Pediatric Craniosynostosis Treatment & Management

In the past 30 years, a better understanding of the pathophysiology and management of craniosynostosis has developed. Currently, surgery is usually cosmetic for infants with fusion of 1-2 sutures that result in a misshapen head. For infants with microcephaly (ie, secondary craniosynostosis), surgery usually is not required.

• In patients with microcephaly, investigate the cause of microcephaly.
• Measure head circumference longitudinally and monitor development. Ensure normal brain growth in patients with primary craniosynostosis.
• Carefully monitor signs and symptoms of elevated intracranial pressure.

  • Examine the fundi and alert parents to report persistent vomiting or lethargy promptly.
  • Should elevated intracranial pressure be suspected, an emergent neurosurgical consult would be appropriate.
• To preserve visual function in patients with elevated intracranial pressure, an emergent ophthalmological consult would be appropriate.
• Address the cosmetic appearance of the skull abnormality.

Surgery is typically indicated for increased intracranial pressure or for cosmetic reasons.

Surgery is usually performed in the first year of life.

• Do not operate in patients without raised intracranial pressure until considering the following:

  • If the shape of the head does not improve by age 2 months, then the abnormality is unlikely to resolve with age. Early referral is helpful if children can be considered for minimally invasive surgery. Infants have a large head relative to body size. Deformity appears more prominent in the young infant and may be less obvious with age.
  • Handle minor deformities conservatively. As the child grows and more hair appears, the visible abnormality may decrease.
• Consideration for cosmetic surgery is dependent on the age of presentation and on which sutures have fused prematurely.

  • Aesthetic surgery is performed in infants aged 3-6 months in the author's practice, although a variety of approaches exist among different surgeons.{{ Ref8}
  • Surgery in younger infants may result in a relatively large loss of blood volume. Accordingly, minimally invasive surgical techniques should be considered.
  • Surgery in infants older than 8 months may be associated with a slowing of skull growth. Thus, the surgical defect may not be covered adequately by bone growth.
• Infants with a defined syndrome causing craniosynostosis should be evaluated early for surgery.

  • Results are best when surgery is performed in infants younger than 6 months.
  • Patients with associated facial deformities may need a staged surgical approach (performed by a team of multidisciplinary specialists).
• Positional molding

  • No surgery is indicated for posterior plagiocephaly secondary to positional molding.
  • The vast majority of infants improve with repositioning maneuvers and physical therapy for torticollis.
  • Some clinicians elect to use molding helmets for severe cases.
• Cranial dysmorphology

  • Often patients present at a later age with nonspecific symptoms of increased intracranial pressure.^[8]
  • Endoscopic correction of sagittal craniosynostosis is well established; however, metopic repair is less well defined.^[9]

• A pediatric neurologist should evaluate the cause of the craniosynostosis and differentiate secondary from primary craniosynostosis. The head circumference should be plotted carefully and monitored from birth onward.
• A geneticist/dysmorphologist should evaluate for associated syndromes.
• A plastic surgeon should evaluate the infant when synostosis is associated with facial deformities.
• A neurosurgeon should evaluate a patient with primary craniosynostosis or elevated intracranial pressure.
• An endocrinologist should evaluate the patient when appropriate.

Following surgery, patients may require some restriction in activity under the direction of the treating neurosurgeon to avoid head injury.