Prenatally Evolving Ectopia Cordis with Successful Surgical Treatment

Sadłecki, Paweł; Krekora, Michał; Krasomski, Grzegorz; Walentowicz-Sadlecka, Małgorzata; Grabiec, Marek; Moll, J; Respondek-Liberska, Maria

doi:10.1159/000326300

Cited by 18 publications

(33 citation statements)

References 8 publications

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“…In 18% (10 cases) there was spontaneous intrauterine demise later on. In 8 cases there was lost follow-up.Finally we found 9 survivors -16%, including the most recent case presented above (as a second one from our Institution) 25 . 4,5,7 .…”

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confidence: 57%

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

Pośpiech-Gąsior

Słodki

Respondek-Liberska

2016

Prenatal Cardiology

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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. Key words: ectopia cordis, Cantrell syndrome, diaphragmatic herniaCorresponding author: majkares@uni.lodz.pl Submitted: 2015Submitted: -12-11, accepted: 2016 INTRODUCTIONCantrell's pentalogy is an anomaly resulting from a defect in embryologic development and consists of the following: a deficiency of the anterior diaphragm, a midline supra-umbilical abdominal wall defect, a defect in the diaphragmatic pericardium, congenital intracardiac abnormalities and a defect of the lower sternum. MATERIAL AND METHODSWe analyzed the published reports of prenatal Cantrell's pentalogy from the literature, since 1984, which were detected in 1 st or second trimester of pregnancy. (Table 1), and focused on the gestational age at the time of diagnoses, number of termination of pregnancies, intrauterine demises and neonatal follow-up. The last case from 2016 from our institution is presented below:A 28-year-old woman (G1, P0) had no history of intake of any teratogens or exposure to unusual environment during the antenatal period. The family history was negative for congenital anomalies or genetic abnormalities, and there was no-cosanguinity. A screening ultrasound at 12 weeks of pregnancy revealed a nuchal translucency of 3,8 mm, nasal bone present and omphalocele (containing liver, stomach and cardiac apex)(Foto 1, 2-cine, 3). Biochemical tests of maternal blood showed PAPPA-A of 0,3 MoM and free bHCG of 0,733 MoM. Chorionic villus sampling was nondiagnostic and amniocentesis showed a karyotype of 46, XX. The pregnant women was offered termination of pregnancy, however, she decided to continue her pregnancy and to deliver at a tertiary obstetrical and cardiac center. Prenatal echocardiography at 18, 20, 27 and 30 th week of gestational age revealed a fourchamber heart with normal flow in the great arteries. Ultrasound biometry was adequate. At 27 th week due to visualization of intestines in fetal chest cavity suggesting diaphragmatic hernia (DH), MRI examination was offered and right anterior DH was confirmed. In the second half of pregnancy, the position of the fetal heart and size of omphalocele changed: there was an improvement in 'niche' in the fetal chest and a decrea...

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confidence: 57%

“…The first case 25 was discharged at the 4th week of postnatal life, but died suddenly 10 months later. The other, presented above, had an extremely difficult postnatal management period, but ended successfully and we do believe its postnatal surgery and intensive care treatment deserves a separate presentation.…”

Section: Discussionmentioning

confidence: 99%

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

Pośpiech-Gąsior

Słodki

Respondek-Liberska

2016

Prenatal Cardiology

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“…In the pathogenesis, lack of completion of the normal developmental process of the sternum and thoracic wall is blamed (5,6). Although ectopia cordis mostly occurs sporadically, familial cases have been reported rarely (7).…”

Section: Discussionmentioning

confidence: 99%

“…Its frequency among all congenital heart diseases is 0.1% (1.5). Ectopia cordis may occur alone or in accompaniment with other congenital heart diseases, central nervous system disorders and disorders including ompholocele, gastrochisis and cleft lip-palate (5). Here, a case of ectopia cordis diagnosed in the prenatal period is presented because it is observed rarely.…”

Section: Introductionmentioning

confidence: 98%

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A rare case of cardiac anomaly: prenatally diagnosed ectopia cordis

et al. 2015

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Ectopia cordis is a rare congenital malformation in which the heart is located partially or totally outside the thoracic cavity. The estimated prevalence of ectopia cordis is 5.5-7.9 per million births and it comprises 0.1% of congenital heart diseases. Ectopia cordis is associated with other congenital heart diseases and various tissue and organ disorders. Common cardiac anomalies associated with ectopia cordis include ventricular septal defect, atrial septal defect, pulmonary stenosis, right ventricular diverticulum, double right ventricular outflow tract and tetralogy of Fallot. Extracardiac anomalies associated with ectopia cordis reported in the literature include omphalocele, gastrochisis, cleft lip and palate, scollosis and central nervous system malformations. Here we report a newborn with ectopia cordis who was diagnosed prenatally. (Turk Pediatri Ars 2015; 50: 129-31)

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Ectopia cordis: A rare congenital anomaly

et al. 2014

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Ectopia cordis (EC) is a rare congenital anomaly associated with the heart positioned outside of the thoracic cavity either partially or completely. The ectopic heart can be found along a spectrum of anatomical locations, including the cervical, thoracic and abdominal regions and in most cases, it protrudes outside the chest through a split sternum. Although the first case of EC was identified during the early 1600s only 91 cases have been reported since then in the literature. This review will discuss the history and prevalence of EC, its etiology, morphology, presentation and symptoms, complications, diagnosis, treatment and management and prognosis.

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Prenatally Evolving Ectopia Cordis with Successful Surgical Treatment

Cited by 18 publications

References 8 publications

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

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

A rare case of cardiac anomaly: prenatally diagnosed ectopia cordis

Ectopia cordis: A rare congenital anomaly

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