Challenges in the management of ectopia cordis

Abstract

ECTOPIA CORDIS, a rare congenital anomaly, presents as a neonatal emergency demanding early surgical intervention. Anesthetic and intensive care management of a patient with ectopia cordis can be challenging. The anatomic defects comprising this disorder include an extrathoracic heart with associated sternal, chest wall, pericardial, and abdominal wall defects in addition to intracardiac anomalies, such as septal defects, tetralogy of Fallot, and ventricular diverticula, and craniofacial, renal, and anorectal anomalies. Despite improvements in surgical, anesthetic, and intensive care management, survivors are consistently rare. 1 Cantrell JR Haller HA Ravitch MM A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynaecol Obstet. 1958; 107: 602 Google Scholar , 2 Dobell ARC Williams HB Long RW Staged repair of ectopia cordis. J Pediatr Surg. 1982; 17: 353-358 Abstract Full Text PDF PubMed Scopus (55) Google Scholar Although the surgical management of ectopia cordis has been well described in the available literature, 3 Hornberger LK Colan SD Lock JE et al. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation. 1996; 94: 32-37 Google Scholar , 4 Leca F Thibert M Khoury W et al. Extrathoracic heart (ectopia cordis): Report of two cases and review of the literature. Int J Cardiol. 1989; 22: 221-228 Abstract Full Text PDF PubMed Scopus (36) Google Scholar , 5 Diaz JH Perioperative management of neonatal ectopia cordis: Report of three cases. Anesth Analg. 1992; 75: 833-837 Crossref PubMed Google Scholar anesthetic management and perioperative critical care need more elaboration. The case of a neonate with ectopia cordis who presented to this referral center is reported with the aim of increasing awareness about this unusual congenital anomaly and conceivably improving future outcomes.