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

• Jung EJ
• Cho MH
• Kim DH
• Byun JM
• Kim YN
• Jeong DH
• Sung MS
• Kim KT
• Lee KB

• KMID: 2386280
• DOI: http://doi.org/10.5468/ogs.2017.60.4.374

Abstract

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.

Keyword

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

MeSH Terms

Adolescent
Cervix Uteri
Coinfection
Diagnosis
Drainage
Dysmenorrhea
Female
Gynecological Examination
Humans
Magnetic Resonance Imaging
Pelvic Pain
Puberty
Suppuration
Uterus
Vagina
Vaginal Discharge
Young Adult

Figure

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  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).

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  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).

Reference

1. Gholoum S, Puligandla PS, Hui T, Su W, Quiros E, Laberge JM. Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). J Pediatr Surg. 2006; 41:987–992.

2. Jindal G, Kachhawa S, Meena GL, Dhakar G. Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis. J Hum Reprod Sci. 2009; 2:87–89.

3. Cox D, Ching BH. Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos. J Radiol Case Rep. 2012; 6:9–15.

4. Ballesio L, Andreoli C, De Cicco ML, Angeli ML, Manganaro L. Hematocolpos in double vagina associated with uterus didelphus: US and MR findings. Eur J Radiol. 2003; 45:150–153.

5. Han B, Herndon CN, Rosen MP, Wang ZJ, Daldrup-Link H. Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Radiol Case Rep. 2015; 5:327.

6. Purslow C. A case of unilateral hæmatokolpos, hæmatometra and hæmatosalpinx. BJOG. 1922; 29:643.

7. Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril. 2007; 87:918–922.

8. Troiano RN. Magnetic resonance imaging of mullerian duct anomalies of the uterus. Top Magn Reson Imaging. 2003; 14:269–279.

9. Nassif J, Al Chami A, Abu Musa A, Nassar AH, Kurdi AT, Ghulmiyyah L. Vaginoscopic resection of vaginal septum. Surg Technol Int. 2012; 22:173–176.

10. Phupong V, Pruksananonda K, Taneepanichskul S, Tresukosol D, Virutamasen P. Double uterus with unilaterally obstructed hemivagina and ipsilateral renal agenesis: a variety presentation and a 10-year review of the literature. J Med Assoc Thai. 2000; 83:569–574.

11. Dias JL, Jogo R. Herlyn-Werner-Wunderlich syndrome: pre- and post-surgical MRI and US findings. Abdom Imaging. 2015; 40:2667–2682.

12. Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, et al. Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging. 2012; 12:4.

13. Jones HW, Rock JA. Te linde's operative gynecology. 11th ed. Philadelphia (PA): Lippincott Williams & Wilkins;2015.

14. Sanghvi Y, Shastri P, Mane SB, Dhende NP. Prepubertal presentation of Herlyn-Werner-Wunderlich syndrome: a case report. J Pediatr Surg. 2011; 46:1277–1280.

15. Kang M, Kim SW, Park JY, Lim HJ, Park CW, Park JS, et al. A case of Herlyn-Werner-Wunderlich syndrome diagnosed in asymptomatic newborn. Korean J Obstet Gynecol. 2012; 55:966–969.