{"paper_id":"7aa500d4-4b0b-4c96-ad37-ad8cefcbf88b","body_text":"Abstract\nObstructive Mullerian anomalies can lead to retrograde menstruation and as such have been shown to cause endometriosis in the adolescent. Appropriate and early diagnosis and timely correction are paramount in preventing progression of severity and avoiding associated complications. Key elements for the diagnosis of obstructive Mullerian anomalies include a careful history, physical exam, laboratory studies, and imaging. Various classification systems have been proposed, which can aid in diagnostic description as well as approach to treatment. The presentation and management of the most well-known anomalies such as obstructed hemivagina ipsilateral renal anomaly syndrome (OHVIRA) and unicornuate uterus with obstructed uterine horn as well as more rare presentations such as cervical agenesis and uterine isthmus agenesis as well as other complex uterine anomalies are discussed.\nAccess this chapter\nTax calculation will be finalised at checkout\nPurchases are for personal use only\nSimilar content being viewed by others\nReferences\nAcien P, Acién M, Sánchez-Ferrer M. Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod. 2004;19:2377–84.\nAcien P, Acién M, Sánchez-Ferrer M. Mullerian anomalies “without a classification”: from the didelphys-unicollis uterus to the bicervical uterus with or without septate vagina, vol. 91. Alicante, Spain: Fertility and Sterility; 2009.\nAcien P, Acien M. The presentation and management of complex female genital malformations. Hum Reprod Update. 2016;22(1):48–69.\nAcien 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.\nAcien P, Acien M, Quereda F, Santoyo T. Cervicovaginal agenesis: spontaneous gestation at term after previous reimplantation of the uterine corpus. Hum Reprod. 2008;23:548–53.\nAnttila L, Penttilä TA, Suikkari AM. Successful pregnancy after in-vitro fertilization and transmyometrial embryo transfer in a patient with congenital atresia of cervix. Hum Reprod. 1999;14:1647–9.\nAppelbaum H, et al.. Congenital Mullerian anomalies. (S. Pfeifer, Ed.) AG, Switzerland: Springer; 2016.\nBermejo C. Three-dimensional ultrasound in the diagnosis of Mullerian duct anomalies and concordance with magnetic resonance imaging. Ultrasound Obstet Gynecol. 2010;35:593–601.\nBrosens I, Brosens J, Benagiano G. Neonatal uterine bleeding as antecedent of pelvic endometriosis. Hum Reprod. 2013;28(11):2893–7.\nCandianai G, Fedele L, Candiani M. Double uterus, blind hemivagina, and ipsilateral renal agenesis: 36 cases and long-term follow-up. Obstet Gynecol. 1997;90(1):26–32.\nChakravarty B, Konar H, Chowdhury NN. Pregnancies after reconstructive surgery for congenital cervicovaginal atresia. Am J Obstet Gynecol. 2000;183:421–3.\nDeffarges JV, Haddad B, Musset R, Paniel BJ. Uterovaginal anastamosis in women with uterine cervix atresia: long term follow up and reproductive performance. Hum Reprod. 2001;16:1772–5.\nDietrich JE, Millar DM, Quint EH. Obstructive reproductive tract anomalies. J Pediatr Adolesc Gynecol. 2014;27:396–402.\nDillon W, et al. Congenital atresia of the cervix. Obstet Gynecol. 1979;54:126–9.\nFedele L, et al. Endometriosis and nonobstructive Mullerian anomalies. Obstet Gynecol. 1992;79:515–7.\nFedele L, et al. Laparoscopically assisted uterovestibular anastamosis in patients with uterine cervix atresia and vaginal aplasia. Fertil Steril. 2008;89:212–6.\nFedele 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.\nFedele L, Bianchi S, Zanconato G, Berlanda N, Bergamini V. Laparoscopic removal of the cavitated noncommunicating rudimentary uterine horn: surgical aspects in 10 cases. Fertil Steril. 2005;83(2):432–6.\nFujimoto VY, Miller JH, Klein NA, Soules MR. Congenital cervical atresia: report of seven cases and review of the literature. Am J Obstet Gynecol. 1997;177:1419–25.\nGrimbizis 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.\nGrimsby GM, Baker LA. The use of autologous buccal mucosa grafts in vaginal reconstruction. Urol Reprod. 2014;15:428.\nHuberlant S, et al. Congenital cervical agenesis: pregnancy after transmyometrial embryo transfer. J Gynecol Obstet Biol Reprod. 2014;43:521–5.\nJayasinghe Y, Rane A, Stalewski H, Grover S. The presentation and early diagnosis of the rudimentary uterine horn. Obstet Gynecol. 2005;105(6):1456–67.\nJiali Tong L, Lang J. Clinical characteristics of 70 patients with Herlyn–Werner–Wunderlich syndrome. Int J Gynecol Obstet. 2013;121:173–5.\nKapczuk PK, Friebe PZ, Iwaniec PK, Kedzia PW. Obstructive Mullerian anomalies in menstruating adolescent girls: a report of 22 cases. J Pediatr Adolesc Gynecol. 2018;31:252–7.\nKriplani A, G K. Laparoscopic-assisted uterovaginal anastamosis in congenital atresia of uterine cervix: a follow up study. J Minim Invasive Gynecol. 2012;19:477–84.\nLai T-H, Wu M-H, Hung K-H, Cheng Y-C, Chang F-M. Successful pregnancy by transmyometrial and transtubal embryo transfer after IVF in a patient with congenital cervical atresia who underwent uterovaginal canalization during cesarean section. Hum Reprod. 2001;16:268–71.\nLee PA, Houk CP, Ahmed SF, Hughes IA, International Consensus Conference on Intersex organized by the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. Consensus statement on management of intersex disorders. Pediatrics. 2006;118:488–500.\nLekovich J, Pfeifer SM. Cervical agenesis. In: Pfeifer SM, editor. Congenital Mullerian anomalies. New York: Springer; 2016. p. 55–62.\nLudwin A, Ludwin I. Comparison of the ESHRE–ESGE and ASRM classifications of Mullerian duct anomalies in everyday practice. Hum Reprod. 2015;30(3):569–80.\nLudwin A, Ludwin I, Bhagavath B, Martins W, Lindheim S. Virginity-sparing management of blind hemivagina in obstructed hemivagina and ipsilateral renal anomaly syndrome. Fertil Steril. 2018;110(5):976–8.\nNabeshima H, Nishimoto M, Shiga N, Utsunomiya H, Yaegashi N. Laparoscopic strassman metroplasty in a postmenarcheal adolescent girl with Herlyn-Werner-Wunderlich Mullerian anomaly variant, obstructed noncommunicating didelphic uterus without gartner duct pseudocyst. J Minim Invasive Gynecol. 2013;20(2):255–8.\nNunley WC Jr, Kitchin JD 3rd. Congenital atresia of the uterine cervix with pelvic endometriosis. Arch Surg. 1980;115:757–8.\nPellerito J. Diagnosis of uterine anomalies: relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology. 1992;185:795–800.\nRichards A, et al. Primary cervico-uterine anastamosis in a patient with agenesis of the uterine isthmus. Fertil Steril. 2017;388\nRock JA, Roberts CP, Jones HW Jr. Congenital anomalies of the uterine cervix: lessons from 30 cases managed clinically a common protocol. Fertil Steril. 2010;94:1858–63.\nSilveira MS, Laufer MR. Persistence of endometriosis after correction of an obstructed reproductive tract anomaly. J Pediatr Adolesc Gynecol. 2013;26:e93–4.\nSmith N, Laufer M. Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril. 2007;87:918–22.\nSociety TA. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Mullerian anomalies and intrauterine adhesions. Fertil Steril. 1988;49(6):944–55.\nSon JK, Taylor GA. Transperineal ultrasonography. Pediatr Radiol. 2014;44:193–201.\nSpitzer RF, Kives S, Allen LM. Case series of laparoscopically resected noncommunicating functional uterine horns. J Pediatr Adolesc Gynecol. 2009;22:e23–8.\nStanheiser J, et al. Mullerian agenesis: diagnosis, treatment, and future fertility. In: Congenital Mullerian Anomalies. Pfeifer SM. (Ed). New York, New York: Springer, Inc.; 2016.\nThabet SMA, Thabet ASMA. Role of new sono-imaging technique ‘sonocolpography’ in the diagnosis and treatment of the complete transverse vaginal septum and other allied conditions. J Obstet Gynecol Res. 2002;28:80–5.\nVercellini P, Daguati R, Somigliana E, Vigano 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.\nYang L-D, Zhang C, Yang L, Wu Y-z, Zhou Q-m. Congenital atresia of uterine isthmus: successful diagnosis and end-to-end anastamosis. J Pediatr Adolesc Gynecol. 2015;28:113–7.\nZhao M, Li P, Li S, Li Q. Use of autologous micromucosa graft for vaginoplasty in vaginal agenesis. Ann Plast Surg. 2009;63:645–9.\nAuthor information\nAuthors and Affiliations\nCorresponding author\nEditor information\nEditors and Affiliations\nRights and permissions\nCopyright information\n© 2020 Springer Nature Switzerland AG\nAbout this chapter\nCite this chapter\nPetrini, A., Pasternak, M., Pfeifer, S.M. (2020). Obstructed Mullerian Anomalies and Endometriosis in the Adolescent. In: Nezhat, C.H. (eds) Endometriosis in Adolescents. Springer, Cham. https://doi.org/10.1007/978-3-030-52984-0_12\nDownload citation\nDOI: https://doi.org/10.1007/978-3-030-52984-0_12\nPublished:\nPublisher Name: Springer, Cham\nPrint ISBN: 978-3-030-52983-3\nOnline ISBN: 978-3-030-52984-0\neBook Packages: MedicineMedicine (R0)","source_license":"CC0","license_restricted":false}