PENTALOGY OF CANTRELL 

 

 

Etiology is uncertain but is thought to involve failure of the lateral body folds to fuse in the thoracic region with variable inferior extension.
 

EMBRYOLOGY

 

Link to Embryology

 

 

ULTRASOUND
  1. Midline supraumbilical abdominal wall defect (omphalocele)
  2. Ectopia cordis
  3. Ventral diaphragmatic defect (hernia)
  4. Apical pericardial defect (free communication with peritoneal cavity)
  5. Cardiovascular malformation (mainly of the ventricular septum)
  6. Bifid sternum (midline sternal defect)

There is a wide range of appearance of this anomaly that have been reported (1,2). Cases not strictly adhering to the above criteria are considered incomplete forms of Cantrell's pentalogy (3).


 

 

REFERENCES
  1. Bayron FX. Ectopia cordis: report of a case with attempted operative correction. J Thorac Surg 1948;17:717-722.
  2. Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602-614.
  3. Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium and heart. Pediatrics 1972;50:7778-7791.