A006 Anesthetic Challenges of an Infant with Apert Syndrome and Tetralogy of Fallot for Craniosynostosis Correction

Background: Apert syndrome (ApS) is characterized by craniosynostosis, craniofacial anomalies, and symmetrical syndactyly. Approximately 10% of Apert syndrome children can have associated congenital cardiac anomalies. It is rare to see ApS with tetralogy of Fallot (TOF). Craniosynostosis corrective surgery is associated with massive bleeding and venous air embolism. The presence of TOF increases the risks of perioperative morbidity and mortality.

Case Description: An 8-month-old infant (weight 6 kg), diagnosed with ApS and TOF was planned for a bifrontal craniotomy with frontal advancement and right frontal orbitotomy. As the child had difficult venous access, it was decided to perform gas induction. After standard monitoring, induction was performed with sevoflurane (4%). An intravenous (IV) line was secured quickly, anesthesia was deepened with fentanyl (1.5 µg/kg), ketamine (1.0 mg/kg), and propofol (1 mg/kg) and sevoflurane concentration was decreased (2%). Intubation was performed using a video laryngoscope. Arterial and the subclavian triple lumen was established. Scalp block, fentanyl, and morphine were given for analgesia. A loading dose of tranexamic acid (20 mg/kg), followed by an infusion (1 mg/kg/h) was given to reduce the bleeding. Scalp dissection and the strip cranioplasty resulted in 150 to 170 mL of blood loss, which was replaced with packed red blood cells (100 mL), fresh frozen plasma (50 mL), and cryoprecipitate (25 mL). A low dose of noradrenaline was started (0.02–0.05 µg/kg/min) to maintain the systemic vascular resistance (SVR). The child remained warm, hemodynamically stable, and the SpO₂ was varying between 85 and 93%. At the end of surgery, the child was extubated and shifted to the neurosurgical ICU. The main anesthetic challenges were the management of difficult airway, C-MAC video laryngoscope helped us to secure the airway. Maintaining the SVR using noradrenaline and avoiding factors which can increase the pulmonary vascular resistance prevented intraoperative “Tet spell.” Transfusion of blood and blood products helped maintaining the hemodynamic stability.

Conclusions: Meticulous planning and administration of titrated anesthetics to maintain both cardiovascular and the cerebrovascular homeostasis is paramount important for a successful outcome.