Herlyn-Werner-Wunderlich syndrome

Case contributed by Dr Hamdy Mohammed Ibrahim

Presentation

Adolescent patient presenting with recurrent lower abdominal pain each month. She has regular menstrual cycles and well developed secondary sexual characteristics. Initial ultrasound noted a pelvic cystic lesion with echogenic contents.

Patient Data

Age: 15 yrs
Gender: Female

Uterine didelphys and the left mullarian 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 mullarian duct derivatives.

Case Discussion

Herlyn-Werner-Wunderlich syndrome encompasses

  • uterine didelphys with vaginal septum causing hemivaginal obstruction
    • consequent unilateral haematocolpos, 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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Case information

rID: 53476
Case created: 20th May 2017
Last edited: 30th May 2017
Inclusion in quiz mode: Included

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