Cransiosysostosis

Cransiosysostosis

  • Premature fusion of one or more cranial sutures requires surgical treatment within the first 9-10 months of life.
  • Premedication: versed 0.5-1mg/kg in response to patient's need, clinical status, and parents' input.
  • Inhalational induction with nitrous oxide and sevoflurane
  • Peripheral IV
  • +/- muscle relaxant/propofol to facilitate intubation of the trachea.
  • In most cases, intracranial pressure is normal pre-operatively but may increase during surgery/general anesthesia. Mild to moderate hyperventilation (i.e., avoiding hypercarbia) is often induced.
  • The surgeon often sutures the endotracheal tube to the alveolar ridge to maintain a secure airway. Ensuring the appropriate depth of the endotracheal is critical, to account for flexion or extension of the head during the surgical repair. Check breath sounds in both fully flexed and extended positions before securing tube.
  • Consider transexamic acid (50 mcg/kg bolus over 15 min, followed by 5 mcg/kg/hr) infusion
  • Consider inserting a second peripheral intravenous catheter and/or an arterial catheter.
  • Ensure that blood has been crossmatched before incision, since blood loss is generally significant enough to require red blood cell transfusion. In rare cases, other blood components/factors may also be required to maintain hemostasis and hemodynamic stability.
  • Patients are at risk for venous air embolism due to the skull bone being diploic; watch for any sudden hemodynamic changes or decreases in end tidal CO2; if noticed alert surgeon immediately so that the field can be flooded with saline to impede continued embolism by eliminating exposure to ambient air.
  • Frequent monitoring of hematocrit and clotting function
  • Balance crystalloid replacement with colloid delivery
  • Awake extubation or post operative intubation/mechanical ventilator support
  • Post operative support/monitoring in the PICU
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This information is meant to serve as an educational resource. Clinicians should use their own professional judgment in the care of any individual patient as the guidance contained in this document may not be appropriate for all patients or all situations.