Prenatal Diagnosis of Exstrophy of the Cloaca with Ectopia Cordis

Abstract 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.

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Pentalogy of Cantrell: Prenatal Diagnosis, Delivery, and Immediate Postnatal Surgical Repair

Journal of Neonatal Surgery ◽

10.21699/jns.v6i2.503 ◽

2017 ◽

Vol 6 (2) ◽

pp. 32 ◽

Cited By ~ 1

Author(s):

Edward Araujo Júnior ◽

Milene Carvalho Carrilho ◽

Bruno Rodrigues Toneto ◽

José Cicero Guilhen

Keyword(s):

Congenital Anomaly ◽

Prenatal Diagnosis ◽

Surgical Repair ◽

Anterior Abdominal Wall ◽

Second Trimester ◽

Ectopia Cordis ◽

Pentalogy Of Cantrell ◽

Parental Counseling ◽

In The Beginning

Pentalogy of Cantrell (PC) is a congenital anomaly characterized by a defect in the lower sternum, anterior diaphragm, and anterior abdominal wall; ectopia cordis; and congenital heart disease. It is a very rare congenital anomaly and the prenatal diagnosis is possible in the beginning of second trimester of pregnancy using the conventional ultrasonography. The prognosis is poor with high rates of perinatal mortality. We present a case report of prenatal diagnosis of PC at 22 weeks and 3 days of gestation. We emphasize the prenatal care follow up in a tertiary reference center, the parental counseling, the planning of delivery, and the management of newborn by a multidisciplinary team, including the description of immediate postnatal surgical repair.

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Prenatal Diagnosis and Management of Ectopia Cordis: Varied Presentation Spectrum

Fetal and Pediatric Pathology ◽

10.1080/15513815.2018.1556367 ◽

2019 ◽

Vol 38 (2) ◽

pp. 127-137 ◽

Cited By ~ 4

Author(s):

Gürcan Türkyilmaz ◽

Sahin Avcı ◽

Tugba Sıvrıkoz ◽

Emircan Erturk ◽

Umut Altunoglu ◽

...

Keyword(s):

Prenatal Diagnosis ◽

Ectopia Cordis ◽

Diagnosis And Management

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Conservative management of Cantrell syndrome: a case report

Journal of Surgical Case Reports ◽

10.1093/jscr/rjaa100 ◽

2020 ◽

Vol 2020 (5) ◽

Author(s):

Marcella Schiavone ◽

Damiano Pizzol ◽

Anna Claudia Colangelo ◽

Mario Antunes

Keyword(s):

Case Report ◽

Prenatal Diagnosis ◽

Abdominal Wall ◽

Low Income ◽

Conservative Management ◽

Gold Standard ◽

Antenatal Screening ◽

Ectopia Cordis ◽

Standard Management ◽

Clinical Conditions

Abstract Cantrell syndrome (CS) is defined as congenital combination of five anomalies: defects at the lower part of the sternum, anterior diaphragm, midline supraumbilical abdominal wall, diaphragmatic pericardium and ectopia cordis. Antenatal screening should be performed to make an accurate prenatal diagnosis. The prognosis is usually poor with a high mortality early in life. The gold standard management is surgery but its prognosis remains poor. In many low-income settings prenatal examinations and surgery treatment are not possible. In the present case, we report a not surgery managed baby affected by CS, with good clinical conditions after 5 months.

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