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Cardiac and Neurologic Anomalies in Robin Sequence: Incidence and Clinical Implications
Melinda Costa, MD; Michael Tu; Kariuki Murage, MD; Sunil Tholpady, MD, PhD; Roberto Flores, MD
Indiana University
2014-02-26
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:
Although the association of syndromic, lower airway, and feeding mechanism anomalies in Robin sequence (RS) is well appreciated, the association of other anomalies is not well described. The purpose of this study is to evaluate the incidence and clinical relevance of cardiac and central nervous system (CNS) anomalies in RS.
Methods:
An 11-year (2001-2012) retrospective review of all infants with RS admitted to the neonatal intensive care unit (NICU) at a tertiary care children's hospital was performed. The presence and type of cardiac and CNS anomalies was assessed. Univariate analysis was used to determine the association between mortality and the anomalies.
Results:
181 patients met inclusion criteria. Of these, 30.9% had at least one cardiac and 25.4% at least one CNS anomaly. 45.9% of patients had either a CNS or cardiac anomaly. The overall mortality rate was 16.6%. Mortality was 39.3% in patients with cardiac anomalies (p<0.001) and 32.6% in patients with CNS anomalies (p<0.001). The odds ratio (OR) for mortality was 2.37 for cardiac and 1.96 for CNS anomalies.
Conclusions:
There is a high incidence of cardiac and CNS anomalies in infants with RS. The presence of either a cardiac or CNS anomaly is associated with a statistically significant, nearly two-fold increase in mortality. These data suggest that cardiac and cranial imaging should be performed during the initial evaluation of infants with RS.
