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Ectopia cordis – case report


Authors: J. Pannová 1;  H. Kováčová 1;  D. Matura 1;  T. Gruszka 2;  J. Pavlíček 2;  P. Delongová 3
Authors place of work: Porodnicko-gynekologická klinika OU a FN, Ostrava, přednosta doc., MUDr. V. Unzeitig, CSc. 1;  Klinika dětského lékařství OU a FN, Ostrava, přednosta doc. MUDr. M. Hladík, Ph. D. 2;  Ústav patologie OU a FN, Ostrava, přednostka doc. MUDr. J. Dvořáčková, Ph. D. 3
Published in the journal: Ceska Gynekol 2015; 80(3): 214-217

Summary

Object:
Case report of ectopia cordis in a fetus at 23 weeks gestation.

Design:
Case repor.

Setting:
Obstetrics and Gynecology Clinic, University of Ostrava and University Hospital in Ostrava.

Methods and results:
We report a case of pentalogy of Cantrell variant in a 23-week fetus with an ultrasound finding of ectopia cordis, associated intracardial defects and omphalocele containing liver and guts. The patient opted for termination of pregnancy by using prostaglandins. Autopsy of the fetus revealed a cleft sternum and thoracic wall defect with nude ectopic heart-thoracic type and omphalocele containing liver and guts. Detailed examination of the heart revealed a double outlet right ventricle with a complete atrioventricular septal defect. Our study describes typical ultrasound findings in a correlation with autopsy findings.

Conclusion:
Ectopia cordis is a rare congenital malformation with an estimated incidence of 1:100 000 live births in developed countries. It is characterized by abnormal heart placement outside the thorax, mostly on the thoracoabdominal side. This form is often associated with pentalogy of Cantrell.

Keywords:
ectopia cordis, pentalogy of Cantrell, omphalocele, cleft sternumbirth weight, mode of delivery


Zdroje

1. Arnaoutoglou, C., Meditskou, S., Keivanidou, A., et al. Ectopia cordis in a fetus with mosaic trisomy 16. J Clin Ultrasound, 2010, 38, 7, p. 386–388.

2. 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, 5, p. 602–614.

3. Carmi, MDR., Boughman, JA. Pentalogy of Cantrell and associated midline anomalies: A possible ventral midline developmental field. Am J Med Genet, 1992, 42, 1, p. 90–95.

4. van Hoorn, JHL., Moonen RMJ., Huysentruyt, CJR., et al. Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach. Eur J Pediatr, 2008, 167, p. 29–35.

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6. Chen, CP., Shihà, JC., Tzen, CY., Wang, W. Three-dimensional ultrasound in the evaluation of complex anomalies associated with fetal ventra midline defects. Ultrasound Obstet Gynecol, 2002, 19, p. 102–104.

7. Chopra, S., Kalpdev, A., Suri, V., et al. Complete ectopia cordis with anencephaly: A case report. J Clin Ultrasound, 2010, 38, 4, p. 212–214.

8. Liang, RI., Huang, SE., Chang, FM. Prenatal diagnosis of ectopia cordis at 10 weeks of gestation using two-dimensional and three-dimensional ultrasonography. Ultrasound Obstet Gynecol, 1997, 10, 2, p. 137–139.

9. Ludwig, K., Salmaso, R., Cosmi, E., et al. Pentalogy of cantrell with complete ectopia cordis in a fetus with asplenia. Pediatr Dev Pathol, 2012, 15, 6, p. 495–498.

10. McMahon, CJ., Taylor, MD., Cassady, CI., et al. Diagnosis of pentalogy of cantrell in the fetus using magnetic resonance imaging and ultrasound. Pediatr Cardiol, 2007, 28, 3, p. 172–175.

11. Morales, JM., Patel, SG., Duff, JA., et al. Ectopia cordis and other midline defects. Ann Thorac Surg, 2000, 70, 1, p. 111–114.

12. Pepper, MA., Fishbein, GA., Teitell, MA. Thoracoabdominal wall defect with complete ectopia cordis and gastroschisis: a case report and review of the literature. Pediatr Dev Pathol, 2013, 16, 5, p. 348–352.

13. Puvabanditsin, S., Di Stefano, V., Garrow, E., et al. Ectopia cordis. Hong Kong Med J, 2013, 19, 5, p.447–450.

14. Tongsong, T., Wanapirak, C., Sirivatanapa, P., Wongtrangan, S. Prenatal sonographic diagnosis of ectopia cordis. J Clin Ultrasound, 1999, 27, 8, p. 440–445.

15. 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, 5, p. 778–792.

Štítky
Dětská gynekologie Gynekologie a porodnictví Reprodukční medicína

Článek vyšel v časopise

Česká gynekologie

Číslo 3

2015 Číslo 3
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