Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-78c5997874-xbtfd Total loading time: 0 Render date: 2024-11-17T17:08:36.415Z Has data issue: false hasContentIssue false

Chapter 9 - Rokitansky Syndrome

Published online by Cambridge University Press:  28 January 2023

By
Gail Busby
Edited by
Naomi S. Crouch  and
Cara E. Williams
Naomi S. Crouch
Affiliation:
St Michael's Hospital, Bristol
Cara E. Williams
Affiliation:
Liverpool Women's Hospital
Get access

Summary

Rokitansky syndrome, or Mayer–Rokitansky–Küster–Hauser syndrome (MRKH) also known as Müllerian agenesis results from embryonic underdevelopment of the Müllerian duct, resulting in agenesis or atresia of the uterus, vagina or both. The vagina is shortened and may appear as just a dimple inferior to the urethra. A single midline uterine remnant may be present, or there may be uterine horns (with or without an endometrial cavity) which do not communicate with the vagina. This condition belongs to the most severe (class 5) of the ESHRE/ESGE classification [1].

Type
Chapter
Information
Paediatric and Adolescent Gynaecology for the MRCOG , pp. 67 - 76
Publisher: Cambridge University Press
Print publication year: 2023

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

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;28:2032–44.Google Scholar
Pizzo, A, Lagana, AS, Sturlese, E, Retto, G, Retto, A, De Dominici, R, et al. Mayer–Rokitansky–Küster–Hauser syndrome: embryology, genetics and clinical and surgical treatment. ISRN Obstetr Gynaecol. 2013;2013:628717.Google ScholarPubMed
Herlin, M, Bjorn, AB, Rasmussen, M, Trolle, B, Peterson, MB. Prevalence and patient characteristics of Mayer–Rokitansky–Küster–Hauser syndrome: a nationwide registry-based study. Hum Reprod. 2016;31:2384–90.CrossRefGoogle ScholarPubMed
Fontana, L, Gentilin, B, Fedele, L, Gernvasini, C, Miozzo, M. Genetics of Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome. Clin Genet. 2017;91:233–46.Google Scholar
Oelschlager, AA, Debiec, K, Appelbaum, H. Primary vaginal dilation for vaginal agenesis: strategies to anticipate challenges and optimize outcomes. Curr Opin Obstet Gynecol. 2016;28:345–9.Google Scholar
American College of Obstetricians and Gynaecologists. Müllerian agenesis: diagnosis, management, and treatment. ACOG Committee Opinion 728. Obstet Gynecol. 2018;131:e35–42.Google Scholar
Cheikhelard, A, Bidet, M, Baptiste, A, Viaud, M, Fagot, C, Khen-Dunlop, N, et al. Surgery is not superior to dilation for the management of vaginal agenesis in Mayer–Rokitansky–Küster–Hauser syndrome: a multicentre comparative observational study in 131 patients. AJOG. 2018;219:281.CrossRefGoogle ScholarPubMed
Callens, N, De Cuypere, G, De Suttter, P, Monstrey, S, Weyers, S, Hoebeke, P, et al. An update on surgical and non-surgical treatments for vaginal hypoplasia. Hum Reprod Update. 2014;20:775–801.CrossRefGoogle ScholarPubMed
Rosen, R, Brown, C, Heiman, J, Leiblum, S, Meston, C, Shabsigh, R, et al. The Female Sexual Function Index (FSFI): a multidimensional self-report instrument for the assessment of female sexual function. J Sex Marital Ther. 2000;26:191–208.Google Scholar
Herlin, M, Bjorn, AB, Jorgensen, LK, Trolle, B, Petersen, MB. Treatment of vaginal agenesis in Mayer–Rokitansky–Küster–Hauser syndrome in Denmark: a nationwide comparative study of anatomical outcome and complications. Fertil Steril. 2018;110:746–53.Google Scholar
Bean, EJ, Mazur, T, Robinson, AD. Mayer–Rokitansky–Küster–Hauser syndrome: sexuality, psychological effects, and quality of life. J Pediatr Adolesc Gynecol. 2009;22:339–46.Google Scholar
Liao, LM, Conway, GS, Ismail-Pratt, I, Bikoo, M, Creighton, SM. Emotional and sexual wellness and quality of life in women with Rokitansky syndrome. Am J Obstet Gynecol. 2011;205(2):117.Google Scholar
Heller-Boersma, JG, Schmidt, UH, Edmonds, K. Psychological distress in women with uterovaginal agenesis (Mayer–Rokitansky–Küster–Hauser syndrome, MRKH). Psychosomatics. 2009;50:277–81.CrossRefGoogle ScholarPubMed
Wagner, A, Brucker, SY, Ueding, E, Grober-Gratz, D, Simoes, E, Rall, K, et al. Treatment management during the adolescent transition period of girls and young women with Mayer–Rokitansky–Küster–Hauser syndrome (MRKHS): a systematic literature review. Opranet J Rare Dis. 2016;11:152.Google Scholar
Friedler, S, Grin, L, Liberti, G, Saar-Ryss, B, Rabinson, Y, Meltzer, S, et al. The reproductive potential of patients with Mayer–Rokitansky–Küster–Hauser syndrome using gestational surrogacy: a systematic review. Reprod Biomed Online. 2016;32:54–61.Google Scholar
Brännström, M, Kahler, PD, Greite, MS, Molne, J, Diaz-Garcia, C, Tullius, SG. Uterus transplantation: a rapidly expanding field. Transplantation. 2018;102:569–77.Google Scholar
Jones, BP, Saso, S, Bracewell-Milnes, T, Thum, M-Y, Nicopoullos, J, Diaz-Garcia, C, et al. Human uterine transplantation: a review of outcomes from the first 45 cases. BJOG. 2019;126:1310–9.Google Scholar
Brännström, M, Johannesson, L, Bokström, H, Kvarnstrom, N, Molne, J, Dahm-Kahler, P, et al. Livebirth after uterus transplantation. Lancet. 2015;385:607–16.Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure [email protected] is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×