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Abstract
Ectopia Cordis is a congenital anomaly in which the heart is located abnormally outside the thoracic cavity. Aetiology of this defect is still unclear with the incidence rate of 8:1,000,000 live births. Clinical manifestations usually present as severe sepsis respiratory distress due to heart failure as a result of an intrinsic abnormality of the heart which makes the management more challenging. Five days-old female newborn was referred to our hospital by a local doctor with “beating lump” outside her chest. The diagnosis of complete thoracic ectopia cordis was made by clinical examination, chest x-ray, and echocardiography. The Echo showed complete AV canal, ASD, mild pulmonary stenosis, and atrioventricular valve regurgitation. Palliative surgery was then undertaken with the initial goal to limit exposure and prevent further infection. The heart is covered with bovine pericardium sutured along the edge of the chest wall defect. Ectopia cordis is rare and has a poor prognosis especially thoracic and cervical types. Definitive surgery, to replace the heart to the thoracic cavity, is difficult to perform because of the hypoplastic thoracic cavity. Attempt to do palliative surgery, such as covering the heart with either skin flap or other material, becomes the choice of treatment in this case although there is no evidence reported of adulthood survivors.
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References
- Shao-tsu VL. Ectopia cordis congenita. The Journal of thoracic and cardiovascular surgery. 1957;5(3):197-212.
- Grosfeld JL. O’Neill JA, Fonkalsrud EW CA. Pediatric surgery. 6th ed. Philadelphia: Mosby; 2006.
- Khoury MJ, Cordero JF, Rasmussen S. Ectopia cordis, midline defects and chromosome abnormalities: An epidemiologic perspective. American journal of medical genetics.1988; 30(3):811-7.
- Puri P. Newborn surgery. 2nd ed. London: Arnold; 2003.
- Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surgery, gynecology & obstetrics. 1958;107(5):602-614.
- Amitabh P, Sharan R, Talapatra A. Ectopia cordis. Journal of clinical neonatology 2012;1(3):166-167.
- Puvabanditsin S, Stefano VD, Garrow E, Wong R, Eng J, Balbin J. Ectopia cordis. Hong Kong medical journal. 2013;19(5):447-450.
- Engum SA. Embryology, sternal clefts, ectopia cordis, and cantrell’s pentalogy. Seminars in pediatric surgery. 2008;17(3):154-160.
- Brewer S, Williams T. Loss of AP-2 a impacts multiple aspects of ventral body wall development and closure. Developmental Biology.2004;267:399-417.
- Magadum S, Shivaprasad H, Dinesh K, Vijay K. Incomplete cantrell’s pentalogy — A case report. Indian Journal of Surgery. 2013;75:350-352.
- Hornberger LK, Colan SD, Lock JE, Wessel DL, Mayer JJE. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation. 1996;94(9 Suppl):II32-7.
- Li X, Guo Y, Ziegler KR, Model LS, Eghbalieh SD, Brenes RA, Kim ST, Shu C, Dardik A.. Current usage and future directions for the bovine pericardial patch. Annals of vascular surgery. 2011;25(4):561-8.
- D’Ambra L, Berti S, Feleppa C, Magistrelli P, Bonfante P, Falco E. Use of bovine pericardium graft for abdominal wall reconstruction in contaminated fields. World journal of gastrointestinal surgery. 2012;4(7):171.
References
Shao-tsu VL. Ectopia cordis congenita. The Journal of thoracic and cardiovascular surgery. 1957;5(3):197-212.
Grosfeld JL. O’Neill JA, Fonkalsrud EW CA. Pediatric surgery. 6th ed. Philadelphia: Mosby; 2006.
Khoury MJ, Cordero JF, Rasmussen S. Ectopia cordis, midline defects and chromosome abnormalities: An epidemiologic perspective. American journal of medical genetics.1988; 30(3):811-7.
Puri P. Newborn surgery. 2nd ed. London: Arnold; 2003.
Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surgery, gynecology & obstetrics. 1958;107(5):602-614.
Amitabh P, Sharan R, Talapatra A. Ectopia cordis. Journal of clinical neonatology 2012;1(3):166-167.
Puvabanditsin S, Stefano VD, Garrow E, Wong R, Eng J, Balbin J. Ectopia cordis. Hong Kong medical journal. 2013;19(5):447-450.
Engum SA. Embryology, sternal clefts, ectopia cordis, and cantrell’s pentalogy. Seminars in pediatric surgery. 2008;17(3):154-160.
Brewer S, Williams T. Loss of AP-2 a impacts multiple aspects of ventral body wall development and closure. Developmental Biology.2004;267:399-417.
Magadum S, Shivaprasad H, Dinesh K, Vijay K. Incomplete cantrell’s pentalogy — A case report. Indian Journal of Surgery. 2013;75:350-352.
Hornberger LK, Colan SD, Lock JE, Wessel DL, Mayer JJE. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation. 1996;94(9 Suppl):II32-7.
Li X, Guo Y, Ziegler KR, Model LS, Eghbalieh SD, Brenes RA, Kim ST, Shu C, Dardik A.. Current usage and future directions for the bovine pericardial patch. Annals of vascular surgery. 2011;25(4):561-8.
D’Ambra L, Berti S, Feleppa C, Magistrelli P, Bonfante P, Falco E. Use of bovine pericardium graft for abdominal wall reconstruction in contaminated fields. World journal of gastrointestinal surgery. 2012;4(7):171.