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