Question

A 12-month-old, 7.0 kg infant with Apert syndrome, bilateral coronal and lambdoidal craniosynostosis is scheduled for a total calvarial vault reconstruction.
Enumerate the perioperative concerns for this patient.

Hint:

Effective management of airway, hemodynamics, and neurological status are key to ensuring a successful perioperative outcome for infants with Apert syndrome.

Answer 1

A 12-month-old infant with Apert syndrome and craniosynostosis requires special perioperative considerations. Apert syndrome is a rare genetic condition characterized by craniosynostosis, syndactyly, and other developmental anomalies. The following perioperative concerns should be addressed:
Step 1: Airway Management:
1. Potential for Difficult Intubation: Due to craniosynostosis and abnormal head shape, airway management may be challenging. A difficult airway cart should be readily available.
2. Risk of Obstructive Apnea: These patients may have associated airway anomalies, including upper airway obstruction, which requires careful monitoring during anesthesia.
Step 2: Hemodynamic Considerations:
1. Blood Loss: Surgical correction of craniosynostosis may involve significant blood loss, particularly in infants. Preoperative assessment should include a baseline hemoglobin level and strategies for blood conservation.
2. Monitoring Blood Pressure: Maintaining stable blood pressure during surgery is crucial to avoid ischemic damage to the brain, especially considering the patient’s young age.
Step 3: Neurological Concerns:
1. Risk of Increased Intracranial Pressure (ICP): The cranial vault reconstruction surgery may elevate ICP. Close monitoring with a team experienced in managing high ICP is essential.
2. Seizure Risk: There may be an increased risk of seizures due to brain malformation, requiring appropriate prophylaxis and monitoring.
Step 4: Fluid and Electrolyte Management:
1. Dehydration Risk: Ensure adequate hydration during surgery, as infants are more susceptible to fluid imbalances.
2. Electrolyte Imbalances: Frequent monitoring of electrolytes should be performed to prevent disturbances during the procedure.
Step 5: Postoperative Care:
1. Pain Management: Postoperative pain should be effectively managed with age-appropriate analgesia, considering both narcotics and non-narcotic options.
2. Postoperative Monitoring: Due to the potential for complications like bleeding or respiratory distress, close monitoring in an intensive care unit (ICU) postoperatively is essential.