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Strabismus > Craniosynostosis 1 > Flashcards

Craniosynostosis 1 Flashcards

1
Q

Two parts of the skull

A

Neurocranium
Viscerocranium

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2
Q

Function of neurocranium

A

Forms the protective case around the brain
-the membranous part, which consists of flat bones that surround the brain
-the cartilaginous part which forms bones of the base of the skull.

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3
Q

Viscerocranium

A

forms the skeleton of the face

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4
Q

How does the skull develop

A

At birth the flat bones of the neurocranium are separated by narrow connective tissue layers known as sutures.
Where two or more bones meet, the sutures are wide and are termed fontanelles.
During the birth process, the sutures and fontanelles permit the bones of the skull to overlap and soon after birth the bones return to their original positions

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5
Q

Does the skull continue to grow

A

A large number of the sutures and fontanelles continue to stay membranous for different periods of time after birth, enabling the skull to continue growing.

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6
Q

What closes after 3 moths of birth

A

The posterior (occipital) fontanelle

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7
Q

What closes after 1.5 years of age

A

The anterior (frontal) fontanelle

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8
Q

What closes after 2 years of age

A

The postero-lateral (mastoid) fontanelle

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9
Q

At birth are flat bones separated by stutures

A

Yes these are fonatelles

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10
Q

What are fontanelles

A

Where flat bones meet and they allow skull & brain to grow

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11
Q

When do fontanelles close

A

Close between 3/12 – 2 years

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12
Q

Craniosynostosis

A

Craniosynostosis is a term given to a range of cranial abnormalities which are caused by premature closure of one or more sutures

It causes abnormally shaped skull and possibly raised intracranial pressure (ICP) with impairment of vision and cerebral functions.

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13
Q

Associated features of craniosynostosis

A

-Raised intracranial pressure (ICP)
-Impairment of cerebral function
-Impairment of vision

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14
Q

Craniosynostosis can cause

A

-Premature fusion of 1 or more sutures
-Inhibit growth of skull in that area
- Force growth in other non-fused areas e.g. abnormally shaped skull

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15
Q

Prevalence

A

1:2000-3000 infants are affected

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16
Q

Congenital v acquired craniosynostosis

A

Congenital (primary)
sporadic
genetically inherited – most commonly autosomal dominant
Recognised feature in over 100 genetic syndromes

Acquired (secondary)
metabolic disorders e.g hypercalcaemia
Mechanical cause e.g Intrauterine compression of skull against maternal pelvis

17
Q

Primary problem

A

the premature fusion is due to developmental error during embryogenesis

18
Q

Types of craniosynostosis

A

Sagittal- scaphocephaly
Metopic- trigonocephaly
Unicoronal- plagiocephaly
Bicoronal- brachycephaly

19
Q

Unicoronal synostosis- plagiocephaly

A

premature fusion of one of the coronal sutures.
greater development on the non-fused side and thus asymmetrical growth of the skull.

20
Q

Unicoronal synostosis- plagiocephaly causes

A

Also known as unicoronal synostosis

On the affected side the forehead is relatively flat and the orbit elevated.
On the non-affected side the forehead tends to be excessively prominent.

21
Q

Unicoronal synostosis- plagiocephaly sides

A

Affected side: forehead flat, orbit & superior orbital rim elevated
Unaffected side: prominent forehead

22
Q

Unilateral coronal synostosis- orthoptic findings

A

Orbits and trochlears are asymmetrical
can produce a mechanical weakness of the superior oblique muscles
vertical strabismus
inferior oblique over-actions
V-pattern
AHP common
amblyopia
astigmatism

23
Q

Unilateral coronal synostosis- treatment

A

Fronto-orbital advancement surgery (FOA)
performed at 4-12 months of age.
Aims to correct the existing deformity and minimise the developing facial asymmetry.
May need further surgery in teenage years
Long term follow up till skeletal maturity to monitor forehead and facial form.

24
Q

Fronto-orbital advancement surgery

A

make an incision over the top of child’s head from ear to ear.
cut through the coronal sutures to remove the front portion of the upper skull and re-shape it by cutting and trimming the bone to form a more normal shape.
the bone will be fixed in place using strong stitches that dissolve very gradually over the next few months while the bone heals.

25
Q

Bicoronal synostosis (Brachycephaly) causes

A

Premature fusion of both coronal sutures
Short anterior-posterior length
Increased lateral & vertical growth

26
Q

Bicoronal synostosis (Brachycephaly) further causes

A

Premature fusion of both coronal sutures
Short antero-posterior skull
Wide & tall skull
Flat & broad face and wide set eyes (hypertelorism).

27
Q

Bicoronal synostosis (Brachycephaly) further causes + ocular

A

50% learning disability
May be associated with Down’s syndrome
May have cleft palate, syndactyly, polydactyly
Proptosis
Hypertelorism – wide set eyes

28
Q

Bicoronal synostosis (Brachycephaly) surgery

A

Fronto-orbital advancement surgery (FOA) and it’s performed at 4-12 months of age

29
Q

Saggital synostosis causes

A

Premature closure of the sagittal suture
Prevents lateral expansion of the skull
Elongation along the anteroposterior axis
Premature fusion of sagittal sutures
Long and narrow skull

30
Q

Scaphocephaly (sagittal synostosis) results from

A

This condition results from premature closure of the sagittal suture, preventing lateral expansion of the skull. Elongation along the anteroposterior axis thus occurs resulting in the skull becomes long and narrow.

31
Q

Saggital synostosis features

A

Strong, prominent forehead
Prominent occipital area
Associated with
Speech & language delay
Raised intracranial pressure

32
Q

Saggital synostosis- surgical intervention

A

Vault expansion surgery:
Indication: raised ICP and improve appearance (most commonly before 10)

Aim: enlarge space within the skull to allow the growing brain develop and reduce ICP

Procedure: insert metal springs thatgradually widen a gap made between the skullbones, which encourage new bone to grow inbetween the two cut surfaces.

33
Q

Oxycephaly causes

A

Tall & narrow (dome-shaped) skull
High forehead
High, narrow palatal arch
↑ICP common- can cause seizures and brain damage
Premature fusion of coronal, sagittal & lambdoidal sutures

34
Q

Oxycephaly causes

A

Most severe form
premature closure of the coronal, saggital and lamboidal sutures
top of the skull is pointed or conical
Tall, narrow skull
High forehead

35
Q

Oxycephaly ocular features

A

-Optic atrophy
-Proptosis (shallow orbits)
-Strabismus
-Amblyopia
-Nystagmus
-Hypertelorism

36
Q

Metopic synostosis (Trigonocephaly) causes

A

Triangular shaped skull
Prominent central ridge down forehead
Orbits slope down & outwards
↑ICP
Mental retardation and developmental delay
Premature fusion of metopic sutures

37
Q

Metopic synostosis (Trigonocephaly) ocular features

A

Bilateral astigmatism
Strabismus
Alphabet pattern

Strabismus (46 decks)

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