Abstract
BACKGROUND: Robin sequence (RS) describes a heterogeneous population with micrognathia, glossoptosis, and upper airway obstruction (UAO). Workup, treatment, outcomes assessment, and research inclusion are widely variable. Despite several classifications and algorithms, none is broadly endorsed. The objective of this investigation was to develop and trial a novel classification system designed to enhance clinical communication, treatment planning, prognostication, and research. METHODS: This is a retrospective cross-sectional study. A classification system was developed with five elements: micrognathia, nutrition, airway, palate, syndrome/comorbidities (MicroNAPS). Definitions and a framework for "stage" assignment (R0-R4) were constructed. Stage "tongue-based airway obstruction" (TBAO) was defined for infants with glossoptosis and UAO without micrognathia. MicroNAPS was applied to 100 infants with at least 1-year follow-up. Clinical course, treatment, airway, and feeding characteristics were assessed. Descriptive and analytic statistics were calculated and a P value less than 0.05 was considered significant. RESULTS: Of the 100 infants, 53 were male. Mean follow-up was 5.0 ± 3.6 years. R1 demonstrated feeding-predominant mild RS for which UAO was managed nonoperatively but gastrostomy tubes were prevalent. R2 was characterized by airway-predominant moderate RS, typically managed with mandibular distraction or tongue-lip adhesion, with few gastrostomy tubes and short lengths-of-stay. R3 denoted severe RS, with similar UAO treatment to R2, but with more surgical feeding tubes and longer admissions. R4 represented a complex phenotype with 33% tracheostomies, protracted hospitalizations, and delayed palatoplasty. R0 ("at risk") and TBAO groups displayed the most variability. CONCLUSIONS: MicroNAPS is easy to use and associated with relevant disease characteristics. We propose its adoption in clinical and research settings.