Mandibular distraction in infants with Pierre Robin sequence improves laryngeal exposure and simplifies airway management for subsequent operations
Sara A. Neimanis, MD, Dahlia Rice, MD, John Faria, MD, Matthew Hirschfeld, MD, Clinton S. Morrison, MD.
University of Rochester, Rochester, NY, USA.
BACKGROUND: Pierre Robin Sequence (PRS) is a congenital condition consisting of retrognathia and glossoptosis causing airway obstruction. The primary treatment goal is airway protection. At our institution mandibular distraction osteogenesis (MDO) is the treatment of choice when non-operative management fails. These patients ultimately undergo three surgical interventions in the first year of life: mandibular distractor placement, distractor removal, and palatoplasty. Many of these children are labeled as “difficult airways” based on diagnosis and we sought to determine if their airway management simplifies with subsequent procedures. METHODS: This is a retrospective review of consecutive patients who underwent MDO for PRS at our institution from 2012-2017. Cormack-Lehane airway grade (1-4), difficulty with intubation, time to extubation, and intra-operative and post-operative airway complications were assessed at each of the three operations: distractor placement, distractor removal, and cleft palate repair. Data was analyzed using Chi-square tests for categorical variables and paired t-tests for continuous variables and was compared at these time points. Statistical significance was considered p<0.05. RESULTS: Sixteen patients were identified. Each underwent distractor placement (average age 4.3 weeks), distractor removal (16.8 weeks), and palatoplasty (46.6 weeks). Four patients had syndromic PRS. None had tracheostomies. Two were intubated prior to distraction for acute airway compromise. All underwent video laryngoscopy and rigid bronchoscopy by an otolaryngologist at the time of distractor placement. After distractor placement, the average time to extubation was 6.3 days. All patients were extubated immediately post-operatively after distractor removal and palate repair. Airway grades were significantly different between the three surgical time points (p=0.028). A significant improvement occurred between distractor placement and palatoplasty (p=0.011). There was no significant airway grade difference between the patients with syndromes and those without. Of the 16 patients, 11 had a numerical improvement in airway grade (69%). Five remained stable from distractor placement to palatoplasty (31%) though these all had grade 1 or 2 airways. The number of intubation attempts decreased from 1.6 to 1.3 to 1.2 for the three operations, respectively. None required reintubation after any operation. CONCLUSIONS: These 16 patients with PRS showed a significant improvement in airway grade between distractor placement and palatoplasty. While we have always believed that the airway improves after MDO based on improvement in the respiratory status of these patients, this shows an objective change in the anatomy. This has implications for these patients for future surgery and intubations that may occur during their lives.
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