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Case report highlights anaesthetic challenges and solutions for airway management in CHARGE syndrome children.
CHARGE syndrome is a rare, complex congenital disorder caused predominantly by mutations in the CHD7 gene, characterized by a multisystem involvement including Coloboma, Heart defects, Atresia of the choanae, Retardation of growth and development, genital anomalies, and ear abnormalities. Each patient presents a unique spectrum of anomalies, posing significant challenges for perioperative management. This case report presents the perioperative anaesthetic management of a 5 year old male child with genetically confirmed CHARGE syndrome (CHD7 mutation: c.6213delA) scheduled for full mouth rehabilitation under general anaesthesia. The child presented with multiple classical features including bilateral coloboma, atrial septal defect (ASD), bilateral sensorineural hearing loss, retrognathia, and global developmental delay. Given the known association with choanal atresia, nasal patency was assessed preoperatively. Airway examination revealed retrognathia and high arched palate, raising concerns for difficult intubation. A multidisciplinary strategy was planned. Inhalational induction with sevoflurane was used to preserve spontaneous ventilation, followed by intravenous propofol and atracurium to ensure smooth intubation while maintaining hemodynamic stability. Anticipating airway difficulty, nasal intubation was performed using a C-MAC video laryngoscope. Intraoperative management emphasized normothermia and normocapnia to ensure cerebral and hemodynamic stability. The surgery proceeded uneventfully, and the child recovered without complications. This case highlights the importance of a tailored, pathophysiology-driven anaesthetic plan and interdepartmental coordination in managing syndromic children. Key takeaways include early identification of airway and systemic risks, detailed preoperative planning, and the use of advanced airway tools to ensure safe outcomes in pediatric patients with complex congenital anomalies.
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NV et al. (2025) studied this question.
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