Advertisement

Pediatric Radiology

, Volume 37, Issue 7, pp 657–665 | Cite as

Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases

  • Cinzia Orazi
  • M. Chiara Lucchetti
  • Paolo M. S. Schingo
  • Paola Marchetti
  • Fabio Ferro
Original Article

Abstract

Background

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised.

Objective

To highlight the imaging diagnostic clues in this rare condition.

Materials and methods

We report on 11 adolescents with this condition.

Results

Sonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents.

Conclusion

Early and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally.

Keywords

Müllerian duct anomaly Renal agenesis Haematocolpos Herlyn-Werner-Wunderlich syndrome MRI Ultrasonography 

References

  1. 1.
    Sadler TW, Langman J (eds) (2000) Langman’s medical embryology, 8th edn. Lippincott Williams & Wilkins, PhiladelphiaGoogle Scholar
  2. 2.
    Gassner I, Geley TE (2004) Ultrasound of female genital anomalies. Eur Radiol 14:107–122CrossRefGoogle Scholar
  3. 3.
    Kiechl-Kohlendorfer U, Geley TE, Unsinn KM et al (2001) Diagnosing neonatal female genital anomalies using saline-enhanced sonography. AJR 177:1041–1044PubMedGoogle Scholar
  4. 4.
    Acien P (1992) Embryological observations on the female genital tract. Hum Reprod 7:437–445PubMedGoogle Scholar
  5. 5.
    American Fertility Society (1988) The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Mullerian duct anomalies and intrauterine adhesions. Fertil Steril 49:944–955Google Scholar
  6. 6.
    Troiano RN, McCarthy SM (2004) Mullerian duct anomalies: imaging and clinical issues. State of the art. Radiology 233:19–34PubMedCrossRefGoogle Scholar
  7. 7.
    Fielding JR (1996) MR Imaging of Mullerian anomalies: impact on therapy. AJR 167:1491–1495PubMedGoogle Scholar
  8. 8.
    Heinonen PK (2000) Clinical implications of the didelphic uterus: long-term follow-up of 49 cases. Eur J Obstet Gynecol Reprod Biol 91:183–190PubMedCrossRefGoogle Scholar
  9. 9.
    Gholoum S, Puligandla PS, Hui T et al (2006) Management and outcome of patients with combined vaginal septum, bifid uterus and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). J Pediatr Surg 41:987–992PubMedCrossRefGoogle Scholar
  10. 10.
    Colavita N, Orazi C, Logroscino C et al (1986) Does MURCS association represent an actual nonrandom complex of malformations? Diagn Imaging Clin Med 55:172–176PubMedGoogle Scholar
  11. 11.
    Tanaka YO, Kurosaki Y, Kobayashi T et al (1998) Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases. Abdom Imaging 23:437–441PubMedCrossRefGoogle Scholar
  12. 12.
    Zurawin RK, Dietrich JE, Heard MJ et al (2004) Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature. J Pediatr Adolesc Gynecol 17:137–141PubMedCrossRefGoogle Scholar
  13. 13.
    Acien P, Arminana E, Garcia-Ontiveros E (1987) Unilateral renal agenesis associated with ipsilateral blind vagina. Arch Gynecol 240:1–8PubMedCrossRefGoogle Scholar
  14. 14.
    Acien P, Acien M, Sanchez-Ferrer M (2004) Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod 19:2377–2384PubMedCrossRefGoogle Scholar
  15. 15.
    Hoeffel C, Olivier M, Scheffler C et al (1997) Uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Case report. Eur J Radiol 25:246–248PubMedCrossRefGoogle Scholar
  16. 16.
    Stassart JP, Nagel TC, Prem KA et al (1992) Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis: the University of Minnesota experience. Fertil Steril 57:756–761PubMedGoogle Scholar
  17. 17.
    Candiani GB, Fedele L, Candiani M (1997) Double uterus, blind hemivagina and ipsilateral renal agenesis: 36 cases and long-term follow-up. Obstet Gynecol 90:26–32PubMedCrossRefGoogle Scholar
  18. 18.
    Purslow CE (1922) A case of unilateral haematocolpos, haematomethra and haematosalpinx. J Obstet Gynaecol Br Emp 29:643Google Scholar
  19. 19.
    Velasquez N, Fernandez GE, Andrade JM et al (2000) Sindrome de utero doble, hemivagina ostruida y agenesia renal homolateral. Rev Obstet Ginecol Venez 60:245–253Google Scholar
  20. 20.
    Siegelman ES, Outwater EK, Banner MP et al (1997) High-resolution MR imaging of the vagina. Radiographics 17:1183–1203PubMedGoogle Scholar
  21. 21.
    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 Frauenheilkd 31:340–347PubMedGoogle Scholar
  22. 22.
    Wunderlich M (1976) Unusual form of genital malformation with aplasia of the right kidney. Zentralbl Gynakol 98:559–562PubMedGoogle Scholar
  23. 23.
    Haddad B, Barranger E, Paniel BJ (1999) Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod 14:1962–1964PubMedCrossRefGoogle Scholar
  24. 24.
    Prada Arias M, Muguerza Vellibre R, Montero Sanchez M et al (2005) Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Eur J Pediatr Surg 15:441–445PubMedCrossRefGoogle Scholar
  25. 25.
    Pieroni C, Rosenfeld DL, Mokrzycki ML (2001) Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. A case report. J Reprod Med 46:133–136PubMedGoogle Scholar
  26. 26.
    Amagai T, Ohkawa H, Kaneko M (1999) Endoscopy septotomy: a new surgical approach to infantile hydrometrocolpos with imperforate hemivagina and ipsilateral renal agenesis. J Pediatr Surg 34:628–631PubMedCrossRefGoogle Scholar
  27. 27.
    Hinckley MD, Milki AA (2003) Management of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. A case report. J Reprod Med 48:649–651PubMedGoogle Scholar
  28. 28.
    Rock JA, Jones HW (1980) The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol 138:339–342PubMedGoogle Scholar
  29. 29.
    Cicinelli E, Romano F, Didonna T et al (1999) Resectoscopic treatment of uterus didelphys with unilateral imperforate vagina complicated by hematocolpos and hematometra: case report. Fertil Steril 72:553–555PubMedCrossRefGoogle Scholar
  30. 30.
    Ballesio L, Andreoli C, De Cicco ML et al (2003) Hematocolpos in double vagina associated with uterus didelphus: US and MR findings. Case report. Eur J Radiol 45:150–153PubMedCrossRefGoogle Scholar
  31. 31.
    Merrot T, Lumenta DB, Tercier S et al (2006) Multicystic dysplastic kidney with ipsilateral abnormalities of genito-urinary tract: experience in children. Urology 67:603–607PubMedCrossRefGoogle Scholar
  32. 32.
    Li S, Qayyum A, Coakley FV et al (2000) Association of renal agenesis and mullerian duct anomalies. J Comput Assist Tomogr 24:829–834PubMedCrossRefGoogle Scholar
  33. 33.
    Burbige KA, Hensle TW (1984) Uterus didelphys and vaginal duplication with unilateral obstruction presenting as a newborn abdominal mass. J Urol 132:1195–1198PubMedGoogle Scholar
  34. 34.
    Scarsbrook AF, Moore NR (2003) MRI appearance of Mullerian duct abnormalities. Clin Radiol 58:747–754PubMedCrossRefGoogle Scholar
  35. 35.
    Pellerito JS, McCarthy SM, Doyle MB et al (1992) Diagnosis of uterine anomalies: relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology 183:795–800PubMedGoogle Scholar
  36. 36.
    Marten K, Vosshenrich R, Funke M et al (2003) MRI in the evaluation of mullerian anomalies. Clin Imaging 27:346–350PubMedCrossRefGoogle Scholar
  37. 37.
    Batt RE, Mitwally MF (2003) Endometriosis from thelarche to midteens: pathogenesis and prognosis, prevention and pedagogy. J Pediatr Adolesc Gynecol 16:337–347PubMedCrossRefGoogle Scholar
  38. 38.
    Sanfilippo JS, Wakim NG, Schikler KN et al (1986) Endometriosis in association with uterine anomaly. Am J Obstet Gynecol 154:39–43PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • Cinzia Orazi
    • 1
  • M. Chiara Lucchetti
    • 3
  • Paolo M. S. Schingo
    • 1
    • 2
  • Paola Marchetti
    • 4
  • Fabio Ferro
    • 3
  1. 1.Department of Diagnostic ImagingI.R.C.C.S. Bambino Gesù Pediatric HospitalRomeItaly
  2. 2.RomeItaly
  3. 3.Operative Unit of Andrological and Gynecological SurgeryI.R.C.C.S. Bambino Gesù Pediatric HospitalRomeItaly
  4. 4.Department of General SurgeryI.R.C.C.S. Bambino Gesù Pediatric HospitalRomeItaly

Personalised recommendations