Abstract
Objective: To analyze the main aspects related to Mullerian malformations, including etiopathogenesis, classification, diagnostic methods, and clinical and surgical approaches for their treatment. Literature review: Mullerian duct anomalies are more common congenital alterations than previously thought, with an estimated prevalence between 0.5% and 6.7% in the general population, reaching up to 16.7% among women with recurrent miscarriage. They originate during the development of the paramesonephric ducts due to fusion failure or lack of septum resorption after duct fusion. Key symptoms include primary amenorrhea, dysmenorrhea, pelvic pain, endometriosis, infertility, sexual dysfunction, and psychological impact such as low self-esteem. Given the significant physical and emotional repercussions, the relevance of this study is justified. Final considerations: Data indicating the best outcomes in the follow-up of these patients from diagnosis to treatment are presented. Self-dilation according to Frank's technique is the most recommended method for uterine and vaginal agenesis, though the best treatment remains controversial. In cases of menstrual flow obstruction, immediate surgical intervention is essential to prevent complications and preserve reproductive health and patient quality of life.
References
AKCAOGLU T, ATILGAN AE. Surgical management of longitudinal vaginal septum: A case report and literature review. Eur J Obstet Gynecol Reprod Biol. 2025 Jun;310:113992.
CHEN N, et al. Update on Mayer-Rokitansky-Küster-Hauser syndrome. Front Med. 2022 Dec;16(6):859-872.
DIXIT R, et al. Mullerian anomalies: revisiting imaging and classification. Insights Imaging. 2025 Feb 17;16(1):40.
FEDELE F, et al. Reconstructive surgery for congenital atresia of the uterine cervix: a systematic review. Arch Gynecol Obstet. 2023 Sep;308(3):685-700.
FORSBERG JG. Cervicovaginal epithelium: its origin and development. Am J Obstet Gynecol 1973;115:1025e43.
GOULIOS C, et al. Management of a twin pregnancy in a didelphys uterus: one fetus in each uterine cavity. BMJ Case Rep. 2020 Aug 25;13(8):e235256.
IBIDAPO-OBE O, et al. Incidental Finding of Leiomyoma in Mayer-Rokitansky-Kuster-Hauser Syndrome. J Investig Med High Impact Case Rep. 2021 Jan-Dec;9:23247096211014690.
KAPCZUK K, et al. Obstructive müllerian anomalies in menstruating adolescent girls: a report of 22 cases. J Pediatr Adolesc Gynecol 2018;31(3):252e7.
LIU D, et al. A successful term birth 10 years after laparoscopic Strassman's metroplasty for bicornuate uterus: A case report. Int J Gynaecol Obstet. 2024 Apr;165(1):384-385.
LUDWIN A, LINDHEIM SR. Unicornuate uterus and the noncommunicating functional horn: continued debate on the diagnosis, classification, and treatment. Fertil Steril. 2020 Apr;113(4):772-773.
MA YC, LAW KS. Pregnancy in a Non-Communicating Rudimentary Horn of Unicornuate Uterus. Diagnostics (Basel). 2022 Mar 21;12(3):759.
MORCEL K, et al. The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: clinical description and genetics. J Gynecol Obstet Biol Reprod 2008;37(6):539e46.
MOUFAWAD G, et al. Reconsidering the best minimally invasive approach for patients with herlyn-werner-wunderlich syndrome: Should we push the frontiers for a better outcome? Front Surg. 2023 Apr 11;10:1158753.
PASSOS I, et al. Obstructive mullerian anomaly: uterus didelphys with cervical and vaginal agenesis. J Minim Invasive Gynecol. 2025 Jul 18:S1553-4650(25)00249-3.
PFEIFER SM, et al. ASRM müllerian anomalies classification 2021. Fertil Steril. 2021 Nov;116(5):1238-1252.
PIZZO A, et al. Mayer-Rokitansky-Kuster-Hauser syndrome: embryology, genetics and clinical and surgical treatment. ISRN Obstet Gynecol 2013;2013:628717.
Practice Committee of the American Society for Reproductive Medicine. Electronic address: asrm@asrm.org. Evidence-based diagnosis and treatment for uterine septum: a guideline. Fertil Steril. 2024 Aug;122(2):251-265.
QIU S, et al. Giant hysteromyoma after vaginoplasty in a woman with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: case report and review of the literature. J Int Med Res. 2021 Dec;49(12):3000605211066394.
SAMANTRAY SR, et al. Laparoscopic Davydov's Colpopoiesis for a Case of Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome. Cureus. 2021 Mar 18;13(3):e13974.
SARAVELOS SH, et al. Prevalence and diagnosis of congenital uterine anomalies in women with reproductive failure: a critical appraisal. Hum Reprod Update 2008;14(5):415e29.
WANG L, et al. Non-communicated rudimentary uterine horn pregnancy and uterine rupture: A case report. SAGE Open Med Case Rep. 2022 Jun 14;10:2050313X221104034.
ZHANG H, et al. MRI presentations of Müllerian duct anomalies in association with unilateral renal agenesis. Clin Radiol. 2023 Mar;78(3):168-174.
ZHANG Y, et al. Laparoscopic metroplasty for unicornuate uterus with a functional noncommunicating rudimentary horn. Int J Gynaecol Obstet. 2020 Apr;149(1):31-36.
