Double‐lumen tube insertion and one‐lung ventilation in Coffin–Siris syndrome: navigating airway challenges in severe scoliosis surgery*

Summary

Coffin–Siris syndrome is a rare genetic condition associated with craniofacial and skeletal anomalies, hypotonia, airway malacia and intellectual disability, posing significant challenges for airway management and ventilation. We report the case of a 13‐year‐old patient with Coffin–Siris syndrome undergoing anterior scoliosis surgery requiring insertion of a double‐lumen tracheal tube and one‐lung ventilation. Pre‐operative assessment identified near‐complete C2‐3 fusion, severe kyphoscoliosis, pulmonary atelectasis and difficult intravenous access. Pre‐operative intranasal dexmedetomidine enabled a smooth inhalational induction with sevoflurane. Anaesthesia was maintained with total intravenous anaesthesia. A 28 Fr left‐sided double‐lumen tracheal tube was inserted on the first attempt using videolaryngoscopy. However, confirming correct tracheal tube position was challenging. Thick secretions and dynamic airway collapse from hypotonia, bronchomalacia, scoliosis‐related distortion and atelectasis limited visualisation. Once correct positioning was confirmed, one‐lung ventilation commenced. Initial high airway pressures improved with ventilatory adjustments, achieving adequate tidal volumes and oxygenation. Surgery was uneventful and the patient was transferred to a high‐dependency unit for observation. We believe this to be the first reported case of successful double‐lumen tracheal tube use and one‐lung ventilation in a patient with Coffin–Siris syndrome. The case highlights the importance of a structured peri‐operative approach to optimise safety in patients with rare syndromes where the evidence base is limited.