characterized by encephalocele, _________ , and _________.

Answer 13

polydactyly, polycystic kidneys

Question 14

Cephaloceles located off midline are usually the result of __________.

Answer 14

ammniotic band syndrome

Question 15

Cephaloceles maybe confused with _________ although they lack a cranial defect.

Answer 15

cystic hygroma

Question 16

Encompasses a wide range of vertebral defects that result from failure of neural tube closure.

Answer 16

spina bifida

Question 17

Spina Bifida occurs most commonly along the _______ and ______ regions.

Answer 17

lumbar, sacral

Question 18

Results from the abnormal cleavage of the prosencephalon (forebrain)

Answer 18

holoprosencephaly

Question 19

Holoprosencephaly is often associated with ______ abnormalities.

Answer 19

facial

Question 20

Facial anomalies identified with holoprosencephaly…

Answer 20

cyclopia, hypertelorism, an absent nose, a flattened nose with single nostril, and proboscios (think trisomy 13)

Question 21

Consists of the combination of hyoptelorism with a normally placed nose with a single nostril.

Answer 21

cebocephaly

Question 22

Severe hypotelorism with a proboscis superior to the eyes.

Answer 22

ethmocephaly

Question 23

Chromosomal anomalies must also be considered if holoprosencephaly is present, especially _________.

Answer 23

trisomy 13

Question 24

The _________ is a fiberous tract that connects the cerebral hemispheres and aids in learning and memory.

Answer 24

corpus callosum

Question 25

Cause of agenisis of the corpus callosum is thought to involve a vascular disruption or inflammatory lesion before _______.

Answer 25

12 weeks

Question 26

Aqueduct stenosis results from obstruction, atresia, or stenosis of the aquduct of sylvius causing _______.

Answer 26

ventriculomegaly

Question 27

The aquaduct of sylvius connects the _____ and _____ ventricles. Which causes the enlargement of the lateral ventricles.

Answer 27

third and forth

Question 28

Aquaductal stenosis is usually sporadic, but may result from __________, such as cytomegalovirus, rubuella, and toxoplasmosis.

Answer 28

intrauterine infection

Question 29

Primary aquaductal stenosis is usually _______ and an autosomial-recessive inheritance.

Answer 29

X-linked

Question 30

Aneurysm of the vein of Galen, also known as…

Answer 30

A vein of Galen malformation

Question 31

Choroid plexus cysts often resolve by ______ weeks gestation.

Answer 31

22 to 26

Question 32

Rare disorder charcterized by clefts in the cerebral cortex.

Answer 32

schizencephaly

Question 33

Schizencephaly has been associated with…

Answer 33

congenital infection, drugs and other toxic exposures, vascular accidents, and metabolic abnormalities.

Question 34

Destruction of the cerebral hemispheres by occlusion of the internal carotid arteries.

Answer 34

hydranencephaly

Question 35

Because microcephaly may manifest late in pregnancy, diagnosis before _______ weeks gestation may be impossible.

Answer 35

24