Branchio-otic syndrome: An opportunity to reassess the paediatric anaesthetists’ approach to the difficult syndromic airway

Abstract

Branchio-oto-renal spectrum disorders are rare genetic entities with variable penetrance and concurrently display a wide phenotypic variation. A common issue to syndromic children is a propensity for difficult bag-mask ventilation, intubation or both. Unfortunately, there is no uniformity of this challenge, assessment strategy or management plan. This case with features
of branchio-otic syndrome provided the opportunity to examine several aspects of paediatric anaesthetic airway management. The child was booked for branchial cyst removal but appeared to have other features of abnormal branchial cleft development. An outline of the executed anaesthetic plan is presented and discussed. Three different techniques were sequentially tried in this patient before the airway was secured. Various case reports in the literature inconsistently describe easy to very difficult airway management in children with branchio-oto-renal spectrum disorders. Branchial arch dysgenesis is almost always associated with difficult direct laryngoscopy. There is undoubtedly no singular way to ideally manage a child with a difficult airway. Many tools for difficult airways are available. However, despite the improvement of difficult paediatric airway equipment, it would appear that for the anaesthetist the flexible bronchoscope remains an indispensable tool.