Regional variations in the presentation and surgical management of Pierre Robin sequence

Abstract

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.

Keywords: Healthcare Cost and Utilization Project; Micrognathia; Pierre Robin sequence; cleft palate; neonatal mandibular distraction osteogenesis; resource utilization; tongue-lip adhesion.