P02.24: Cloacal exstrophy: images during first trimester of pregnancy

Abstract

A 26-year-old woman underwent a prenatal diagnosis ultrasonography at 11 weeks of gestation. The fetus had a crown-rump length of 54mm, with normal nuchal translucency (0,94 mm). Multiple fetal abnormalities were seen, included a large abdominal defect with evisceration of liver and bowel. The bladder was not visualized and there was a single umbilical artery. The parents denied amniocentesis procedure, and the patient decided to search a second opinion in another prenatal diagnosis unit. After the delivery, cloacal exstrophy was confirmed.The following findings are described for prenatal diagnosis of cloacal exstrophy: Absent bladder (vesical exstrophy), lower abdominal wall defect with genitourinary anomalies and distal ileum prolapsed. Also we can see the protrusion of the ileum seems like a trunk of elephant, the so called ''elephant trunk sign'', but this can be seen on the second trimester. However, the images of this anomaly in the first trimetre, are very rare.We show the images that we could see on the first echography.Supporting information can be found in the online version of this abstract Methods:In an attempt to characterize portal system and shunt developmental variations and their possible impact on outcome, the diameter of the shunt was compared with the diameter of the umbilical vein and the entire portal vasculature was assessed.Absence of the normal intrahepatic umbilical vein and ductus venosus has been described in cases with extrahepatic insertion of the umbilical vein. In these prenatally and postnatally diagnosed cases, the umbilical vein was described to enter into the right atrium, the inferior vena cava or the iliac vein. The majority of these cases were associated with other anomalies. Results: A 24-year-old primagravida woman was referred for a rutin first trimester scan at 13 weeks of gestation. Biometry in standard planes showed appropriate fetal growth. Biometry in standard planes showed appropriate fetal growth. The anatomical survey of the fetus and sequential segmental analysis of heart performed. A ductus venosus could not be demonstrated during venous blood flow studies. Dilated umbilical vein-3,3 mm bypassing the liver and connecting directly to the right atrium. Stenosis of A.pulmonalis and the initial expansion of the urinary bladder to sense megaciste were detected. Subsequent chorionic villus sampling showed a normal 46 XX karyotype and then a pregnancy was aborted. If the shunt is narrow, the portal system will have developed normally. A wide shunt is associated with underdevelopment or absence of the portal system. In cases of ADV with extrahepatic shunt, prognosis is determined by the severity of associated anomalies, the diameter of the shunt and development of the portal system. Conclusions: This report suggests that the detection of the DV agenesis in the first trimester is possible.We suggest that fetuses with absent ductus venosus warrant a careful search for associated anomalies. Fetal karyotyping should be offered and serial examinations are recommended.In cas...