Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos

Area of Science:

• Reproductive Medicine
• Medical Imaging
• Congenital Anomalies

Background:

• Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare congenital condition affecting Mullerian duct development.
• It is characterized by a duplicated uterus and vagina with one side obstructed, often accompanied by a kidney anomaly on the same side.
• While Mullerian duct anomalies affect 2-3% of women, OHVIRA accounts for a small subset, with few cases involving pyocolpos.

Observation:

• Symptoms typically manifest post-menarche, presenting as dysmenorrhea and a palpable pelvic mass due to hematocolpos.
• Initial diagnostic work-up often involves ultrasonography, which may reveal a pelvic fluid collection that can mimic other pathologies.
• The condition is associated with a uterine didelphys and ipsilateral renal agenesis.

Findings:

• Pelvic MRI is essential for characterizing the uterine didelphys and vaginal septum, aiding in surgical planning.
• Abdominal imaging confirms the presence of renal agenesis, a key component of the syndrome.
• The pelvic mass represents a collection of menstrual blood within the obstructed hemivagina.

Implications:

• Accurate and timely diagnosis of OHVIRA is critical for effective management.
• Early intervention can help prevent serious complications such as endometriosis and adhesions.
• Preventing complications associated with OHVIRA is vital for preserving reproductive potential and avoiding subsequent infertility.