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

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 pube...

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Detalles Bibliográficos
Autores principales: Del Vescovo, Riccardo, Battisti, Sofia, Di Paola, Valerio, Piccolo, Claudia L, Cazzato, Roberto L, Sansoni, Ilaria, Grasso, Rosario F, Zobel, Bruno Beomonte
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3314562/
https://www.ncbi.nlm.nih.gov/pubmed/22405336
http://dx.doi.org/10.1186/1471-2342-12-4
Descripción
Sumario: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.