Herlyn-Werner-Wunderlich syndrome

Case contributed by Salman S. Albakheet
Diagnosis certain


History of right kidney agensis diagnosed by ultrasound, presents with pelvic pain.

Patient Data

Age: 20 years
Gender: Female

Abdomen and pelvis CT scan


Agenesis of the right kidney. Left kidney is normal.

Separated divergent uterine horns are seen with retained fluid density within the right uterine horn and cervix denoting hematometrocolpos, while the left uterine horn is within normal. 

MRI Pelvis


Normal size, shape and signal intensity of left uterus with normal endometrial interface. Normal wall thickness and normal cervix. Hour-glass enlarged right uterus measuring 13.3 cm in longest diameter filled with high fluid content in T1 and low in T2 representing subacute hemorrhage. Such hour-glass appearance represents dilated endometrium and narrowing at the level of internal os of the cervix and closed outer end of the cervix. This dilatation did not extend to involve the vagina. Normal size, shape and signal intensity of both ovaries with no ovarian lesion are seen.

Case Discussion

Herlyn-Werner-Wunderlich syndrome is a rare Müllerian duct anomaly consisting of a didelphic uterus, a hemivaginal septum, and ipsilateral renal agenesis.

The diagnosis should be suspected in adolescent females with a pelvic mass and ipsilateral renal agenesis.

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