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

  • Laura Indiveri University of the Witwatersrand
  • Ayesha Noor Mohamed University of the Witwatersrand
  • Analee Milner University of the Witwatersrand
Keywords: Branchio-otic syndrome, paediatric, difficult airway, flexible bronchoscopy

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.

Author Biographies

Laura Indiveri, University of the Witwatersrand

Department of Anaesthesiology, Faculty of Health Sciences, Charlotte Maxeke Johannesburg Academic Hospital,
University of the Witwatersrand, South Africa

Ayesha Noor Mohamed, University of the Witwatersrand

Department of Anaesthesiology, Faculty of Health Sciences, Charlotte Maxeke Johannesburg Academic Hospital,
University of the Witwatersrand, South Africa

Analee Milner, University of the Witwatersrand

Department of Anaesthesiology, Faculty of Health Sciences, Charlotte Maxeke Johannesburg Academic Hospital,
University of the Witwatersrand, South Africa

Published
2019-07-08
Section
Case Studies