Pentalogy of Cantrell
Pronunciation: pen-TAL-oh-jee of can-TREL
Rare congenital syndrome involving midline defects of the chest wall, heart, diaphragm, sternum, and abdominal wall. Requires complex surgical management.
Full Definition
Pentalogy of Cantrell is a rare congenital syndrome characterized by five midline developmental defects: ectopia cordis (heart outside the chest), anterior diaphragmatic defect, sternal cleft, pericardial defect, and abdominal wall defect (typically omphalocele or gastroschisis). This complex condition results from defective development of the septum transversum and ventral folding during embryogenesis. The severity varies widely, with complete pentalogy being extremely rare and often incompatible with life. Incomplete forms may present with varying combinations of defects. Surgical management requires staged reconstruction involving cardiac surgery, thoracic surgery, and abdominal wall repair, often necessitating multidisciplinary coordination and innovative surgical techniques.
Usage
Usage note: Specify which components of the pentalogy are present when documenting incomplete forms.
In Context
- "Prenatal diagnosis of pentalogy of Cantrell prompted delivery at a specialized center with pediatric cardiac surgery capabilities." — Obstetric consultation note
- "Staged reconstruction for incomplete pentalogy of Cantrell began with sternum repair and abdominal wall closure." — Surgical planning conference