Authors :
Maha Lhaloui; Hassnaa Sarhane; Kawtar Bahida; Nouhaila Yartaoui; Fatima Zahra Belouzza; Amina Etber; Nisrine Benouicha; Aziz Baydada
Volume/Issue :
Volume 10 - 2025, Issue 8 - August
Google Scholar :
https://tinyurl.com/4jywe294
Scribd :
https://tinyurl.com/mr2mm9nx
DOI :
https://doi.org/10.38124/ijisrt/25aug113
Note : A published paper may take 4-5 working days from the publication date to appear in PlumX Metrics, Semantic Scholar, and ResearchGate.
Note : Google Scholar may take 30 to 40 days to display the article.
Abstract :
The congenital malformation results from an abnormal development of the Müllerian ducts during
embryogenesis. Primary amenorrhea and primary infertility are the main symptoms leading patients to seek medical
consultation. Pelvic ultrasound remains the gold standard for diagnosis. The therapeutic approach is multidisciplinary, and
treatment should only be considered once the patient has reached adequate awareness and emotional maturity.
Keywords :
Mayer-Rokitansky-Kusterhauser Syndrom, Infertility, Primary, Amenorrhea.
References :
- https://doi.org/10.1016/j.ando.2020.07.691
- Morcel K, Guerrier D, Watrin T, Pellerin I, Levêque J. Le syndrome de Mayer-Rokitansky-Küster-Hauser (MRKH) : clinique et génétique. J Gynecol Obstet Biol Reprod 2008;37(6):539–46.
- Morcel K, Watrin T, Pasquier L, Rochard L, Le Caignec C, Dubourg C, et al. Utero-vaginal aplasia (Mayer-Rokitansky-Küster-Hauser syndrome) associated with deletionsin known DiGeorge or DiGeorge-like loci. Orphanet J Rare Dis 2011;6:9
- Choussein S, Nasioudis D, Schizas D, Economopoulos KP. Mullerian dysgenesis: a critical review of the literature. Arch Gynecol Obstet 2017;295(6):1369–81.
- Adil R, Qayyum A. Mayer-Rokitansky-Kuster-Hauser syndrome. J Ayub Med Coll Abbottabad 2013;25(1–2):208–9.
- Pizzo A, Lagana` AS, Sturlese E, Retto G, Retto A, De Dominici R, et al. MayerRokitansky-Kuster-Hauser syndrome: embryology, genetics and clinical and surgical treatment. ISRN Obstet Gynecol 2013;2013:628717 [Published online 2013 Feb 4]
- Labrosse J, Peigné M. Aménorrhées. EMC - Traité de Médecine Akos 2024;28(1):1-13 [Article 1-0230].
- Carson SA, Simpson JL, Malinak LR, Elias S, Gerbie AB, Buttram VC, et al. Heritable aspects of uterine anomalies. II. Genetic analysis of Mullerian aplasia. Fertil Steril 1983;40: 86—90.
- Timmreck LS, Gray MR, Handelin B, Allito B, Rohlfs E, Davis AJ, et al. Analysis of cystic fibrosis transmembrane conductance regulator gene mutations in patients with congenital absence
- Guitron-Cantu A, Lopez-Vera E, Forsbach-Sanchez G, LealGarza CH, Cortes-Gutierrez EI, Gonzalez-Pico I. Gonadal dysgenesis and Rokitansky syndrome. A case report. J Reprod Med 1999;44:891—3.
- Gorgojo JJ, Almodovar F, Lopez E, Donnay S. Gonadal agenesis 46,XX associated with the atypical form of Rokitansky syndrome. Fertil Steril 2002;77:185—7.
- Gardo S, Papp Z, Gaal J. XO-XX Mosaicism in the RokitanskyKuster-Hauser syndrome. Lancet 1971;2:1380—1.
- Linquette M, Gasnault JP, Dupont-Lecompte J, Lefebvre J. A case of utero-vaginal aplasia with polycystic kidney and mosaic XX = XXX. Bull Fed Soc Gynecol Obstet Lang Fr 1968;20:26—8.
- Aydos S, Tukun A, Bokesoy I. Gonadal dysgenesis and the Mayer-Rokitansky-Kuster-Hauser syndrome in a girl with 46,X,del(X)(pter–>q22:). Arch Gynecol Obstet 2003;267:173—4
- Rousset P, Raudrant D, Peyron N, Buy JN, Valette PJ, Hoeffel C. Ultrasonography and MRI features of the Mayer-Rokitansky-Ku¨ ster-Hauser syndrome. Clin Radiol 2013;68(9):945–52
- Fiaschetti V, Taglieri A, Gisone V, Coco I, Simonetti G. Mayer-RokitanskyKuster-Hauser Syndrome diagnosed by Magnetic Resonance Imaging. Role of Imaging to identify and evaluate the uncommon variation in development of the female genital tract. J Radiol Case Rep 2012;6(4):17–24 [Published online 2012 Apr 1].
- Yoo R-E, Cho JY, Kim SY, Kim SH. Magnetic resonance evaluation of Mu¨llerian remnants in Mayer-Rokitansky-Ku¨ ster-Hauser syndrome. Korean J Radiol 2013;14(2):233–9 [Published online 2013 Feb 22].
- https://asso-mrkh.org/
- Frank T. The formation of an artificial vagina without operation. Am J Obstet Gynecol 1938;35:1053–5.
- Fedele L, Bianchi S, Frontino G, Fontana E, Restelli E, Bruni V. The laparoscopic Vecchietti’s modified technique in Rokitansky syndrome: anatomic, functional, and sexual long-term results. Am J Obstet Gynecol 2008;198, 377 e1-6.
- Dargent D, Marchiole P, Giannesi A, Benchaib M, Chevret-Measson M, Mathevet P. Laparoscopic Davydov or laparoscopic transposition of the peritoneal colpopoeisis described by Davydov for the treatment of congenital vaginal agenesis: the technique and its evolution. Gynecol Obstet Fertil 2004;32:1023–30
The congenital malformation results from an abnormal development of the Müllerian ducts during
embryogenesis. Primary amenorrhea and primary infertility are the main symptoms leading patients to seek medical
consultation. Pelvic ultrasound remains the gold standard for diagnosis. The therapeutic approach is multidisciplinary, and
treatment should only be considered once the patient has reached adequate awareness and emotional maturity.
Keywords :
Mayer-Rokitansky-Kusterhauser Syndrom, Infertility, Primary, Amenorrhea.