Indications
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The main indications for surgical intervention for craniosynostosis are the prevention of potential neurologic impairment and correction of deformity. Increased intracranial pressure, hydrocephalus, mental retardation, visual abnormalities, and learning disabilities all can be associated with craniosynostosis. Generally, the more sutures that are fused (as in the syndromic forms of craniosynostosis), the greater the likelihood of neurologic compromise. If there is any evidence of neurologic compromise, urgent surgical intervention should be performed.
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A secondary indication for surgical intervention is esthetic improvement of the skull shape, although for many patients this is the primary indication for treatment. Deformities of the skull as a result of craniosynostosis are best treated with cranial vault reshaping, preferably at an early age, before the calvarial bones have fully ossified.
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We prefer to perform open cranial vault reshaping on patients with multiple suture synostoses or severe single suture synostosis deformities, especially in older children The open approach of the affected skull via bicoronal incision provides the necessary exposure to address these deformities properly.
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We prefer to perform endoscopic cranial vault reshaping on patients with mild to moderate single suture synostoses before 4 to 6 months of age. The main benefits of this approach are diminished blood loss and limited incisions. Other benefits include decreased scarring and shorter hospital stays.
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We perform endoscopic repair for patients 3 to 6 months old with mild to moderate sagittal synostosis. We reserve the open approach for sagittal synostosis for patients with severe deformity.
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We perform endoscopic repair for patients 3 to 6 months old with mild metopic synostosis. We perform open cranial vault reshaping with frontoorbital advancement for patients with moderate to severe metopic synostosis.
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We perform endoscopic repair for patients 3 to 6 months old with mild unicoronal synostosis. We perform open cranial vault reshaping with frontoorbital advancement for patients with moderate to severe unicoronal synostosis.
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We perform open cranial vault reshaping for patients 3 to 6 months old with mild to moderate to severe lambdoid synostosis.
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We perform open cranial vault reshaping with or without frontoorbital advancement for patients with syndromic craniosynostosis or multiple suture–involved nonsyndromic craniosynostosis.
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Treatment of midface deformity and associated hypertelorbitism is beyond the scope of this chapter, but such treatments are critical aspects of care in most patients with syndromic craniosynostosis.
Contraindications
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Significant medical comorbidities that would preventing a safe anesthetic should be adequately dealt with before surgical intervention.
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No cranial vault reshaping should be performed if there is questionable soft tissue coverage present over the calvaria, or if there is active infection present in any of the layers of the scalp.
Planning and positioning
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Preoperative evaluation includes obtaining laboratory values (complete blood count) and crossmatching for 1 to 2 U of packed red blood cells. Three-dimensional reconstruction of a computed tomography (CT) scan of the head is obtained preoperatively in all patients. Blood products usually are not required for endoscopic patients; however, most patients undergoing open vault reshaping require transfusion of blood products.
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Patients are given preoperative antibiotics immediately before skin incision.
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For anterior skull exposure, the patient is positioned supine.
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For vertex and posterior skull exposure, the patient is positioned in the sphinx position.
