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The Herlyn-Werner-Wunderlich Syndrome - A Case Report with Radiological Review.

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Herlyn-Werner-Wunderlich (HWW) syndrome, a rare congenital anomaly, presents with uterus didelphys, obstructed hemivagina, and renal agenesis. Early diagnosis and intervention are crucial to prevent complications like endometriosis and infertility.

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DidelphisHematometraMullerian Ducts

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Area of Science:

  • Reproductive Medicine
  • Pediatric Surgery
  • Medical Imaging

Background:

  • Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly involving Mullerian and mesonephric ducts.
  • Characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis.
  • Presentation varies, often delayed, with symptoms including pelvic pain and dysmenorrhea due to hematocolpos or hematometra.

Observation:

  • A case of a 13-year-old patient with HWW syndrome presenting with dysmenorrhea and a palpable hypogastric mass.
  • Imaging (USG and MRI) revealed left renal agenesis, uterus didelphys with left hematometra and hematocervix, and a dilated left fallopian tube.
  • Unique features included left cervical and vaginal atresia (classification 1.2) alongside left renal agenesis.

Findings:

  • The case highlights the diagnostic utility of imaging modalities in identifying HWW syndrome.
  • Demonstrates a specific subtype (1.2) with associated renal and Mullerian duct anomalies.
  • Confirms the link between uterine and vaginal abnormalities and renal anomalies.

Implications:

  • Early diagnosis of HWW syndrome is essential for timely intervention.
  • Associated renal anomalies should be investigated in patients with uterine and vaginal abnormalities.
  • Prompt management can mitigate risks of endometriosis and infertility.