Decannulation and Airway Outcomes With Maxillomandibular Distraction in Treacher Collins and Nager Syndrome.

Background: Treacher Collins syndrome is a rare disorder (1/50,000 live births) with features that include hypoplastic orbitozygomatic complex with downward slanting eyes, and maxillary/mandibular retrusion. Obstructive sleep apnea and tracheostomy-dependence are common. This study presents the outcomes of skeletal distraction on avoidance of tracheostomy and decannulation in this patient population.
Methods: The authors reviewed charts of all patients with Treacher Collins syndrome who underwent craniofacial reconstruction from 2003 to 2016. Primary outcome measures included decannulation of tracheostomy dependent patients and avoidance of tracheostomy. Secondary outcome measures included cephalometric parameters, polysomnography scores, and airway exposure scores on direct laryngoscopy.
Results: Twenty-five patients underwent mandibular and maxillary advancement to resolve upper airway obstruction. Mandibular distraction was performed in 24 of 25 patients, and maxillary distraction in 14 of 25 patients. Maxillary distraction was combined with mandibular distraction in 13 of 17 to accomplish greater advancement and counter-clockwise rotation of the entire maxillary-mandibular complex. Six of 7 patients, 85.7%, avoided a tracheostomy and 39% (7 of 18) were decannulated. Cephalometric changes in sella-nasion-A point, sella-nasion-B , occlusal plane angle, and posterior airway space were equivalent between the groups who were able to clear their obstruction and those who were not.
Conclusions: Treacher Collins is a very challenging disease in which to resolve airway obstruction. Thus, thorough evaluation of the entire airway for all levels of obstruction is critical to successful outcomes. Future collaborative efforts between multiple institutions can help to increase our understanding and effective management of this rare disease.