Airway management in a patient with Cockayne syndrome

A 23‐year‐old man, 112 cm tall and weighing 33 kg, was listed for an elective Achilles tendon lengthening procedure under general anaesthesia. He was known to have Cockayne syndrome.

Cockayne syndrome is a rare, inherited multisystem condition presenting with short stature, visual and hearing impairment, learning disability [1], and potentially challenging airway anatomy [2, 3, 4]. Figure 1 demonstrates aspects of his airway anatomy including a short neck with limited extension, prominent dentition and a small mouth but with good mouth opening. On pre‐operative airway assessment, he was found to have a Mallampati grade of III.

Figure 1.

Airway anatomy typical of Cockayne syndrome, including a short neck, limited neck extension, prominent dentition and a small mouth.

General anaesthesia was induced with target‐controlled infusions of propofol and remifentanil following pre‐oxygenation. Our ‘plan A’ was to site a supraglottic airway. However, facemask ventilation with a size 2 oropharyngeal airway was challenging and the capnography trace was very limited, so we administered atracurium for muscle relaxation and moved to our ‘plan B’. Facemask ventilation was easier with the onset of the muscle relaxant, in combination with the use of a smaller oropharyngeal airway (size 1.5). We used a videolaryngoscope (Glidescope™, Verathon, Bothell, USA) with a hyperangulated blade (LoPro Size 3, Verathon, Bothell, USA) and obtained a full view of the glottis, through which we passed a paediatric bougie (Size 10Ch Portex Tracheal Tube Guide, Smiths Medical, Glasgow, UK). We attempted to railroad a 6.0 mm internal diameter cuffed tracheal tube (Portex, Smiths Medical, Glasgow, UK), but could not pass it distal to the cords, possibly due to subglottic stenosis. We then successfully intubated the trachea with a 5.0 mm internal diameter tracheal tube (MICROCUFF, Avanos Medical, Alpharetta, Georgia, USA). The rest of the anaesthetic and operation were uncomplicated. Following emergence from general anaesthesia, the patient's trachea was extubated uneventfully, and he was discharged home later the same day.

Much of the existing literature relating to airway management in Cockayne syndrome is from before the era of videolaryngoscopy [2, 3, 4]. This case highlights the potential airway management challenges in the context of contemporary anaesthetic techniques. Based on our experience, videolaryngoscopy is beneficial, but the likelihood of subglottic stenosis, which was also observed by other authors [2, 3], means that having access to a variety of smaller internal diameter endotracheal tubes is essential. On reflection, rocuronium may have been a better choice of neuromuscular blocking agent than atracurium in this case, given the ability to rapidly reverse paralysis if required.