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Herlyn-Werner-Wunderlich Syndrome

Reference work entry

Herlyn-Werner-Wunderlich syndrome is a rare Müllerian anomaly consisting of a didelphic uterus, a hemivaginal septum, and ipsilateral renal agenesis. The diagnosis should be suspected in adolescent females with a pelvic mass and ipsilateral renal agenesis.

Synonyms and Related Disorders

Blind hemivagina with ispsilateral renal agenesis

Genetics/Basic Defects

  1. 1.

    Anatomical defect is caused by a failure of lateral fusion of Müllerian ducts (didelphic/septate uterus and double vagina) combined with a failure of vertical fusion between the Müllerian duct and the urogenital sinus (hemivaginal septum) ipsilateral to the side of the renal agenesis.

     
  2. 2.
    Various types of uterine anomalies secondary to nondevelopment or failure of fusion of the distal segments of the Müllerian ducts
    1. a.

      Hypoplasia/agenesis of the uterus

       
    2. b.

      Unicornuate uterus

       
    3. c.

      Didelphic uterus

       
    4. d.

      Bicornuate uterus

       
    5. e.

      Septate uterus

       
    6. f.

      Arcuate uterus

       
     
  3. 3.
    Various types of vaginal anomalies secondary to the different...

Keywords

Esophageal Atresia Renal Agenesis Imperforate Anus Urogenital Sinus Vaginal Septum 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Gholoum, S., Puligandla, P., Hui, T., et al. (2006). Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). Journal of Pediatric Surgery, 41, 987–992.PubMedCrossRefGoogle Scholar
  2. Heinonen, P. K. (1982). Longitudinal vaginal septum. European Journal of Obstetrics, Gynecology, and Reproductive Biology, 13, 253–258.PubMedCrossRefGoogle Scholar
  3. Heinonen, P. K. (2000). Clinical implications of the didelphic uterus: Long-term follow-up of 49 cases. European Journal of Obstetrics, Gynecology, and Reproductive Biology, 91, 183–190.PubMedCrossRefGoogle Scholar
  4. Herlyn, U., & Werner, H. (1971). Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Geburtshilfe und Frauenheilkunde, 31, 340–347.PubMedGoogle Scholar
  5. Madureira, A. J., Maiz, C. M., Bernardes, J. C., et al. (2006). Case 94: Uterus didelphys with obstructing hemivaginal septum and ipsilateral renal agenesis. Radiology, 239, 602–606.PubMedCrossRefGoogle Scholar
  6. OMIM #192050: Uterus bicornis bicollis with partial vaginal septum and unilateral hematocolpos with ipsilateral renal agenesis. Updated June 7, 1995.Google Scholar
  7. Orazi C, Lucchetti C, Schingo PMS, et al: Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 37:657–665, 2007.PubMedCrossRefGoogle Scholar
  8. Rana, R., Pasrija, S., & Puri, M. (2008). Herlyn-Werner-Wunderlich syndrome with pregnancy: A rare presentation. Congenital Anomalies, 48, 142–143.PubMedCrossRefGoogle Scholar
  9. Rock, J. A., & Jones, H. W., Jr. (1980). The double uterus associated with an obstructed hemivaginal and ipsilateral renal agenesis. American Journal of Obstetrics and Gynecology, 138, 339–342.PubMedGoogle Scholar
  10. Troiano, R. N., & McCarthy, S. M. (2004). Müllerian duct anomalies: Imaging and clinical issues. Radiology, 233, 19–34.PubMedCrossRefGoogle Scholar
  11. Vercellini, P., Daguati, R., Somigliana, E., et al. (2007). Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: Institutional case series and a systematic literature review. Fertility and Sterility, 87, 719–724.PubMedCrossRefGoogle Scholar
  12. Wiersma, A. F., Peterson, L. F., & Justema, E. J. (1976). Uterine anomalies associated with unilateral renal agenesis. Obstetrics and Gynecology, 47, 654–657.PubMedGoogle Scholar
  13. Wunderlich, M. (1976). Unusual form of genital malformation with aplasia of the right kidney. Zentralblatt für Gynäkologie, 98, 559–562.PubMedGoogle Scholar
  14. Zurawin, R. K., Dietrich, J. E., Heard, M. J., et al. (2004). Didelphic uterus and obstructed hemivaginal with renal agenesis (case report) and review of the literature. Journal of Pediatric and Adolescent Gynecology, 17, 137–141.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

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