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2010-3-25-14 Prune-belly syndrome © Julien www.TheFetus.net


Prune-belly syndrome

Emmanuel Julien, MD.

Centre hospitalier Le Mans, France.

 
Case report

This a case of a 30-year-old G4P3 with non-contributive personal and family history. The first trimester ultrasound scan performed at 13 weeks of gestation showed dilated urinary bladder and normal nuchal translucency. Amniocentesis was suggested to the patient but she refused the procedure.

Follow-up ultrasound scans at 17 and 20 weeks showed abnormally dilated urinary bladder, decreased amount of the amniotic fluid and small echogenic kidneys. Ureters were not dilated. This was a male fetus with a single umbilical artery.

Surprisingly, we found a normal amount of the amniotic fluid on the ultrasound scan performed at 23 weeks. We thought that the uretral valve opened and that was the reason for the improvement of the ultrasound findings.

Subsequent ultrasounds did not show any signs of decreased amount of amniotic fluid or pulmonary hypoplasia. Both kidneys were growing and seemed well differentiated. Lower portions of both ureters were dilated.

Patient delivered at 39 weeks of gestation. The neonate's weight was 2600 grams.

The examination of the neonate revealed the fistula in the umbilical cord which connected cord with the urinary bladder via urachus. The reason for the lower urinary tract obstruction was a uretral stenosis caused by a prostatic gland.

The baby is now 3 weeks old and is still voiding via fistula in the urachus. The cathetrization of the baby was unsuccessful. The surgical correction is scheduled but the outcome and prognosis for the kidney function is uncertain.

Images 1,2: Images show a dilated ureters.




Images 3,4: Image 3 shows a dilated ureter. Image 4 shows dilated urinary bladder with single umbilical artery.



Images 5,6: Image 1 shows a dilated urinary bladder. Image 2 shows a transverse view of the fetal abdomen with dilated bladder, kidneys are without a pyelectasis.



Images 7,8: Image 7 shows a bladder dilation. Image 8 shows a male genitalia.



Images 9,10: Image 9 shows a sagittal view of the bladder and genitalia. Image 10 shows a normal four-chamber-view.



Image 11: Neonate after delivery, the wet stain on the sheet was caused by voiding via umbilical cord.




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