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Robin Sequence: Mortality, Causes of Death, and Clinical Outcomes
Melinda Costa, MD; Michael Tu; Kariuki Murage, MD; Sunil Tholpady, MD; William Engle, MD; Roberto Flores, MD
Indiana University
2014-03-01
Presenter: Melinda Costa
Affidavit:
This submission represents the original work of the authors. The presenting fellow has made primary contribution to the design, data analysis, and editing of the study.
Director Name: Robert L. Flores, MD
Author Category: Fellow Plastic Surgery
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial
Purpose:
We report etiology of and risk factors for mortality in infants with Robin sequence (RS) and identify characteristics associated with quality of life outcomes. We hypothesize that through improvements in airway monitoring, treatment, and neonatal care, death due to airway obstruction is uncommon.
Methods:
An 11-year retrospective review of infants with RS admitted to the neonatal intensive care unit was performed. The primary outcome was mortality rate. Secondary outcomes to assess quality of life were emergency room (ER) visit and hospital admission rates. Significant variables were identified via univariate analysis.
Results:
181 infants were identified. Mean follow up was 35 months. Patient characteristics included 32.6% isolated, 31.5% syndromic, 38.1% gastrointestinal, 32.6% pulmonary, 30.9% cardiac, 25.4% central nervous system, and 69.6% ¡Ý 2 organ system anomalies.
Mortality was 16.6%; two deaths were related to airway obstruction. There were no deaths in isolated RS (p=0.002). Mortality was statistically associated with cardiac anomalies (p<0.001), central nervous system anomalies (p=0.001), and ¡Ý 2 organ system abnormalities (p=0.001). Variables associated with an increased rate of ER visits were cardiac anomalies (p=0.04) and ¡Ý 2 organ system abnormalities (p=0.04). The presence of ¡Ý 2 organ system abnormalities (p=0.04) was associated with an increased hospital admission rate.
Conclusions:
Mortality and negative quality of life measures in RS are not directly related to respiratory obstruction. Isolated RS was not associated with mortality. The presence of additional organ system anomalies are associated with mortality; risk assessment should include identification of organ system anomalies outside the tongue base.
