title: Pentalogy of Cantrell: Case Report With Review of the Literature.
abstract: BACKGROUND
  Pentalogy of Cantrell is a rare, congenital disorder characterized by lower sternal defects, diaphragmatic defect, pericardial defect, supraumbilical abdominal wall abnormalities, and/or intracardiac defects. The collective defects result from failure of either differentiation or migration of mesenchymal or mesodermal structures during the embryonic phase of development. Mortality of the disease complex is high, and treatment, when appropriate, revolves around surgical correction of the associated defects.
  
  
  OBJECTIVE
  This article presents a case of pentalogy of Cantrell and examines the literature to report the most current evidence relative to the embryology and pathophysiology. In addition, the methods of pre- and postnatal diagnosis, management, and prognostic indicators are examined.
  
  
  METHODS
  Case report was gathered from the medical records and is provided as it occurred. The literature was searched for evidence of best management strategies as well as care implications for families.
  
  
  RESULTS
  A female newborn was delivered at (Equation is included in full-text article.)weeks' gestation secondary to premature onset of labor. Prenatal ultrasonography identified an abdominal wall defect, diaphragmatic hernia, sternal defect, ventricular septal defect, and open neural tube defect. Examination immediately after delivery confirmed prenatal findings and a diagnosis of pentalogy of Cantrell was assigned.
  
  
  CONCLUSIONS
  Patients with the diagnosis of pentalogy of Cantrell should receive antenatal counseling relative to mortality and morbidity risks. An interprofessional approach in the immediate timeframe after delivery facilitates timely diagnostics and offers families prompt confirmation of antenatal findings.
  
  
  CONCLUSIONS
  Future research can focus on further elucidating genetic etiologies of pentalogy of Cantrell.
authors.affiliation: University of North Carolina System, Neonatal Nurse Practitioner Program, East Carolina University, College of Nursing, and University of North Carolina at Chapel Hill, (Dr Jnah); Neonatal Nurse Practitioner Program, University of North Carolina System, and Department of Graduate Nursing Science, East Carolina University, College of Nursing, Chapel Hill (Dr Newberry); and University of North Carolina System, and Division of Neonatology and Perinatal Medicine, University of North Carolina at Chapel Hill (Dr England).
authors.forename: Amy J
authors.initials: AJ
authors.lastname: Jnah
authors.forename: Desi M
authors.initials: DM
authors.lastname: Newberry
authors.forename: Amanda
authors.initials: A
authors.lastname: England
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D062071:Infant, Extremely Premature; D007231:Infant, Newborn; D058502:Pentalogy of Cantrell / Q000175:diagnosis / Q000196:embryology / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D011247:Pregnancy; D011296:Prenatal Diagnosis; D011379:Prognosis; D055815:Young Adult