Herlyn-Werner-Wunderlich syndrome: a rare cause of pelvic pain in young females

The Herlyn-Werner-Wunderlich syndrome (HWW) is a rare congenital anomaly of the female urogenital tract that associates Mullerian and Wolffian duct anomalies. It is characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. With normal external genitalia, diagnosis is often delayed until after menarche with most affected patients presenting with dysmenorrhea, increasing pelvic pain and a palpable mass secondary to the associated hematocolpos or hematometra. The diagnosis and treatment at an early stage can relieve symptoms, prevent acute and long-term complications and preserve fertility. Although the ultrasound imaging (US) is the first choice for detection, magnetic resonance imaging (MRI) is suggested for definitive diagnosis and characterization and is considered the gold standard for pre-operative planning. In this paper, we reported the case of a 13 years old girl with known left renal agenesis, presenting to our hospital with increasing pain in the lower abdomen and dysmenorrhea. US confirmed the absence of the left kidney and showed an image suggestive of hematocolpos on the left pelvic side. For further evaluation we performed a pelvic MRI that revealed didelphys uterus and double vagina, the left one was distended by fluid exhibiting a signal intensity similar to methemoglobin suggesting a blood collection (hematocolpos) due to the presence of a longitudinal septum responsible for the obstruction. On the basis of the imaging findings the case was diagnosed as HWW syndrome. The patient underwent surgery with transvaginal resection of the vaginal septum and drainage of retained blood without perioperative or postoperative complications.