PEDIATRICS II Lecturer: Martha bolanos, MD

S4-05c: Hydrocephalus Date: 05-11-2021

OUTLINE
I. CSF Physiology
a. Subtopic A
i. Sub-subtopic
b. Subtopic B
c. Subtopic C
II. Hydrocephalus
a. Causes
b. Clinical Manifestations
III. Dandy walker malformation
a. Diagnosis
b. Differential Diagnosis
c. Management
d. Prognosis
IV. Post Quiz

References: Lecture Recording and Powerpoint

Presentation
Figure 1 The ventricular system of the human brain
Legend:
● Ventricular system: choroid plexus is usually
Reference textbook
located in the lateral ventricle (site of production of
Audio from lecture recording
CSF); from the ventricle it will now flow to the
Nice-to-Know
frontal horn, lateral horn, and occipital horn and
TG Notes
through the foramen of monro to your third
Important
ventricle then cerebral aqueduct then to your
Revised/Corrected from previous trans fourth ventricle and it will be absorbed in your
Foramen of Magendie and Luschka.
I. CSF PHYSIOLOGY
● CSF is formed primarily by the choroid plexus in II. HYDROCEPHALUS
the ventricular system (LV. 3rd, and 4th ventricle) ● Diverse group of conditions that result from
● But 25% originate from extra choroidal surfaces, impaired circulation and or absorption of CSF
including the capillary endothelium within the brain ● Obstructing or non communicating hydrocephalus-
parenchyma resulting from obstruction within the ventricular
● Neurogenic control of CSF formation: adrenergic system (there is a mass lesion present)
system diminishes CSF production, excitation of ● Communicating or non obstructive hydrocephalus-
cholinergic nerves may double the normal CSF result form obliteration of the subarachnoid
production rate cisterns or malfunctions of the arachnoid villi.
● Normal child: CSF production = 20 ml/hr ○ Example: If you have meningitis or
● Total volume of CSF hemorrhage so you may have gliosis in the
○ Infant - 50ml subarachnoid spaces so the arachnoid villi
○ Adult - 150 ml cannot absorbed the csf therefore resulting in
this type of hydrocephalus

CAUSES OF HYDROCEPHALUS
● Acquired hydrocephalus:
● Dysfunctional SAS
○ SAH or infection
○ Spinal Cord Tumor
○ Disseminated Tumor
○ Choroid Plexus Tumor
● Ventricular obstruction: obstruction in any part of
the ventricular system
○ IVH (specially in preterm babies) or infection
(ventriculitis)
○ parenchymal brain tumor (usually the cause of
ventricular obstruction)

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○ choroid plexus tumor
● Vascular
○ vascular malformation
○ disordered cerebral venous function
● Congenital hydrocephalus/ genetic
abnormalities
○ more common cause in newborns
● Congenital or developmental hydrocephalus
○ congenital aqueductal stenosis- most common
cause
○ neural tube defects (myelomeningocele or chiari
II malformation
○ posterior fossa malformations
○ developmental cysts
○ congenital foramen of monro atresia
● Genetic Abnormalities associated with
Pediatric Hydrocephalus
○ x linked hydrocephalus with AS
○ Nonsyndromic AR hydrocephalus
○ Fried-type syndromic MR
○ Walker- warburg syndrome
○ NT defects
○ Primary ciliary dyskinesias and other ciliopathies
○ RAS- opathies
○ VACTERL-H
● Obstructive HCP
○ Aqueductal stenosis (most common in
newborns)- x linked recessive trait, associated
with spina bifida occulta, NF
○ Aqueductal gliosis- secondary to neonatal
meningitis or SAH in PT, intrauterine viral
infections, mumps meningoencephalitis
○ vein of galen formation- vascular malformation,
occurs during the newborn age
○ space occupying lesions in the posterior fossa
○ malformations in the posterior fossa- chiari
malformation and dandy walker syndrome
Figure 2 Left image:Ultrasound showing congenital HCP
with Aqueductal Stenosis
Right image: MRI: very small area beside the blue color
because of the partial aqueductal stenosis causing HCP
● Ultrasound of anterior fontanelle is done in infants
since it is less expensive and you can do it without
sedating the patient. ct scan or mri requires
sedation and it is hard to monitor the bp when
inside the machine
● Some are diagnosed perinatally (when patient is
still inside the womb)
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S4-05c: Hydrocephalus
Figure 3 Example of baby with congenital HCP
● Very prominent head and the setting sun eyes
because of the compression in the brainstem
● ultrasound shows aqueductal stenosis; there is
enlargement of lateral ventricle, 3rd ventricle, as
well as temporal horns.
● congenital hydrocephalus usually occurs during
the neural proliferation stage
CLINICAL MANIFESTATIONS
● Infant: increasing HC (some may be born with big
head already), open and bulging AF, dilated scalp
veins, broad forehead, setting-sun eye signs
● hyperreflexia, spasticity, clonus, bilateral Babinski
signs
● Child: irritability, lethargy, poor appetite, vomiting,
headache. Gradual personality change and
deterioration in academic performance
● Serial HC monitoring (check baby book of the
patient), Macewen sign
● Papilledema, abducens nerve palsy, pyramidal
tract signs (LE)
● Foreshortened occiput- chiari malformation
● Prominent occiput- dandy walker malformation
● Chiari Malformation
○ Type 1- displacement of cerebellar tonsils into
the cervical canal. Patients may have normal
growth initially then when adolescent stage
occurs symptoms happen. Late manifestation
during adolescence or adult life with recurrent
headache, neck pain, urinary frequency, and
progressive LE spasticity
● Chiari II malformation
○ progressive hydrocephalus with
myelomeningocele (mass in the back of the
patient usually thoracic or lumbosacral in
location)
○ failure of pontine flexure during embryogenesis,
resulting in elongation of the 4th ventricle and
kinking of the brainstem, with displacement of
the inferior vermis, pons, and medulla into the
cervical canal
○ present during infancy with stridor, weak cry, and
apnea
○ management: ventriculoperitoneal shunting of
hydrocephalus
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Figure 4 Types of Chiari Malformation

● Chiari I- the cerebellum is descended to the
cervical canal; there will be obstruction and patient
will develop enlargement of central canal in the
cervical cord
● Chiari II- pons, medulla is down on the cervical
canal
Figure 5 Physical Manifestations of Neural Tube Defects
● Check always the back of the babies to check for
spinal deformities, tufts of hair,dimple( dermal
sinus tract
III. DANDY-WALKER MALFORMATION
● Cystic expansion of the 4th ventricle in the
posterior fossa
● Diagnostics:
○ Plain skull x ray: separation of sutures, erosion
of the posterior clinoids, beaten- silver
appearance
○ UTZ/CT/MRI
● Differential Diagnosis:
○ Thickened cranium, metabolic and degenerative
disorders producing megalencephaly, gigantism,
NF, familial megalencephaly
● Management: depends on the cause
Figure 6 Patient with Dandy-Walker Malformation
● Initiallyyou may think that the baby has
hydrocephalus secondary to cerebral aqueduct
stenosis but when you do your neuroimaging you
find this cyst in the posterior fossa which is
diagnostic of dandy walker malformation
DIAGNOSIS
● History
○ Age of onset
○ Family history
○ Past history of prematurity (usually they have
intraventricular hemorrhage and this can cause
post hemorrhagic hydrocephalus) or CNS
infection
● Physical Examination
○ Skin: Neurocutaneous lesions (NF)
○ Inspection, palpation, and auscultation of the
skull and spine
○ Occipitofrontal HC is compared with previous
measurements
○ Size and configuration of the anterior fontanel
○ Inspect back for abnormal midline skin defects:
tufts of hair, lipoma, or angioma
○ Prominence of the forehead
○ Abnormalities in the shape of the head, esp. the
occiput
○ Cranial bruit
○ Transillumination of the skull
○ Fundoscopy: chorioretinitis (patient may have
TORCH), papilledema
○ Plain skull film- separation of sutures, erosion of
posterior clinoids, beaten-silver appearance
○ Cranial UTZ
○ CT scan/ MRI
DIFFERENTIAL DIAGNOSIS
● Macrocephaly
○ Megalencephaly (brain volume is big; but patient
is not smart and this may be secondary to
lysosomal diseases, aminoacidurias,
leukodystrophies
○ Chronic anemia
○ Rickets
○ Osteogenesis imperfecta
○ Epiphyseal dysplasia
○ Chronic subdural collections
■ parietal bone prominence
○ Increased brain mass

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 S4-05c: Hydrocephalus
■ Sotos syndrome, overgrowth syndromes, NF a. 50ml
○ Familial megalencephaly b. 150 ml
○ hydranencephaly c. 200 ml
d. 250 ml
MANAGEMENT e. None of the above
● Depends on the cause
● Medical management if patient cannot undergo 6. Dandy-Walker Malformation is synonymous to the
surgery we can give: Chiari Type III Malformation.
○ acetazolamide, furosemide to lower CSF a. True
production but you should monitor electrolytes b. False
● Surgery
○ ventriculoperitoneal shunt- under the skin; no 7. CSF production is primarily done in the choroid
cosmetic problems plexus specifically in the 5th and 7th Ventricle
○ endoscopic third ventriculostomy- new a. True
procedures; open third ventricle to remove b. False
obstruction; advantage is you would not leave
any device in the patient 8. The prominence or size of the occiput can
● Complications of shunting differentiate a Chiari Malformation from a
○ shunt malfunction Dandy-Walker Malformation.
○ shunt infection a. True
b. False
PROGNOSIS
● When shunting is done late cognitive functions are
not reversed anymore
Answers: 1D,2A,3B,4C,5A, 6B, 7B, 8A
● Depends on the cause
● Increased risk for various developmental
disabilities
● Decreased IQ **********End of Transcription**********
● Memory problems
● Visual problems (strabismus, visuospatial
abnormalities, visual field defects, optic atrophy
● May need long term follow up in a multidisciplinary
setting

IV. POST QUIZ

1. Cystic expansion of the 4th ventricle in the
posterior fossa?
a. Chiari 1
b. Chiari 2
c. HCP with C.A. stenosis
d. Dandy walker

2. Displacement of cerebellar tonsils into the

cervical canal:
a. Chiari 1
b. Chiari 2
c. HCP
d. Dandy walker

3. Progressive hydrocephalus with

myelomeningocele?
a. Chiari 1
b. Chiari 2
c. HCP
d. Dandy walker

4. Normal child CSF production?

a. 10ml
b. 15 ml
c. 20 ml
d. 25 ml

5. Total volume of CSF in an infant?

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