Craniosynostosis

Question 1

Presentations of CRANIOSYNOSTOSIS

Answer 1

Restrict bone growth & elevated ICP —-> affect cognitive function and behavioural delays

Optic nerve compression —-> papilloedema, optic nerve atrophy, blindness

Shallow orbits ——> exorbitism, proptosis, dystopia, strabismus,

Hydrocephalus —-> d/t constriction of cranial base & diminished venous drainage

Severe midface hypoplasia ——> osa, malocclusion, dental crowding

Question 2

Difference between Apert and Crouzon syndromes

Answer 2

Apert affects bilateral coronal sutures. Associated with 
midface hypoplasia
cleft palate
syndactyly
thick facial skin
IQ often affected

Crouzon affects bilateral coronal sutures. WITHOUT other associated features.
IQ normal

Question 3

Sequence of surgical treatment for craniosynostosis

Answer 3

1. Increased ICP - early suturectomy & expansion of cranial vault
   (as early as rules of 10s for GA)
2. Craniectomy + anterior cranial vault reconstruction + fronto-orbital advancement
   (before 12months)
3. Repeat craniofacial expansion (prn)
4. Monobloc, Le Fort III procedures for the residual forehead retrusion, hypertelorism, midface deficiency (once orbitozygomatic development is completed, age 5-7)
5. Orthognathic surgery for final malocclusion (upon skeletal maturity)

Question 4

Common syndromic craniosynostosis

Answer 4

Crouzon
Apert
Pfeiffer - cloverleaf
Binder - cloverleaf skull without craniosynostosis