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Open Access Highly Accessed Case report

Herlyn-werner-wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis

Riccardo Del Vescovo*, Sofia Battisti, Valerio Di Paola, Claudia L Piccolo, Roberto L Cazzato, Ilaria Sansoni, Rosario F Grasso and Bruno Beomonte Zobel

Author Affiliations

Department of Radiology, Campus Bio-Medico Univeristy of Rome, Italy

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BMC Medical Imaging 2012, 12:4  doi:10.1186/1471-2342-12-4

Published: 9 March 2012

Abstract

Background

Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian structures, and it is characterized by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Müllerian duct anomalies (MDA) are infrequently encountered in a routine clinical setting.

Cases presentation

two cases of HWW syndrome in adolescents and a differential diagnosis for one case of a different MDA, and the impact of magnetic resonance (MR) imaging technology to achieve the correct diagnosis.

Conclusions

MR imaging is a very suitable diagnostic tool in order to perform the correct diagnosis of HWW syndrome.