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Objectives/Hypothesis To estimate the current birth prevalence of isolated and syndromic Pierre Robin sequence (iPRS and sPRS), including demographic variations. To assess for regional variations in surgical airway interventions for PRS, and to determine the mean length of stay (LOS), cost of admission, complication rate, and rate of associated procedures related to tongue-lip adhesion (TLA), neonatal mandibular distraction osteogenesis (MDO), and tracheotomy. Study Design Retrospective cross-sectional study. Methods The 2006 and 2009 Kids Inpatient Databases were used to identify newborns and infants with PRS; analysis using cross tabulations and linear regression modeling was performed. Results In 2006 and 2009, the estimated birth prevalence of iPRS was 1.8:10,000 live births and sPRS 1.4:10,000 live births. The highest rate was in whites and the lowest in non-Hispanic blacks. There were 145 TLAs (36%), 176 MDOs (43%), and 85 tracheotomies (21%). The Northeast favored a TLA strategy; the Midwest favored MDO. The mean LOS for TLA was 24.5 days, MDO 36.7 days, tracheotomy (iPRS) 44.9 days, and tracheotomy (sPRS) 53.0 days. Conclusions The birth prevalence of PRS may be higher than previously described, especially in whites. Surgical management strategies vary between regions. The overall cost of a TLA admission is lower than an MDO or tracheotomy admission, owing primarily to shorter LOS. This study was limited by not taking into account outpatient expenses (nursing care, monitoring) or need for further airway/feeding intervention over subsequent admissions. Level of Evidence 2c Laryngoscope, 124:2818-2825, 2014 [ABSTRACT FROM AUTHOR]

Objective: There is considerable variation in opinion regarding the optimal management of patients with Pierre Robin sequence (PRS). No single method of airway intervention or feeding strategy is universally appropriate and effective. This study was performed to examine methods used for airway and feeding management and to identify specific problems encountered. Study Design: A retrospective study of 252 patient charts between 1989 and 1997 at Children's Hospital of Wisconsin. Methods: Patient information was collected regarding perinatal history, genetics evaluation, and airway and feeding evaluations and intervention. A group of 47 patients was determined as having PRS. Results: Secondary respiratory difficulties, defined as respiratory abnormalities in addition to the expected PRS obstruction, were identified in 23% of patients. Also, intrinsic feeding abnormalities not associated with airway obstruction were identified in 11% of patients. Analysis by Fisher's Exact Test revealed patients with a syndromic diagnosis to have a significantly higher rate for tracheotomies and gastrostomy tube placement ( P = .041, and P = .0004, respectively). Syndromic patients were also found to have significantly lower Apgar scores and longer hospital stays. Positioning techniques, tongue-lip adhesion, and tracheotomy were also employed effectively with specific indications and specific difficulties that need to be considered. Conclusion: Patients with PRS require thorough airway and feeding evaluation. Those with additional syndromic diagnoses demonstrate higher rates of more invasive interventions. Patients with PRS must undergo individualized approaches with consideration of multiple factors for successful management. [ABSTRACT FROM AUTHOR]