Treacher Collins Syndrome and Tracheostomy: Decannulation utilizing Mandibular Distraction Osteogenesis

Background: Research on mandibular distraction (MDO) as a treatment for Robin Sequence (RS) related airway obstruction commonly includes Treacher Collins (TC) within the RS study population. Although RS and TC both present with retrognathia, glossoptosis and airway obstruction, there a distinct anatomic differences between the RS and TC mandible which may affect surgical outcomes. We present out center’s clinical outcomes of TC patients treated with MDO in comparison to historic data on RS.

Methods: A single center, twenty-year retrospective review was conducted on all patients with TC treated with MDO. Recorded variables included: age of MDO, number of distraction procedures, type of device, presence of tracheostomy and complications. Literature review of clinical outcomes of MDO in the RS population demonstrated age of distraction under one year, average of one distraction per patient and avoidance of tracheostomy in over 90% of patients.

Results: 24 patients with TC who underwent MDO were included in our analysis. The follow up time was 9.2 years (range 1.7-17 years). The mean age of the first MDO was 4.97 years and the mean number of distractions was 1.42. Distraction devices were external in 67% and internal in 33%. 19 patients (79%) had a tracheostomy prior to MDO and only 9 (47%) patients were decannulated within one year of distraction. An additional 5 patients were decannulated several years later after further jaw reconstruction. Complication were divided into major, such as ankylosis, device failure and minor such as pin infection, hypertrophic scar. Ankylosis was noticed in 20% of patients without relationship to the vector of distraction or device type, one patient had parotid cutaneous fistula and 16% had device failure. 

Conclusions: Compared to the RS population, TC patients undergo MDO at a later age, require more distraction and have less successful decannulation. Further surgery is required to effectively treat airway obstruction. The incidence of major complications is higher. When assessing clinical outcomes in RS, TC should be assessed as a separate category due to the complexity of surgical care in this patient population.