- Craniosynostosis is the premature fusion of calvarial sutures, occurring in 1 in 2500 births. It can be syndromic or non-syndromic. The most common types are sagittal, unilateral coronal, bilateral coronal, metopic, and lambdoid synostoses. - Diagnosis involves clinical examination, skull X-rays or CT scans to identify the fused suture. Early surgery (before 12 months) is usually recommended to reshape the skull and allow brain growth. Procedures depend on the suture involved and may require staged repairs for complex cases. - Long term follow up after surgery addresses development, genetics counseling, and monitoring of vision, speech,

1. CraniosynostosisCraniosynostosis
Dr Hemin M. Hama AmeenDr Hemin M. Hama Ameen
NeurosurgeonNeurosurgeon
25-1-201625-1-2016

2. Infant skullInfant skull
 Flexible; to get through the vagina during birth.Flexible; to get through the vagina during birth.
 Expansile; to accommodate rapid brain growth.Expansile; to accommodate rapid brain growth.
 Anterior fontanelle close by 2.5 years.Anterior fontanelle close by 2.5 years.
 Posterior fontanelle close by 2-3 months.Posterior fontanelle close by 2-3 months.
 Spheniod by 2-3 months and mastoid by 1 year.Spheniod by 2-3 months and mastoid by 1 year.
 90% of adult size achieved by age of 1 year, 96%90% of adult size achieved by age of 1 year, 96%
by the age of 6 years.by the age of 6 years.

3. AnatomyAnatomy

4. HistoryHistory
 In the late 1700s Sommerring noted that bone growth
in the skull occurred primarily at suture lines and that
when this growth site was prematurely bridged with
bone, an abnormal skull shape developed.
 Similar observations were made by Otto2 in 1830 and
Virchow3 in 1821.
 Moss noted that surgeons operating on the skulls of
children presumed to have craniosynostosis would
occasionally find patent cranial vault sutures, despite
what appeared to be typical craniosynostosis skull
deformities.

5.  The functional matrix theory, in which the
functional enlargement or development of an
organ system is the primary force in changing
its overall shape and determining its final form.

6. Suture growthSuture growth
 Suture grow perpendicular to suture lineSuture grow perpendicular to suture line

7. Sutrure closureSutrure closure
 Ossification; by age of 8 years.Ossification; by age of 8 years.
 Union; by the age of 20 years.Union; by the age of 20 years.
 Early closure cause bone growth parallel to theEarly closure cause bone growth parallel to the
suture.suture.

8. DefinitionDefinition
 Premature fusion of calvarial sutures.Premature fusion of calvarial sutures.
 1 in 2500 live births.1 in 2500 live births.
 Syndromic and non-syndromic.Syndromic and non-syndromic.
 Phenotypic and non-phenotypic.Phenotypic and non-phenotypic.
 Primary and secondary.Primary and secondary.
 Most common, sagittal, unilateral coronal,Most common, sagittal, unilateral coronal,
bilateral coronal, metopic and lambdoid.bilateral coronal, metopic and lambdoid.

10. Sagittal synostosisSagittal synostosis
“Boat-Head”
)Scaphocephaly,
dolichocephaly(

11. Coronal synostosisCoronal synostosis
“Bent-Head”
)Plagiocepahly if unilateral,
brachycephaly if bilateral(

12. Metopic synostosisMetopic synostosis
“Triangle-Head”
)Trigonocephaly(

13. Lambdoid synostosisLambdoid synostosis
Mattress
“Slant-Head”
)Occipital Plagiocephaly(

15. Primary craniosynostosis, suturePrimary craniosynostosis, suture
involvedinvolved
0%
10%
20%
30%
40%
50%
60%
1967 1982 1994
Sagittal
Coronal
Metopic
Lamboidal
Multiple

16. Clinical examinationClinical examination
 OFCOFC
 Head shape (from above, side)Head shape (from above, side)
 Ear and facial symmetryEar and facial symmetry
 Palpate suture lines & fontanellesPalpate suture lines & fontanelles
 Look for ridgingLook for ridging
 Look for associated anomaliesLook for associated anomalies
 Skull X-ray or CTSkull X-ray or CT

17. TypesTypes
 PrimaryPrimary
 Secondary:Secondary:
11..MicrocephalyMicrocephaly
22..PrematurityPrematurity
33..VP ShuntingVP Shunting
4.4. PositioningPositioning
 SyndromicSyndromic

18. PrematurityPrematurity
 Deformational ScaphocephalyDeformational Scaphocephaly
 Impaired mobility & prolonged positioningImpaired mobility & prolonged positioning
 Persists until adulthoodPersists until adulthood
 Prevention:Prevention:
 Donut-shaped head supportsDonut-shaped head supports
 waterbed mattresseswaterbed mattresses
 Does not warrant interventionDoes not warrant intervention

19. V-P shuntingV-P shunting
 ScaphocephalyScaphocephaly
 Chronic hydrocephalus thickens the skullChronic hydrocephalus thickens the skull
 Once decompression with shunt, the sutureOnce decompression with shunt, the suture
fusesfuses

20. MicrocephalyMicrocephaly
 Surgical correction not indicatedSurgical correction not indicated
 Abnormal OFCAbnormal OFC
 in primary craniosynostosis, OFC remains normalin primary craniosynostosis, OFC remains normal
yet oddly shapedyet oddly shaped
 Rare cases of multisutural craniosynostosisRare cases of multisutural craniosynostosis
restricting head growth, but manifests withrestricting head growth, but manifests with
increased ICPincreased ICP

21. Positional deformationPositional deformation
 Most common causeMost common cause
 Usually forehead asymmetryUsually forehead asymmetry
 Sometimes associated with torticollisSometimes associated with torticollis
 Usually acts on coronal or lamboidal sutureUsually acts on coronal or lamboidal suture
 40% of newborns40% of newborns

22. DifferencesDifferences
Deformational True
Incidence Common Rare
Occipital
Flattening
Yes Yes
Suture Open Closed/Ridged
Fronto-
parietal
Bossing
Ipsilateral Contralateral
Ipsilateral Ear Forward /
Down
Back / Down
Head Shape

23. SyndromicSyndromic
 10-20 % of cases10-20 % of cases
 Autosomal DominantAutosomal Dominant
 Linked to Chromosome 10qLinked to Chromosome 10q
 Multi-sutural, complex casesMulti-sutural, complex cases
If a suture is fused, check hands, feet,
big toe and thumb

25. Crouzon’sCrouzon’s
 Normal intellect
 Normal extremities
 5 % have acanthosis nigricans
 30 % have progressive hydrocephalus

26. Apert’sApert’s
“Crouzon’s with Hand Involvement“Crouzon’s with Hand Involvement””
• Varying intellect (50 % with MR)
• Mitten Glove Syndactyly
• Cervical vertebral anomalies
• Rare hydrocephalus

27. True Craniosynostosis/SurgeryTrue Craniosynostosis/Surgery
 Single Suture Synostosis: Confirm by exam andSingle Suture Synostosis: Confirm by exam and
skull x-raysskull x-rays
 Complex cases: CT or 3D CTComplex cases: CT or 3D CT
 X-Ray: Fused sutures have a broad ridge ofX-Ray: Fused sutures have a broad ridge of
overgrowth of solid bone along a previousovergrowth of solid bone along a previous
suture, or suture is completely obliteratedsuture, or suture is completely obliterated
 Ridge is especially characteristic of fused sagittalRidge is especially characteristic of fused sagittal
suturesuture

28. ManagementManagement
 Surgery VS conservative management.Surgery VS conservative management.

29. The Decision to OperateThe Decision to Operate
 Raised ICP in 1/3 of cases, but no neuroRaised ICP in 1/3 of cases, but no neuro
impairment. 8% Sagittal, 6% metopic and 12%impairment. 8% Sagittal, 6% metopic and 12%
in unilateral coronal.in unilateral coronal.
 Cosmetic considerations usually most importantCosmetic considerations usually most important
 affects peer acceptance, parent-child bonding, self-affects peer acceptance, parent-child bonding, self-
image and copingimage and coping

30. ImagingImaging
 Skull X-raySkull X-ray
 CT scanCT scan
 3D CT scan3D CT scan

31. SurgerySurgery
 If not part of syndrome, the earlier the operationIf not part of syndrome, the earlier the operation
the betterthe better
 At the latest 6-12 months (by 12 months, skull isAt the latest 6-12 months (by 12 months, skull is
90% of adult size)90% of adult size)
 For coronal suture, operate before 2 months becauseFor coronal suture, operate before 2 months because
of facial symmetry and visual system developmentof facial symmetry and visual system development
 Procedure depends on continuing skull growthProcedure depends on continuing skull growth
 Hospitalization for 3-10 daysHospitalization for 3-10 days

32. SurgerySurgery
 Unilateral coronal suture: difficult. OrbitalUnilateral coronal suture: difficult. Orbital
relocation as well.relocation as well.
 Syndromic or multi-suture cases: staged repairs.Syndromic or multi-suture cases: staged repairs.

41. Metopic synostosisMetopic synostosis

47. EndoscopyEndoscopy

48. Laser scan imageLaser scan image

52. Conservative Therapy forConservative Therapy for
Deformational PlagiocephalyDeformational Plagiocephaly
 Re-positioning
 If no improvement
 by 6 months….
 Helmet Molding

53. Custom Made for each head
24/7wear for 4 months

54. Ocular morbidityOcular morbidity
 Pre- and post-op impairments seen withPre- and post-op impairments seen with
unilateral coronal and metopic synostosesunilateral coronal and metopic synostoses
 dissociated movementsdissociated movements
 amblyopiaamblyopia
 refractive errorsrefractive errors
 Ophthalmological involvement in work-up andOphthalmological involvement in work-up and
follow-upfollow-up

55. Long term follow upLong term follow up
 SpeechSpeech
 Genetic CounselingGenetic Counseling
 Feeding / SwallowingFeeding / Swallowing
 OphthalmologyOphthalmology

56. ThanksThanks