Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare anomaly characterized by Müllerian duct anomalies (MDA) associated with mesonephric duct anomalies 3. This entity is also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). It is not to be confused with the Wunderlich syndrome.
Presentation is typically after menarche with pelvic pain due to the obstructed hemivagina. There may be a palpable pelvic mass.
Herlyn-Werner-Wunderlich (HWW) syndrome is characterized by a triad of type III Müllerian duct anomaly (uterus didelphys), obstructed hemivagina and mesonephric duct anomalies. Most often the latter manifests as renal agenesis 3 but duplicated kidneys, dysplastic kidneys, rectovesical bands or crossed fused ectopia may also be seen.
Irrespective of imaging modality, the typical findings are:
- duplication of the uterus, cervix and vagina
- unilateral hematocolpos or hematometrocolpos
- absent kidney on the same side as the uterovaginal obstruction
- Gartner duct cysts
Treatment and prognosis
Treatment is surgical, with resection of the septum dividing the two hemivaginas in order to relieve the obstruction. Subsequent successful pregnancy in the obstructed uterus has been reported 5.
When uterovaginal obstruction results in significant dilatation, the imaging differential may include a large adnexal mass such as a cystadenoma or endometrioma.
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- 2. Madureira AJ, Mariz CM, Bernardes JC et-al. Case 94: Uterus didelphys with obstructing hemivaginal septum and ipsilateral renal agenesis. Radiology. 2006;239 (2): 602-6. doi:10.1148/radiol.2392031187 - Pubmed citation
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- 4. Tanaka YO, Kurosaki Y, Kobayashi T et-al. Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases. Abdom Imaging. 23 (4): 437-41. Abdom Imaging (link) - Pubmed citation
- 5. Altchek A, Paciuc J. Successful pregnancy following surgery in the obstructed uterus in a uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: case report and literature review. J Pediatr Adolesc Gynecol. 2009;22 (5): e159-62. doi:10.1016/j.jpag.2009.02.001 - Pubmed citation
- 6. Troiano RN, Mccarthy SM. Mullerian duct anomalies: imaging and clinical issues. Radiology. 2004;233 (1): 19-34. doi:10.1148/radiol.2331020777 - Pubmed citation
- 7. Ahmad Z, Goyal A, Das CJ et-al. Herlyn-Werner-Wunderlich syndrome presenting with infertility: Role of MRI in diagnosis. Indian J Radiol Imaging. 2013;23 (3): 243-6. doi:10.4103/0971-3026.120283 - Free text at pubmed - Pubmed citation