Herlyn-Werner-Wunderlich syndrome

Case contributed by Hamdy Mohammed Ibrahim
Diagnosis certain


Recurrent lower abdominal pain. Initial ultrasound noted a pelvic cystic lesion.

Patient Data

Age: 15 years
Gender: Female

Uterine didelphys and the left Mullerian duct derivatives are distended with fluid consistent with blood signal intensity. There is ipsilateral kidney agenesis and the contralateral kidney is hypertrophied with normal-appearing contralateral Mullerian duct derivatives.

Case Discussion

Herlyn-Werner-Wunderlich syndrome encompasses

  • uterine didelphys with vaginal septum causing hemivaginal obstruction
    • consequent unilateral hematocolpos, hematometra, hemosalpix and occasionally hemoperitoneum
  • contralateral Mullerian duct is well developed so that the patient presentation is often vague as there is no amenorrhea.
  • ipsilateral renal agenesis with compensatory hypertrophy of the contralateral kidney

This patient underwent resection of the left hemiuterus with improvement of her symptoms.

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