Hydrocephalus

Question 1

5 most common etiologies to hcph in peds?

Answer 1

1. congenital w/o MMC 38%
2. congenital with MMC 29%
3. perinatal hemorrhage 11%
4. tumor 11%
5. previous infection 7.6%
6. trauma/SAH 4.7%

Question 2

signs of hcph in young children?

Answer 2

• cranium enlarges more than face
• abnormalities in head circumference
• poor head control
• irritability
• N/V - nausea vomiting
• full/bulging fontanelle
• frontal bossing (forhead prominent)
  *Enlarged and engorged scalp veins
• Mavewens sign
• 6th nerve palsy
• SETTING SUN SIGN
• hyperactive reflexes
• irregular respirations w apneic spells
  *splaying of cranial sutures

Question 3

Why is the scalp veins enlarged and engorged in child hcph?

Answer 3

Reversed flow from intracerebral sinuses.

Question 4

What is Macewens sign?

Answer 4

cracked pot sound on percussing dilated/engorged scalp veins in young child hcph.

Question 5

What is the perinaud s syndrome?

Answer 5

Pressure at the suprapineal region give:
*upward gaze palsy
* read p 109

Question 6

What is OCF?

Answer 6

occipital-frontal circumference

Question 7

Rule of thumb for normal OCF?

Answer 7

Equals distance from crown to rump.

Question 8

What is a normal head growth?

Answer 8

it follows a parallell line to normal curve over time.
*NOT more than 1.25cm growth for several weeks.
*NOT approaching +2SD
*NOT out of proportion compared to length and weight curves and within normal limits for age.

Question 9

typical radiological findings of benign external hydrocheplaus/external hydrocephalus? When does it resolve?

Answer 9

• enlarged subarachnoidal space over frontal poles in first year of life.
• normal/minimally enlarged ventricles.
• cortical vein sign
• usually resolve spontaneously by 2yo.

Question 10

Clinical signs of benign external hcph

Answer 10

• normal child, perhaps a bit late in motorf due to large head
• enlarged cisterns and widening of anterior interhemispheric fissure.
• usually resolve spontaneously by 2yo.

Question 11

Known associations to benign external hcph?

Answer 11

• some craniosynostoses esp. plagiocephaly
• following IVH
• following superior vena cava obstruction.

Question 12

what needs to be done for a child w benign external hcph?

Answer 12

Parents need to change side for the child when sleeping not to risk head moulding.

Question 13

How is L1 syndromes passed on?

Answer 13

L1 syndromes stand for mutations in the L1CAM membrane bound receptor that plays a sign role in CNS deveklopment for axonal migration to appropriate target locations through intergin cell adhesion molecules and MAP kinase signal cascade.

It is X bound (Xq28) and passed to sons through their phenotypically normal mother. Classical phenotypic expression will skip single generations.

Question 14

syndrome connected to x-linked inhereted hcph?

Answer 14

L1-syndrome.
L1CAM membrane bound receptor

Question 15

Mmonic for classical L1 syndrome

Answer 15

CRASH
C- corpus callosum hypoplasia
R- retardation
A- adducted thumbs
S- spastic paralys
H- hydrocephalus
MASA
M- mental handicap
A- aphasia
S- shuffling gait
A- adducted thumbs
HSAS
H- hydrocephalus with
S- stenosis of the
A- aqueduct of
S- sylvi
The spectrum also involves agenesis of CC and spastic paraparesis type 1.

Question 16

Arrested hcph

Answer 16

No progression or deleterious sequelae due to hcph that would require the presence of a CSF shunt.

Question 17

When should someone w arrested hcph be advised to search help?

Answer 17

• H/A
• vomiting
• ataxia
• visual symptoms

Question 18

What needs to be done for pt w apparent shunt independence?

Answer 18

• follow closely. Reports of death as long as 5 years after apparent shunt independence.

Question 19

When to remove a shunt?

Answer 19

only if an apparent non functioning shunt get infected. and even then, follow closely.

Question 20

What is entrapped 4th ventricle?

Answer 20

4th ventricle w no communication to 3rd v nor basal cisterns.

Question 21

how common is entrapped 4th ventricle?

Answer 21

2-3% of shunted pt

Question 22

When can entrapped 4th ventricle occur?

Answer 22

• after side ventricle shunting for many years and especially after infection. (fungal especially)
• Dandy walker syndrome

Question 23

presentation of entrapped 4th ventricle

Answer 23

• H/A
• swallowing difficulties
• If the 4th venricle compress the facial colliculus; facial biplegia and/or bilateral abducens paresis might occur.
• reduced level of consiousness
• nausea/vomiting
• “incidental finding”

Question 24

what is special with the arachnoid villi in hcph onset from infantile age?

Answer 24

they are usually not patent. You can never use a Torkildsen shunt then. ? a VCS shouldnt work either I guess?

Question 25

What type of hcph is caused by aqueductal stenosis?

Answer 25

triventricular hcph.

Question 26

What are the most common etiologies for aqueductal stenosis?

Answer 26

• Inflammation following hemorrhage or infection (syphilis, TB)
• Neoplasm - especially brainstem astrocytoma and tectal situated gliomas. LIPOMAS
• Quadrigeminal plate arachnoidal cysts.

Question 27

How many percent of congenital hcph is casued by aqueduct stenosis?

Answer 27

70%

Question 28

How old is a child USUSALLY when a hcph is developed from a congenital aqueductstenosis?

Answer 28

Usually at birth, but up to 3 mo.

Question 29

What are the three options for treating non- tumoral AqS?

Answer 29

• Shunting
• Torkildsen shunting
• Endoscopic third ventriculostomy