Obstet Gynecol Sci.  2017 Jul;60(4):374-377. 10.5468/ogs.2017.60.4.374.

Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

  • 1Department of Obstetrics and Gynecology, Inje University Busan Paik Hospital, Busan, Korea. 107526@paik.ac.kr
  • 2Paik Institute for Clinical Research, Inje University Busan Paik Hospital, Busan, Korea.


Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the urogenital tract, which is characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents at puberty with pelvic pain, dysmenorrhea, and a vaginal or pelvic mass. Although rare, it may present with purulent vaginal discharge due to secondary infection of the obstructed hemivagina, making diagnosis difficult. A careful pelvic examination to identify the cervix and vagina is the key to the diagnosis of Müllerian duct anomalies and magnetic resonance imaging can provide additional useful information. The optimal treatment is full excision and marsupialization of the obstructing vaginal septum so that both uteri can drain through the patent vagina. The authors report a case of a 22-year-old female with an unusual presentation of Herlyn-Werner-Wunderlich syndrome complicated by pyocolpos, which was successfully managed by vaginal septum resection and drainage of pus.


Herlyn-Werner-Wunderlich syndrome; Renal agenesis; Urogenital anomalies; Uterus didelphys

MeSH Terms

Cervix Uteri
Gynecological Examination
Magnetic Resonance Imaging
Pelvic Pain
Vaginal Discharge
Young Adult


  • Fig. 1 (A) Bulging of the right lateral vaginal wall (arrow) with presence of one cervix (Cx). (B) Pus drainage occurring after the incision of the obstructed vaginal septum. (C) Wide excision of the vaginal septum until two separate cervices are reached. (D) Laparoscopic findings of uterine didelphys and endometriotic lesions on the uterine serosa (arrow) (E) Laparoscopic findings of thin band adhesions (arrow).

  • Fig. 2 Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis. Coronal T2-weighted magnetic resonance image demonstrates (A) two separate uterine cavities (arrows) with two separate cervices (arrowheads). (B) Dilatation of right hemivagina (arrow) and high signal intensity fluid with internal debris (asterisk), suggestive of pyocolpos. (C) Absent right kidney (arrow).


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