1. Characterize intracranial arachnoid cysts.

G7 p.222:100mm

a. Origin: c_____

congenital

b. C_____ _____ _____ produce _____.

cells of lining; CSF

c. age: y_____ _____

young patients

d. incidence per 1000 autopsies_____

5

e. symptoms of s_____, h_____

seizures, headache

f. treatment: s_____, d_____, f_____

shunt, drain, fenestrate

g. path: s_____ a_____ m_____

split arachnoid membrane

2. True or False. Acute deterioration in patients with known arachnoid cysts usually signifies

G7 p.223:65mm

a. rapid increase in cyst size

false

b. postictal state

false

c. rupture into subdural space

false

d. rupture of bridging veins and cyst bleed

true

3. Complete the following about arachnoid cysts:

G7 p.222:177mm

a. The location of the only extradural type of arachnoid cyst is in the_____ cyst.

intra sellar

b. A retrocerebellar arachnoid cyst might mimic a_____ _____syndrome.

Dandy-Walker

c. The most common location for an arachnoid cyst is the_____ _____.

sylvian fissure

d. The next most common location is the _____ _____.

cerebellopontine angle

e. They are associated with ventriculo megaly in _____%.

64%

G7 p.224:45mm

f. The best treatment is probably_____ of_____.

shunting of cyst

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4. Complete the following regarding intraspinal cysts:

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a. If you find one ventrally think_____ _____.

neurenteric cyst

b. If you find one dorsally think_____ _____.

arachnoid cyst

5. Intracranial lipomas

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a. are usually found in the_____ _____

midsagittal plane

b. especially in the_____ _____.

corpus callosum

c. They are frequently associated with _____

agenesis

d. of the_____.

corpus callosum

e. They may less frequently involve the

     i. _____ _____

tuber cinereum

     ii. and the_____ _____.

quadrigeminal plate

6. True or False. Characteristics of intracranial lipomas include

G7 p.225:145mm

a. Association with_____abnormalities

congenital

b. On CT they have a _____density.

low

c. Differential diagnosis is

     i. d_____ c_____

dermoid cyst

     ii. t_____

teratoma

     iii. g_____

geminoma

d. On MRI they have a _____intensity on T1

high (like fat)

e. On MRI they have a _____intensity on T2.

low

7. Intracranial lipomas may present clinically with

G7 p.225:178mm

a. s_____

seizures

b. h_____ d_____

hypothalamic dysfunction

c. h_____

hydrocephalus

d. m_____ r_____

mental retardation

8. Hypothalamic hamartomas

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a. are frequent or rare?

rare

b. are neoplastic or nonneoplastic?

nonneoplastic

c. consist of a mass of_____ _____

neuronal tissue

d. that arises from the

     i. in_____ h_____ or

inferior hypothalamus

     ii. t_____ c_____

tuber cinereum

9. Hypothalamic hamartomas clinically

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a. may present with a special type of seizure called _____, which means _____ seizure

gelastic; laughing

b. may also have_____ attacks

rage

c.

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     i. may also present with p_____ p_____

precocious puberty

     ii. due to release of g_____ r_____ h_____

gonadotropin releasing hormone

     iii. formed within the_____ cells

hamartoma

10. Complete the following about neurenteric cysts:

G7 p.227:100mm

a. A neurenteric cyst is a central nervous system (CNS) cyst lined with_____

endothelium

b. resembling the_____ or_____ tract.

gastrointestinal or respiratory

c. Regions affected are usually the_____ or_____ areas.

cervical or thoracic

G7 p.227:115mm

d. Histology is a cyst lined with c_____- c_____ e_____

cuboidal-columnar epithelium

e. with m _____-s_____ g_____ c_____.

mucin-secreting goblet cells

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11. Complete the following about craniofacial development:

G7 p.228:105mm

a. The anterior fontanelle closes by age _____.

2.5 years

b. Head size is 90% of adult size at age _____

1 year

c. The head stops enlarging by age _____

7 years

G7 p.228:130mm

d. The skull is_____ at birth.

unilaminar

e. Diploë appears by the _____year and

fourth

f. reaches a maximum at age_____

35 years

g. Diploic veins form at age_____.

35 years

h. Air cells in the mastoid occur in _____ year.

sixth

12. True or false. Craniosynostosis

G7 p.228:172mm

a. has been proven to occur after shunting.

false

b. of one suture does not cause ↑ ICP.

false—11 % have ↑ ICP

13. Complete the following about craniofacial development:

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a. The most common craniosynostosis is _____.

sagittal

b. The male to female ratio is

80:20

c. The resulting skull shape is

dolichocephalic/scaphocephalic/boat shape

d. Surgery should be done within the age range of_____.

3 to 6 months

e. The strip craniectomy should be_____ cm wide.

3

14. Complete the following regarding coronal synostosis:

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a. Incidence of patients with craniosynostosis who have coronal synostosis is _____%.

18%

b. In which is it more common, males or females?

females

15. Coronal suture synostosis (CSS)

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a. plus syndactyly is called_____ syndrome.

Apert

b. Unilateral CSS is called_____.

plagiocephaly

c. CSS plus hypoplasia of the face is called_____ disease.

Crouzon

d. Plagiocephaly has an unusual orbit appearance on x-ray called the_____ _____ _____.

harlequin eye sign

e. Plagiocephaly

     i. Forehead on affected side is _____ or _____

flattened or concave

     ii. Supraorbital ridge has a _____margin.

higher

16. Harlequin eye sign

G7 p.230:40mm

a. occurs in u_____ c_____ suture closure

unilateral coronal

b. seen on_____ _____ _____.

anteroposterior skull x-ray

c. The abnormal bony structure is the_____ _____

supraorbital margin

d. and is_____ than on the normal side.

higher

17. Complete the following about craniofacial development:

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a. What suture is closed to produce trigonocephaly?

metopic

b. It is usually associated with an abnormality of the_____ chromosome.

19p

18. Characterize lambdoid synostosis.

G7 p.230:100mm

a. Male to female ratio is_____.

4:1

b. Side involved most frequently is_____.

right side

c. The frequency of involvement is_____% right.

70%

d. Does it have a ridge or an indentation to palpation?

not a ridge like the sagittal or coronal synostosis, but it has an indentation

19. Considering lambdoid synostosis:

G7 p.230:160mm

Fig. 8.1

illustration by Tony Pazos

a. Differentiate from positional flattening by looking at the ears from the_____ _____ _____ _____.

top of the head

b. In lambdoid synostosis you will see the ipsilateral ear_____ _____.

lags behind

c. In positional flattening you will see the ipsilateral ear is _____ _____.

pushed forward (If flat side of occipital bone is same side as the posteriorly positioned ear it is a case of lambdoid synostosis; if not it is a case of positional flattening.)

20. Answer the following concerning lambdoid synostosis treatment:

G7 p.231:85mm

a. True or False. All require surgery.

false (Only 15% won’t respond to repositioning.)

b. True or False. Surgery is indicated early (i.e., in 3 to 6 months).

false (One can observe for 3 to 6 months for improvement.)

c. Ideal age for surgery is_____ to_____ months.

6 to 18

d. Early surgery is indicated for s_____ d_____ and e_____ i_____ p_____.

severe disfigurement and elevated intracranial pressure

21. Describe oxycephaly.

G7 p.231:155mm

a. Definition:_____ _____

tower skull

b. Occurs if there is fusion of_____ _____

multiple sutures

c. Is there elevated ICP?

yes

d. What is the status of the sinuses?

underdeveloped sinuses

22. Complete the following about craniofacial development:

G7 p.232:60mm

a. Cranium bifidum is another name for _____ or_____.

encephalocele or meningocele

b. What type does not produce a visible soft tissue mass?

basal encephalocele

c. Definition: an extension of_____ structures outside the normal_____ of the skull

normal, confines

G7 p.232:75mm

d. A nasal polypoid mass in a newborn should be considered an_____ until proven otherwise.

encephalocele

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23. Complete the following about encephalocele:

G7 p.232:130mm

a. Incidence of the basal form of encephalocele is _____%.

1.5%

b. May exit the skull via a defect in

     i. c_____ p_____

cribriform plate

     ii. f_____ c_____

foramen cecum

     iii. s_____ o_____ f_____

superior orbital fissure

c. Treatment is by a combined i_____ and t_____ approach

intracranial and transnasal

24. Compare Chiari types I and II.

G7 p.233:100mm

a. medulla-caudal dislocation

Chiari I, no

Chiari II, yes

b. into cervical canal

Chiari I, tonsils

Chiari II, vermis, medulla,

fourth ventricle

c. myelomeningocele

Chiari I, no

Chiari II, yes

d. hydrocephalus

Chiari I, no

Chiari II, yes

e. medullary kink

Chiari I, no

Chiari II, 55%

f. cervical nerves

Chiari I, normal

Chiari II, upward

g. age at presentation

Chiari I, adult

Chiari II, infant

h. symptoms

Chiari I, neck pain

Chiari II, hydrocephalus, respiratory distress

25. Complete the following about Chiari malformation:

G7 p.234:25mm

a. Chiari I has how many abnormalities?

1—with many names

b. List four names this abnormality has been called.

     i. t_____ h_____

tonsillar herniation

     ii. c_____ d_____ of c1

caudal displacement of cerebellum

     iii. p_____ e_____ of t_____

peglike elongation of tonsil

     iv. c_____ e_____

cerebellar ectopia

26. Chiari I

G7 p.234:25mm

a. has how many deformities?

1

b. is known by the following names

     i. e_____

ectopia

     ii. e_____

elongation

     iii. d_____

displacement

     iv. h_____

herniation

c. symptoms

     i. o_____ h_____

occipital headaches

     ii. c_____ p_____

cervical pain

27. What is the particular eye sign associated with Chiari I?

Downbeat nystagmus is considered a characteristic of this condition in 47%, but it can also occur in Chiari II.

G7 p.235:130mm

28. What percentage of Chiari I patients have hydrosyringomyelia?

20 to 30% of Chiari I patients have a syrinx.

G7 p.236:1 5mm

29. Characterize the location of tonsils and Chiari I.

G7 p.236:1 5mm

a. Normal range related to foramen magnum

     i. high

8 mm above

     ii. low

5 mm below

     iii. mean

1 mm above

b. Chiari I range is

     i. high

3 mm below

     ii. low

29 mm below

     iii. mean

13 mm below

c. Symptoms can occur with tonsils at _____ mm below.

2

d. Usual level considered cutoff for diagnosis is_____ mm below.

5

30. Possible better correlation with symptoms of tonsillar herniation is the degree of brain stem compression

G7 p.236:100mm

a. at the_____ _____

foramen magnum

b. as seen on the_____

axial

c. T_____ W1 MRI.

2

31. The best results from surgery occur if treated within years of onset of symtoms.

2

G7 p.237:160mm

32. Complete the following concerning Chiari I:

G7 p.237:182mm

a. The most common postop complication is

     i. _____ _____

respiratory depression

     ii. in _____%.

15%

b. Occurs within how many days of surgery?

5

c. Occurs mostly at what time of day?

night

d. Death can occur from s_____ a_____.

sleep apnea

e. Other risks of surgery include

     i. c_____ f_____ l_____

cerebrospinal fluid leak

     ii. injury to_____ _____

posterior inferior cerebellar artery (PICA)

     iii. h_____ of c_____ h_____

herniation of cerebellar hemispheres

33. Complete the following concerning Chiari I:

G7 p.238:30mm

a. Operative results

     i. Main benefit may be to_____ progression

arrest

     ii. Best results in patients with syndrome_____ syndrome

cerebellar

     iii. which consists of

G7 p.238:55mm

t_____ a_____

truncal ataxia

l_____ a_____

limb ataxia

n_____

nystagmus

d_____

dysarthria

b. Which responds better: pain or weakness?

pain

G7 p.238:55mm

34.Factors that correlate with a worse outcome are

G7 p.238:63mm

a. a_____

atrophy

b. s_____

scoliosis

c. symptoms that are lasting more than_____ _____

2 years

35. Which Chiari malformation is associated with myelomeningocele?

Chiari II

G7 p.238:108mm

36. Study Chart. Chiari II anatomical abnormalities: A to Z.

atlas assimilation

beaking of tectum

bony abnormalities

cerebellar folia poorly

myelinated

cervical medullary junction

compression

craniolacunia

corpus callosum agenesis

degenerated lower CN nuclei

enlarged massa intermedia

falx hypoplasia

fourth ventricle trapped

fusion of cervical vertebrae

gyri miniaturized

hydrocephalus

heterotopia

hydromyelia

Klippel-Feil deformity

low attachment of tentorium

massa intermedia enlarged

medulla oblongata

“z” bend microgyria

nuclei of lower CN

degenerated

platybasia

peg of cerebellar tonsils

septum pellucidum absent

syringomyelia

tectum beaking (fusion)

tentorium low attachment

Z-shaped bend of medulla

G7 p.238:137mm

37. Considering Chiari II, presenting symptoms are due to dysfunction of

G7 p.239:15mm

a. b_____ s_____

brain stem

b. l_____ c_____ n_____

lower cranial nerves

38. Finding on presentation of Chiari II

Hint: n2 chiari two

G7 p239:30mm

a. n_____

nystagmus—down beat

b. n_____ _____

nasal regurgitation

c. c_____

cyanosis

d. h_____

hoarseness

e. i_____ _____ _____

impaired ventilatory drive

f. a_____ _____, _____

apneic spells, aspiration

g. r_____, _____ _____

regurgitation, respiratory arrest

h. i_____ _____

inspiratory stridor

i. t_____ _____ _____ _____ _____

tenth nerve (vagus) vocal cord paralysis

j. w_____ _____

weak arm—weak cry

k. o_____

opisthotonus

39. Complete the following regarding Chiari II:

G7 p.240:68mm

a. The most common cause of mortality is_____ _____.

respiratory arrest

b. The mortality at 6 years follow-up is _____%.

40%

c. Range of mortality

     i. Infants in poor condition (i.e., cardiopulmonary arrest, vocal cord paralysis, and/or arm weakness mortality) is _____%.

71%

     ii. There is gradual onset of symptoms in _____%.

23%

     iii. The worst prognostic factor for response to surgery is b_____v_____ c_____ p_____.

bilateral vocal cord paralysis

40. Complete the following regarding Dandy-Walker malformation (DWM):

G7 p.240:138mm

a. It is caused by a_____ of the f_____ of L_____ and M_____.

atresia of the foramina of Luschka and Magendie (old theory)

b. Results in

     i. agenesis of_____

vermis

     ii. large_____ _____ _____, which communicates with the

posterior fossa cyst

     iii. _____ _____, which becomes

fourth ventricle

     iv. _____.

enlarged

41. To differentiate DWM from retrocerebellar arachnoid cyst observe for

G7 p.241:28mm

a. v_____ a_____

vermian agenesis

b. cyst opens into f_____ v_____

fourth ventricle

c. enlarged p_____ f_____

posterior fossa

d. elevation of the t_____ H_____

torcular Herophili

42. What is Dandy-Walker pathogenesis?

G7 p.241:50mm

a. D_____

Dilation of fourth ventricle

b. A_____

Agenesis of vermis

c. N_____

Membrane of fourth ventricle

d. D_____

Dysembryo genesis

e. Y_____

Hydrocephalus

43. DWM patients

G7 p.241:60mm

a.

     i. Hydrocephalus is present in_____%

70 to 90%

     ii. and _____% of hydrocephalus patients have DWS.

2 to 4%

b. A common associated abnormality is

G7 p.241:92mm

     i. _____ of the_____ _____ in

agenesis of the corpus callosum

     ii. _____%.

17%

c. and c_____ a_____

cardiac abnormalities

d. If treatment is necessary, you must shunt the ventricle, the cyst, or both?

cyst

e. If aqueductal stenosis you should shunt _____also.

ventricle

f. But shunting the lateral ventricle alone

G7 p.241:122mm

     i. is _____

contraindicated

     ii. because it might cause_____ _____.

upward herniation

g. To avoid _____ herniation

upward

G7 p.241:125mm

h. you must not shunt the _____ alone.

ventricle

G7 p.241:125mm

44. What is the prognosis in DWM?

G7 p.241:152mm

a. Seizures occur in _____%.

15%

b. Mortality occurs in _____to _____%.

12 to 50%

c. Normal IQ is _____%.

50%

45. True or False. Aqueductal stenosis is seen only in children.

false (Adults can present with symptoms as well.)

G7 p.241:179mm

46. What are the causes of aqueductal stenosis?

Hint: aqectal

G7 p.242:20mm

a. a_____

astrocytoma of brain stem

b. q_____

quadrigeminal plate mass

c. e_____

inflammation, infection

d. c_____

congenital atresia

e. t_____

tumors

f. a_____

arachnoid cysts

g. l_____

lipoma

47. Complete the following concerning aqueductal stenosis:

G7 p.242:45mm

a. It is associated with congenital hydrocephalus in _____%.

70%

b. MRI may show absence of

     i. n_____ f_____ v_____ in the

normal flow void

     ii. a_____ of S_____.

aqueduct of Sylvius

c. MRI with contrast should be used to rule out_____

tumor

d. Follow-up should be for at least _____

2 years

e. in order to rule out_____.

tumor

G7 p.243:22mm

48. True or False. A patient with aqueductal stenosis of adulthood may have the following symptoms:

G7 p.242:100mm

a. headache

true

b. visual disturbances

true

c. decline of mental function

true

d. gait disturbance

true

e. papilledema (sign)

true

f. ataxia

true

g. urinary incontinence

true

49. What are the treatment options for aqueductal stenosis?

G7 p.242:175mm

a. ventriculoperitoneal_____ _____

CSF shunting

b. T_____ _____ _____ _____

Torkildsen shunt in adults

c. ETV = _____ _____ _____

endoscopic third ventriculostomy

50. With neural tube defects there are classification systems. Give examples of

G7 p.243:45 mm

a. neurulation defects

     i. a_____

anencephaly

     ii. m_____

myelomeningocele

b. postneurulation defects

     i. m_____

microcephaly

     ii. h_____

hydranencephaly

     iii. h_____

holoprosencephaly

     iv. l_____

lissencephaly

     v. s_____

schizencephaly

c. spinal defects

     i. d_____

diastematomyelia

     ii. s_____

syringomyelia

51. Complete the following about neural tube defects:

G7 p.243:45mm

a. Failure to fuse the anterior neuropore results in_____.

anencephaly

b. Failure to fuse the posterior neuropore results in_____.

myelomeningocele

c. The definition of microcephaly is head circumference_____ _____ _____ below the mean.

2 standard deviations

d. In hydranencephaly the cortex is replaced by_____.

CSF

e. Failure to cleave can result in_____.

holoprosencephaly

52. Complete the following about neural tube defects:

G7 p.243:45mm

a. Give examples of neurulation defects.

     i. a_____

anencephaly

     ii. c_____

craniorachischisis

     iii. m_____

myelomeningocele

b. These defects are due to _____ of the neural tube

nonclosure

53. Complete the following about neural tube defects:

G7 p.243:70mm

a. Name five postneurulation defects.

     i. h_____

hydranencephaly

     ii. l_____

lissencephaly (most severe)

     iii. h_____

holoprosencephaly

     iv. a_____ of_____ _____

agenesis of corpus callosum

     v. d_____

diastematomyelia

b. Which is the most severe?

lissencephaly

54. Complete the following regarding lissencephaly:

G7 p.243:120mm

a. It is an example of an abnormality of neuronal_____.

migration

b. It results in an abnormality of the _____ convolutions

cortical

c. called_____.

agyria

55. Name the key features of schizencephaly.

G7 p.243:155mm

a._____ which communicates with _____

cleft; ventricle

b. lined with_____ _____

gray matter

c. Two types are

     i. o_____ l_____

open lipped

     ii. c_____ l_____

close lipped

56. Complete the following about neural tube defects:

G7 p.243:160mm

a. In schizencephaly, the cleft wall is lined with cortical_____ _____.

gray matter

b. In porencephaly, a cystic lesion is lined with _____ or _____ tissue.

connective or glial

57. Hydranencephaly

G7 p.244:49mm

a. is a _____-neurolation defect.

post-

b. Cranium is filled with_____.

CSF

c. Is there a small or large head?

macrocrania

d. Most common etiology is_____ _____ _____.

bilateral ICA infarcts

58. Angiography

G7 p.244:137mm

a. of anterior circulation shows_____ _____.

no flow

b. of posterior circulation shows _____.

normal

59. Complete the following about neural tube defects:

G7 p.244:150mm

a. What are the three types of holoprosencephaly? Please list in order of decreasing severity.

     i. a_____

alobar (single ventricle) most severe

     ii. s_____

semilobar

     iii. l_____

lobar (least severe)

b. They occur because of

     i. failure to_____

cleave

     ii. of the_____ _____

telencephalic vesicle

60. List the risk factors for neural tube defects.

G7 p.245:120mm

a. B_____ i_____

B₁₂ insufficiency

b. c_____

cocaine—maternal use

c. D_____

Depakene—use during pregnancy

d. f_____ a_____ i_____

folic acid insufficiency

e. f_____

fever in first trimester

f. h_____e_____

heat exposure—maternal hot tub, sauna

g. o_____

obesity before and during pregnancy

h. v_____ a_____

valproic acid use during pregnancy

     i. v_____

vitamins—prenatal up folic acid and B₁₂

61. What are the tests for prenatal detection of neural tube defects?

G7 p.245:160mm

a. serum _____ _____ (If high at 15 to 20 weeks be suspicious for neural tube defects.)

alfa fetoprotein (If high at 15 to 20 weeks be suspicious for neural tube defects.)

     i. u_____,

ultrasonography

     ii. which can detect what % of spina bifida cases?

90%

b. a_____

amniocentesis

62. For prenatal detection of neural tube defects

G7 p.245:168mm

a. test mother’s serum for_____ _____.

alpha fetoprotein

b. Has a success rate for

     i. spina bifida open _____% and

91%

     ii. anencephaly_____ %.

100% may be missed

     iii. Closed spinal dysraphism_____ _____ _____.

may be missed

c. An overestimate of gestational age will make us think that a high alpha fetoprotein level is _____.

normal

d. Real-time imaging is by_____.

ultrasonography

e. Identifies _____% of s_____ b_____.

90% of spinal bifida

f. Obtaining fluid from the womb is called _____.

amniocentesis

g. It carries a risk of fetal loss of _____%.

6%

63. Characterize agenesis of the corpus callosum.

G7 p.246:70mm

a. On computed tomographic scan the typical appearance is as follows:

     i. Third ventricle is_____

expanded

     ii. Lateral ventricles are_____.

separated

     iii. Atria and occipital horns are _____.

dilated

b. Corpus callosum forms at age_____ _____ after conception and forms from_____ to_____.

2 weeks; rostral to caudal

64. Complete the following concerning the bundles of Probst:

G7 p.246:115mm

a. They are aborted beginnings of the_____ _____

corpus callosum

b. bulging into the_____ _____.

lateral ventricles

65. Complete the following regarding agenesis of the corpus callosum:

G7 p246:155mm

a. Does it always have clinical significance?

no, it may be an incidental finding

b. Underlying cause may be an abnormality of a_____.

chromosome

66. List the features of spina bifida occulta.

Hint: bifidaocculta

G7 p.247:145mm

a. b_____

bifida

b. i_____

incidental

c. f_____

foot deformity

d. i_____

innocuous

e. d_____

diastematomyelia

f. a_____

atrophy of leg

g. o_____

occurs in 20 to 30% of people

h. c_____

cutaneous stigmata

i. c_____

clinical importance often nil

j. u_____

urinary incontinence

k. l_____

lipoma, leg weakness

l. t_____

tethered cord

m. a_____

absent spinous process

67. Complete the following regarding myelomeningocele:

G7 p.248:28mm

a. The anterior neuropore closes at gestational age day_____.

25

b. The posterior neuropore closes at gestational age day_____.

28

68. Complete the following concerning myelomeningocele (MM):

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a. Incidence if no previous child has a MM equals_____ % or_____ per 1000.

0.2%, 2

b. One previous MM child equals _____% or_____ per 1000.

2%, 20

c. Two previous MM children equals _____ %or _____ per 1000.

6%, 60

d. Associated hydrocephalus equals incidence of _____%.

80%

e. Associated Chiari II occurs in_____ children with MM.

most

69. Answer the following about myelomeningocele:

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a. What is the incidence of meningocele or myelomeningocele?

1 to 2/1000 live births (0.2%)

b. Does the risk increase in families with one affected child?

yes (The risk does increase to 2 to 3% in families with one previous myelomeningocele child.)

c. Does the risk increase in families with two affected children?

yes (It further increases to 6 to 8% in families with two previous affected children.)

70. True or False. All children born with myelomeningocele have an associated Chiari II malformation.

false (Not all, but most, have Chiari II.)

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71. True or False. Closure of myelomeningocele may result in the need for CSF shunting.

true

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72. Meningomyelocele patients develop allergy to_____.

latex

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73. True or False or Uncertain. Intrauterine closure of mm defect reduces

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a. Chiari II defect

true

b. hydrocephalus

uncertain

c. neurological dysfunction

false

74. Complete the following concerning myelomeningocele:

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a. If ruptured start_____ (n_____ and g_____).

antibiotics (nafcillin and and gentamicin)

b. Perform surgery within_____ to_____ hours.

24 to 36 hours

c. Better functional outcome occurs if children have spontaneous _____ of _____ _____.

movement of lower extremities

d. Do multiple anomalies occur in myelomeningocele?

yes (average 2 to 2.5 additional anomalies in myelomeningocele)

75. Complete the following about myelomeningocele and early closure:

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a. True or False. Results in improvement of neurological functions.

false (Early closure does not result in improvement of neurological function.)

b. True or False. Results in lower infection rate

true (It does result in a lower infection rate.)

c. Myelomeningocele should be closed within 12, 24, or 36 hours?

24

76. Considering late problems in myelomeningocele repair, possible late problems include

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a. brain _____

hydrocephalus— malfunctioning shunt

b. cervicomedullary junction_____

Chiari II compressing medulla

c. cord_____

syrinx

d. cauda_____

tethered cord

77. Characterize myelomeningocele outcome without treatment and with treatment.

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a. survive infancy without treatment ____% with treatment ____%

15 to 30%, 85%

b. normal IQ without treatment ____% with treatment____%

70%, 80%

c. ambulatory without treatment ____% with treatment ____%

50%, 40 to 85%

d. continence without treatment _____ with treatment ____%

rare, 3 to 10%

78. For each of the following what are the facts to know concerning lipomeningocele?

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a. age for surgery

2 months is appropriate for surgery

b. band

thick fibrovascular band constricts

c. conus

is split

d. dura

is dehiscent

e. epidural fat versus

lipoma is distinct from epidural fat

f. placode

lipoma attached to neural placode

g. neuro exam

is normal 50%

h. sensory loss

most common neurological abnormality

i. stigmata

cutaneous

j. urologicexam

should be done preop

79. True or False. Lipomyelomeningocele is associated with tethered cord.

true

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80. Study Chart.

lipomeningocele

steps in surgical treatment:

untether the cord

Xomed

CUSA (Cavitron Ultrasonic

Surgical Aspirator)

recording from anal sphincter

free up sides from

attachment to dura

reduce bulk of fat using CUSA

in the midline

tie dura open to sides

place bovine pericardial graft

as dural substitute

G7 p.251:90mm Courtesy of Dr. David Frim

81. True or False. The most common location of a dermal sinus tract is the

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a. occipital region

false

b. cervical region

false

c. thoracic region

false

d. lumbosacral region

true

82. What is the most likely cause of dermal sinus?

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a. Failure of the_____ ectoderm

cutaneous

b. to_____

separate

c. from the_____ -ectoderm

neuro

d. at the time of_____

closure

e. of the_____ _____.

neural groove

83. Dermal sinus items to know include

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a. Location most common is the_____ area

lumbosacral

b. Results from _____ of_____ of _____ _____

failure of separation of cutaneous ectoderm

c. from_____ _____

neural ectoderm

d. appears as a_____

dimple:

     i. hair?

with or without hairs

     ii. midline?

close to midline

     iii. skin stigmata?

yes

e. First manifestation is_____ dysfunction

bladder

f. Tract always courses_____ from lumbosacral area

cephalad

84. True or False. An epidermoid cyst contains hair follicles and sweat glands.

false (An epidermoid cyst contains stratified squamous epithelium with keratin from desquamated epithelium. A dermoid cyst is lined with dermis and contains sebum and hair.)

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85. What is a major difference between epidermoid cyst and dermoid cyst?

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a. Epidermoid cyst is

     i. lined with s_____ s_____ e_____

stratified squamous epithelium

     ii. and contains only_____.

keratin (from desquamated epithelium)

b. Dermoid cyst is

     i. lined with_____

dermis

     ii. and contains_____ _____ such as

skin appendages

     iii. hair follicles?

yes

     iv. sebaceous glands?

yes

86. True or False. A dermal sinus tract is a potential pathway for intradural infection such as meningitis or abscess.

true

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87. Radiologic evaluation of dermal sinus

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a. If seen at births do_____

ultrasound

b. If first seen later do_____

MRI

88. Given the above, indicate whether the dermal sinus tract should be excised at the given locations.

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a. lumbar

yes

b. sacral

yes, though controversial

c. coccygeal

no

89. Complete the following concerning the cranial dermal sinus:

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a. The track extends_____.

caudally

b. If the dermal sinus tract enters the skull they do so_____ to the torcula.

caudal

90. True or False. Klippel-Feil syndrome results from failure of

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a. primary neurulation

false

b. secondary neurulation

false

c. dysjunction

false

d. segmentation

true (Klippel-Feil results from abnormal segmentation of the cervical somites between 3 and 8 weeks gestation.)

91. Klippel-Feil syndrome

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a. results from failure of_____ of _____ _____ at gestational age of _____.

segmentation of cervical somites; 3 to 8 weeks

b. Clinical triad

     i. Hairline is_____.

low

     ii. Neck is_____.

short

     iii. Motion is_____.

limited

c. Limitation of range of motion of the neck occurs only if more than_____ segments are fused.

3

d. True or False. Other congenital abnormalities may also be present.

true

e. True or False. Klippel-Feil causes symptoms related to fused vertebrae.

false (No symptoms ever attributed to the fused vertebrae.)

92. True or False. Anomalies seen in association with Klippel-Feil include

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a. Sprengel deformity

true

b. webbing of the neck

true

c. basilar impression

true

d. unilateral absence of the kidney

true

93. Possible systemic congenital abnormalities include (be specific)

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a. g_____

enitourinary—absence of one kidney

b. c_____

cardiopulmonary

94. List six presenting signs and symptoms of tethered cord syndrome.

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Fig. 8.2

Illustration by Tony Pazos

95. True or False. In a patient with myelomeningocele with worsening scoliosis, spasticity, worsening gait, or deteriorating urodynamics, shunt evaluation is not part of the primary workup.

false (In a myelomeningocele patient with worsening clinical symptoms, shunt evaluation and confirmation of normal intracranial pressure should be one of the first modalities of intervention.)

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96. True or False.

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a. Progressive scoliosis is not seen in conjunction with tethered cord syndrome.