Wunderlich syndrome : a case report and literature review of didelphic uterus, blind hemivagina and ipsilateral renal agenesis

Abstract

Synopsis We report a case of Wunderlich syndrome, complex of didelphic uterus, blind hemivagina and ipsilateral renal agenesis. This syndrome is one of the rare uterine malformations and is often difficult to have an accurate preoperative evaluation. The patient was a 20-year-old nulliparous woman, with repeated abdominal pain and low-grade fever, which persisted on and off from menarche. Buldging of the left-sided lateral vaginal wall was found in the pelvic exam. Trasvaginal ultrasonography revealed fluid retention inside the vaginal wall and two separate horns of uterine corpus. Magnetic resonance imaging of the pelvis confirmed the finding of didelphic uterus with blind hemivagina, and intravenous pyelography added the finding of ipsilateral renal agenesis. She underwent laparoscopic exploration and resection of vaginal septum, and purulent retained material was drained from her blind vagina. The removed vaginal wall contained both columnar and squamous epithelium on the obstructed side. This histologic finding proved this case Wunderlich syndrome, in which the hemato- or pyocolpos was derived from cervical glandular epithelium. The symptoms of the patient were completely relieved and have not recurred postoperatively for 6 months. Recent reports and studies have proven that the appropriate treatments for this type of malformation are laparoscopic exploration and resection of vaginal septum, instead of hemihysterectomy with ipsilateral hemicolpectomy, which had been performed in the past years. Early accurate diagnosis followed by appropriate treatment not only relieves symptoms, but also avoids complications such as endometriosis or chronic pelvic inflammation, which decrease the reproductive potential and occasionally require more extensive surgical intervention. [Adv Obstet Gynecol, 56(2) : 85-90, 2004(H16.5)]