Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases.

BACKGROUND: Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised. OBJECTIVE: To highlight the imaging diagnostic clues in this rare condition. MATERIALS AND METHODS: We report on 11 adolescents with this condition. RESULTS: Sonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents. CONCLUSION: Early and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally.

Isolated tubal torsion: a rare cause of pelvic pain at menarche. Sonographic and MR findings.

Isolated torsion of the fallopian tube is a rare clinical entity, especially in adolescents and at menarche. The diagnosis is essentially made at laparoscopy or at laparotomy because of nonspecific clinical signs. We present a case of isolated tubal torsion in a 12-year-old girl a few days after menarche, highlighting the sonographic and MR findings. Both techniques demonstrated the enlarged and tortuous fallopian tube with normal ovaries and uterus, but MR was also able to characterize contained blood and absent vascular supply. Although this condition is uncommon it should be considered as a cause of acute pelvic pain in adolescents because of the possibility of salvage surgery with early diagnosis. Sonography and MRI have a complementary role in this diagnosis.