Airway Management in a Patient With Treacher Collins Syndrome Requiring Emergent Cesarean Section

Abstract

To the Editor: Treacher Collins syndrome also referred to as mandibulofacial dysostosis, is characterized by maxillary, zygomatic, and mandibular hypoplasia and known to be associated with difficult intubation. We report the airway management in a patient with Treacher Collins syndrome requiring emergent cesarean delivery owing to cord prolapse. The patient did not have either a preoperative assessment, or a clinical history in the hospital, and therefore no anesthetic strategy had been considered beforehand. Because there was no time to perform regional anesthesia because of the cord prolapse, the patient received general anesthesia. Taking into account that exclusive reliance on direct laryngoscopy was a high-risk strategy (mouth opening 2 cm; unable to protrude the lower incisors past the upper incisors; Mallampati class 4), we decided to insert a LMA-Fastrach™ (which was inserted on the first attempt) and the patient breathed spontaneously thereafter. No blind intubation through the LMA could be achieved because of resistance, despite of applying Chandy's maneuver (1). As backup in the event the LMA could not be satisfactorily inserted, or that ventilation could not be achieved, we were prepared to perform jet ventilation leaving emergent tracheostomy as the last option. Both weaning and LMA-Fastrach removal were uneventful. Craniofacial abnormalities often affect airway management making mask ventilation and tracheal intubation difficult or even impossible to achieve if there are also temporomandibular joint abnormalities. Airway morphology may change as patients mature or as a result of surgical interventions (2). The difficulty in intubation increases as the patient with Treacher Collins syndrome ages and requires reevaluation of the airway prior to each anesthetic (3). Different strategies including use of the classical laryngeal mask airway (LMA), [which is not always successful (4)] and the Bullard laryngoscope (5) have been described for airway management in patients with Treacher Collins syndrome. Ferson et al. (6) reported the use of LMA-Fastrach as a conduit for blind intubation in 254 patients with difficult-to-manage airways and showed a success rate for this device of 75.5% on the first attempt and 96.5% within five attempts, reaching the success rate 100% with the added use of a fiberoptic bronchoscope. However, in patients with distorted airway anatomy related to previous surgeries, Ferson et al. electively used a fiberoptic guided intubation (6), although it should be noted that none of these patients had Treacher Collins syndrome. A new device that has been successfully used to intubate the tracheas of patients with difficult airways (none with Treacher Collins syndrome) is the LMA-CTrach (7). Nevertheless, there is no published evidence that any of these new devices (including the LMA-Fastrach) are successful in adult patients with craniofacial abnormalities as Treacher Collins syndrome. In such patients, a previous preoperative assessment together with a complete clinical history (including records of previous surgeries or anesthetics) and even a reevaluation of the airway are advised to best plan anesthetic strategy and airway management. Paloma Morillas, MD Inocencia Fornet, MD, PhD Isabel De Miguel, MD Francisco J. Palacio, MD, PhD Marïa A. López, MD Department of Anesthesia and Intensive Care Medicine La Paz University Hospital Madrid, Spain [email protected]