Canadian Journal of Anesthesia, Vol 33, 364-370, Copyright © 1986 by Canadian Anesthesiologists' Society

Clinical Reports: Anaesthesia for Treacher Collins and Pierre Robin Syndromes: A Report of three Cases

¹ Departments of Anesthesiology, Pediatrics and Surgery, Medical Center Hospital and The University of Texas Health Science Center at San Antonio, Texas

Address correspondence to: Dr. Deborah K. Rasch, Department of Anesthesiology, The University of Texas Health Science Center, 7703 Floyd Curl Drive, San Antonio, Texas 78284.

We present three patients with Treacher Collins or Pierre Robin syndromes who had historical and physical evidence of airway obstruction, difficulty feeding, and sleep disturbances. These preoperative findings correlated with difficult airway management intraoperatively. Based on this experience, we recommend that children with obstructive symptoms have laryngoscopy prior to anaesthetic induction. If the glottic opening is visualized, inhalational induction can proceed. If the glottic structures cannot be visualized, then the anaesthetist must choose between awake oral or nasal intubation, elective tracheostomy, or fiberoptic intubation. In all cases, a tracheostomy tray should be ready and a surgeon experienced in paediatric tracheostomy should be in attendance. After intubation, anaesthesia is best maintained with oxygen and a potent inhalational agent. Extubation should only be done with the patient fully awake and with emergency airway equipment immediately available. Postoperatively, these patients should be transferred to an intermediate care area or intensive care unit where they can be observed closely since delayed complications of airway obstruction are common in this group of patients.

Key Words: GENETIC FACTORS: Treacher Collins syndrome, Pierre Robin anomaly, mandibulofacial dysostosis • COMPLICATIONS: cor pulmonale, airway obstruction