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2007-10-25-13 Currarino syndrome, partial form © Cuillier www.thefetus.net/
Currarino syndrome, partial form 

Fabrice Cuillier, MD*; E. Kaufmann, MD**; H. Randrianaivo, MD***.

*    Department of Gynecology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France;
**   Department of Gynecology, GHSR, 97600 Saint-Pierre, Ile de la Réunion, France;
***  Anatomo-pathologist, GHSR, 97600 Saint-Pierre, Ile de la Réunion, France.

 

Case report

This is a 39-year-old woman G2P1, with a noncontributive personal and familial history. Her ultrasound scan at 13 weeks was normal, but the biochemical triple test wasn"t done. An amniocentesis was performed at 18 weeks (46 XX, Inv 9). At 26 weeks the patient was seen in our antenatal unit and the ultrasound examination showed several abnormalities:

  • dilatation of the fourth cerebral ventricle (Images 1, 2);

  • complete agenesis of the corpus callosum (Images 3, 4, 5, 6);

  • thinned renal parenchyma with a dilatation of the renal pelvices (Images 7, 8) and the upper pole of ureters were distended (Image 9);

  • bilateral kidney duplication was suspected (Image 9);

  • bilateral ureterocele (Images 10, 11);

  • partial sacral agenesis was suspected (Video 8A, 8B).

  • sacral cyst was present (Images 12, 13);

  • the umbilical cord was thin, but with normal number of the umbilical vessels.

The parents opted for the termination of the pregnancy. The agenesis of the corpus callosum with the dilatation of the lateral and fourth ventricles, bilateral ureteral duplication with the megaureters and bilateral ureteroceles, and a partial sacral agenesis were found. The sacral radiography of the parents was normal. The final diagnosis was the partial form of Currarino syndrome.

Images 1, 2: 26 weeks of pregnancy; the images show transverse scans of the fetal posterior fossa of the skull - the fourth ventricle is dilated.

 

Images 3, 4: 26 weeks of pregnancy; the images show transverse scans of the fetal head. The lateral ventricles are dilated and the cavum septum pellucidum is missing. 

 

Images 5, 6: 26 weeks of pregnancy; the images show sagittal scans of the fetal head with the complete agenesis of the corpus callosum.

 

Images 7, 8: 26 weeks of pregnancy; the images show thinned renal parenchyma with a dilatation of the renal pelvises.

 

Images 9: 26 weeks of pregnancy; transverse scan of the fetal abdomen - bilateral kidney duplication was suspected.

Images 10, 11: 26 weeks of pregnancy; transverse scans of the fetal abdomen showing the ureterocele.

 

Images 12, 13: 26 weeks of pregnancy; transverse scans show a fetal sacral cyst.

 

 

Video 1, 2: The video 1 shows transverse scan of the fetal head with the agenesis of the corpus callosum (lateral ventricles are dilated, cavum septum pellucidum is missing). The video 2 shows sagittal scan of the sacral region of the fetus with the partial sacral agenesis.

 

Video 3: 26 weeks of pregnancy; the video shows a transverse scan of the fetal abdomen. A thinned renal parenchyma with a dilatation of the renal pelvises and megaureters are visible.

 

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