Pediatric Craniosynostosis Treatment & Management

Updated: Dec 04, 2018
  • Author: Raj D Sheth, MD; Chief Editor: George I Jallo, MD  more...
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Treatment

Medical Care

In the past 30 years, a better understanding of the pathophysiology and management of craniosynostosis has developed. Currently, surgery is usually for cranial deformity correction for infants with fusion of 1-2 sutures that results 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 appearance of the skull abnormality.

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Surgical Care

Surgery is typically indicated for increased intracranial pressure or for correction of the cranial deformity.

Surgery is usually performed in the first year of life.

Sagittal synostosis before and after cranial vault Sagittal synostosis before and after cranial vault surgery and the associate improvement of scaphocephaly.

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 synostosis correction is dependent on the age of presentation and on which sutures have fused prematurely.

  • Surgery to correct the cranial or craniofacial deformity is performed in infants aged 3-6 months in the author's practice, although a variety of approaches exist among different surgeons. [10]

  • Surgery in younger infants may result in a relatively large loss of blood volume. Accordingly, minimally invasive surgical techniques should be considered. One promising area of investigation is the use of intraoperative tranexamic acid (TXA). A randomized double-blind study published in 2011 concluded that patients undergoing surgical correction of craniosynostosis treated with erythropoietin prior to surgery and TXA during the operation sustained lower volumes of blood loss and received less transfused blood at statistically significant rates than did the control patients. [11]

  • A prospective review of 89 children younger than 12 months with sagittal craniosynostosis treated with either endoscopically assisted or open repair found reduced operative times, shorter hospital stays, lower rates of blood transfusion, and excellent outcomes for the patients undergoing endoscopically assisted strip craniectomies followed by helmet therapy. Endoscopic procedures are typically reserved for patients younger than 6 months owing to the difficulties associated with the use of such techniques in older patients with thicker bone. [12]

  • Endoscopic metopic synostosis repair is less well defined, but some evidence in the literature suggests that minimally invasive endoscopy-assisted techniques and postoperative cranial molding may be used even in the treatment of infants with multiple-suture nonsyndromic craniosynostosis. [13]

  • 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). [14, 15, 16]

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. [10]

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Consultations

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.

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Activity

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

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