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2006-04-04-8 Answer to the case of week © Fox

Answer to the case of the week #170

June 23 - July 6, 2006

David Fox, MD

Riverside Methodist Hospital, Columbus, Ohio, USA

This is a 36-year-old, G2P1, referred to our unit at 26 weeks’ gestation. The first trimester screening revealed a thick nuchal translucency. After that, an amniocentesis was performed with normal result (46XX).The ultrasound findings were:

  • left-sided multicystic, dysplastic kidney
  • mild contra lateral hydronephrosis
  • single umbilical artery
  • midline septated structure posterior to the bladder
  • dense ascites
  • normal amniotic fluid volume

The patient was followed semi-weekly with biophysical profiles, and biweekly for growth scans. A follow-up ultrasound revealed a second, smaller septated cystic structure- a mirror image of the first- anterior to the initial cystic structure and posterior to the bladder. By 31 weeks’ gestation the ascites had completely resolved as the initial cystic lesion increased in relative size. The anus could not be visualized. At 31 weeks’ gestation, fetal testing became non-reassuring, and a cesarean delivery was performed of a 1283 gram female with one and five-minute Apgar scores of 5 and 9, respectively. The infant had an imperforate anus and absent vaginal opening.

In the immediate postnatal period, the infant was noted to have hyperbilirubinemia and excessive bleeding from phlebotomy, and the latter led to a diagnosis of disseminated intravascular coagulation of uncertain etiology. This resolved after 1-2 days, allowing for colostomy. Exploration of the abdominal cavity revealed uterus didelphys. The uterus drained a large amount of fluid that was felt to be a mixture of urine and uterine/vaginal secretions. The left kidney was multicystic dysplastic. The sigmoid colon was extremely distended.


Transverse view of fetal pelvis. Note the bladder on left and the didelphic uterus with hydrometrocolpos on the right.

Left image: Left multicystic kidney disease.  Right image: Intrafetal umbilical vein

Left multicystic kidney disease and mild hydronephrosis on the right kidney

Renal Doppler arteries

Cerebral and umbilical artery Doppler

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