What is the treatment for Creniosynostosis (premature fusion of cranial sutures)?

Treatment for Craniosynostosis

Early surgical intervention is crucial in the treatment of craniosynostosis, with the goal of improving craniofacial form and reducing complications. 1

Diagnosis and Assessment

• Craniosynostosis diagnosis is primarily clinical, based on:

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

• Initial imaging recommendations:

  • Transcranial Ultrasound (TCUS) for infants under 12 months
  • CT or MRI for:
    • Inconclusive TCUS results
    • Surgical planning
    • Complex or syndromic craniosynostosis
    • Children older than 12 months
    • Evaluation of intracranial complications 1

Surgical Treatment

Timing of Surgery

Surgical intervention should be performed early, typically within the first year of life, for optimal outcomes. This is particularly important because:

• 17% of children with single-suture craniosynostosis have elevated intracranial pressure
• 38% have borderline intracranial pressure 2
• Early intervention can prevent developmental delay, facial abnormality, sensory dysfunction, and neurological complications 3

Surgical Approaches

1. Single-suture craniosynostosis:

   • Linear excision of the affected suture 1
   • Less invasive suturectomy-based techniques for younger infants 4

2. Multiple-suture or complex craniosynostosis:

   • More extensive cranial vault remodeling 1
   • Traditional open cranial vault remodeling for older children or complex cases 4

3. Asymmetrical deformities (predominantly unilateral):

   • Unilateral approach in infancy yields excellent results 5

4. Mild symmetrical deformities:

   • Bilateral orbital advancement in infancy gives satisfactory results 5

5. Severe symmetrical deformities:

   • Consider delaying major craniofacial surgery until age 7 or older
   • Earlier cranial surgery may still be advisable to address intracranial pressure 5

Multidisciplinary Management

A team approach is essential, involving:

• Neurosurgery
• Plastic/craniofacial surgery
• Ophthalmology
• Developmental pediatrics
• Genetics 1

This is particularly important for syndromic cases, which require more extensive evaluation and management.

Follow-up and Monitoring

Regular follow-up is necessary to assess:

• Cranial growth and development
• Neurological status
• Vision and hearing
• Developmental milestones 1

Special attention should be paid to monitoring for:

• Increased intracranial pressure (especially in multiple-suture or syndromic cases)
• Recurrent craniosynostosis
• Persistent intracranial hypertension
• Airway obstruction
• Cranial vault maldevelopment 1

Common Pitfalls and Caveats

1. Delayed diagnosis: Failure to recognize craniosynostosis early can lead to increased risk of intracranial hypertension and poorer surgical outcomes.

2. Underestimating intracranial pressure: Even single-suture craniosynostosis can be associated with elevated intracranial pressure, particularly with midline suture involvement (sagittal or metopic) 2.

3. Inappropriate surgical timing: While early intervention is generally preferred, the optimal timing may vary based on the type and severity of craniosynostosis 5.

4. Inadequate follow-up: Regular monitoring is essential to detect and address potential complications, especially in complex cases 1.

5. Radiation exposure: When CT is necessary for surgical planning, low-dose techniques should be employed to minimize radiation exposure risk, particularly in young infants 1.