Herlyn Werner Wunderlich Syndrome with Vagino-umbillical Fistula: A Most Unique Presentation
The Herlyn–Werner–Wunderlich (HWW) syndrome is an uncommon Mullerian duct anomaly composed of a triad of obstructed hemivagina, renal agenesis, uterine didelphys with a prevalence of <1/1,000,000 females. Aside from its usual presentation of cyclic hypogastric pain and abdominal mass, there has b...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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World Scientific Publishing
2021-03-01
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| Series: | Fertility & Reproduction |
| Subjects: | |
| Online Access: | http://www.worldscientific.com/doi/epdf/10.1142/S2661318221300014 |
| Summary: | The Herlyn–Werner–Wunderlich (HWW) syndrome is an uncommon Mullerian duct anomaly composed of a triad of obstructed hemivagina, renal agenesis, uterine didelphys with a prevalence of <1/1,000,000 females. Aside from its usual presentation of cyclic hypogastric pain and abdominal mass, there has been no other reported case in literature of the syndrome presenting with a vagino-umbilical fistula. Presented here is a unique case of a 35-year-old woman presenting with purulent, foul-smelling discharge extruding from a fistula on her umbilicus and her vagina associated with a tender hypogastric mass. Three-dimensional transvaginal ultrasonography aided the diagnosis of HWW syndrome with a concomitant vagino-umbilical fistula. Excision of the vaginal septum together with antibiotic coverage resolved her symptoms. The syndrome’s embryopathogenesis, classification, diagnostics, definitive surgical management and other issues during follow up are discussed. |
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| ISSN: | 2661-3182 2661-3174 |