• Marten N Basta, Pamela A Mudd, Stephanie M Fuller, Luv R Javia, and Jesse A Taylor.
• *Division of Plastic Surgery, The Perelman School of Medicine at the University of Pennsylvania and Children's Hospital of Philadelphia, Philadelphia, PA †Division of Otolaryngology, Children's National Health System and George Washington University Medical Center, Washington, DC ‡Division of Otolaryngology, Children's Hospital of Philadelphia §Division of Cardiothoracic Surgery, The Perelman School of Medicine at the University of Pennsylvania and Children's Hospital of Philadelphia, Philadelphia, PA.
• J Craniofac Surg. 2015 Nov 1; 26 (8): e788-91.

BackgroundLong-segment congenital tracheal stenosis (CTS) is characterized by segmental tracheal stenosis, complete tracheal rings, and absent posterior pars membranosa for >50% of tracheal length. Slide tracheoplasty on cardiopulmonary bypass (CPB) has traditionally been the procedure of choice for airway reconstruction. Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. The authors and others, have demonstrated the efficacy of mandibular distraction osteogenesis (MDO) to avoid tracheostomy in severe cases of PRS.MethodsThe authors present a unique case of the multidisciplinary management of long-segment CTS and concomitant PRS via total airway reconstruction off CPB, involving our otolaryngology, cardiothoracic, and plastic surgery teams.ResultsThis 36-week baby girl, prenatally diagnosed with PRS and polyhydramnios concerning for airway obstruction, was delivered via planned ex utero intrapartum treatment (EXIT). Tracheostomy was aborted because of long-segment CTS. A 2.5-French endotracheal tube (ETT) was temporarily sutured in before transfer to our facility for definitive airway management.Bilateral MDO was performed without complication at 2 weeks old (distraction to 20mm by postoperative day 25). At 6 weeks old, delayed slide tracheoplasty avoiding cardiopulmonary bypass was followed by an uneventful recovery. Most recent follow-up demonstrates airway patency without signs of obstruction.ConclusionsThis patient's case is the first reporting combined MDO and slide tracheoplasty to relieve multilevel neonatal airway obstruction. Unique and challenging, it demonstrates the importance of multidisciplinary management of complex neonatal airway obstruction.

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