ANESTHETIC MANAGEMENT FOR A DIFFICULT AIRWAY IN A CASE OF TEMPLE BARAITSER SYNDROME
AUTHORS: A. MARTÍN, E. FERNÁNDEZ, F. REINOSO, A. DÍAZ, J. YAGÚE.
BACKGROUND: Anaesthesiologists are trained to solve complicated situations amongst which are the intubation of patients with difficult airways. In paediatric patients these situations can also occur. Temple Baraitser syndrome is a rare syndrome with a prevalence of less than 1/1000000. 
CASE REPORT
A four-year-old infant (20kg) with Temple Baraitser and Lennox syndrome, with chronic respiratory failure that requires nightly BIPAP, presented a huge superior and inferior gingival hyperplasia that was obstructing breathing.
Palliative surgery to reduce lower gingival hyperplasia is performed.
Preoperative assessment was carried out thoroughly with routine investigations. Mallampati classification was not possible. In the oral cavity, gingival hyperplasia, haphazardly arranged teeth, reduced tongue mobility and an open mouth at rest were seen. High difficulty is anticipated when ventilating and intubating.
A slow inhalational induction, with gradually increasing sevofluorane (2 to 6%) was performed and 100% oxygen, while maintaining spontaneous breathing. There was good ventilation and intravenous access was established.
Intravenous fentanyl (1microg/kg) and propofol (1mg/kg) were administered. Intubation was tried two times with direct laringoscopy, The view was Cormack and Lehane grade IV. A second experienced anaesthetist was called. We finally performed a fibre-optic orotracheal intubation and a size 6.0 endotraqueal tube was inserted.
Surgery went on without complication and the patient was extubated uneventfully when spontaneous ventilation was recovered. After surgery upper airway obstruction improved significantly. However, high-flow nasal cannula (HFNC) was need during three days to maintain oxygen saturations above 90%.
DISCUSSION: Paediatric airway deserves a special section among the difficult airway algorithms, the most recent guidelines already have a separate section on this issue. Fibre-optic intubation remains the “gold standard” for accomplishing tracheal intubation in a difficult paediatric airway. Not only intubation, but also extubation is a dangerous moment, as a laryngospasm on a paediatric patient with a difficult airway might be a risky situation.
LEARNING POINTS: It is important for the anaesthesiologist to know the management of a difficult airway in paediatric and adult patients and the differences between both cases.
REFERENCES
https://www.das.uk.com/guidelines/paediatric-difficult-airway-guidelines
Paediatric difficult airway management: what every anaesthetist should know! Br. J. Anaesth. April 19, 2016


09 / ANESTHETIC MANAGEMENT FOR A DIFFICULT AIRWAY IN A CASE OF TEMPLE BARAITSER SYNDROME