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

Year: 2026
Type: article
Study design: Case Report
Abstract: 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.
DOI: 10.1002/anr3.70117
Language: English
Field: Medicine
Open Access status: bronze
OpenAlex ID: W7225174451
FWCI:
Cites: 5
Cited by:
Related to: 10