Herlyn-Werner-Wunderlich syndrome (uterus didelphys, blind hemivagina and ipsilateral renal agenesis) - a case report

Abstract

Uterovaginal duplication with obstructed hemivagina and ipsilateral renal agenesis is referred to as the Herlyn-Werner-Wunderlich (HWW) syndrome. A 17 year old woman presented with right pelvic pain and dysmenorrhea, present since menarche at 13 and worsening over the past year. Ultrasound examination revealed a right pelvic mass (5x5 cm), double endometrial echoes, and hematocolpos. A right pelvic mass, agenesis of the right kidney, double uterus, and blind hemivagina with hematocolpos were detected by magnetic resonance imaging and intravenous pyelography. A right tubo-ovarian abscess with dense adhesions and a double uterus were observed on diagnostic laparoscopy. Adhesiolysis was carried out and purulent material irrigated. After a course of antibiotics, a vaginal septum resection was performed and the pyocolpos drained. She remained symptom free after four months of follow-up. Prompt and accurate diagnosis and treatment of this syndrome can significantly improve the lives of sufferers and prevent future complications. (J Turkish-German Gynecol Assoc 2010; 11: 107-9)

The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

“…The exact etiopathogenesis of HWW syndrome is still not known. Wolffian ducts play an important role in the development of internal genital organs and give rise to kidneys [8]. They are inductor elements for adequate fusion of the Mullerian ducts.…”

Pyometra and Pregnancy with Herlyn-Werner-Wunderlich Syndrome

“…Herlyn-Werner-Wunderlich syndrome is extremely rare. To our knowledge, there are five cases of pregnancies associated to HWWS, [12][13][14][15] seven cases of HWWS with pyocolpos, [16][17][18][19][20][21][22] one case of pregnancy in HWWS with pyocolpos, 23 and two cases of pregnancy with pyocolpos in a uterus didelphys. 24,25 According to the European Society of Human Reproduction and Embryology (ESHRE) and the European Society for Gynecological Endoscopy (ESGE) 26 consensus on the Classification of Female Genital tract congenital anomalies, HWWS appears to be included in Class U3B uterine anomaly, class C2 cervix anomaly, and class V3 vaginal anomaly.…”

Herlyn-Werner-Wunderlich syndrome: a rare cause of acute urinary retention in an adolescent girl

“…Uterus didelphys represents failure of fusion of the paired Mullerian ducts. Obstructed hemivagina results from a transverse vaginal septum, which represents vertical fusion defect consequent to non-resorption of tissue between the vaginal plate that originates from the urogenital sinus and the caudal aspect of the paired Mullerian ducts 2. The renal agenesis on the same side of the obstructed hemivagina reflects the close embryological association of Wolffian duct in development of Mullerian ducts, whereby developmental anomaly of one Woffian duct may cause unilateral renal agenesis associated with obstructed hemivagina.…”

“…The most common presenting symptoms of HWWS are cyclical dysmenorrhoea at the onset of puberty, associated with vaginal or pelvic mass. Rarely, it may have delayed presentations of primary infertility, ischiorectal swelling, pyometra or urinary obstruction 1 2…”