Craniosynostosis is the premature fusion of skull sutures, impairing normal brain and skull development. It can cause increased intracranial pressure and hydrocephalus. The most common types are sagittal and coronal synostosis. Diagnosis involves physical exam, imaging like CT. Treatment is early surgical release of fused sutures and reconstruction to allow brain growth, using techniques like barrel stave osteotomies or endoscopic suturectomies. Follow up addresses development, genetics counseling and specialty care for associated issues.

1. SURGICAL MANAGEMENT OF
CRANIOSYNOSTOSIS
PRESENTEDBY
DR ANKIT JAIN

2. • DEFINITION: Theprocess of premature closing of suturecausing
problems with normal brain and skulldevelopment.
• Craniosynostosis are frequently associated with impaired central
nervous system function due to
1) raised ICT,2)Hydrocephalus, 3) Brainanomalies.
• Incidence: 3.4 per 10,000 births
• Males – sagittal and metopic synostosis
• Female- coronal
• In general : MC-sagittal synostosis, 2nd MC-coronal

3. CRANIALANATOMY
• Normal infant skull isflexible.
• 4 Suture, 4 fontanalle.
Langman’sMedical Embryology

4. TIMING OF CLOSUREOF SUTURES AND
FONTANELLES
SUTURES AND FONTANELLES
1. Metopic suture
2. Coronal, saggital, lambdoid
3. Anterior fontanelle
4. Posterior fontanelle
5. Mastoid fontanelle
6. Sphenoid fontanelle
TIMING OFCLOSER
- 9 months-2 yrs
- 40 years
- 18 - 24 months
- 3 – 6 months
- 1 yr
- 2 – 3 months

5. ETIOLOGY:
• Exactetiology unknown
• Sporadic most cases
• Riskfactors-Advanced maternal age
Maternal smoking
Male sex
Fertility treatment.. Etc
Hypothesis – it suggest that abnormal development of the base of
the skull creates exaggerated forces on the dura that act to disrupt
normal suture development. ( Moss’stheory)

6. CLASSIFICATION OF CRANIOSYNOSTOSIS
Genetic classification
• Isolated- unknown, uterine constraint, FGFR3mutation.
• Syndromic cs-FGFR1-Pfeiffer syndrome
FGFR2-Apert’s syndrome, Crouzons syndrome
TWIST-Saethre chotzen syndrome.

7. PRIMARY VSSECONDARY
a. Primary defect of ossification
b. Head asymmetric
c. Brain continue to grow in
areaswhere sutures are open
d. Most individuals normal
neurologically
e. Surgical good prognosis
Ex:simple –coronal,sagittal..
compound- syndromic
• Primary • Secondary
a. Secondary to brain
malformation
b. Head symetric
c. Growth of brainimpaired
d. Neurologically abnormal
e. No benefit from surgery
Ex:malformation-microcephaly,
holoprocencephaly

8. TYPESOFCRANIOSYNOSTOSIS

10. Cephalic Index

11. A) sagittalsuture
• SCAPHOCEPHALY/ DOLICHOCEPHALY
- Most common type
- Features- broad forehead
prominent occiput
small/absent AF
biparietal narrowing
ridging of the sagittalsuture
- Sporadic –MCin male
- Not produce – raised ICT/hydrocephalus
- Labour- CPD

12. Saggital craniosynostasis
Clinical photograph-Lateral and Superior view of achild with sagittal
craniosynostosis demonstrating frontal and occipital bossing.

13. B) CORONALSUTURE
• ANTERIOR/ FRONTALPLAGIOCEPHALY
- 2NDMC (18%)
- Associated with Apertsyndrome
- U/L flattening: Plagiocephaly
- B/Lflattening: Brachycephaly.
- Mc-female

14. Unilateral coronal synostosis
• Prematurely fused one coronal suture,
• Flattening of the ipsilateral frontal and parietal bones
• Bulging of the contralateral frontal and parietal bones
• Bulging of the ipsilateral squamous portion of the temporal bone,
• Ipsilateral ear displaced anteriorly compared withthe
contralateral ear
• Radiographic findings include the “harlequin” orbitdeformity
(elevation of supra orbital margin )due to elevation of the greater
and lesser wings of thesphenoid

16. Bilateral coronal synostosis
1 Fused bilateral coronal suture.
2 Recessed superior orbital rim.
3 Prominent frontal bone.
4Flattening of occiput
5Anteriorly displaced skull vertex.
6 Shortened anterior cranialfossa.
7 Harlequin deformity of greater wing of sphenoid.
8 Protrusion of squamous portion oftemporal bone

17. C) LAMBDOID SUTURES
- Occipital plagiocephaly
- 10-20%ofcso
- M:f-4:1
- U/L- posterior plagiocephaly
- Right side mc
- Flat occiput
- Ipsilateral forehead bulge(rhomboid skull)
- B/L- posterior brachycephaly
Brachycephaly with b/l antero inferior displacedear.

19. D) METOPICSUTURE
- Trigonocephaly
- Incidence- 4-10%, M>F
- Ch19p abnormality
- Pointed fore head and midline ridge
hypotelorism
Ridging of metopic suture. 2, Temporal narrowing. 3, Patent
coronal suture displaced anteriorly. 4, Compensatory bulging of
the parieto-occipital region. 5, Narrowed bizygomatic
dimension. 6, Posterior displacement of the superolateral
orbital rim.

20. • D) MIXEDTYPE
1.TURRICEPHALY:coronal+sagittal
- Tower like tall pointedskull
- Leadsto acrocephaly/oxycephaly
- No room for braingrowth
- ICT–needs shuntinghydrocephalus

21. 2. CLOVERLEAF
-Multiple suture involved
-Also called Kleeblattschadel deformity.
-Three bulges-two temporal and top
-Pronounced constrictions in both sylvian
fissures

22. CRANIOSYNOSTOSIS SYNDROME
• Crouzon’ssyndrome
- Inherited asautosomal dominant trait
- Pattern- B/Lcloser of coronal
- Mutation of gene coding FGFR2,FGFR3
- C/F- Brachycephaly
significant hypertelorism, proptosis,
maxillary hypoplasia, beakednose

23. Apert’s syndrome
• Crouzon’s with Hand Involvement
• 1 in 100,000 to 160,000 live births, mutationFGFR2
• Varying intellect (50 %with MR)
• Cervical vertebral anomalies
• Syndactaly-2,3,4 finger
• multiple suture involve

24. Pfeiffer’s syndrome
•ADInheritance
•Clover leaf skull in 20%
•Intelligence is reported to benormal
•C/F: eyesprominent & wide spaced
broad thumbs & great toes and areshort.
•Mutation of gene coding for FGFR1,FGFR

25. Carpenter’s syndrome
•Autosomal recessive.
•Syndactyly of feet
•Intellectual disability iscommon
•Sagittal and lambdoid suture closesfirst
coronal last
•Cardiacabnormalities, corneal opacities.

26. Consequence ofcraniosynostosis
•Intracranial hypertension
Neurologic symptoms of elevatedICP
( Headaches,vomiting, sleep disturbances, feeding difficulties, behavioral changes,and diminished
cognitive function).
•Hydrocephalus- 4%to 18%.(Communicating)
multiple-suture craniosynostosis >>nonsyndromic single suture craniosynostosis
•Ophthalmologic Effects
Papilloedema, optic nerve atrophy, and even loss of vision may occur withprolonged,
untreated elevated ICP.

27. • Diagnosis
(A) Detailed history
• Birth history , sleepingposition.
• Headtilt , torticollis (deformationalplagiocephaly)
• Delayed developmental mile stone
• family history abnormal head shape or multiple systemicproblems
(eg,cardiac, genitourinary, musculoskeletal)

28. (B)Clinical Examination
• HC(micro/macrocephaly),
• Headshape (from above, side)
• Palpate suture lines & fontanelles (Look forridging)
• Earand facial symmetry, neck, spine, digits, and toes
• Look for associated anomalies (cvs,genitourinary, musculoskeletal)
• Fundus examination

29. (C) Radiological Evaluation
• Plain radiography-AP and lateral views of the skull -bony bridging
acrossthe suture ,sclerosis, straightening and narrowing of the suture
and loss of suture clarity
• CTscanHead-more accurate . structural abnormalities (e.g.,
hydrocephalus, agenesisof the corpuscallosum).
• 3DCTscanning accurately delineate the craniofacial deformity and
plan surgical reconstruction.

30. Treatment
• Primary objectives in nonsyndromic craniosynostosis are release of
the involved (fused) suture and reconstruction of all dysmorphic
skeletal components

31. Timing of surgery
Early operation(3-6 months) Better compliance of brain ,dura andscalp
Calvarium is much more malleable, easier to shape and providinga
better outcome
Rapid brain growth reshape the bone

32. Indications
• Correction of cosmetic abnormality
• Early treatment of intracranial hypertension
• Optimizing brain growth
• Severe proptosis and impending corneal damage
• of cosmetic abnormality

33. Basic Mechanisms
• Passive reshapement
1. Generous removal of bone
2. Strip craniectomy
3. Morcellation
• Active reshapement
1. Fronto orbital advancement
2. Cranial vault reshapement

34. ANTERIOR CRANIOSYNOSTOSIS

35. BARREL STAAVE OSTEOTOMY

36. Pi procedure

37. McComb’s approach for management of sagittal
synostosis in the older infant
Occipital reduction–biparietal widening”
Occipital protuberance is reduced
Biparietal diameter widened
height of the vertex is lowered

39. ENDOSCOPIC SUTURECTOMY

40. Late Intervention
Closer the cranium is to the adult size, the less
overcorrection for reconstruction and the better the
ultimate skull shape.
Higher risk of recurrent deformity
Surgical correction more complex

41. DISTRACTION DEVICES
Based on distraction osteogenesis
1mm/day upto 20-30 mm
Kept for 6-8 weeks
Ex-spring and cranial vault distractor( KLS Martin)

42. Conservative Therapy for Deformational
Plagiocephaly
Re-positioning
If no improvement by 6 months….
Helmet Molding

43. Long Term Follow-‐Up
Speech
Genetic Counseling
Feeding / Swallowing
Ophtha