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Spectrum of Cantrell’s Pentalogy: Case Series From a Single Tertiary Care Center and Review of the Literature

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Abstract

The pentalogy of Cantrell (PC) was first described in 1958. It includes five anomalies: a deficiency of the anterior diaphragm, a midline supraumbilical abdominal wall defect, a defect in the diaphragmatic pericardium, various congenital intracardiac abnormalities, and a defect of the lower sternum. Five patients showing the PC spectrum are reported. The report presents the prenatal diagnosis, the postnatal course, and the patients’ outcome at a tertiary care center from June 2001 to May 2012. A literature review and the management plan for this group of patients also are discussed. All patient data were obtained via electronic medical records retrospectively after approval by the institutional review board at the home institution. The patients in the study were three males and two females. For all of the patients, a prenatal diagnosis had been determined. The mean gestational age at delivery was 36.6 weeks. One patient had associated cranial and spine malformations. All the patients had associated congenital heart disease but a normal karyotype. Four of the five patients died in the first year of life. The ages at death ranged from 0 to 259 days (mean, 46.2 ± 51.8 days). The patients who did not survive had withdrawal of care due to increased morbidity, associated complications, or parental wishes. The pentalogy of Cantrell is a wide spectrum of associations. Patients with the complete PC together with complex congenital heart disease or extracardiac malformations may have a poor prognosis. Incomplete PC cases may have a better outcome based on associated anomalies. Prenatal counseling plays a very important role in the decision-making process for the families and has a significant impact on the postnatal management. A multidisciplinary team approach is essential for successful postpartum outcomes.

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Correspondence to Sawsan Awad.

Electronic supplementary material

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Patient B: Long-axis view of the thorax and abdomen demonstrating the ectopia cordis and omphalocele (AVI 17,193 kb)

Patient B: Cross-section of the thorax demonstrating the apical portion of the heart outside the thorax (AVI 41,682 kb)

Patient B: Zoomed view of the heart demonstrating side-by-side great vessels and the aorta overriding the ventricular septal defect (VSD) (AVI 16,157 kb)

Patient C: Short-axis view of the thorax demonstrating the sternal cleft (AVI 26,238 kb)

Patient D: Long-axis view of the thorax and abdomen demonstrating an abnormal cardiac axis with the apex directed anteriorly and superiorly. A cystic structure in the left hemithorax can be seen (AVI 14,444 kb)

Patient D: Short-axis view of the thorax demonstrating dextroposition of the heart and the cystic structure in the left hemithorax (AVI 21,967 kb)

Patient D: Two-dimensional echocardiogram and color Doppler of the short-axis view of the thorax demonstrating the dextroposition of the heart and the cystic structure in the left hemithorax (AVI 12,571 kb)

Movie clip 1a

Patient B: Long-axis view of the thorax and abdomen demonstrating the ectopia cordis and omphalocele (AVI 17,193 kb)

Movie clip 1b

Patient B: Cross-section of the thorax demonstrating the apical portion of the heart outside the thorax (AVI 41,682 kb)

Movie clip 1c

Patient B: Zoomed view of the heart demonstrating side-by-side great vessels and the aorta overriding the ventricular septal defect (VSD) (AVI 16,157 kb)

Movie clip 2

Patient C: Short-axis view of the thorax demonstrating the sternal cleft (AVI 26,238 kb)

Movie clip 3a

Patient D: Long-axis view of the thorax and abdomen demonstrating an abnormal cardiac axis with the apex directed anteriorly and superiorly. A cystic structure in the left hemithorax can be seen (AVI 14,444 kb)

Movie clip 3b

Patient D: Short-axis view of the thorax demonstrating dextroposition of the heart and the cystic structure in the left hemithorax (AVI 21,967 kb)

Movie clip 3c

Patient D: Two-dimensional echocardiogram and color Doppler of the short-axis view of the thorax demonstrating the dextroposition of the heart and the cystic structure in the left hemithorax (AVI 12,571 kb)

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Mallula, K.K., Sosnowski, C. & Awad, S. Spectrum of Cantrell’s Pentalogy: Case Series From a Single Tertiary Care Center and Review of the Literature. Pediatr Cardiol 34, 1703–1710 (2013) doi:10.1007/s00246-013-0706-4

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Keywords

  • Cantrell’s pentalogy
  • Cantrell’s syndrome
  • Pentalogy of Cantrell