Obstructed Hemivagina

Obstructed hemivagina in association with a double uterus and renal anomaly has been described as early as 1922. Since then, this anomaly has been described in the literature associated with several different names: double uterus with obstructed hemivagina and ipsilateral renal agenesis, obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, and Herlyn-Werner-Wunderlich syndrome. Diagnosis should be suspected in an adolescent female with escalating dysmenorrhea as menstruation occurs from the patent outflow tract, but hematocolpos, hematometra, and retrograde menstruation occur in the obstructed side leading to pain. Treatment is typically primary resection of the vaginal septum with marsupialization to unite the vaginal cavities and relieve the obstruction. Alternative therapies include hemihysterectomy with hemocolpotomy, and drainage and decompression of the hematocolpos with hormonal suppression of menses. Fertility may be affected by extent of endometriosis and adhesive disease, but infertility is not common. Pregnancies occur more commonly in the non-obstructed horn, but may be present in the horn ipsilateral to obstruction in a significant percentage of cases. Similar content being viewed by others

Purslow CE. A case of unilateral haematocolps, haematometria, and haematosalpinx. J Obstet Gynaecol Br Emp. 1922;29:643. Fedele L, Motta F, Frontino G, Restelli E, Bianchi S. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases. Hum Reprod. 2013;28(6):1580–3. Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril. 2007;87(4):918–22. Tong J, Zhu L, Lang J. Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome. Int J Gynaecol Obstet. 2013;121(2):173–5. Attar R, Yıldırım G, Inan Y, Küzılkale O, Karateke A. Uterus didelphys with an obstructed unilateral vagina and ipsilateral renal agenesis: a rare cause of dysmenorrhoea. J Turk Ger Gynecol Assoc. 2013;14(4):242–5. Schutt AK, Barrett MR, Trotta BM, Stovall DW. Perioperative evaluation in Herlyn-Werner-Wunderlich syndrome. Obstet Gynecol. 2012;120(4):948–51. Heinonen PK. Clinical implications of the didelphic uterus: long-term follow-up of 49 cases. Eur J Obstet Gynecol Reprod Biol. 2000;91:183–90. Cetinkaya SE, Kahraman K, Sonmezer M, Atabekoglu C. Hysteroscopic management of vaginal septum in a virginal patient with uterus didelphys and obstructed hemivagina. Fertil Steril. 2011;96(1):e16–8. Herlyn U, Werner H. Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Geburtshilfe Frauenheilkd. 1971;31:340–7. Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, Grasso RF, Zobel BB. Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging. 2012;12:4. Vercellini P, Daguati R, Somigliana E, Viganò P, Lanzani A, Fedele L. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review. Fertil Steril. 2007;87(4):719–24. 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(5):987–92. Grimbizis GF, Campo R. Congenital malformations of the female genital tract: the need for a new classification system. Fertil Steril. 2010;94(2):401–7. Acien P, Acien MI. The history of female genital tract malformation classifications and proposal of an updated system. Hum Reprod Update. 2011;17(5):693–705. Oppelt P, Renner SP, Brucker S, Strissel PL, Strick R, Oppelt PG, et al. The VCUAM (Vagina Cervix Uterus Adnex-associated Malformation) classification: a new classification for genital malformations. Fertil Steril. 2005;85(5):1493–7. American Fertility Society. The AFS classification of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Mullerian anomalies and intrauterine adhesions. Fertil Steril. 1988;49:944–55. Berger-Chen S, Ritch JM, Kim JH, Evanko J, Hensle TW. An unusual presentation of uterine didelphys and obstructed hemivagina. J Pediatr Adolesc Gynecol. 2012;25(6):e129–31. Khong TL, Siddiqui J, Mallinson P, Horton D, Gandhi J, Daniel R. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis-role of sonographically guided minimally invasive vaginal surgery. Eur J Pediatr Surg. 2012;22(2):171–3. Heinonen PK. Pregnancies in women with uterine malformation, treated obstruction of hemivagina and ipsilateral renal agenesis. Arch Gynecol Obstet. 2013;287:975–8. Rock JA, Jones Jr HW. The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol. 1980;138:339–42. Candiani GB, Fedele L, Candiani M. Double uterus, blind hemivagina, and ipsilateral renal agenesis: 36 cases and long-term follow-up. Obstet Gynecol. 1997;90:26–32. Paulozzi LJ, Lary JM. Laterality patterns in infants with external birth defects. Teratology. 1999;60:265–71. Fantel AG, Person RE, Burroughs-Gleim C, Shepard TH, Juchau R, Backler B. Asymmetric development of mitochondrial activity in rat embryos as a determinant of the defect patterns induced by exposure to hypoxia, hyperoxia, and redox cyclers in vitro. Teratology. 1991;44:355–62. Fantel AG, Juchau R, Tracy JW, Burroughs CJ, Person RE. Studies of mechanisms of niridazole-elicited embryotoxicity: evidence against a major role for covalent binding. Teratology. 1989;39:63–74. Pereira N, Anderson SH, Verrecchio ES, Brown MA, Glassner MJ. Hemivaginal septum resection in a patient with a rare variant of Herlyn-Werner-Wunderlich syndrome. J Minim Invasive Gynecol. 2014;21(6):1113–7. Wu TH, Wu TT, Ng YY, Ng SC, Su PH, Chen JY, et al. Herlyn-Werner-Wunderlich syndrome consisting of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis in a newborn. Pediatr Neonatol. 2012;53(1):68–71. Haddad B, Barranger E, Paniel BJ. Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod. 1999;14(8):1962–4. Olive DL, Henderson DY. Endometriosis and mullerian anomalies. Obstet Gynecol. 1987;69:412–5. Fedele L, Bianchi S, Di Nola G, Franchi D, Candiani GB. Endometriosis and nonobstructive müllerian anomalies. Obstet Gynecol. 1992;79(4):515–7. Rock JA, Zacur HA, Dlugi AM, Jones HW. Pregnancy success following surgical correction of imperforate hymen and complete transverse septum. Obstet Gynecol. 1982;59:448. Kim TE, Lee GH, Choi YM, Jee BC, Ku SY, Suh CS, et al. Hysteroscopic resection of the vaginal septum in uterus didelphys with obstructed hemivagina: a case report. J Korean Med Sci. 2007;22:766–9. Shih CL, Hung YC, Chen CP, Chien SC, Lin WC. Resectoscopic excision of the vaginal septum in a virgin with uterus didelphys and obstructed unilateral vagina. Taiwan J Obstet Gynecol. 2010;49(1):109–11. Nassif J, Al Chami A, Abu Musa A, Nassar AH, Kurdi A, Ghulmiyyah L. Vaginoscopic resection of vaginal septum. Surg Technol Int. 2012;22:173–6. Dorais J, Milroy C, Hammoud A, Chaudhari A, Gurtcheff S, Peterson CM. Conservative treatment of a Herlyn-Werner-Wunderlich müllerian anomaly variant, noncommunicating hemiuterus with Gartner duct pseudocyst. J Minim Invasive Gynecol. 2011;18(2):262–6. Wang S, Lang JH, Zhu L, Zhou HM. Duplicated uterus and hemivaginal or hemicervical atresia with ipsilateral renal agenesis: an institutional clinical series of 52 cases. Eur J Obstet Gynecol Reprod Biol. 2013;170:507–11. Sanfilippo JS, Wakim NG, Schikler KN, Yussman MA. Endometriosis in association with uterine anomaly. Am J Obstet Gynecol. 1986;154:39–43. Acien P, Acien M, Sanchez-Ferrer M. Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod. 2004;19:2377–84. Propst AM, Hill III JA. Anatomic factors associated with recurrent pregnancy loss. Semin Reprod Med. 2000;18:341–50. Author information Authors and Affiliations Corresponding author Editor information Editors and Affiliations Rights and permissions Copyright information © 2016 Springer International Publishing Switzerland About this chapter Cite this chapter Pereira, N., Pfeifer, S.M. (2016). Obstructed Hemivagina. In: Pfeifer, S. (eds) Congenital Müllerian Anomalies. Springer, Cham. https://doi.org/10.1007/978-3-319-27231-3_12 Download citation DOI: https://doi.org/10.1007/978-3-319-27231-3_12 Published: Publisher Name: Springer, Cham Print ISBN: 978-3-319-27229-0 Online ISBN: 978-3-319-27231-3 eBook Packages: MedicineMedicine (R0)