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Craniofacial

Cranial Sutures

Anatomy & Development

  • Metopic fuses by 8 months – 1 year 
  • Other sutures fuse in adulthood 

Craniosynostosis

  • Premature fusion of cranial sutures 

Types

Name 

Suture(s) Involved 

Features 

Brachycephaly 

Bicoronal 

Usually syndromic 

Trigonocephaly 

Metopic 

  • Bifrontal narrowing 
  • Metopic ridge 
  • Retrusion of superiorlateral orbital rims 
  • Hypotelorism 

Scaphocephaly 

Sagittal 

Most common craniosynostosis 

Posterior plagiocephaly 

Lambdoid 

Least common 

  • Unilateral posterior flattening 
  • Ipsilateral mastoid bossing  
  • Protuberance of the contralateral forehead (unlike plagiocephaly) 
  • Ear position no reliable 

Kleeblattschädel (“cloverleaf”) deformity

Coronal

Lambdoid

Sagittal 

Craniosynostosis of multiple sutures (coronal, lambdoid, portion of the sagittal suture) 

Syndromes with Craniosynostosis

Syndrome 

Genetics 

Mutation 

Craniosynostosis/Head Anomalies 

Limb Anomalies 

Apert 

AD 

FGFR2 

  • Bicoronal synostosis
  • Turribrachycephaly 
  • Hydrocephalus 
  • Cognitive delay 

Syndactyly hands/feet 

Can’t spread fingers APART 

Antley-Bixler 

AR

FGFR 

  • Craniosynostosis 
  • Choanal atresia  

Radiohumeral synostosis 

Crouzon

 

AD 

FGFR2 

  • Bicoronal craniosynostosis 
  • Brachycephaly 
  • Exorbitism 
  • Maxillary/midface hypoplasia 
  • Beaked nose 

Normal 

CAN pick up croutons (unlike Apert) 

Pfieffer

 

AD

FGFR2 

  • Turribrachycephaly 
  • Bicoronal craniosynostosis 
  • Exorbitism 
  • Down-slanting eyes 

Broad thumbs/toes 

Mild syndactyly 

Muenke 

AD

FGFR3 

3 little monkeys sitting in a tree” 

  • Coronal synostosis 
  • Increased intracranial pressure 
  • Sensorineural hearing loss 

“Monkey Ears” 

Abnormal middle phalanges 

Saethre-Chotzen 

AD

TWIST1 

“Tongue Twister!” 

  • Asymmetric brachycephaly 
  • Low frontal hairline  
  • Eyelid ptosis 
  • Maxillary hypoplasia 
  • Prominent ear crus 

Like Harry Potter Slitherin  

Syndactyly 

Work-up

CT head

Deformational Plagiocephaly

Presentation 

  • Head asymmetry 
    • Unilateral occipital flattening 
    • Ipsilateral frontal bossing 
    • Ipsilateral anterior shift of ear 

Treatment 

  • <8 months → Positional change 
  • >8 months → Helmet therapy 

Persistent Calvarial Defects

Treatment = Autologous, split calvarial bone graft (will grow with child) 

  • Other less desirable treatment options (expensive, become unstable with growth, risk of infection) 
    • Titanium mess  
    • Porous polyethylene 
    • PMMA (must saline irrigation to dissipate heat) 
     

Complications 

Contaminated Graft 

    • Bone  
      • Wash with triple antibiotic solution and use graft 
    • Non-Bone  
      • Chlorhexadine