SP27. Mandibular Distraction in Patients with Pierre Robin Sequence: A Multi-surgeon Experience

Abstract

PURPOSE: Mandibular distraction osteogenesis (MDO) is rapidly becoming a standard of care for management of patients with severe Pierre Robin Sequence. The tongue is brought forward to alleviate the airway obstruction. This study will look at an institutional, multi-surgeon experience with MDO over ten years. METHODS: This study was conducted as a retrospective chart review including all patients who underwent MDO at the authors’ institution from 2012 to 2022. Demographics, preoperative and postoperative respiratory and feeding status, and distraction data were collected. Primary outcomes were achievement of full oral feeds, avoidance of a gastrostomy tube (GT), avoidance of a tracheostomy, discharge from hospital on room air and complications. A significance value of 0.05 was utilized. RESULTS: Twenty-eight patients met inclusion criteria. Three craniofacial surgeons performed all interventions. The average age at MDO was 135 days, mean activation phase was 13.6 days, mean distraction length was 14.9 mm and mean consolidation phase was 64.2 days. MDO showed a significant improvement in apnea-hypopnea index (AHI) (p < 0.001) and %O2 Nadir (p < 0.001) on postoperative polysomnography. Syndromic patients were associated with discharge with a GT and negatively associated with avoidance of tracheostomy. Patients with preoperatively diagnosed tracheomalacia and subglottic stenosis were negatively associated with avoidance of a tracheostomy. Preoperative diagnosis of gastroesophageal reflux disease (GERD) was negatively associated with extubation immediately to room air (RA) following index procedure. A shorter duration of intubation following index procedure was associated with discharge on RA and a longer duration of intubation was associated with unilateral pan-facial nerve palsy. A longer activation phase was associated with discharge with a GT and a shorter activation phase was associated with discharge on full oral feeds. The ability to discharge on RA was associated with a shorter latency phase, shorter activation phase, and decreased distance of distraction. Repeat MDO was associated with postoperative infection requiring operative management. CONCLUSIONS: The goal of MDO is to achieve full oral feeds with no respiratory support. Several interesting findings are described in this study. When assessing patients preoperatively it is important to note the higher rate of failure to avoid a tracheostomy in patients with a syndrome and those with tracheomalacia and subglottic stenosis noted on airway evaluation. Syndromic patients were also noted to be more likely to be discharged with a GT. Several different latency periods were used in this study, and it appears that not only is a short latency period safe, but that it may be correlated with successful discharge on RA. This should be tempered with the finding that a decreased time spent intubated following surgery, decreased distance distracted and time spent in activation was also associated with discharge on RA, which may be a function of the severity of micrognathia in these patients. Of note, patients that spent a longer period intubated following surgery experienced a higher rate of facial nerve palsy. The reason for this correlation is unclear but may represent more severe cases that resulted in more forced retraction during surgery.