Purpose: Patients with Treacher-Collins or Nager syndromes may present with mandibular hypoplasia causing posterior collapse of the tongue base and compromised oropharyngeal airway. Despite mandibular advancement with orthognathic procedure or distraction into proper occlusion, airway obstruction may persist. For this select population, we studied the outcome after genioplasty distraction and hyoid advancement performed to optimize epiglottal positioning.
Methods: Part I (Airway Outcome): Treacher-Collins or Nager Syndrome patients with obstructive sleep apnea or tracheostomy dependency underwent genioplasty distraction and hyoid advancement (n=8). Airway outcome was assessed by comparison of preoperative and 1 year follow-up: 1) direct laryngobronchoscopy, 2) sleep studies, and 3) tracheostomy dependence. Part II (Genioplasty Outcome): Three groups were studied: Group I (Distraction Genioplasty, Syndromic)(n=7), Group II (Conventional Genioplasty, Syndromic)(n=8), and Group III (Conventional Genioplasty, Non-syndromic)(n=10). Lateral cephalometric measurements were used to assess horizontal and vertical advancement and relapse.
Results: Part I (Airway Outcome): Epiglottal position was optimized by the procedure in all patients based on direct endoscopic assessment. All 5 patients with obstructive sleep apnea had resolution of symptoms and 2 of 3 patients achieved removal of their tracheostomy. For Part II (Genioplasty Outcome): Mean advancement for Groups I, II, and III was 25mm, 14mm, and 8mm, respectively. Follow-up horizontal advancement for Groups I, II, and III were 18mm, 4mm, and 6mm, respectively. Cephalometric measurements showed a horizontal relapse for Groups I, II, and III of 10%, 62%, and 11%, respectively with a similar relapse for the distraction syndromic and conventional nonsyndromic groups but a larger relapse for the conventional syndromic group.
Conclusions: Data suggests that 1) genioplasty distraction allows for a greater advancement and decreased relapse rate than acute procedures alone; and 2) genioplasty distraction with hyoid advancement is a useful technique for resolution of obstructive sleep apnea or to achieve tracheostomy removal in those syndromic patients who have already undergone mandibular advancement to achieve class I occlusion.