What is the Survival Rate in Prenatally Detected Cantrell’s Pentalogy?

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Cantrell’s pentalogy is a congenital defect characterized by uncompleted fusion of the anterior chest wall, resulting in an extrathoracic location of the heart. Ultrasound diagnosis during the first trimester of prenatal life is possible, and termination of pregnancy is usually chosen by pregnant women. We analysed 57 fetuses: 56 from literature and one additional recent case from our institute (from 2016) to evaluate what was the survival rate reported after prenatal diagnosis, including the possibility to terminate the pregnancy, intrauterine deaths and neonatal deaths. We found 10 survivors - 18% since 1984.

Despite dismal prognosis of fetal ectopia cordis, there is a chance for postnatal survivorship probably due to evolving anatomical structures, not only in the first trimester of pregnancy but also during the following weeks of prenatal life.

1. 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.

2. Desselle C, Herve P, Toutain A, Lardy H, Sembely C, Perrotin F. Pentalogy of Cantrell: sonographic assessment. J Clin Ultrasound. 2007;35:216–220.

3. Khoury MJ, Cordero JF, Rasmussen S. Ectopia cordis, midline defects and chromosome abnormalities: an epidemiologic perspective. Am J Med Genet. 1988;30:811–817.

4. Respondek-Liberska M, Janiak K, Kaczmarek P, Borowski D, Czichos E. The significance of cardiography in the diagnosis of cardiac ectopia of the fetus. Ginekol Pol. 1998;69:139-144. Polish.

5. Kaplan LC, Matsouka R, Gilbert EF, Opitz JM, Kurnit DM. Ectopia cordis and cleft sternum: evidence for mechanical teratogenesis following rupture of the chorion or yolk sac. Am J Med Genet. 1985;21:187–202.

6. Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: a case report and review of the syndrome. Pediatrics 1972;50:778–786.

7. Pekin AT, Kerimoğlu OS, Yilmaz SA, Kebapcilar AG, Bakbak BG, Celik C. Pentalogy of Cantrell accompanied by scoliosis and pes equinovarus deformity at 12 weeks gestation. Clin Exp Obstet Gynecol. 2015;42:392–394.

8. Haynor DR, Shuman WP, Brewer DK, Mack LA. Imaging of fetal ectopia cordis: roles of sonography and computed tomography. J Ultrasound Med. 1984;3:25–27.

9. Achiron R, Shimmel M, Farber B, Glaser J. Prenatal sonographic diagnosis and perinatal management of ectopia cordis. Ultrasound Obstet Gynecol. 1991;1:431–434.

10. Dillon E, Renwick M. The antenatal diagnosis and management of abdominal wall defects: the northern region experience. Clin Radiol. 1995;50:855–859.

11. Hsieh YY, Lee CC, Chang CC, Tsai HD, Hsu TY, Tsai CH. Prenatal sonographic diagnosis of Cantrell’s pentalogy with cystic hygroma in the first trimester. J Clin Ultrasound. 1998;26:409-412.

12. Bognoni V, Quartuccio A, Quartuccio A. First-trimester sonographic diagnosis of Cantrell’s pentalogy with exencephaly. J Clin Ultrasound. 1999;27:276–278.

13. Tongsong T, Wanapirak C, Sirivatanapa P, Wongtrangan S. Prenatal sonographic diagnosis of EC. J Clin Ultrasound. 1999;27:440–445.

14. Respondek-Liberska M, Janiak K, Wloch A. Fetal echocardiography in ectopia cordis. Pediatr Cardiol. 2000;21:249-252.

15. Colpaert C, Bogers J, Hertveldt K, Loquet P, Dumon J, Willems P. Limb-body wall complex: 4 cases illustrating the importance of examining placenta and umbilical cord. Pathol Res Pract. 2000;196:783–790.

16. Mittermayer C, Bernaschek G, Lee A. Diagnosis of ectopia cordis with three-dimensional power Doppler ultrasound in first-trimester pregnancy. Ultrasound Obstet Gynecol. 2002;20:203–205.

17. Smrcek JM, Gembruch U, Krokowski M, Berg C, Krapp M, Geipel A, Germer U. The evaluation of cardiac biometry in major cardiac defects detected in early pregnancy. Arch Gynecol Obstet. 2003;268:94-101.

18. Onderoğ lu L, Baykal C, Tulunay G, Talim B, Kale G. Prenatal diagnosis of Cantrell’s pentalogy: a case report. Turk J Pediatr. 2003;45:357–358.

19. Staboulidou I, Wüstemann M, Schmidt P, Günter HH, Scharf A. Increased fetal nuchal translucency as a predictor of Cantrell’s pentalogy - case report. Z Geburtshilfe Neonatol. 2005;209:231-234.

20. Gomez SG, Bermulez Sosa MT, Palma Hernandez E, del Castillo Salceda LF, Pinzon Muslera O, Hernandez Cortes B, Mendez Machado G. Ectopia cordis: multidisciplinary approach with successful result. Ginecol Obstet Mex. 2006;74:439–443.

21. Zidere V, Allan LD. Changing findings in pentalogy of Cantrell in fetal life. Ultrasound Obstet Gynecol. 2008;32:835–837.

22. Peixoto-Filho FM, do Cima LC, Nakamura-Pereira M. Prenatal diagnosis of Pentalogy of Cantrell in the first trimester: is 3-dimensional sonography needed? J Clin Ultrasound. 2009;37:112-114.

23. Barbee K, Wax JR, Pinette MG, Cartin A, Blackstone J. First-trimester prenatal sonographic diagnosis of ectopia cordis in a twin gestation. J Clin Ultrasound. 2009;37:539–540.

24. Hannoun A, Usta IM, Sawaya F, Nassar AH. First trimester sonographic diagnosis of ectopia cordis: a case report and review of the literature. J Matern Fetal Neonatal Med. 2011;24:867–869.

25. Sadłecki P, Krekora M, Krasomski G, Walentowicz-Sadłecka M, Grabiec M, Moll J, Respondek-Liberska M. Prenatally evolving ectopia cordis with successful surgical treatment. Fetal Diagn Ther. 2011;30:70–72.

26. Di Spiezio Sardo A, Paladini D, Zizolfi B, Spinelli M, Nappi C. Pentalogy of Cantrell: embryofetoscopic diagnosis. J Minim Invasive Gynecol. 2013;20:248–251.

27. Sepulveda W, Wong AE, Simonetti L, Gomez E, Dezerega V, Gutierrez J. Ectopia cordis in a first-trimester sonographic screening program for aneuploidy. J Ultrasound Med. 2013;32:865–871.

28. Shibata Y, Terada K, Igarashi M, Suzuki S. Body stalk anomaly complicated by ectopia cordis in the first trimester. J Clin Diagn Res. 2014;8:OD06-7.

29. Türkçapar AF, Sargin Oruc A, Öksüzoglu A, Danışman N. Diagnosis of pentalogy of cantrell in the first trimester using transvaginal sonography and color Doppler. Case Rep Obstet Gynecol. 2015;2015:179298.

30. Donofrio MT, Levy RJ, Schuette JJ, Skurow-Todd K, Sten MB, Stallings C, Pike JI, Krishnan A, Ratnayaka K, Sinha P, du Plessis AJ, Downing DS, Fries MI, Berger JT. Specialized delivery room planning for fetuses with critical congenital heart disease. Am J Cardiol. 2013;111:737–747.

31. Rajewska J, Gawrych E, Wegrzynowski J, Konefał H, Rybkiewicz M. Cantrell's pentalogy - a case report. Ginekol Pol. 2010 Nov; 81(11): 860-4.

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