Treacher Collins Syndrome and Tracheostomy: Decannulation Using Mandibular Distraction Osteogenesis. Issue 3 (September 2018)

• Record Type: Journal Article
• Authors: Ali-Khan, Safi; Runyan, Christopher; Nardini, Gil; Shetye, Pradip; Staffenberg, David; McCarthy, Joseph G.; Flores, Roberto L.
• Is Part Of: Annals of plastic surgery. Volume 81:Issue 3(2018)
• Publication Date: 2018-09
• Subjects: Treacher Collins syndrome -- Pierre Robin sequence -- neonatal distraction -- mandibular distraction osteogenesis -- distraction -- micrognathia -- tracheostomy -- decannulation -- airway outcomes -- airway obstruction -- nanognathia -- glossoptosis -- syndromic micrognathia -- Nager syndrome; Surgery, Plastic -- Periodicals; 617.95205
• DOI: 10.1097/SAP.0000000000001514
• Languages: English
• ISSNs: 0148-7043

Abstract

Abstract : Introduction: Treacher Collins syndrome (TC) and Pierre Robin sequence (RS) are associated with hypoplastic mandible, glossoptosis, and consequent airway obstruction. Although TC and RS are often grouped together, airway outcomes of bilateral mandibular distraction osteogenesis (MDO) have not been specifically studied in TC. The purpose of this study is to report on the clinical outcomes of MDO in the TC patient population. Materials and Methods: A twenty-year single-institution retrospective review of all patients with TC who underwent bilateral MDO was performed. Twenty-four patients were identified after exclusion due to different diagnoses or insufficient medical records. Data on comorbidities, airway status, MDO operations, and complications were collected. Data were compared with published clinical outcomes in RS and data for 13 RS patients from our institution. Results: Surgical success, defined as prevention of imminent tracheostomy or successful decannulation within 1 year after primary distraction, was observed in 21% of TC patients and 65% of RS patients ( P = 0.01). Repeat distraction was necessary for 11 TC patients (46%) and 1 RS patient. Complications were divided into minor, moderate, and major based on need for invasive management. Overall, 67% of TC patients had complications, 20% of which were major. Conclusions: Clinical outcomes to airway function after MDO are significantly inferior in patients with TC compared with patients with RS. Repeat MDO and longer course to decannulation are more prevalent in patients affected by TC.