Genetics of Crouzon Syndrome Treatment & Management

Updated: May 06, 2015
  • Author: Harold Chen, MD, MS, FAAP, FACMG; Chief Editor: Maria Descartes, MD  more...
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Treatment

Medical Care

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  • A high prevalence of visual impairment in patients with craniosynostotic syndromes such as Crouzon syndrome was reported; almost half of the cases were due to potentially correctable causes, including amblyopia and ametropia. [16] Optic atrophy remains an important cause of visual impairment.
  • Early detection of eye problems to reduce amblyopia by correction of refractory errors and timely treatment of strabismus and patching is indicated. Optic atrophy remains an important cause of visual impairment before decompressive craniectomy. [17]
  • To relieve airway obstruction, a nasal continuous positive airway pressure device may be needed.
  • Close otologic and audiologic follow-up is indicated to detect sensorineural hearing loss.
  • Management of speech may be necessary.
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Surgical Care

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  • The goal is to stage reconstruction to coincide with facial growth patterns, visceral function, and psychosocial development.
  • Surgical treatment varies according to the variable expressivity of the disease and usually begins during a child’s first year with fronto-orbital advancement with cranial decompression. Subsequent development of midfacial hypoplasia needs correction. Procedures for this purpose include the Le Fort III osteotomy or its segmental variants, monobloc frontofacial advancement, or bipartition osteotomy. [18]
  • Early craniectomy with frontal bone advancement is most often indicated to prevent or treat increased intracranial pressure because newborns with Crouzon syndrome develop multiple suture synostoses and fused synchondroses.
  • Fronto-orbital and midfacial advancements help in the cosmetic reconstruction of facial dysmorphisms.
  • A new technique, craniofacial disjunction, followed by gradual bone distraction (Ilizarov procedure) has been reported to produce complete correction of exophthalmos and improvement in the functional and aesthetic aspects of the middle third of the face without the need for bone graft in patients aged 6-11 years.
  • Adult Crouzon syndrome, often presenting with marked midface hypoplasia and exorbitism, can be corrected by orbital decompression and zygomaticomaxillary advancement. [18]
  • The following treatments may be necessary:
    • Shunting procedures for hydrocephalus
    • Tracheostomy for airway compromise
    • Myringotomy to drain middle ear secretions secondary to distorted nasopharynx
    • Orthodontic management
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Consultations

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  • Neurosurgeon
  • Neuroradiologist
  • Plastic surgeon
  • Oromaxillofacial surgeon
  • Craniofacial anesthesiologist
  • Orthodontist
  • Dentist
  • Orthopedist
  • Ophthalmologist
  • Clinical geneticist
  • Speech, physical, and occupational therapists
  • Psychosocial team
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Diet

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  • No special diet is required.
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Activity

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  • Restriction of activity is not necessary.
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