Trends in Natural Decannulation in Patients with Robin Sequence: A Twenty-five Year Retrospective Review

Abstract

Objective: Robin sequence (RS) consists of micrognathia and glossoptosis that result in upper airway obstruction (UAO). In RS patients who undergo tracheostomy, long-term goals include natural decannulation (ND) without further surgical airway intervention. The objective of this study was to identify long-term trends in the rate and length of time to ND. Methods: Retrospective chart review on 144 patients with RS treated from 1995 to 2020 at a pediatric tertiary care center. Patients were grouped by year of tracheostomy. Demographic data, UAO management, postoperative care, complications, and time to decannulation were recorded. Results: Thirty-six patients met the inclusion criteria. Tracheostomy was performed at a median age of 45.5 days. 19 (53%) patients experienced ND at a median time of 66.1 months. ND rate was higher in non-syndromic patients (93% non-syndromic vs 27% syndromic; P < .0001) and during the first study period (1995-2006: 78%, 2007-2020: 28%; P = .003). Cox proportional-hazard regression demonstrated that white race [aHR 0.15 (0.03-0.8); P = .023] and higher birthweight [aHR 0.9 (0.8-0.98); P = .018] were associated with a higher likelihood of ND while a syndromic diagnosis had a negative association with ND [aHR 12.5 (3.3-50.0); P < .001]. Conclusions: Our study documented that ND in patients with RS who underwent tracheostomy was significantly associated with ethnicity, birthweight, and syndromic status. The negative impact on successful ND was most observed in patients with syndromic associations. Additionally, ND rates are lower in the 2007 to 2020 subgroup. We suspect this is because alternative management techniques such as tongue lip adhesion and mandibular distraction osteogenesis became primary surgical treatment in severe RS upper airway obstruction at our institution starting in 2007.