Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases
- 1.4k Downloads
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.
To highlight the imaging diagnostic clues in this rare condition.
Materials and methods
We report on 11 adolescents with this condition.
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.
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.
KeywordsMüllerian duct anomaly Renal agenesis Haematocolpos Herlyn-Werner-Wunderlich syndrome MRI Ultrasonography
- 1.Sadler TW, Langman J (eds) (2000) Langman’s medical embryology, 8th edn. Lippincott Williams & Wilkins, PhiladelphiaGoogle Scholar
- 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
- 18.Purslow CE (1922) A case of unilateral haematocolpos, haematomethra and haematosalpinx. J Obstet Gynaecol Br Emp 29:643Google Scholar
- 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