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Journal Information

Online ISSN
1305-3124

Established
1993

Editors-in-Chief
​Cihat Şen, ​Nicola Volpe

Editors
Daniel Rolnik, Mar Gil, Murat Yayla, Oluş Api

Statistics Editor
Resul Arısoy

A rare congenital anomaly: ectopia cordis and Cantrell pentalogy

Salih Serin, Deniz Cemgil Arıkan, Murat Bakacak, Önder Ercan, Bülent Köstü, Fazıl Avcı

Article info

A rare congenital anomaly: ectopia cordis and Cantrell pentalogy. Perinatal Journal 2014;22(3):SE20 DOI: 10.2399/prn.14.S001084

Author(s) Information

Salih Serin,
Deniz Cemgil Arıkan,
Murat Bakacak,
Önder Ercan,
Bülent Köstü,
Fazıl Avcı

  1. Kahramanmaraş Sütçü İmam Üniversitesi Tıp Fakültesi, Kadın Hastalıkları ve Doğum Anabilim Dalı- Kahramanmaraş TR
Correspondence

Deniz Cemgil Arıkan, Kahramanmaraş Sütçü İmam Üniversitesi Tıp Fakültesi, Kadın Hastalıkları ve Doğum Anabilim Dalı- Kahramanmaraş TR,

Publication History
Conflicts of Interest

No conflicts declared.

Objective

In this study, we aimed to present two cases of ectopia cordis which diagnosed in the prenatal period.

Introduction 

Ectopia Cordis (EC) is a very rare congenital malformation which appears as development of midline defect. EC may be present as an isolated malformation or a component of Cantrell’s Pentalogy.

Case 1 

A 22-years-old pregnant at 15 weeks 1 day gestational age admitted to our fetal medicine centre. Prenatal ultrasound showed a fetus with bilateral hydrothorax, EC, and a large omphalocele with evisceration of the heart (Figure 1). Cantrell’s pentalogy with thoracoabdominal EC was diagnosed and pregnancy was terminated.

Case 2 

A 32-years-old pregnant at 11 weeks 3 days gestational age admitted to our fetal medicine centre. Ultrasound done at our fetal medicine centre revealed absent sternum, ectopia cordis, absent diaphragm, and omphalocele (Figure 2). Cantrell’s pentalogy with thoracoabdominal EC was diagnosed and pregnancy was terminated at 12 weeks and 3 days of gestational age.

Results

The diagnosis of EC in the prenatal period with monitoring of the fetal heart outside the thorax. EC may be associated with intrinsic heart disease and other midline abnormalities. The vast majority of cases has been reported aneuploidi, particularly Trisomi 18, chromosomal analysis is recommended.

Conclusion 

The ectopia cordis can be diagnosed with careful ultrasound examination of the early weeks of pregnancy. The ectopia cordis is especially high risk for Trisomi 18. We suggest that you should keep in mind this condition and amniocentesis should be offered.
 

Keywords

Ectopia Cordis; Cantrell’s Pentalogy; prenatal diagnosis

File/Dsecription
Figure 1
Prenatal ultrasound of first patient showed a foetus with bilateral hydrothorax, EC, and a large omphalocele with evisceration of the heart.
Figure 2
Prenatal ultrasound of second patient showed a foetus with bilateral hydrothorax, Ectopia Cardis, and a omphalocele with evisceration of the heart.