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Elsa AACN > Ch 20 congenital and acquired hydrocephalus > Flashcards

Ch 20 congenital and acquired hydrocephalus Flashcards

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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
aqueductal stenosis
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”)
26
Dandy Walker syndrome
70-80% of children with DWS develop hydrocephalus
27
Cause of hydrocephalus in people with DWS?
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
28
Prematurity Intraventricular Hemorrhage (IVH)
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
29
Which IVH grades are associated with hydrocephalus?
Grades III and IV
30
Internal hydrocephalus
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 ```
31
external hydrocephalus
accumulation of CSF and other forms of hydrocephalus OUTSIDE of ventricles do not necessarily involve increased ICP involves subarachnoid spaces
32
communicating hydrocephalus
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
33
non communicating hydrocephalus
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
34
normal pressure hydrocephalus
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
35
risk factors of idiopathic NPH
HTN, vascular disease, diabetes
36
mortality rates of congenital hydrocephalus
0.7/ 100,000
37
death rate highest in what group in congenital hydrocephalus
infants
38
Neural tube defects prevalence
1-10/1000 births
39
why have rates of neural tube defects decreased?
increased emphasis on folate acid | shunting
40
Myelomeningocele accounts for ?% of all forms of congenital hydrocephalus
70%
41
Myelomeningocele ratio of Male : Female
Females more frequently slightly
42
Aqueductal stenosis incidence rate
5-10/100,000
43
Aqueductal stenosis accounts for ?% of all forms of congenital hydrocephalus
5-15% of congenital hydrocephalus
44
aqueductal stenosis is usually detected during infancy because of
head size control gaze
45
Dandy walker syndrome rate
3-5/100,000
46
Dandy walker syndrome accounts for ?% of all forms of congenital hydrocephalus
5-10%
47
when is Dandy walker detected?
prenatal diagnosis | 1st year of life
48
why is dandy walker detected during 1st year of life
head size poor control gaze problems
49
Dandy walker is often fatal or non fatal
fatal (50%)
50
prematurity IVH
determined by grade and not incidence | lowest birth weight and gestational age usually have highest incidence and severity
51
normal pressure hydrocephalus incidence rate
5-6/100,000
52
normal pressure hydrocephalus overall prevalence rate
0.5% (usually underestimated)
53
incidence of NPH increases with?
age
54
idiopathic NPH prevalence rate?
2-6%
55
How is NPH diagnosed?
headaches urinary incontinence gait abnormality mental decline HUGMe
56
How is NPH treated
reversible with shunting
57
what determines outcome in spina bifida myelomeningocele?
``` severity of chiari II malformation direct effects on cerebellar functions tectal beaking hypogenesis of corpus callosum level of spinal lesion ```
58
higher level spinal defects are associated with
paraplegia of lower limbs inability to ambulate more severe brain malformation
59
in other congenital hydrocephalus, severity is related to
hydrocephalus | underlying disorder
60
severity of aqueductal stenosis depends on?
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
severity of Dandy walker depends on?
cystic malformation | degree of underdevelopment of cerebellar vermis
62
manifestation of DWS?
gait | upper extremity control
63
prematurity IVH severity outcomes depend on?
prematurity complications
64
in idiopathic NPH, severity is dependent on?
increase in ICP | compression of ventricles on other parts of the brain
65
When is spina bifida myeloningocele identified usually?
at birth because of the spinal lesion | if lesion is myeloningocele, then concerns for chiari II malformation and hydrocephalus
66
When is aqueductal stenosis and DWS identified usually?
infancy because of head circumference head control gaze abnormality
67
IVH is identified on
routine ultrasound
68
hydrocephalus is a chronic or acute problem?
chronic problem after treatment
69
treatment of hydrocephalus
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
where is the shunt usually placed?
right posterior ventricle with valve that drains fluid into the peritoneal cavity
71
what is endoscopic third ventriculostomy (ETV)
the floor of 3rd ventricle is perforated to drain CSF into an open CSF space, usually the subcistern
72
NP results for spina bifida
``` impairment in fine motor skills (2/2 chiari II malformation) ID attention ADHD I ```
73
congenital hydrocephalus attention skills
difficulties with orienting and disengaging stronger regulation when oriented and engaged (disorder of posterior attention systems)
74
congenital hydrocephalus NP profile
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
spina bifida quality of life affected by what factors
orthopedic and bladder complications
76
DWS NP pattern
severe difficulties with upper motor coordination due to cerebellar hypoplasia ID
77
premature IVH
children with grades IV IVH with shunted hydrocephalus performed worse than those who are not shunted
78
idiopathic NPH NP
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
employment in congenital hydrocephalus
independent and can work, but underemployed
80
Transition from childhood to adolescence to adulthood in congenital hydrocephalus is
challenging, and can feel isolating
81
NPH in older adults is often mistaken for
dementia or PD
82
diastomyelia
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
hydrocephalus ex vacuo
expansion of the ventricles with no increase in ICP | arrested or compensated hydrocephalus
84
Ventriculomegaly
enlarged ventricles
85
meningocele
meninges protrude through the spinal cord
86
brain of children with meningocele is normal or abnormal
normal, but some may develop aqueductal stenosis
87
myelomeningocele
protrusion of meninges through incompletely formed spinal cord with a sac containing CSF and CNS material associated with chiari II malformation
88
what is the primary cause of hydrocephalus in children
myelomeningocele
89
children with hydrocephalus secondary to spina bifida myelomeningocele and aqueductal stenosis show what kind of NP?
similar patterns except in motor domain (spina bifida myelomeningocele has MORE difficulty with fine motor skills)
90
hydrocephalus affects white matter? gray matter?
widespread effects on white matter, gray matter stretching of white matter axons compressive effects on cortical structures
91
spina bifida myelomeningocele and Dandy Walker syndrome both show what impairment?
fine motor skills
92
In spina bifida myelommeningocele and aqueductal stenosis
verbal skills > than spatial skills

Elsa AACN (35 decks)

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