Craniosynostosis & Hydrocephalus 1

Head shape abnormalities

Skull growth

- After birth skull suture remain open to allow brain and skull growth.
- Skull sutures
o Metopic suture (between frontal bones)
o Coronal suture (between frontal and parietal bones)
o sagittal suture( between parietal bones)
o lambdoid suture (between parietal and occipital bones)
- Skull grows perpendicular to sutures.
o sagittal suture run from anterior to posterior,
so it permit skull to grow laterally.
o Coronal permit skull to grow posteroanterior growth.
- if suture close prematurely the skull will grow in all directions
except perpendicular to the that’s suture .
o So skull to compensate will grow more parallel to closed suture.

skull grow perpendicular to sutures

Premature Suture fused Description Terminology causes

Sagittal Boat skull (AP diameter of skull is long Scaphocephaly
leading to frontal bossing and occipital
cupping and the biparietal diameter is
shortened)
Metopic Triangular skull Trigonencephaly
Bilateral Coronal Broad and short skull Brachycephaly Down syndrome
Unilateral coronal Anterior Asymmetric skull (Flattened Anterior plagiocephaly
lateral forehead over involved suture,
prominent opposite forehead)
Unilateral lambdoid posterior Asymmetric skull Posterior plagiocephaly
Bilateral lambdoid Broad and short skull Brachycephaly
 Craniosynostosis & Hydrocephalus 2

Define shape ? Scaphocephaly

Define shape? Trigonencephaly
What is cause? sagittal Synostosis
What is the cause? Metopic Synostosis ( premature closure of sagittal suture)
(premature closure of Metopic suture)

Define shape? Brachycephaly

Define shape ? Anterior plagiocephaly What is cause? premature closure of Bilateral
Coronal suture
What is cause ? premature closure of Associated condition? Down syndrome
Unilateral (right) coronal suture

Abnormalities of head shape can be

positional(especially plagiocephaly) .
for example in child with rickets.

Treatment: keep child sleep on other

side and follow up.

Define shape ? posterior plagiocephaly

What is cause ? premature closure of Unilateral lambdoid suture
 Craniosynostosis & Hydrocephalus 3

Craniosynostosis
- Definition: Craniosynostosis is a condition in which one or more of the fibrous sutures in an
infant skull prematurely fuses by ossification.
- Primary craniosynostosis
o Due to Primary defect of ossification.
o Primary simple craniosynostosis
 One suture only fused prematurely
 Will have abnormal head shape only.
 Normal ICT (intra-cranial tension)
o Primary complex craniosynostosis
 Multiple sutures involved.
 Head circumference will be small.
 Brain will try to growth put will be resisted by skull, so intracranial tension (ICT)
will increase, and later on brain atrophy will result.
 Diagnosis:
 Brain CT (bone window) is the best.
 Skull X-ray : will shows silver- beaten appearance (indicate high ICP)
 complication: quadriplegic spastic blind CP (cerebral palsy)
 Treatment: suturectomy silver- beaten appearance
- Secondary craniosynostosis (more common)
o Due to brain atrophy
o Normal intra-cranial tension
o Diagnosis: brain CT
- Syndromic craniosynostosis
o Some syndrome associated with raised ICP,
and some not.
o Examples
 Crouzon syndrome (associated with high ICP).
 Apert Syndrome
 Down syndrome
- These syndrome may present with Kleeblattschädel
or cloverleaf skull

Kleeblattschädel (cloverleaf) skull (yellow raw) shows silver-beaten

appearance which represent printing of
sulci and gyri on skull due to raised ICP.
 Craniosynostosis & Hydrocephalus 4

Hydrocephalus
 Definition: state of widening of ventricular system due to CSF excess that result from imbalance
between production and absorption.
 CSF
o Normal CSF volume is 50 ml in infant, and 150 ml in adult.
o Rate of CSF production = 20 ml/h
o CSF produce from choroid plexus (mainly from lateral ventricle), from
lateral ventricle CSF cross Foramen of Monro to reach the third ventricle
then pass through Aqueduct of Sylvius to the fourth ventricle, then pass
Foramina of Magendie & Luschka to reach subarachnoid space .
o CSF absorbed from subarachnoid space and drain to superior sagittal
sinus. (this process is passive depend on pressure gradient)
o Any obstruction between ventricles and subarachnoid space is
obstructive hydrocephalus.
 Normal Intraventricular pressure = up to 120-180 mm H2O
 Classification of hydrocephalus:
o Obstructive hydrocephalus: results from obstruction of the flow of CSF (intraventricular
or extraventricular)
o Communicating hydrocephalus: occurs when full communication exists between the
ventricles and subarachnoid space.
 Causes of Obstructive (Non-communicating) hydrocephalus
o Congenital
 X-linked Aqueductal stenosis
 forking of Aqueduct of sylvius In Neurofibromatosis and tuberous
 Dandy- Walker Syndrome sclerosis, there will be hamartomas
 Arnold Chiari malformation change in brain that are like tumors
 Vein of Galen malformation obstruct CSF circulation.
o Acquired
 Meningitis (due to gliosis)
 Hemorrhage (due to gliosis)
 Tumors (especially Posterior fossa tumors)
 Systemic disease (eg. Neurofibromatosis, tuberous sclerosis, Achondroplasia )
 Causes of communicating (non-obstructive) hydrocephalus
o Increase secretion (rare) In Achondroplasia, there problem with growth
of longitudinal bone, where membranous
 Choroiditis
bone (as skull) is grow normally, so they have
 Choroid congestion
relative macrocephaly.
o Decrease absorption (more common)
 Subarachnoid hemorrhage Achondroplasia patients are prone to get
 Subarachnoid adhesions (Post Meningitis) hydrocephalus because they have small
 Sinus thrombosis foramen magnum.

Hemorrhage and Meningitis can cause both obstructive and communicating hydrocephalus.
 Craniosynostosis & Hydrocephalus 5

Q1/Child present with hydrocephalus, the lateral & third ventricles are enlarged, the fourth ventricle
is normal, where is the obstruction?
Aqueduct of Sylvius

Q2/Child present with hydrocephalus, the fourth ventricle is hugely enlarged in posterior fossa, what
is the diagnosis?
Dandy- Walker syndrome

Q3/ patient with hydrocephalus and examination shows scar on the back, what is the diagnosis?
Arnold chiari malformation

Clinical picture of hydrocephalus

- Presentation depends on sutures, is it open or closed.

 Infant Presentation (before closure of fontanels and sutures)
o Progressive increase in skull circumference
o Widened fontanels
o Wide separation of sutures
o transillumination
o Dilated tortuous scalp veins & stretched shiny scalp skin
o Sunset appearance (paralysis of upgaze ) due to impingement of the dilated
suprapineal recess on the tectum .
o skin will be pulled upward by increased skull circumference
o Positive Mc Ewen’s (cracked pot sign) on percussion of skull because of separation
of sutures. (put your hand on skull bone beside suture line, percuse skull by other
hand closely to first hand… if you feel like cracked pot, this is positive Mc Ewen’s)
o Craniotabes
o Examination of the back to R/O Arnold chiari malformation which associated with
meningomyelocele (which cause flaccid paralysis of lower limb)
o Motor & mental deficit, seizures may be associated (with prolong severe
untreated hydrocephalus) due to brain atrophy.
 Older children presentation (after closure of fontanels and sutures)
o Symptoms & Signs of increased intracanial pressure:
 Headache
 Irritability
 deterioration of scholastic performance
 vomiting
 Pyramidal signs
 abducent nerve palsy
 papilledema
 Child deterioration after shunt
o deterioration of school performance (is the earliest sign of shunt dysfunction)
o symptoms of elevated ICP
 Craniosynostosis & Hydrocephalus 6

Differential diagnosis

- causes of increase intracranial pressure (ICP)

o Space occupying lesion (brain tumors)
o CNS infection (meningitis)
o Primary complex Craniosynostosis
o Pseudo tumor cerebri
 Hypervitaminosis A
 Hypervitaminosis D
 Outdated tetracyclines
 Hypo-parathyroidism
 Idiopathic (seen in Adolescent obese females)
- Causes of increased skull circumference (Macrocephaly)
o Rickets
o Chronic hemolytic anemia
o Skeletal dysplasia (Achondroplasia)
o Chronic sub dural collection
o Hydranencephaly (vascular insult in-utero causing the brain tissue dies and the skull fills
with fluid and leave only a brain stem )
o Megalencephaly (enlargement of brain parenchyma)
 Familial (Autosomal Dominant )
 Anatomic Megalencephaly
o Sotos syndrome
o Tuberous sclerosis
 Metabolic / Neurodegenerative disorders:
o Tay Sachs
o Mucopolysaccharidosis
o MSUD (Maple syrup urine disease)
o MLD (Metachromatic leukodystrophy)
o Alexanders disease
o Canavan disease

Investigations:

- X-ray skull
- Brain imaging: U/S, CT, MRI
- TORCH screen
- Fundus examination
 Craniosynostosis & Hydrocephalus 7

Management

- Medical
o Acetazolamide ± Furosemide (used for preparation patient for surgery)
o Anti-epileptic drugs (if have seizure)
- Surgical
o V-P shunt
 Usually the shunt will be 30 cm longer, this because the child will grow.
 Well-function shunt will remain for 3 years, then it need to be replaced.
o The second shunt will replaced after age of 9 years.
 If shunt gets complication, it can be revised and replaced by new one.

Complication of shunt

1- Shunt infection (Bacterial colonization, staph. and strept .are most common organisms)
2- Kinking, obstruction or malfunction of shunt
o To test the shunt dysfunction, you have to compress over shunt tube on the neck
 If shunt fill with fluid (resist compression) this indicated obstruction in lower
part.
 If shunt compressed but not recoil (not back) this indicate obstruction in upper
part.