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Herlyn-Werner-Wunderlich syndrome: clinical considerations and management
  1. Wagner Horst1,2,
  2. Rafael Cardoso de Melo3,4,
  3. Giulia Theilacker1 and
  4. Betina Schmitt5
  1. 1School of Medicine, Faculdade Estacio de Jaraguá do Sul, Jaraguá do Sul, Santa Catarina, Brazil
  2. 2Health and Environment Postgraduation Program, UNIVILLE, Joinville, Santa Catarina, Brazil
  3. 3Radiology, Hospital Divina Providência, Porto Alegre, Rio Grande do Sul, Brazil
  4. 4Radiology, Complexo Hospitalar Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil
  5. 5School of Medicine, PUCPR, Curitiba, PR, Brazil
  1. Correspondence to Wagner Horst; horst.wagner{at}estacio.br

Abstract

Herlyn-Werner-Wunderlich syndrome (HWWS), defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct malformation, usually diagnosed after menarche, when symptoms related to haematocolpos arise. We report a case of a 14-year-old patient who presented to the emergency department complaining of proctalgia and pelvic pain treated in our medical centre. Ultrasound and abdomino-pelvic MRI imaging studies confirmed the diagnosis. Treatment was surgical incision of the vaginal septum. At the follow-up visit, after the initial procedure, excess vaginal tissue was excised using a hysteroscopic approach during diagnostic vaginoscopy. Vaginoscopy-assisted treatment of the patient proved to be a safe and effective minimally invasive treatment modality that resulted in symptomatic relief and fertility preservation. In conclusion, although premenarche is asymptomatic in the vast majority of cases, HWWS would be optimally diagnosed in childhood to avoid acute late complications, although it is usually first diagnosed after menarche as a result of haematocolpos. Gynaecologists should consider the syndrome in the presence of pelvic mass, renal agenesis, menstrual changes and cyclic pelvic pain.

  • obstetrics and gynaecology
  • radiology
  • congenital disorders
  • surgery
  • ultrasonography

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Footnotes

  • Twitter @waghorst

  • Contributors WH and BS conceived of the study. BS and GT wrote the manuscript with support from WH and RCdM analysed imaging findings. WH helped supervise the project. All authors contributed to refinement of the case report and approved the final manuscript. All authors agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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