PMC5106532_01
Female with Pentology of Cantrell. Axial prenatal sonogram image of the fetus at week 33 with ectopia cordis (arrowhead) and omphalocele (arrow) containing liver.
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This sonogram showed a female fetus with a large upper anterior abdominal wall defect (omphalocele) containing liver and stomach with marked ectopia cordis (Figure 1).
Female with Pentology of Cantrell. Cross-table lateral radiograph of the thorax and abdomen of the infant shortly after birth. The ectopia cordis (arrow) on the left is severe with an adjacent smaller extruding gastrointestinal omphalocele (arrowhead).
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On post partum day 1, a cross table lateral radiograph was taken before the omphalocele was repaired and the heart enclosed in surrounding fascia and subcutaneous tissue (Figure 2).
Female with Pentology of Cantrell. 64-slice volume-rendered MDCT image of the patient 7 months postpartum, showing the external position of the anterior heart beneath the skin (arrow).
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The infant was monitored for the next 18 months, during which she was hospitalized for worsening respiratory distress marked by hypoventilation and cyanosis intensified by various infections and secondary complications. 3D multidetector computed tomography (dual-source 64 slice MDCT, Siemens Medical Systems ) was performed at 7 months to assess the cardiac, lung and abdominal abnormalities using 64 slice with 3D post processing (Figure 3, Figure 4, Figure 5).
Female with Pentology of Cantrell. Thoracic cross-section of the patient 7 months postpartum, showing the ectopia cordis (arrow) external to the rib cage and diffuse chronic lung disease due to pulmonary hypoplasia (asterisks).
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The infant was monitored for the next 18 months, during which she was hospitalized for worsening respiratory distress marked by hypoventilation and cyanosis intensified by various infections and secondary complications. 3D multidetector computed tomography (dual-source 64 slice MDCT, Siemens Medical Systems ) was performed at 7 months to assess the cardiac, lung and abdominal abnormalities using 64 slice with 3D post processing (Figure 3, Figure 4, Figure 5).
Female with Pentology of Cantrell. 64 slice volume rendered (a) and reconstructed maximum intensity projection (MIP, b) MDCT images of the patient 7 months after birth showing ectopia cordis (arrow) with CT angiography. The external position of the heart and subpulmonary stenosis caused by the indenting sternum (arrowheads) are notable with post-stenotic main pulmonary artery dilatation (asterisk).
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The infant was monitored for the next 18 months, during which she was hospitalized for worsening respiratory distress marked by hypoventilation and cyanosis intensified by various infections and secondary complications. 3D multidetector computed tomography (dual-source 64 slice MDCT, Siemens Medical Systems ) was performed at 7 months to assess the cardiac, lung and abdominal abnormalities using 64 slice with 3D post processing (Figure 3, Figure 4, Figure 5). This was localized to the region where the right ventricular outflow tract crossed posterior to the closed sternum (Figure 5).