1. Complete the following statements about hydrocephalus:

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a. Incidence of congenital hydrocephalus is_____%.

0.2%

b. Size of normal temporal horns should be no wider than _____ mm.

2 mm

c. Width of brain (internal diameter) compared with largest width of frontal horns should normally be_____.

2 times or more

d. Therefore, a ratio of frontal horns to internal diameter of _____% suggests hydrocephalus.

>50%

2. True or False. Indicate if the following are considered “true” hydrocephalus:

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a. hydrocephalus ex vacuo

false

b. obstructive hydrocephalus

true

c. communicating hydrocephalus

true

3. True or False. The following are characteristics of hydranencephaly:

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a. preneurulation defect

false

b. cause may be from infection

true

c. cause may be from bilateral internal carotid artery (ICA) infarcts

true

d. electroencephalography (EEG) shows no cortical activity

true

e. transillumination specific and very helpful

false

4. Complete the following regarding hydranencephaly:

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a. Hydranencephaly is defined as total or near total absence of the_____.

cerebrum

b. It occurs before or after neurulation?

postneurulation

c. The most common cause is_____ _____ _____.

bilateral ICA infarcts

d. Other causes are

     i. n_____ h_____

neonatal herpes

     ii. i_____

infection

     iii. t_____

toxoplasmosis

e. The best way to differentiate hydranencephaly from maximal hydrocephalus is to perform an_____.

EEG

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f. Other tests include

     i. c_____ t_____

computed tomography (CT)

     ii. m_____ r_____ i_____

magnetic resonance imaging (MRI)

     iii. a_____

angiography

5. What are key features regarding benign external hydrocephalus (also known as external hydrocephalus)?

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a. Subarachnoid spaces are_____.

enlarged over frontal poles in first year of life

b. Ventricles are_____.

normal or minimally enlarged

c. They are distinguished from subdural hematorma by the_____ _____ _____.

cortical vein sign

d. It usually spontaneously_____by_____.

resolves; 2 years of age_____.

6. Complete the following concerning external hydrocephalus:

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a. It occurs in what age group?

infants in first year of life

b. What is the cortical vein sign?

MRI or CT shows veins extending from brain to inner table of skull

c. The cortical vein sign helps differentiate_____from_____.

benign external hydrocephalus from subdural hematoma

d. Postulated cause of benign external hydrocephalus (BEH) is_____.

defect in CSF reabsorption

e. BEH usually resolves by age_____.

2

f. Concern is caused by_____.

large head size

7. “X” linked hydrocephalus

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a. is a type of_____hydrocephalus that

inherited

b. occurs in _____% of patients with hydrocephalus.

2%

c. Gene is located on_____.

Xq28

d. It causes abnormality in_____membrane receptor and

LICAM

e. produces classical syndromes including

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     i. c_____ c_____ h_____

corpus callosum hypoplasia

     ii. r_____

retardation

     iii. a_____ t_____

adducted thumbs

     iv. s_____ p_____

spastic paralysis

     v. h_____

hydrocephalus

8. Complete the following regarding radiographic finding of L1 syndrome:

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a. Large

     i. p_____ h_____

posterior horn

     ii. m_____ i_____

massa intermedia

     iii. q_____ p_____

quadrigeminal plate

b. Small (hypoplastic)

     i. c_____ c_____

corpus callosum

     ii. c_____ v_____

cerebellarvermis

c. Rippled

     i. v_____ w_____

ventricular wall

d. Which feature is pathognomonic? r_____ v_____ w_____

rippled ventricular wall

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e. Available treatment for retardation?

none

9. True or False. Shunt dependency is likely in hydrocephalus due to

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a. aqueductal stenosis

true

b. spina bifida

true

c. communicating hydrocephalus (i.e., secondary to arachnoidal adhesions)

false (shunt independence more likely to occur)

10. True or False. With respect to a disconnected or nonfunctioning shunt:

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a. A disconnected shunt may continue to function by CSF flow through a subcutaneous fibrous tract.

true

b. If in doubt, better to watch, not shunt.

false

c. Patients with a nonfunctioning shunt should not be followed with serial CT scans but possibly with serial neuropsychological evaluations.

false

11. True or False. When deemed “arrested” no further follow-up is needed.

false, deterioration can still occur

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12. True or False. With regard to “arrested hydrocephalus”:

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a. It is interchangeable with the term “uncompensated hydrocephalus.”

false

b. Arrested hydrocephalus satisfies the following criteria in the absence of a cerebrospinal fluid (CSF) shunt

false

     i. ventriculomegaly nonprogressive

true

     ii. normal head growth curve

true

     iii. continued psychomotor development

true

13. Hydrocephalus-radiologic criteria:

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a. skull

     i. inner table shows_____ _____ cranium

beaten copper

     ii. sella shows_____

erosion

b. ventricles

     i. Frontal horns ballooning look like M_____ M_____.

Mickey Mouse

     ii. Frontal horns’ percent of brain width is>_____%.

50%

     iii. Temporal horns’ width is >_____mm.

2 mm

     iv. Anteroposterior (AP) view shows_____.

disproportion of ventricle size and cortical sulci

     v. Third ventricle on AP view shows_____ _____.

bowing laterally

     vi. Third ventricle on lateral view shows_____ _____ _____ _____.

bowing down into sella

c. brain

     i. transependymal_____

edema

     ii. corpus callosum is_____

thin/atrophic

     iii. and shows_____

stretching

     iv. and_____ _____

upward bowing

14. Regarding the characteristics of the etiology of hydrocephalus:

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a. True or False. There is excess production of CSF.

True

b. True or False. There is impaired absorption of CSF.

true

c. True or False. It is congenital without myelomeningocele.

true

d. Congenital with myelomeningocele usually occurs with_____.

Chiari II

e. Chiari I if a cause has_____ _____ _____ _____.

fourth ventricle outlet obstruction

f. Aqueductal stenosis presents symptoms in_____.

infancy

g. Secondary aqueductal stenosis is due to_____ _____, _____, or _____.

intrauterine infection, hemorrhage, or tumor

h. Atresia of foramina of Luschka and Magendie is called_____ – _____ _____.

Dandy-Walker syndrome

15. Complete the following concerning etiologies of hydrocephalus:

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a. Chiari II is associated with_____.

myelomeningocele

b. Aqueductal stenosis usually manifests itself in which age group?

infancy

c. Of postop pediatric post-fossa tumor patients,_____% develop hydrocephalus and need a shunt.

20%

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d. This may be delayed for up to_____.

1 year

e. Dandy-Walker malformation occurs in what percent of patients with hydrocephalus?

2.4%

16. Conditions that may mimic hydrocephalus are

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     i. h_____

hydranencephaly

     ii. a_____

atrophy

     iii. a_____ of c_____ c_____

agenesis of corpus callosum

     iv. s_____ o_____ d_____

septo optic dysplasia

17. List signs and symptoms of hydrocephalus in young children.

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     i. h_____

hydrocephalus

     ii. y

young children

     iii. d_____

diplopia on lateral gaze (abducens palsy)

     iv. r_____

respiratory pattern irregular

     v. o_____

outward protrusion of fontanelle

     vi. c_____

cracked pot sound of Macewen

     vii. e_____

enlargement of cranium relative to face

     viii. p_____

poor head control, Parinaud syndrome

     ix. h_____

hyperactive reflexes

     x. a_____

abducens nerve palsy, apneic spells

     xi. l_____

large head

     xii. u_____

upward gaze palsy

     xiii. s_____

scalp veins prominent

     xiv. s_____

setting sun sign

     xv. s_____

splaying of cranial sutures (seen on plain skull x-rays)

18. List the signs and symptoms of active hydrocephalus in older children/adults with rigid cranial vault.

Hint: hcp

headache, nausea, vomiting changes in gait, and urine control

papilledema, upward gaze or abducens palsy

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19. Occipital frontal circumference (OFC) in the normal child should equal the distance from crown to_____.

rump

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20. For the indicated ages give the expected normal head circumference pattern.

Hint: At 33 weeks the circumference is 33 cm. In a child younger than 33 weeks the head circumference is greater in cm than the age of the child in weeks old. After 33 weeks head circumference growth slows so that at 40 weeks of age the head circumference is 36 cm.

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a. premature (ages in weeks)

     i. 28

29 cm

     ii. 29

30 cm

     iii. 30

31 cm

     iv. 31

31.5 cm

     v. 32

32 cm

     vi. 33

33 cm

     vii. 34

33.5 cm

     viii. 35

34 cm

     ix. 36

34.5 cm

     x. 37

35 cm

     xi. 38

35 cm

     xii. 39

35.4 cm

     xiii. 40

36 cm

b. full term (ages in months)

Hint: Note the pattern; with each month head circumference increases by 1 cm.

     i. 1

40 cm

     ii. 2

42 cm

     iii. 3

43 cm

     iv. 4

44 cm

     v. 5

45 cm

     vi. 6

46 cm

c. What is the upper limit of head circumference for a baby?

     i. 28 weeks gestational age

29 cm

     ii. 33 weeks gestational age

33 cm

     iii. 2 months old

42 cm

     iv. 3 months old

43 cm

     v. 4 months old

44 cm

     vi. 6 months old

46 cm

21. Answer the following about the treatment of hydrocephalus:

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a. True or False. Hydrocephalus is a medically treated condition.

false (mainly to be treated surgically)

b. Diuretic therapy can include a_____ and f_____.

acetazolamide and furosemide

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c. Be sure to watch for the complication of_____ _____.

electrolyte imbalances

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d. Role of spinal taps in hydrocephalus is to t_____.

temporize (Hydrocephalus after intraventricular hemorrhage may be only transient, and serial taps [ventricular or lumbar] may temporize until resorption resumes, but lumbar taps can be performed only for communicating hydrocephalus.)

e. Critical protein level of CSF is_____.

100 mg/dL (If reabsorption does not resume when protein content of CSF is < 100 mg/dL, then it is unlikely that spontaneous resorption will occur and a shunt will usually be necessary.)

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22. Complete the following concerning spinal taps and hydrocephalus:

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a. Protein above_____ will not be absorbed.

100 mg/dL

b. Protein below absorbed. _____ may be

100 mg/dL

23. Complete the following concerning surgery and hydrocephalus:

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a. Third ventriculostomy when looking into ventricle

     i. Where is thalamostriate vein?

lateral wall

     ii. Where is septal vein?

medial wall

     iii. Where is choroid plexus?

enters foramen of Monro

b. Where is puncture of third ventricle to occur?

anterior to mammillary bodies

c. Into the_____ cistern

interpeduncular

d. Watch out for_____.

basilar artery

e. Success rate is _____%,

approximately 50% (60 to 90% range) for aqueductal stenosis

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f. but only 20% for_____ _____.

preexisting pathology

24. Concerning shunts and hydrocephalus, what type of shunts do you know?

Hint: palmt

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a. v_____ s_____

ventriculoperitoneal shunt

b. v_____ a_____

ventriculo-jugular vein–right cardiac atrial

c. l_____

lumboperitoneal

d. m_____ s_____

miscellaneous shunts–ventriculopleural

e. T_____ s_____

Torkildsen shunt (ventricle–cisterna magna)

25. What is shunt usage priority?

Hint: palmt

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a. most often used_____ _____

ventriculoperitoneal shunt

b. abdominal abnormality_____ _____

ventriculoatrial shunt

surgery

peritonitis

morbid obesity

c. pseudotumor cerebri_____ _____

lumboperitoneal shunt-small ventricles

d. alternative_____ _____

miscellaneous shunts

e. acquired obstructive hydrocephalus_____ _____

Torkildsen shunt

26. Which are the miscellaneous shunts?

Hint: gupc

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     i. g_____

ventricle to gall bladder shunt

     ii. u_____

ventricle to ureter or bladder shunt

     iii. p_____

ventriculopleural shunt

     iv. c_____

cyst shunt (arachnoid cyst or subdural hygroma cavity to peritoneum)

27. Name six possible shunt complications.

Hint: odesma

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     i. o_____

obstruction

     ii. d_____

disconnection of shunt parts

     iii. e_____

erosion through skin

     iv. s_____

seizures–5.5% first year, 1.1% after 3 years

     v. m_____

metastases of tumor cells

     vi. a_____

allergy to silicone

28. What are ventriculoperitoneal shunt complications?

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Hint: h²alo³mvps

     i. h_____

hernia–inguinal 17%

     ii. h_____

hydrocele

     iii. a_____

CSF ascites

     iv. l_____

lengthen catheter with growth (preventable)

     v. o_____

obstruction by omentum or debris

   by peritoneal cyst (infection or talc from surgical gloves) severe peritoneal adhesions malposition of catheter tip collapsed ventricular wall choroid plexus

     v. o_____

obstruction or strangulation of intestine

     vii. o_____

overshunting

     viii. m_____

migration of tip to:

scrotum

perforation of stomach,

bladder, diaphragm

     ix. v_____

volvulus

     x. p_____

peritonitis

     xi. s_____

subdural hematoma

29. What are ventriculoatrial shunt complications?

Hint: liverssh

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     i. l_____

lengthening in children

     ii. i_____

infection

     iii. v_____

vascular

   perforation

   thrombophlebitis

   pulmonary microemboli

     iv. e_____

shunt embolus

     v. r_____

retrograde blood flow

     vi. s_____

superior vena cava obstruction

     vii. s_____

subdural hematoma

     viii. h_____

hypertension (pulmonary)

30. What are lumboperitoneal shunt complications?

Hint: Carols

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     i. C_____

Chiari l malformation (70% made worse)

     ii. a_____

arachnoiditis and adhesions

     iii. r_____

radiculopathy (from tube hard to control)

     iv. o_____

overshunting (sixth and seventh cranial nerve dysfunction)

     v. l_____

leakage of CSF

     vi. s_____

scoliosis due to laminectomy (14% in children)

31. When do you tap the shunt?

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a. to study CSF for

     i. i_____

infection

     ii. c_____

cytology

     iii. b_____

blood

b. or to assess function

     i. measure p_____

pressure

     ii. instill c_____

contrast

c. inject m_____

medication

32. When tapping a shunt, what is normal CSF pressure as measured from the ventricle?

less than 15 cm of CSF in relaxed recumbent position

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33. How often does the patient have to pump the shunt?

Patient must not touch the pump unless instructed to do so.

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34. What are acute symptoms of undershunting?

Hint: salvadib h

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a. s_____

seizures

b. a_____

ataxia

c. l_____

lethargy

d. v_____

vomiting

e. a_____

apnea

f. d_____

diplopia

g. i_____

irritability

h. b_____

bradycardia

i. h_____

headache

35. What are signs of acute increase in intracranial pressure?

Hint: p⁴b²

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a. p_____

Parinaud syndrome

b. p_____

palsy of abducens

c. p_____

papilledema

d. p_____

prominent scalp veins

e. b_____

blindness or field cut

f. b_____

bulging fontanelle

36. What are complications of overshunting?

Hint: s⁴i

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a. s_____

slit ventricles 12%

b. s_____

subdural hematoma

c. s_____

sylvian aqueduct occlusion

d. s_____

skull changes— craniosynostosis or microcephaly

e. i_____

intracranial hypotension

37. Intracranial hypotension

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a. When patient is erect, column of CSF produces a s_____ e_____.

siphon effect

b. Diagnose by documenting a drop in ICP when patient changes from_____ to_____ position.

supine to erect

38. Slit ventricles can be diagnosed by frontal-occipital horn ratio of less than

0.2

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39. Name categories of patients with slit ventricles.

Hint: pahms

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a. p_____

pseudotumor cerebri

b. a_____

asymptomatic slit ventricles

c. h_____

intracranial hypotension

d. m_____

migraine

e. s_____

slit ventricle syndrome

40. Complete the following concerning hydrocephalus and subdural hematomas (SDs):

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a. Cause of SD in patients with shunts is_____ of the brain and_____ _____ _____ _____ _____.

collapse; tearing of the bridging veins

b. Risk factors

     i. b_____ a_____

brain atrophy

     ii. l_____-s_____ h_____

long-standing hydrocephalus

     iii. n_____ v_____ p_____

negative ventricular pressure

41. If subdural hematoma develops as a shunt complication the subdural is located on

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a. the same side as the shunt_____%

32%

b. opposite side of the shunt_____%

21%

c. bilaterally_____%

47%

42. Treatment for subdural hematoma that occurs due to shunting for hydrocephalus could include

Hint: bcdht

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a. b_____

burr holes

b. c_____

craniotomy

c. d_____

drainage–subdural peritoneal shunt

d. h_____

higher pressure shunt

e. t_____

tie off shunt

43. True or False. In VP shunt and laparoscopic surgery, abdominal insufflation can increase ICP.

true

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44. What are the symptoms of normal pressure hydrocephalus?

Hint: dig

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a. d_____

dementia (wacky)

b. i_____

incontinence of urine (wet)

c. g_____

gait disturbances (wobbly)

45. What is the etiology?

Hint: mistapa

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a. m_____

meningitis

b. i_____

idiopathic

c. s_____

subarachnoid hemorrhage

d. t_____

trauma

e. a_____

aqueductal stenosis

f. p_____

posterior fossa surgery

g. A_____

Alzheimer disease

46. In clinical triad, which symptom precedes the others?

Gait disturbance

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47. Note the clinical features of NPH as expected (+) or not expected (−).

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a. Wide-based gait

+

b. Shuffling steps

+

c. Unsteadiness on turning

+

d. Difficult initiating steps

+

e. Feel glued to the floor

+

f. Ataxia of limbs

−

g. Slowness of thought

+

h. Unwitting urinary incontinence

−

i. Papilledema

−

j. Seizure

−

k. Headaches

−

48. True or False. Concerning cisternography for normal pressure hydrocephalus (NPH), what finding predicts a 75% improvement with a shunt? Radionucleotide in the ventricle at

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a. 24 hours

false

b. 48 hours

false

c. 72 hours

true (late scan 48 to 72 hours)

49. In NPH what is the sequence in which symptoms are likely to improve with shunting?

Hint: igd

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a. i_____

incontinence

b. g_____

gait

c. d_____

dementia

50. Blindness in hydrocephalus may be due to

Hint: pop

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a. p_____

papilledema—chronic—optic atrophy

b. o_____ _____ _____

optic chiasm compression dilation of third ventricle

c. p_____ _____ _____ _____

posterior cerebral artery occlusion compressed at tentorial edge

51. Blindness clinical criteria for localization are_____ _____ and _____ _____.

pregeniculate blindness and postgeniculate blindness

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a. Characteristics for pre-_____

pregeniculate blindness

     i. o_____ n_____ a_____ — s_____

optic nerve atrophy—severe

     ii. p_____ r_____ —p_____

pupillary reflexes—poor

     iii. due to p_____, h_____, a_____

pressure, hypotension, anemia

b. Characteristics for p_____ b_____

postgeniculate blindness

     i. o_____ n_____ a_____ — m_____

optic nerve atrophy—minimal

     ii. p_____ r_____ —n_____

pupillary reflexes—normal

     iii. due to_____ _____ _____

hypoxia macular sparing in PCA occlusion, no macular sparing in trauma to occiput

52. Cortical blindness may be associated with

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a. Anton syndrome = d_____ of v_____ d_____

denial of visual deficit

b. Riddoch phenomenon = a_____ of m_____ o_____, but n_____ a_____ of s_____ o_____

appreciation of moving objects, but no appreciation of stationary objects

53. Patients with shunt for hydrocephalus should prior to conception

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a. have up-to-date_____ or_____

CT or MRI

b. have assessment of any m_____

medications

c. if prospective mother’s hydrocephalus is accompanied by a neural tube defect (NTD), her child could be born with an NTD incidence of_____ to _____%

2 to 3%

d. have genetic c_____

counseling

e. start v_____

vitamins

f. avoid excessive h_____

heat

54. If shunt malfunctions during pregnancy, you may

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a. in the first two trimesters_____ the VP shunt

revise

b. in the third trimester use a_____-_____or a_____-_____ shunt

ventriculo-atrial, ventriculo-pleural

55. During labor and delivery

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a. Use p_____ a_____.

prophylactic antibiotics

b. If patient is asymptomatic deliver via_____.

vagina

c. If patient is symptomatic deliver via_____.

cesarean

d. In light of increased cranial pressure avoid_____.

epidurals