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Prenatal Diagnosis of Umbilicoportosystemic Shunts: Report of 11 Cases and Review of the Literature

Guillaume Gorincour1, Pierre Droullé2 and Laurent Guibaud1

1 Department of Pediatric and Fetal Imaging, Hôpital Debrousse, 29 Rue Soeur Bouvier, Cedex 05, Lyon 69322, France.
2 Department of Radiology, Maternité A. Pinard, Nancy, France.



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Fig. 1. Illustration shows hemodynamic theory for umbilical flow in embryo at 6 weeks' gestation. Arrow shows direction of flow. 1 = sinus venosus, 2 = common cardinal vein, 3 = posterior cardinal vein, 4 = parietal embryonic vein, 5 = superior mesenteric vein, 6 = vitelline vein, 7 = vitellomesenteric trunk, 8 = left umbilical vein, 9 = vitelloumbilical anastomosis, 10 = right umbilical vein, 11 = umbilical anastomosis, 12 = common prehepatic umbilical vein, 13 = liver, 14 = intestine beyond umbilical loop. (Reprinted with permission from [18].)

 


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Fig. 2. Female fetus at 24 weeks' gestation with direct entry of umbilical vein into right atrium. Transverse oblique sonographic image shows persistent prehepatic umbilical vein (arrowhead) joining right atrium (star).

 


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Fig. 3. One-day-old male neonate with shunt between umbilical vein and subcutaneous venous network. Profile view of postnatal angiogram obtained after umbilical vein catheterization shows subcutaneous venous network (small arrows) joining veins and superior vena cava (arrowhead), but also thin portal system (large arrow).

 


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Fig. 4A. Female fetus at 21 weeks' gestation with umbilicoportomesentericoiliac shunt. Coronal sonographic image shows aberrant anastomosis with iliac vein (arrow); note dilatation of inferior vena cava (star) and right atrium.

 


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Fig. 4B. Female fetus at 21 weeks' gestation with umbilicoportomesentericoiliac shunt. Coronal view of postmortem angiogram after umbilical vein catheterization (arrow) shows dilated superior mesenteric vein (star) joining iliac vein connection with inferior vena cava.

 


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Fig. 5. Male fetus at 32 weeks' gestation with shunt between vein and inferior vena cava. Transverse oblique sonographic image shows persistent right umbilical vein (star) joining inferior vena cava (arrow) at level of right kidney (RK).

 


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Fig. 6. Male fetus at 32 weeks' gestation with intrahepatic shunt. Transverse oblique sonographic images show multiple anastomoses (arrowheads, left) between persistent right umbilical vein (star, right) and right (arrowhead, right) and middle hepatic veins.

 

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