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

Research output: Contribution to journalArticle

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 syndromeis 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.

Original languageEnglish
Pages (from-to)657-665
Number of pages9
JournalPediatric Radiology
Volume37
Issue number7
DOIs
Publication statusPublished - Jul 2007

Keywords

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

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Pediatrics, Perinatology, and Child Health
  • Radiological and Ultrasound Technology

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