A rare urogenital malformation “Herlyn-Werner-Wunderlich syndrome” (Ohvira syndrome) discovered during an acute pelvic pain

A rare urogenital malformation “Herlyn-Werner-Wunderlich syndrome” (Ohvira syndrome) discovered during an acute pelvic pain DOI: https://doi.org/10.18203/2320-1770.ijrcog20230829Keywords: OHVIRA syndrome, Obstructed hemivagina, Didelphic uterus, Hematocolpos, Renal agenesis, Vaginal septumAbstract The Herlyn-Werner-Wunderlich syndrome (OHVIRA syndrome) is a rare urogenital malformation, characterized by a blind hemivagina, a didelphic uterus, and homolateral renal agenesis, it results from a defect in the development of the muller and Wolf ducts during the embryonic period, of unknown origin, the diagnosis is most often made at puberty during the first menstruation, sometimes late, particularly for non-obstructive forms: infertility or obstetrical complications, the diagnosis is based on pelvic ultrasound especially with 3D reconstructions and pelvic magnetic resonance imaging (MRI) which remains the standard gold for the detection of mullerian anomalies, laparoscopy can also play a double diagnostic and prognostic role in order to study the consequences of the blood reflection (hematosalpinx, inflammatory pelvis, endometriosis. In this work we report the case of an OHVIRA syndrome diagnosed late at the age of 36 years in a multiparous woman with acute pelvic pain simulating the picture of a torsion of the adnexa, we will discuss through this case the clinical, diagnostic and therapeutic aspects of this uterine malformation. Metrics References Buttram VC, Gibbons WE. Mullerian anomalies: a proposed classification (an analysis of 144 cases). Fertil Steril. 1979;32:40-6. Troiano RN, McCarth SM. Mullerian duct anomalies: imaging and clinical issues. Radiology. 2004;233:19-34. Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies. Hum Reprod. 2013;28(8):2032-44. 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. Acien P, Acien M, Sanchez-Ferrer ML. Mullerian anomalies ‘‘without a classification’’: from the didelphys-unicollis uterus to the bicervical uterus with or without septate vagina. Fertil Steril. 2009;91:2369-75. Ballesio L, Andreoli C, De Cicco ML, Angeli ML, Manganaro L. Hematocolpos in double vagina associated with uterus didelphys: US and MRI findings. Eur J Radiol. 2003;45:150-3. Sharma R, Mishra P, Seth S, Agarwal N. OHVIRA Syndrome Diagnostic Dilemmas and Review of Literature. J South Asian Federation Obstet Gynaecol. 2020;12(6). Mullen RD, Behringer RR. Molecular genetics of Müllerian duct formation, regression and differentiation. Sex Dev. 2014;8(5):281-96. Ceccaldi PF, Ducarme G, Dedecker F, Harika G, Gabriel R, Quéreux C, et al. Hematocolpos due to obstructed hemivagina. About three cases]. Gynecol Obstet Fertil. 2006;34(6):510-3. Simon C, Martinez L, Pardo F, Tortajada M, Pellicer A. Mullerian defects in women with normal reproductive outcome. Fertil Steril. 1991;56:1192-3. Karagozov I. Herlyn-Werner-Wunderlich syndrome. Akush Ginekol (Sofiia). 1983;22:70-6. Zhu L, Chen N, Tong JL, Wang W, Zhang L, Lang JH. New classification of Herlyn-WernerWunderlich syndrome. Chin Med J (Engl). 2015;128:222-5. David A, Gudi SN, Shankar R. Herlyn–Werner–Wunderlich syndrome: premenarche. J South Asian Feder Obst Gynae. 2017;9(2):207-10. Watanabe Y, Etoh T, Nakai H. Adenocarcinoma of the lower female genital tract in patients with Herlyn-Werner-Wunderlich syndrome. AJOG. 2012;e5-6. Braverman P, Breech L. Committee on adolescence. Clinical report—gynecologic examination for adolescents in the pediatric office setting. Pediatrics. 2010;126(3):583-90. de Groot JA, Tierney Wolgemuth BS, Sanfilippo J. Mullerian anomalies in the pediatric and adolescent population: diagnosis, counseling and treatment options. Ann Infert Rep Endocrin. 2019;2(1):1016. Carrington BM, Hricak H, Nuruddin RN, Secaf E, Laros RK Jr, Hill EC. Mullerian duct anomalies: MR imaging evaluation. Radiology. 1990;176:715-20. Olpin JD, Heilbrun M. Imaging of Mullerian duct anomalies. Clin Obstet Gynecol. 2009;52(1):40-56. Philibois O, Guye E, Richard O, Tardieu D. Role of laparoscopy in vaginal malformation - An experience in 22 children. Surg Endosc. 2004;18:87-91. Loumaye F, Cheruy C, Cristinelli S, Foidart JM, Nisolle M. Comment je traite... un utérus didelphe associé à un hémi-vagin borgne. Rev Med Liege. 2007;62(7-8):480-3. Cheng C, Subedi J, Zhang A. Vaginoscopic incision of oblique vaginal septum in adolescents with OHVIRA syndrome. Sci Rep. 2019;9:20042.