Case of the Week # 35

L. Gourand MD; V. Mirlesse MD; B. Bessieres MD; F. Jacquemard MD; R. Levy MD; F. Daffos MD

September 29-October 13, 2000

Service de Medecine Foetale, Institut de Puericulture, 26 Boulevard Brune 75014 Paris, France

This second trimester fetus was referred for a cystic pelvic mass. The following images were obtained:

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It was subsequently decided to punction the mass. These are the images that were obtained after the punction. A subtle finding is now visible that was not appreciated before the punction. That finding led to propose a new diagnosis. Laboratory results obtained on the fluid supported the new diagnosis.

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Answer

This second trimester fetus was referred for a cystic pelvic mass. The following images were obtained which demonstrated a cyst behind the bladder

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It was subsequently decided to drain the mass. These are the images that were obtained after the punction. A subtle finding is now visible that was not appreciated before the punction. There is some anomaly of the sacrum on the left of the cyst in the image below. Note that the sacrum is irregular due to a small defect. That finding led to propose a new diagnosis. Laboratory results obtained on the fluid supported the new diagnosis.

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Before the drainage the working diagnosis was that of of hydrocolpos. However the sacral defect changed that.

In OMIM the association of "sacrum" and "meningocele" or "teratoma" provided the diagnosis of Currarino syndrome or triad. Other differential diagnoses include:

312800 Sacral defect with anterior sacral meningocele
#176450 Currarino syndrome
*600145 Sacral defect with anterior meningocele  
*142994 Homeo box gene HB9; HLXB9

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