Anesthesia for Treacher Collins syndrome: a review of airway management in 240 pediatric cases

OBJECTIVES: To review airway management with anesthesia for children with Treacher Collins syndrome (TCS) and determine whether intubation was more difficult with increasing age. BACKGROUND: Treacher Collins syndrome is a rare disorder of craniofacial development characterized by maxillary, zygomatic, and mandibular dysplasia. TCS is associated with difficult intubation, but reports of airway management are limited to case reports and small cases series. Children with TCS may require multiple general anesthetics, and it has been suggested that intubation becomes more difficult with increasing age. METHODS: A retrospective case note review of children with TCS from birth to 18 years undergoing anesthesia from 1971 to 2011 in a single center was performed. Demographic data, procedure type, anesthesia type, method of airway management, modified Cormack-Lehane (MCL) grade of laryngoscopic view, and any other descriptions of airway difficulty or complications were collated. RESULTS: Of 59 patients with TCS, 35 children underwent a total of 240 anesthetics, most commonly for craniofacial surgery. Final airway management consisted of face mask 17%, laryngeal mask airway 16%, endotracheal intubation 49%, and 18% had a preexisting tracheostomy. The laryngeal mask airway provided an adequate airway in all cases when it was used. MCL grade was recorded in 97 cases involving 28 patients: 7% grade 1, 9% grade 2a, 31% grade 2b, 26% grade 3, and 27% grade 4. Fifteen (54%) patients were MCL grade 4 on at least one occasion. Failed intubation occurred in 6 (5%) of 123 cases of planned intubation. The procedure was canceled in two cases (0.8%) because of failure to intubate. Intubation techniques other than conventional direct laryngoscopy were used in 41% of cases. MCL grade increased with increasing age (P = 0.007). CONCLUSIONS: Most children with TCS have difficult laryngoscopic views with many requiring specialized intubation techniques. Direct laryngoscopy becomes more difficult with increasing age. The laryngeal mask airway is a good choice of airway when endotracheal intubation is not required.