Pentalogy of Cantrell: Embryofetoscopic Diagnosis

Sardo, Attilio Di Spiezio; Paladini, D.; Zizolfi, Brunella; Spinelli, Marialuigia; Nappi, Carmine

doi:10.1016/j.jmig.2012.10.010

Cited by 3 publications

(1 citation statement)

References 21 publications

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“…Nuchal translucency Nuchal translucency was measured and reported in 44 cases: the results were abnormal (>2.5 mm, including cystic hygroma) in 39 cases (88.63%) [1,[10][11][12][13]16,17,22,25,26,[28][29][30][34][35][36]41,[43][44][45][46][47]50,53,54,56,57].…”

Section: Intracardiac Anomaliesmentioning

confidence: 99%

Cantrell syndrome in the first trimester of pregnancy: imagistic findings and literature review

et al. 2020

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Pentalogy of Cantrell (POC) is a rare condition characterized by complete or partial expression of 5 types of congenital birth defects originating in (1) the lower sternum, (2) the anterior diaphragm, (3) the diaphragmatic pericardium, (4) the midline/ventral supraumbilical abdominal wall region, and (5) the heart. POC can be diagnosed antenatally by means of ultrasonography (US), the task being difficult if defects are minor. Advances in the field of US and the introduction of the first morphology trimester as state-of-the-art pregnancy monitoring, facilitate the early diagnosis of this condition. We performed a systematic review on 67 reported cases of POC diagnosed in the first trimester of pregnancy (published from January 1980 to July 2019). The aim of our systematic review was twofold: to assess the main US findings in the first trimester of pregnancy and to increase awareness of early diagnostic possibilities. Our study showed that POC can be diagnosed in the first trimesterof pregnancy based on key US findings such as the association between omphalocele and ectopia cordis. When these two anomalies are present, increased nuchal translucency can also be considered a marker of POC in the first trimester.

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Section: Intracardiac Anomaliesmentioning

confidence: 99%

Cantrell syndrome in the first trimester of pregnancy: imagistic findings and literature review

et al. 2020

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Pentalogy of Cantrell

Newberry

England

2015

Advances in Neonatal Care

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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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Pentalogy of Cantrell: Embryofetoscopic Diagnosis

Cited by 3 publications

References 21 publications

Cantrell syndrome in the first trimester of pregnancy: imagistic findings and literature review

Cantrell syndrome in the first trimester of pregnancy: imagistic findings and literature review

Pentalogy of Cantrell

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

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