- 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,
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.
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.
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.
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
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
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
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
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.
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