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Pediatric Radiology
Published in: Pediatric Radiology 7/2007

01-07-2007 | Original Article

Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases

Authors: Cinzia Orazi, M. Chiara Lucchetti, Paolo M. S. Schingo, Paola Marchetti, Fabio Ferro

Published in: Pediatric Radiology | Issue 7/2007

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Abstract

Background

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised.

Objective

To highlight the imaging diagnostic clues in this rare condition.

Materials and methods

We report on 11 adolescents with this condition.

Results

Sonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents.

Conclusion

Early and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally.
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Metadata
Title
Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases
Authors
Cinzia Orazi
M. Chiara Lucchetti
Paolo M. S. Schingo
Paola Marchetti
Fabio Ferro
Publication date
01-07-2007
Publisher
Springer-Verlag
Published in
Pediatric Radiology / Issue 7/2007
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-007-0497-y

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