What is the treatment for craniosynostosis?

Treatment of Craniosynostosis

Surgical intervention is the primary treatment for craniosynostosis, with the specific surgical approach determined by the type of craniosynostosis, age of the patient, and presence of associated complications such as increased intracranial pressure. 1, 2

Diagnosis and Initial Evaluation

• Clinical diagnosis is the first step, based on:

  • Abnormal skull shape
  • Ridging along suture lines
  • Fontanelle abnormalities
  • Signs of increased intracranial pressure 1

• First-line imaging for infants under 12 months:

  • Transcranial Ultrasound (TCUS) is recommended as the initial imaging modality
  • No radiation exposure
  • Can be performed at bedside for unstable infants
  • Cost-effective compared to CT/MRI 1

• Advanced imaging indications:

  • Surgical planning (CT examination)
  • Complex or syndromic craniosynostosis
  • Evaluation of intracranial complications
  • Children older than 12 months where TCUS is less reliable 1

Surgical Treatment Options

Timing of Surgery

• Early surgical intervention is crucial, typically performed before 12 months of age (median age 12.8 months in recent studies) 2
• Earlier intervention (before 6 months) may be necessary in cases with:
  • Multiple suture involvement
  • Signs of increased intracranial pressure
  • Syndromic craniosynostosis 3, 4

Surgical Techniques

The specific surgical approach depends on the affected suture(s) and severity:

1. Frontoorbital Advancement (40.5% of procedures)

   • Primary technique for metopic and coronal synostosis
   • Addresses anterior cranial vault and orbital deformities 2

2. Biparietal Remodeling (32.4% of procedures)

   • Often used for sagittal synostosis (scaphocephaly)
   • Focuses on reshaping the middle portion of the skull 2

3. Total Cranial Vault Remodeling (22.9% of procedures)

   • Used for complex or multiple suture involvement
   • More extensive reconstruction of the entire cranial vault 2

4. Posterior Procedures (4% of procedures)

   • Posterior distraction or expansion
   • May be used as initial procedure in cases of severe increased intracranial pressure
   • Can allow deferral of anterior procedures until 12 months of age 4

5. Endoscopic-assisted suturectomy

   • Less invasive option for younger infants (typically <6 months)
   • Requires postoperative helmet therapy 5

Special Considerations for Complex Cases

Syndromic Craniosynostosis

• Higher complication rates compared to isolated craniosynostosis:

  • Secondary procedures (36.8% vs 13.5%)
  • Perioperative complications (11.3% vs 5.0%)
  • Hydrocephalus (42.1% vs 22.4%)
  • Seizures (11.8% vs 2.9%) 3

• Often requires staged procedures:

  • Initial posterior skull release or decompression for increased intracranial pressure
  • Later fronto-orbital advancement (around 12 months)
  • Potential midface advancement procedures for midface hypoplasia (64.8% of syndromic cases) 3, 4

Monitoring and Follow-up

• Regular neurological assessment recommended yearly 6
• Complete evaluation with fundoscopy and brain/skull imaging for:
  • Skull morphology suggestive of craniosynostosis
  • Clinical symptoms of intracranial hypertension
  • Lower brainstem compression
  • Compression of upper cervical cord 6

Outcomes and Complications

• With proper multidisciplinary approach, approximately 73.7% of patients achieve satisfactory craniofacial form 3
• Potential complications include:
  • Recurrent craniosynostosis requiring reoperation (5% in one large series)
  • Persistent intracranial hypertension
  • Airway obstruction
  • Cranial vault maldevelopment 3

Early surgical intervention by an experienced multidisciplinary team (neurosurgery, plastic surgery, maxillofacial surgery) is essential to achieve optimal outcomes and minimize complications in the treatment of craniosynostosis 2, 7.