Acta Scientific Women's Health (ASWH)

Case Report Volume 6 Issue 6

Case Report: Diagnosing and Treating a 11 Year Old Girl with Herlyn-werner-wunderlich Syndrome

Amrita Razdan Kaul*

Senior Consultant, OBG Department, Asian Institute of Medical Sciences, Faridabad, Haryana, India

*Corresponding Author: Amrita Razdan Kaul, Senior Consultant, OBG Department, Asian Institute of Medical Sciences, Faridabad, Haryana, India.

Received: April 10, 2024; Published: May 16, 2024

Abstract

Introduction: Herlyn- Werner-Wunderlich syndrome is a very rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina and mesonephric duct anomalies. Most often the later manifests as renal agenesis [1]. Incidence of this condition is 0.1-3% [2].

The patient is usually asymptomatic in early childhood, onset of symptoms occur after menarche and common complaints are progressive dysmenorrhea, recurrent pain abdomen and palpable lump over lower abdomen. Patient can also present with urinary problems like recurrent UTI, vesico- ureteric reflux, increased risk of hypertension, compensatory hypertrophy of existing kidney, in severe cases CKD and ESRD which occur as complication of unilateral solitary kidney.

Case Report: Case report presented here is of an 11 year old girl who presented with pain lower abdomen and pelvic region, fever, palpable tender mass over right lower abdomen and urinary complaints. Further evaluation of the case with biochemical parameters and radiological modalities like USG and MRI confirmed the diagnosis as HWW syndrome complicated with CKD after which surgical management of the case was followed.

Conclusion: Duplication of the uterus and cervix with obstructed hemivagina and iplsilateral renal agenesis (presence of both) should prompt the clinicians to think in the direction of HWW syndrome. Early diagnosis and prompt treatment of the condition can prevent possible serious complication in future.

Keywords: Herlyn Werner Wunderlich Syndrome; Müllerian Duct; Wolffian Duct; Uterus Didelphys

References

  1. Mandava Anitha., et al. “OHVIRA syndrome”. Journal of Paediatrics and Adolescent Gynaecology (2012).
  2. Aveiro Ana Cristina., et al. BMJ Case report (2011).
  3. Burgis J. “Obstructive mullerian anomalies: case report, diagnosis and management”. American Journal of Obstetrics and Gynecology (2001).
  4. Wilson D and Bordoni B. Embriology, Mullerian ducts (Para mesonephric ducts): Stat Pearls: Orlando, FL, USA, (2021).
  5. Zhu L. “New classification of HWWS”. Chinese Medical Journal (Engl) (2015).
  6. Jindal G., et al. “Uterus didelphys with unilateral obstructed hemivagina, hematocolpos, hematosalpinx with unilateral renal agenesis”. Journal of Reproduction Science (2009).
  7. Zhu L., et al. “New classification of Herlyn -Werner-Wunderlich syndrome”. Chinese Medical Journal (Engl) (2015).
  8. Vercellini P., et al. “Asymmetrical 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 (2007).
  9. Angotti R., et al. “Herlyn- Werner- Wunderlich syndrome: an early onset case report and review of literature”. International Journal of Surgery Case Reports (2015).
  10. Rechberger T., et al. “Congenital anomalies of the female reproductive tract- diagnosis and management”. Cureus (2024).
  11. Guifeng Jia., et al. “A case report on Herlyn- Werner- Wunderlich syndrome with spontaneous abortion”. Medicine (Baltimore)36 (2018): e12004.

Citation

Citation: Amrita Razdan Kaul. “Case Report: Diagnosing and Treating a 11 Year Old Girl with Herlyn-werner-wunderlich Syndrome".Acta Scientific Women's Health 6.6 (2024): 05-07.

Copyright

Copyright: © 2024 Amrita Razdan Kaul. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.




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