title: Robin sequence: mortality, causes of death, and clinical outcomes.
abstract: BACKGROUND
  The authors report the cause of and risk factors for mortality in infants with Robin sequence and identify characteristics associated with quality-of-life outcomes.
  
  
  METHODS
  The authors performed an 11-year retrospective review of all infants with Robin sequence treated at a neonatal intensive care unit. Patient characteristics were correlated to mortality and quality-of-life measures. Emergency room visits and hospital admissions were used to assess quality-of-life outcomes. Significant variables were identified by means of univariate analysis.
  
  
  RESULTS
  One hundred eighty-one consecutive infants were identified. Patient characteristics included the following: isolated, 32.6 percent; syndromic, 31.5 percent; gastrointestinal, 38.1 percent; pulmonary, 32.6 percent; cardiac, 30.9 percent; central nervous system, 25.4 percent; and two or more organ system anomalies, 69.6 percent. Mortality was 16.6 percent; two deaths were related to airway obstruction problems. There were no deaths in isolated Robin sequence (p = 0.002). Mortality was statistically associated with cardiac anomalies (p < 0.001), central nervous system anomalies (p = 0.001), and two or more organ system abnormalities (p = 0.001). Variables associated with an increased rate of emergency room visits were cardiac anomalies (p = 0.04) and two or more organ system abnormalities (p = 0.04). The presence of two or more organ system abnormalities (p = 0.04) was associated with an increased hospital admission rate.
  
  
  CONCLUSIONS
  Mortality and negative quality-of-life measures in Robin sequence are not directly related to respiratory obstruction. Isolated Robin sequence confers no increased risk of mortality. There is a high incidence of cardiac and central nervous system anomalies, which are significantly associated with mortality. Cardiac and cranial imaging should be performed during the initial evaluation of infants with Robin sequence.
  
  
  METHODS
  Risk, III.
authors.affiliation: Indianapolis, Ind. From the Division of Plastic Surgery and the Department of Pediatrics, Riley Hospital for Children, Indiana University School of Medicine.
authors.forename: Melinda A
authors.initials: MA
authors.lastname: Costa
authors.forename: Michael M
authors.initials: MM
authors.lastname: Tu
authors.forename: Kariuki P
authors.initials: KP
authors.lastname: Murage
authors.forename: Sunil S
authors.initials: SS
authors.lastname: Tholpady
authors.forename: William A
authors.initials: WA
authors.lastname: Engle
authors.forename: Roberto L
authors.initials: RL
authors.lastname: Flores
mesh_terms: D002423:Cause of Death; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010855:Pierre Robin Syndrome / Q000401:mortality* / Q000628:therapy; D011788:Quality of Life; D012189:Retrospective Studies; D012307:Risk Factors