page_title: Pentalogy of Cantrell
section_title: Presentation
hierarchical_section_title: Pentalogy of Cantrell / Presentation
caption_attribution_description: English: The images are in the public domain and thus free of any copyright restrictions. As a matter of courtesy we request that the content provider (Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities) be credited and notified in any public or private usage of this image.
context_page_description: Pentalogy of Cantrell is a rare syndrome that causes defects involving the diaphragm, abdominal wall, pericardium, heart and lower sternum.
context_section_description: There are five characteristic findings in pentalogy of Cantrell: 
  an abdominal wall defect,
  lower sternal defect,
  congenital heart malformations,
  absence of the diaphragmatic pericardium,
  and an anterior diaphragmatic defect.
  Abdominal wall defects in pentalogy of Cantrell occur above the umbilicus (supraumbilical) and in the midline, and have a wide range of presentations. Diastasis recti, hernias, and omphalocele have all been described in conjunction with the pentalogy.
  Sternal defects too have a range of presentations, from absence of the xiphoid process to shortened or cleft sternum. If the sternal defect is large enough, the neonate may have ectopia cordis, in which the heart is located outside of the thorax.
  Many congenital heart malformations have been described in conjunction with pentalogy of Cantrell. The most common is ventricular septal defect, found in 72% of cases. Others include atrial septal defect, cardiac diverticulum, pulmonic stenosis, double outlet right ventricle, tetralogy of Fallot, dextrocardia, and transposition of the great vessels.
caption: A baby with isolated omphalocele, one of the defects included in the pentalogy of Cantrell.
exif: {}