Preparation for Surgery for Syndromic Craniosynostosis in Children

Indications for Surgery

• Signs of elevated ICP: The failure to promptly recognize elevated ICP and restricted brain growth due to suture fusion will lead to a poor outcome. It is important to make an early decision about the timing and extent of the first surgical intervention.
• Rapid abnormal growth of skull: In the presence of restricted skull growth, exposed orbits, or severe airway obstruction, surgery should be carried out as soon as possible.
• Age vs. clinical situation: When making a decision about early surgery, the clinical situation must be balanced against the increased risks associated with neonatal surgery. Multidisciplinary decisions must be taken on this aspect and have to be tailored to each patient. In the neonate, as long as the baby shows no signs of elevated ICP, airway obstruction, or proptosis, there is no rush for immediate surgery.
• Availability of craniofacial team: Because of the rarity and variable course of patients with syndromic craniosynostosis, they should be managed only by experienced multidisciplinary craniofacial teams, who have the expertise in the decision-making process for such children.

Preoperative Orders

• Assess for tolerance of surgery: Any child requiring cranial surgery for syndromic craniosynostosis is facing major surgery lasting at least 4–6 hours and significant blood loss. Preoperative assessment must therefore prepare them for this procedure by evaluating the child’s general health, especially nutritional, cardiac, and respiratory status.
• Cross-match for blood products: Blood and clotting factors must be available to allow for whole blood volume replacement if required.
• Risk of infection from airways: Preoperative nasal and throat swabs are taken. Prophylactic antibiotics are routinely given.

Anesthetic Considerations

• Airway anomalies: In children with abnormal airways or anomalies of the craniocervical junction, fiberoptic intubation and delayed extubation should be considered. Preoperative tracheostomy may be necessary.
• Blood loss: Depending on the preference of the team, the use of tranexamic acid and cell savers may be considered to reduce blood loss and transfusion requirements
• Positioning: The patient should be carefully positioned by both the surgeon and the anesthesiologist, who must take into account head position (patient may be prone or supine), airway compromise, pressure areas, and exposure keratitis. Suturing the eyelids closed may be required at the beginning of the procedure.

Devices to Be Implanted

• Bone fixation systems: For cranial reconstruction procedures bone fixation will be required and will vary with the individual team’s preference. Absorbable and nonabsorbable screws and plates may be used. No particular fixation has been shown to be superior, and it is individual surgical experience that is of most value in determining the choice of skull fixation.

Ancillary/Specialized Equipment

• Craniotome and drill: Standard craniotomy equipment is required for cranial reconstruction. Power drills for craniotomy and osteotomy are required, dependent on the surgeon’s preference.