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Elsa AACN > Ch 20 congenital and acquired hydrocephalus > Flashcards

Ch 20 congenital and acquired hydrocephalus Flashcards

1
Q

Definition of hydrocephalus

A

accumulation of cerebral spinal fluid in or around the ventricles

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2
Q

Result of hydrocephalus?

A 
ventricular expansion
pressure on other parts of the brain
impairment of gait
atypical eye movements
headache
vomiting
cognitive difficulty
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3
Q

Is hydrocephalus usually a primary or secondary condition

A

secondary

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4
Q

Can hydrocephalus be acquired or congenital?

A

either congenital or acquired

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5
Q

Cause of hydrocephalus

A

obstruction that blocks the normal flow of CSF in the brain

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6
Q

What can cause ventricular dilation

A

inadequate absorption or overproduction of CSF

loss of brain tissue because of atrophy

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7
Q

When ventricular dilation occurs, what happens?

A

stretching and enlargement of periventricular white matter fibers –>
demyelination and disruption of development of cerebral white matter

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8
Q

What is the function of CSF flow?

A

flows in and around the brain and spinal cord
cushion from injury
provide nutrients.
remove potentially toxic metabolic byproducts

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9
Q

hydrocephalus can be understood as a subcortical disconnection syndrome because

A

because of the injury to the long periventricular pathways that support communication

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10
Q

Is hydrocephalus an isolated condition?

A

No. It’s usually secondary to other disease process

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11
Q

classification of hydrocephalus is based on

A

age of origin
site of disruption
whether there is increased and sustained ICP

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12
Q

Types of congenital hydrocephalus?

A

4 types of congenital hydrocephalus

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13
Q

Which 4 types of congenital hydrocephalus

A

Spina bifida myelomenigocele
aqueductal stenosis
dandy-walker syndrome
prematurity intravanetricular hemorrhage

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14
Q

Congenital hydrocephalus are (name 3 characteristics)

A

obstructive
non communicating
internal

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15
Q

Spina bifida myelomeningocele

A

nonlethal neural type defect that occurs in first 30 days of gestation
most common form of spina bifida
most common cause of congenital hydrocephalus

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16
Q

Spina bifida myelomeningocele accounts for how many % of childhood cases?

A

70%

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17
Q

What happens in spina bifida? describe

A

in spina bifida, the portion of the neural tube that eventually forms the vertebrae column does not fuse during embryogenesis

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18
Q

the degree of closure of the neural tube can lead to…

A

different kinds of spinal lesions apparent at birth

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19
Q

myelomeningocele

A

open neural tube defect
open spinal cord
CNS is exposed
with meningeal cyst

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20
Q

examples of closed spinal defects

A

meningocele
spina bifida occulta (closed asymptomatic)
closed spinal dysraphism covered with lipoma (fatty tumor)
diastomyelia (defects of the vertebrae)

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21
Q

spina bifida occulta

A

common form of spinal abnormality with no protruding spinal sac that us usually not overtly apparent at birth

usually found out with back pain, identified on radiology

rarely associated with hydrocephalus or other brain abnormalities

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22
Q

what happens during spina bifida myelomeningocele

A

spinal cord protrudes through the meninges
hydrocephalus develops in 90% of infants
- Chiari II malformation of hindbrain
- obstruction of 4th ventricle

abnormal corpus callosum
abnormal cerebellum

C4CC
Chiari
4th ventricle
Corpus callosum
Cerebellum
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23
Q

Chiari II malformation is ubiquitous in association with

A

myelomeningocele

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24
Q

What is Chiari II malformation?

A

cerebellar and brainstem extends into skull and bulges through foramen magnum (hole at base of skull where spinal cord passes)

puts pressure on parts of the brain and spinal cord

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25
Q

aqueductal stenosis

A

narrowing of the aqueduct of Sylvius/cerebral aqueduct which blocks the flow of CSF in the ventricular system.

causes hydrocephalus by congenital narrowing of the adqueduct of Sylvius / cerebral aqueduct

cerebellum usually normal

consequence - more likely to have difficulty looking up (“upward gaze palsy”)

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26
Q

Dandy Walker syndrome

A

70-80% of children with DWS develop hydrocephalus

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27
Q

Cause of hydrocephalus in people with DWS?

A

partial or complete absence of the cerebellar vermis (medial part of the cerebellum)

cystic enlargement of the 4th ventricle

cyst formation of the base of the skull (posterior fossa).

C4P
Cerebellar vermis MISSING!
4th ventricle
Posterior fossa cyst

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28
Q

Prematurity Intraventricular Hemorrhage (IVH)

A

children with prematurity IVH develop hydrocephalus because of hemorrhage involving geminal matrix shortly after birth

happens in very low birth weight infants

hydrocephalus usually non progressive in these case

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29
Q

Which IVH grades are associated with hydrocephalus?

A

Grades III and IV

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30
Q

Internal hydrocephalus

A

obstructive hydrocephalus
involves formamen of monro
narrowing of aqueduct of sylvius

results in: 
accumulation of CSF WITHIN the ventricles
increase in ICF
ventricular expansion
compression of brain
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31
Q

external hydrocephalus

A

accumulation of CSF and other forms of hydrocephalus OUTSIDE of ventricles
do not necessarily involve increased ICP
involves subarachnoid spaces

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32
Q

communicating hydrocephalus

A

non obstructive
abnormality in CSF absorption
occurs when the flow of cerebrospinal fluid (CSF) is blocked after it exits the ventricles.
result from a thickening of the arachnoid around the base of the brain, which blocks the flow of CSF from the spinal to the cortical subarachnoid spaces.
ventricular enlargement with accumulated CSF (e.g. dementia)

ICP not always increased

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33
Q

non communicating hydrocephalus

A

obstructive form of hydrocephalus associated with congenital disorders
can be associated with disorders (e.g. cysts) that obstruct the outflow of CSF into subarachnoid space

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34
Q

normal pressure hydrocephalus

A

adult onset
accumulation of CSF in the ventricles
increase ICP
may have no obvious cause, but can be due to insult (e.g. tumor, hemorrhage, TBI)
hydrocephalus is usually a secondary condition

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35
Q

risk factors of idiopathic NPH

A

HTN, vascular disease, diabetes

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36
Q

mortality rates of congenital hydrocephalus

A

0.7/ 100,000

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37
Q

death rate highest in what group in congenital hydrocephalus

A

infants

38
Q

Neural tube defects prevalence

A

1-10/1000 births

39
Q

why have rates of neural tube defects decreased?

A

increased emphasis on folate acid

shunting

40
Q

Myelomeningocele accounts for ?% of all forms of congenital hydrocephalus

A

70%

41
Q

Myelomeningocele ratio of Male : Female

A

Females more frequently slightly

42
Q

Aqueductal stenosis incidence rate

A

5-10/100,000

43
Q

Aqueductal stenosis accounts for ?% of all forms of congenital hydrocephalus

A

5-15% of congenital hydrocephalus

44
Q

aqueductal stenosis is usually detected during infancy because of

A

head size
control
gaze

45
Q

Dandy walker syndrome rate

A

3-5/100,000

46
Q

Dandy walker syndrome accounts for ?% of all forms of congenital hydrocephalus

A

5-10%

47
Q

when is Dandy walker detected?

A

prenatal diagnosis

1st year of life

48
Q

why is dandy walker detected during 1st year of life

A

head size
poor control
gaze problems

49
Q

Dandy walker is often fatal or non fatal

A

fatal (50%)

50
Q

prematurity IVH

A

determined by grade and not incidence

lowest birth weight and gestational age usually have highest incidence and severity

51
Q

normal pressure hydrocephalus incidence rate

A

5-6/100,000

52
Q

normal pressure hydrocephalus overall prevalence rate

A

0.5% (usually underestimated)

53
Q

incidence of NPH increases with?

A

age

54
Q

idiopathic NPH prevalence rate?

A

2-6%

55
Q

How is NPH diagnosed?

A

headaches
urinary incontinence
gait abnormality
mental decline

HUGMe

56
Q

How is NPH treated

A

reversible with shunting

57
Q

what determines outcome in spina bifida myelomeningocele?

A 
severity of chiari II malformation
direct effects on cerebellar functions
tectal beaking
hypogenesis of corpus callosum
level of spinal lesion
58
Q

higher level spinal defects are associated with

A

paraplegia of lower limbs
inability to ambulate
more severe brain malformation

59
Q

in other congenital hydrocephalus, severity is related to

A

hydrocephalus

underlying disorder

60
Q

severity of aqueductal stenosis depends on?

A

usually pure form of early hydrocephalus
may present with no other brain malformation
abnormality in cerebellum and corpus callosum will have more severe outcomes

61
Q

severity of Dandy walker depends on?

A

cystic malformation

degree of underdevelopment of cerebellar vermis

62
Q

manifestation of DWS?

A

gait

upper extremity control

63
Q

prematurity IVH severity outcomes depend on?

A

prematurity complications

64
Q

in idiopathic NPH, severity is dependent on?

A

increase in ICP

compression of ventricles on other parts of the brain

65
Q

When is spina bifida myeloningocele identified usually?

A

at birth because of the spinal lesion

if lesion is myeloningocele, then concerns for chiari II malformation and hydrocephalus

66
Q

When is aqueductal stenosis and DWS identified usually?

A

infancy because of head circumference
head control
gaze abnormality

67
Q

IVH is identified on

A

routine ultrasound

68
Q

hydrocephalus is a chronic or acute problem?

A

chronic problem after treatment

69
Q

treatment of hydrocephalus

A

shunt implantation that diverts the flow of CSF around the site of blockage

endoscopic third ventriculostomy (ETV)

medication to absorb excessive fluid

surgery to replace spinal defect

70
Q

where is the shunt usually placed?

A

right posterior ventricle with valve that drains fluid into the peritoneal cavity

71
Q

what is endoscopic third ventriculostomy (ETV)

A

the floor of 3rd ventricle is perforated to drain CSF into an open CSF space, usually the subcistern

72
Q

NP results for spina bifida

A 
impairment in 
fine motor skills (2/2 chiari II malformation)
ID
attention
ADHD I
73
Q

congenital hydrocephalus attention skills

A

difficulties with orienting and disengaging
stronger regulation when oriented and engaged
(disorder of posterior attention systems)

74
Q

congenital hydrocephalus NP profile

A

NVLD
verbal IQ > non verbal IQ

strengths -
verbal skills, rote verbal material, facial recognition, vocab, grammar, decoding, spelling, procedural learning

deficits -
processing speed, attention, motor skills, memory and learning, math, EF (more attention/motor)

mood: hypersocial

75
Q

spina bifida quality of life affected by what factors

A

orthopedic and bladder complications

76
Q

DWS NP pattern

A

severe difficulties with upper motor coordination due to cerebellar hypoplasia
ID

77
Q

premature IVH

A

children with grades IV IVH with shunted hydrocephalus performed worse than those who are not shunted

78
Q

idiopathic NPH NP

A

variable, diffuse pattern
improves with shunting more likely in patients who have not shown severe gait difficulties, dementia or stroke
deficits on motor based tasks, attention, memory, spatial skills, concept formation

79
Q

employment in congenital hydrocephalus

A

independent and can work, but underemployed

80
Q

Transition from childhood to adolescence to adulthood in congenital hydrocephalus is

A

challenging, and can feel isolating

81
Q

NPH in older adults is often mistaken for

A

dementia or PD

82
Q

diastomyelia

A

developmental deformity in which the spinal cord is separated into two parts by a rigid or fibrous septum. The deformity is often accompanied by abnormal development of the vertebrae.

83
Q

hydrocephalus ex vacuo

A

expansion of the ventricles with no increase in ICP

arrested or compensated hydrocephalus

84
Q

Ventriculomegaly

A

enlarged ventricles

85
Q

meningocele

A

meninges protrude through the spinal cord

86
Q

brain of children with meningocele is normal or abnormal

A

normal, but some may develop aqueductal stenosis

87
Q

myelomeningocele

A

protrusion of meninges through incompletely formed spinal cord with a sac containing CSF and CNS material

associated with chiari II malformation

88
Q

what is the primary cause of hydrocephalus in children

A

myelomeningocele

89
Q

children with hydrocephalus secondary to spina bifida myelomeningocele and aqueductal stenosis show what kind of NP?

A

similar patterns except in motor domain (spina bifida myelomeningocele has MORE difficulty with fine motor skills)

90
Q

hydrocephalus affects white matter? gray matter?

A

widespread effects on white matter, gray matter
stretching of white matter axons
compressive effects on cortical structures

91
Q

spina bifida myelomeningocele and Dandy Walker syndrome both show what impairment?

A

fine motor skills

92
Q

In spina bifida myelommeningocele and aqueductal stenosis

A

verbal skills > than spatial skills

Elsa AACN (35 decks)

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