CTICasesPentalogy of Cantrell
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Imaging findings
The patient's heart is outside of the thoracic cavity, showing four chambers and relatively normal communication between them, including an atrial septal defect. The 'horn-like' structures are actually the atrial appendages. There is also an omphalocele apparatus overlying the abdomen after reduction.
Key takeaways
This patient has a sternal cleft, ectopia cordis, absent pericardium, an anterior diaphragmatic defect, an omphalocele, and an atrial septal defect, comprising the full pentalogy of Cantrell. This is an incredibly rare congenital condition, known prenatally, with a very poor prognosis. The baby was delivered via c-section but unfortunately passed away.
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