Hydrocephalus Flashcards
5 most common etiologies to hcph in peds?
- congenital w/o MMC 38%
- congenital with MMC 29%
- perinatal hemorrhage 11%
- tumor 11%
- previous infection 7.6%
- trauma/SAH 4.7%
signs of hcph in young children?
- 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
Why is the scalp veins enlarged and engorged in child hcph?
Reversed flow from intracerebral sinuses.
What is Macewens sign?
cracked pot sound on percussing dilated/engorged scalp veins in young child hcph.
What is the perinaud s syndrome?
Pressure at the suprapineal region give:
*upward gaze palsy
* read p 109
What is OCF?
occipital-frontal circumference
Rule of thumb for normal OCF?
Equals distance from crown to rump.
What is a normal head growth?
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.
typical radiological findings of benign external hydrocheplaus/external hydrocephalus? When does it resolve?
- enlarged subarachnoidal space over frontal poles in first year of life.
- normal/minimally enlarged ventricles.
- cortical vein sign
- usually resolve spontaneously by 2yo.
Clinical signs of benign external hcph
- 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.
Known associations to benign external hcph?
- some craniosynostoses esp. plagiocephaly
- following IVH
- following superior vena cava obstruction.
what needs to be done for a child w benign external hcph?
Parents need to change side for the child when sleeping not to risk head moulding.
How is L1 syndromes passed on?
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.
syndrome connected to x-linked inhereted hcph?
L1-syndrome.
L1CAM membrane bound receptor
Mmonic for classical L1 syndrome
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.
Arrested hcph
No progression or deleterious sequelae due to hcph that would require the presence of a CSF shunt.
When should someone w arrested hcph be advised to search help?
- H/A
- vomiting
- ataxia
- visual symptoms
What needs to be done for pt w apparent shunt independence?
- follow closely. Reports of death as long as 5 years after apparent shunt independence.
When to remove a shunt?
only if an apparent non functioning shunt get infected. and even then, follow closely.
What is entrapped 4th ventricle?
4th ventricle w no communication to 3rd v nor basal cisterns.
how common is entrapped 4th ventricle?
2-3% of shunted pt
When can entrapped 4th ventricle occur?
- after side ventricle shunting for many years and especially after infection. (fungal especially)
- Dandy walker syndrome
presentation of entrapped 4th ventricle
- 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”
what is special with the arachnoid villi in hcph onset from infantile age?
they are usually not patent. You can never use a Torkildsen shunt then. ? a VCS shouldnt work either I guess?
What type of hcph is caused by aqueductal stenosis?
triventricular hcph.
What are the most common etiologies for aqueductal stenosis?
- Inflammation following hemorrhage or infection (syphilis, TB)
- Neoplasm - especially brainstem astrocytoma and tectal situated gliomas. LIPOMAS
- Quadrigeminal plate arachnoidal cysts.
How many percent of congenital hcph is casued by aqueduct stenosis?
70%
How old is a child USUSALLY when a hcph is developed from a congenital aqueductstenosis?
Usually at birth, but up to 3 mo.
What are the three options for treating non- tumoral AqS?
- Shunting
- Torkildsen shunting
- Endoscopic third ventriculostomy
