Tailored Sedation for Oesophageal Dilatation in Goldenhar Syndrome: Avoiding Airway Instrumentation with High-flow Nasal Cannula (HFNC) Support

Goldenhar syndrome, or oculo-auriculo-vertebral spectrum, is a rare congenital anomaly involving craniofacial dysmorphia, vertebral deformity, and atypical outer ear, and is classically complicated by challenging airway management. In this case report, the anaesthetic management of a nine-year-old male with Goldenhar syndrome who also had a recurrent benign oesophageal stricture with odynophagia has been described. The patient also had many previous oesophageal dilatations and had generalised anatomical deformities such as asymmetrical facial deformity, mandibular hypoplasia, absent pinnae, scoliosis, and tracheal shift. Due to the presence of extremely high-risk airway complications, sedation was the procedure of choice without general anaesthesia. Intravenous sedation by ketamine, fentanyl, and midazolam was employed for the procedural sedation, and the maintenance of adequate ventilation as well as oxygenation during the procedure was with a High-Flow Nasal Cannula (HFNC). The anaesthetic team had a difficult airway cart and rescue airway devices on hand, even though invasive airway management was not employed. The procedure proceeded successfully with stable haemodynamics and satisfactory spontaneous ventilation. This case sets an example of the need for individualised anaesthetic planning, appropriate use of sedation, and the value of HFNC in the management of syndromic children with difficult airways.