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19 Cards in this Set

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• Differentiate familial large head from hydrocephalus
Familial large head
Head larger than 95th percentile (larger than 2 SD)
-follow normal growth pattern
everything is normal, mom and dad just gave you a big head!

Other large heads
Mostly due to hydrocephalus
• Differentiate obstructive and non-obstructive hydrocephalus
Blockage of this flow anywhere within the substance of the brain (i.e. before the CSF passes through the foramina of Luschka and Magendie) results in obstructive or non-communicating hydrocephalus

Overproduction or decreased absorption at the arachnoid villi results in communicating or non-obstructive hydrocephalus.
Rapid increase in head size (as seen by plotting serial head circumference measurements against a normal set of curves)
Large bulging fontanel (in an upright child)
hydrocephalus in infants
what 2 malformations are commonly seen in Hydrocephalus in Older Children
Foreshortened occiput (with Chiari malformation)

Prominent occiput (with Dandy-Walker malformation)
describe a CHIARI MALFORMATION

does it cause non-obstructive or obstructive hydrocephaly?
Displacement of the cerebral tonsils into the cervical canal  usually no sx until adolescence or adulthood. (Recurrent headaches, neck pain, urinary frequency, and progressive lower extremity spasticity. Etiology unknown.

Non-communicating or Obstructive
describe a Dandy Walker Malformation

does it cause non-obstructive or obstructive hydrocephaly?
Dandy-Walker Syndrome: a cystic expansion of the 4th ventricle with failure of formation of the roof of the forth ventricle  90% have hydrocephalus. Other associated anomalies include agenesis of the posterior cerebellar vermis and of the corpus collosum.

Non-communicating or Obstructive
tx for hydrocephalus?
Acetazolamide and furosemide reduce CSF production, but are not definitive solutions

V/P* shunting is palliative not curative (complicated by infection or obstruction)
which is more greatly affected in hydrocephalus: motor or intellect?
Motor performance is more greatly affected than intellect because the gray matter of the brain is less affected by the hydrocephalus than the white matter (spasticity or coordination defects)
Which of the following will you find in a child with familial macrocephaly?

Rapid increase in head growth
Cracked pot sound on percussion
1st degree relative with large head
History of meningitis
1st degree relative with large head
Which of the following is seen in an older child with untreated hydrocephalus?

Gaze disturbance
Rapid increase in head size
Bulging fontanelle
Normal funduscopic exam
Gaze disturbance
Most common congenital anomaly of nervous system?

due to?
Neural Tube Defects

FOLATE DEF
Anencephaly
Failure of anterior neural tube closure with the presence of exposed rostral mass of neural tissue
Encephalocele
Malformation involving defective closure of a portion of the neural tube in association with a bony skull defect
Midline defect with failure of closure of the posterior vertebral arches and laminae (typically lumbosacral)
Without protrusion of meninges or spinal cord
Ordinarily asymptomatic and found incidentally on X-ray in 5 to 15% of the general population
Spina Bifida Occulta
define a Meningocele.

Sx?
Midline defect in which the meninges herniate through a defect in the posterior vertebral arches, unaccompanied by neural tissue

Many patients have **gait abnormalities or loss of bladder control***, particularly during periods of rapid growth
define a Myelomeningocele

how do sx vary based on location?
Midline defect of the posterior vertebral arches with herniation of meninges and neural tissue

High lesions (above T11) are associated with higher morbidity, lower intelligence, and greater disability than low lesions (below L3)

Mid-sacral to mid-lumbar region:
flaccid paralysis of the legs
lack of touch and pain sensation
clubfeet and subluxed hips
Chiari II malformation with hydrocephalus (~80%)

Low sacral region:
bladder and bowel incontinence
perineal anesthesia
no motor involvement
A child is born with a low lumbar midline defect. MRI shows herniation of meninges through a spinal defect without neural tissue. What should you expect?

He will develop worsening spasticity in his legs
He may have intermittent incontinence as he grows
80% are associated with Chiari II malformation
Lack of touch and pain sensation in the legs
correct: He may have intermittent incontinence as he grows

myelomeningiocele:
80% are associated with Chiari II malformation
Lack of touch and pain sensation in the legs
On the boards you will be asked about the most common cause of neural tube defects. You’ll answer:

Maternal drug use
TORCH infection
Radiation
Malnutrition
B9 deficiency
B9 deficiency
A 5 month infant is brought to your office. At your visit a few months ago, you noted some flattening of the back of his skull. You advised repositioning. On review of yesterday’s CT scan, you note a premature fusion of bilateral lambdoid sutures. You advise the parents:

He should receive OMT
He needs an MRI
He should receive surgery
OMT won’t be enough, he’ll need helmet therapy
He should receive surgery