Peculiarities of differential diagnostics and surgical treatment of complications in female patients with Mayer—Rokitansky—Küster—Hauser syndrome

Khardikov A.V.; Petrov S.V.; Petrova A.S.

doi:https://doi.org/10.17116/rosakush202424061107

Khardikov A.V.

Kursk State Medical University

ORCID: 0000-0002-7394-4622

Petrov S.V.

Arivamed LLC

ORCID: 0009-0007-8755-4805

Petrova A.S.

Kursk State Medical University

ORCID: 0009-0001-6809-4328

Peculiarities of differential diagnostics and surgical treatment of complications in female patients with Mayer—Rokitansky—Küster—Hauser syndrome

Authors:

Khardikov A.V., Petrov S.V., Petrova A.S.

More about the authors

Journal: Russian Bulletin of Obstetrician-Gynecologist. 2024;24(6): 107‑111

DOI: 10.17116/rosakush202424061107

Read: 816 times

Contact the author

Table of contents

To cite this article:

Khardikov AV, Petrov SV, Petrova AS. Peculiarities of differential diagnostics and surgical treatment of complications in female patients with Mayer—Rokitansky—Küster—Hauser syndrome. Russian Bulletin of Obstetrician-Gynecologist. 2024;24(6):107‑111. (In Russ.)
https://doi.org/10.17116/rosakush202424061107

Подписка 2026 Российский вестник акушера-гинеколога (Russian Bulletin of Obstetrician-Gynecologist)

Использование инфографики авторами научных работ

Abstract:

A clinical observation is presented — a retrospective analysis of the medical history of a patient with Mayer—Rokitansky—Küster—Hauser syndrome and a myoma that developed from uterine ru-diments. The reasons leading to diagnostic errors in the examination of patients with complicated forms of Mayer—Rokitansky—Küster—Hauser syndrome are indicated, and an algorithm for diagno-sis and treatment is proposed that allows increasing their effectiveness. To optimize diagnostics and differential diagnostics of complications in Mayer—Rokitansky—Küster—Hauser syndrome, it is necessary to use clinical and anamnestic data, transvaginal, transabdominal, and transrectal ultra-sound, magnetic resonance imaging, tumor marker testing, laparoscopy, and histological examina-tion of the removed specimen (biopsy). The optimal volume of surgical treatment is removal of the fibroid and uterine rudiments on both sides. If their complete removal is impossible, regular monitoring is indicated for early diagnostics and treatment of possible relapse.

Keywords:

Mayer-Rokitansky-Küster-Hauser syndrome

uterine aplasia

uterine rudiments

fi-broid

Authors:

Khardikov A.V.

Kursk State Medical University

ORCID: 0000-0002-7394-4622

Petrov S.V.

Arivamed LLC

ORCID: 0009-0007-8755-4805

Petrova A.S.

Kursk State Medical University

ORCID: 0009-0001-6809-4328

Received:

20.12.2023

Accepted:

19.01.2024

Close metadata

References:

Batyrova ZK, Uvarova EV, Kumykova ZKh, Kruglyak DA, Bol’shakova AS, Sadelov IO, Zaretskaya NV, Trofimov DYu. Modern molecular — genetic concepts of Mayer—Rokitansky—Küster—Hauser syndrome. Reproduktivnoe zdorov’e detei i podrostkov. 2022;18:2:25-30. (In Russ.).
Kruglyak DA, Buralkina NA, Ipatova MV, Batyrova ZK, Uvarova EV. Vaginal and uterine aplasia (Mayer—Rokitansky—Küster—Hauser syndrome): etiology, pathogenetic aspects and theories of defect formation (literature review). Ginekologiya. 2018;20:2:64-66. (In Russ.). https://doi.org/10.26442/2079-5696_2018.2.64-66
Sibirskaya EV, Karachentsova IV, Panasenko TS. Mayer—Rokitansky—Küster—Hauser syndrome. Effektivnaya farmakoterapiya. 2022;18:24:38-42. (In Russ.).
Uvarova EV. Clinical recommendations (project). Congenital malformations of the vagina and/or uterus in children and adolescents. Reproduktivnoe zdorov’e detei i podrostkov. 2021;17:3:31-56. (In Russ.).
Fontana L, Gentilin B, Fedele L, Gervasini C, Miozzo M. Genetics of Mayer—Rokitansky—Kuster—Hauser (MRKH) syndrome. Clin Genet. 2017;91:2:233-246.
Makiyan ZN, Adamyan LV, Asaturova AV, Yarygina NK. Uterine rudiments: Clinical and morphological variants and optimization of surgical treatment. Akusherstvo i ginekologiya. 2019;12: 126-132. (In Russ.).
Asaturova AV, Fayzullina NM, Bobkova MV, Arakelyan AS, Tregubova AV, Smolnova TU, Adamyan LV. Morphological features and the functional state of connective tissue of the uterine rudiments in reproductive age patients with Mayer—Rokitansky—Küster—Hauser syndrome. Clin exp morphology. 2020;9:4:24-30. https://doi.org/10.31088/CEM2020.9.4.24-30
Adamyan LV, Kulakov VI, Khashukova AZ. Malformations of the uterus and vagina. Moscow: Meditsina. 1998;320. (In Russ.).
Forsnes E V, Derrough KR, O’Donoghue M. A mitotically active myoma in Mayer—Rokitansky—Küster—Hauser syndrome. Obstet Gynecol. 1999;94:862.
Makiyan ZN, Adamyan LV, Yarygina NK, Asaturova AV. Giant uterine fibroids in uterine and vaginal aplasia. Akusherstvo i ginekologiya. 2020;8:149-152. (In Russ.).
Blontzos N, Iavazzo S, Vorgias G, Kalinoglou N. Leuomyoma development in Mayer–Rokitansky—Küster—Hauser syndrome: a case report and narrative review of the literature. Obstet Gynecol Sci. 2019;62:4:294-297.
Shalini Mahana Valecha, Prajakta Katdare, Uday Kargar, Prajakta Shende. Multiple fibroid: Case of MRKH Syndrome with absent uterus: recommendations for management. Indian J Clin Pract. 2014;24:9:856-858.
Sharma R, Guleria K, Suneja A, Bhaskaran S, Tanveer N. Giant leiomyoma with extensive myxoid degeneration in Mayer—Rokitansky—Küster—Hauser syndrome. Int J Gynaecol Obstet. 2017;138:1:125-127.
Favre-Inhofer A, Rafii A, Carbonnel M, Revaux A, Ayoubi JM. Uterine transplantation: Review in human research. J Gynecol Obstet Hum Reprod. 2018;47:6:213-221.
Beecham CT, Skiendzielewski J. Myoma in association with Mayer—Rokitansky—Küster—Hauser syndrome. Am J Obstet Gynecol. 1977;129:346-348.
Garifullova Yu.V., Zhuravleva VI, Kalimulina GN. A rare clinical case of fibroids in Mayer—Rokitansky—Küster—Hauser syndrome. Prakticheskaya meditsina. 2021;19;2:S0—S3. (In Russ.). https://doi.org/10.32000/2072-1757-2021-2-80-83
Amaratunga T, Kirkpartrick I, Yan Y, Karlichi F Ectopic pelvic fibroid in a woman with uterine agenesis and Mayer—Rokitansky—Küster—Hauser syndrome. Ultrasound Q. 2017;33:3:237-241.
van Troost MHW, Geomini PMAJ, Bongers MY. Laparoscopic removal of a large uterine leiomyoma in a patient with Mayer—Rokitansky—Küster—Hauser syndrome: a case report. Gynecol Surg. 2012;9:445-447. https://doi.org/10.1007/s10397-012-0747-3
Papa G, Andreotti M, Giannubilo SR, Cesari R, Ceré I, Tranquilli AL. Case report and surgical solution for a voluminous uterine leiomyoma in a woman with complicated Mayer—Rokitansky—Küster—Hauser syndrome. Fertil Steril. 2008;90:5:2014.e5-6. https://doi.org/10.1016/j.fertnstert.2008.04.061