Herlyn-Werner-Wunderlich syndrome: 3D ultrasonographic diagnosis in premenarche

Uterine malformations are a heterogeneous group of congenital anomalies resulting from abnormal development of Mullerian ducts. Herlyn-Werner-Wunderlich syndrome is a rare condition that includes the triad of uterus didelfus, blind hemivagina and ipsilateral renal agenesis. We report a case with premenarche diagnosis and treatment and make a review of the illness. A premenarche 11year old female was referred for pediatric nephrology and neurodevelopment consultation due to prenatal diagnosis of left renal agenesis. A pelvic ultrasound was performed and an anomalous uterus and a cystic pelvic mass was incidentally discovered. A 3D trans-rectal ultrasound identified a bicolis didelfus uterus and mucocolpos. A unidigital vaginal exam revealed a single cervix with soft bulging of the left apical vaginal wall. Using monopolar knife, an incision was done allowing drainage of the cavity. A part of the apical vaginal septum was removed and a normal cervix was seen. A high degree of clinical suspicion is important for the diagnoses. 3D ultrasound or MRI are the imaging diagnostic exams of choice, helped by a careful bimanual pelvic examination. An early diagnosis is important to prevent chronic pelvic pain and perhaps severe endometriosis.