Rokitansky syndrome in combination with ovariocele in an eight-year-old girl newly diagnosed during laparoscopic appendectomy
Severinov D.A., Zakutaeva L.Yu., Statina M.I., Skirda T.V.
Rationale. Mayer–Rokitansky–Küster–Hauser syndrome is a congenital disorder characterized by aplasia of the uterus and upper vagina in women with normal secondary sex characteristics and a normal female karyotype (46, XX).
Case report. An 8-year-old girl with her mother were admitted to the emergency department of the regional hospital with moderate abdominal pain, nausea, and a body temperature increase to 37.2°C. Upon examination, appendiceal symptoms were positive. Laboratory and instrumental studies revealed neutrophilic leukocytosis, and ultrasound scan identified appendicitis. An emergency laparoscopic appendectomy with an examination of the pelvic organs revealed the absence of the uterus, and dilated tortuous veins approaching the adnexa on both sides, forming a vascular plexus in the area of the absent uterus. An intraoperative consultation with a gynecologist was held: Mayer–Rokitansky–Küstner–Hauser syndrome (uterus aplasia/agenesis) was suspected. The postoperative period was uneventful. The girl was discharged on the fifth postoperative day. Follow-up period is 2 years, menses are absent, no pain. The girl is being monitored by a gynecologist at the regional hospital.
Conclusion. Thus, the described case of a combination of developmental malformation of the reproductive system organs in a girl (Mayer–Rokitansky–Küstner–Hauser syndrome with ovariocele) is a rare variant of the association of defects.
Authors’ contributions. Severinov D.A. – study concept and design, manuscript editing; Zakutaeva L.Yu., Statina M.I. – material collection and processing; Skirda T.V. – manuscript writing.
Conflicts of interest. The authors declare no conflict of interest.
Funding. The authors did not receive additional funding.
Generative Artificial Intelligence. No generative artificial intelligence technologies were used while writing this article.
Patient Consent for Publication. Written informed consent for the publication of the case and anonymous medical information was obtained from the child's legal representative.
For citation: Severinov D.A., Zakutaeva L.Yu., Statina M.I., Skirda T.V. Rokitansky syndrome in
combination with ovariocele in an eight-year-old girl newly diagnosed during laparoscopic appendectomy.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2026; (7): 193-197 (in Russian)
https://dx.doi.org/10.18565/aig.2026.31
Keywords
Mayer–Rokitansky–Küstner–Hauser syndrome
uterus aplasia
vagina aplasia
children
laparoscopic appendectomy
References
- Кругляк Д.А., Буралкина Н.А., Ипатова М.В., Батырова З.К., Уварова Е.В. Аплазия влагалища и матки (синдром Майера–Рокитанского–Кюстнера–Хаузера): этиология, патогенетические аспекты и теории формирования порока (обзор литературы). Гинекология. 2018; 20(2): 64-6. https://dx.doi.org/10.26442/2079-5696_2018.2.64-66 [Kruglyak D.A., Buralkina N.A., Ipatova M.V., Batyrova Z.K., Uvarova E.V. Aplasia of the vagina and uterus (Mayer–Rokitansky–Küster–Hauser syndrome): etiology, pathogenetic aspects and theories of defect formation (literature review). Gynecology. 2018; 20(2): 64-6 (in Russian). https://dx.doi.org/10.26442/2079-5696_2018.2.64-66].
- Сибирская Е.В., Караченцова И.В., Никифорова П.О., Меленчук И.А., Аннакулиева А.С., Ковалева А.А. Абсолютное маточное бесплодие (синдром Майера–Рокитанского–Кюстера–Хаузера). Эффективная фармакотерапия. 2025; 21(4): 74-80. https://dx.doi.org/10.33978/2307-3586-2025-21-4-74-80 [Sibirskaya E.V., Karachentsova I.V., Nikiforova P.O., Melenchuk I.A., Annakulieva A.S., Kovaleva A.A. Absolute uterine infertility (Mayer–Rokitansky–Küster–Hauser syndrome). Effective Pharmacotherapy. 2025; 21(4): 74-80 (in Russian). https://dx.doi.org/10.33978/2307-3586-2025-21-4-74-80].
- Ющенко М.А., Робул А.В., Елгина С.И., Мозес В.Г., Рудаева Е.В., Железная А.А., Мраморнова П.Н., Мозес К.Б. Синдром Майера–Рокитанского–Кюстера–Хаузера (клинический случай). Мать и дитя в Кузбассе. 2022; 2(89): 35-9. https://dx.doi.org/10.24412/2686-7338-2022-2-35-39 [Yushchenko M.A., Robul A.V., Elgina S.I., Mozes V.G., Rudaeva E.V., Zheleznaya A.A., Mramornova P.N., Mozes K.B. Mayer–Rokitansky–Küster–Hauser syndrome (a clinical case). Mother and child in Kuzbass. 2022; 2(89): 35-9 (in Russian). https://dx.doi.org/10.24412/2686-7338-2022-2-35-39].
- Oyania F., Commander S.J., Mugarura R., Situma M. 3-year-old with Mayer–Rokitansky–Küster–Hauser syndrome and anorectal malformation: a case report. Int. J. Surg. Case Rep. 2023; 106: 108120. https://dx.doi.org/10.1016/j.ijscr.2023.108120
- Гаврилюк В.П., Северинов Д.А., Зубкова Ю.А. Краткосрочные исходы симультанной герниопластики паховых грыж при лапароскопической аппендэктомии у детей: когортное исследование. Вопросы современной педиатрии. 2024; 23(1): 21-7. https://dx.doi.org/10.15690/vsp.v23i1.2651 [Gavrilyuk V.P., Severinov D.A., Zubkova Yu.A. Short-term outcomes of simultaneous inguinal hernia repair at laparoscopic appendectomy in children: cohort study. Current Pediatrics. 2024; 23(1): 21-7 (in Russian). https://doi.org/10.15690/vsp.v23i1.2651].
- Хворостов И.Н., Синицын А.Г. Хронический абдоминальный синдром с локализацией в правой подвздошной области у детей: хирургические аспекты. Российский педиатрический журнал. 2020; 23(3): 198-203. https://dx.doi.org/10.18821/1560-9561-2020-23-3-198-203 [Khvorostov I.N., Sinitsyn A.G. Chronic abdominal syndrome with localization in the right iliac region in children: surgical aspects. Russian Pediatric Journal. 2020; 23(3): 198-203 (in Russian). https://dx.doi.org/10.18821/1560-9561-2020-23-3-198-203].
- Herlin M.K., Petersen M.B., Brännström M. Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome: a comprehensive update. Orphanet J. Rare Dis. 2020; 15: 214. https://dx.doi.org/10.1186/s13023-020-01491-9
- Батырова З.К., Уварова Е.В., Кумыкова З.Х., Кругляк Д.А., Большакова А.С., Саделов И.О., Зарецкая Н.В., Трофимов Д.Ю. Современные молекулярно-генетические представления о синдроме Майера–Рокитанского–Кюстера–Хаузера. Репродуктивное здоровье детей и подростков. 2022; 18(2): 25-30. https://dx.doi.org/10.33029/1816-2134-2022-18-2-25-30 [Batyrova Z.K., Uvarova E.V., Kumykova Z.H., Kruglyak D.A., Bolshakova A.S., Sadelov I.O., Zaretskaya N.V., Trofimov D.Yu. Modern molecular genetic concepts in Mayer–Rokitansky–Küster–Hauser syndrome. Pediatric and Adolescent Reproductive Health. 2022; 18(2): 25-30 (in Russian). https://dx.doi.org/10.33029/1816-2134-2022-18-2-25-30].
- Herlin M.K. Genetics of Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome: advancements and implications. Front. Endocrinol. 2024; 15: 1368990. https://dx.doi.org/10.3389/fendo.2024.1368990
- Фармонова Р.Ф., Норжигитов А.М. Морфологические аспекты острого аппендицита у детей. Central Asian Journal of Multidisciplinary Research and Management Studies. 2024; 1(15): 13-5. https://dx.doi.org/10.5281/zenodo.13927858 [Farmonova R.F., Norzhigitov A.M. Morphological aspects of acute appendicitis in children. Central Asian Journal of Multidisciplinary Research and Management Studies. 2024; 1(15): 13-5 (in Russian). https://dx.doi.org/10.5281/zenodo.13927858].
- Lin X., Kang J., Zhu L. Recent advances in vaginal atresia: a literature review. Biomedicines. 2025; 8; 13(1): 128. https://dx.doi.org/10.3390/biomedicines13010128
- Бабина А.С. Синдром Майера–Рокитанского–Кюстера–Хаузера как причина бесплодия. Способы лечения. Международный журнал гуманитарных и естественных наук. 2022; 4-1(67): 58-60. https://dx.doi.org/10.24412/2500-1000-2022-4-1-58-60 [Babina A.S. Mayer–Rokitansky–Küster–Hauser syndrome as a cause of infertility. Methods of treatment. International Journal of Humanities and Natural Sciences. 2022; 4-1(67): 58-60 (in Russian). https://dx.doi.org/10.24412/2500-1000-2022-4-1-58-60].
- Mentessidou A., Mirilas P. Surgical disorders in pediatric and adolescent gynecology: vaginal and uterine anomalies. Int. J. Gynecol. Obstet. 2023; 160(3): 762-70. https://dx.doi.org/10.1002/ijgo.14362
- Chen N., Song S., Bao X., Zhu L. Update on Mayer–Rokitansky–Küster–Hauser syndrome. Front. Med. 2022; 16(6): 859-72. https://dx.doi.org/10.1007/s11684-022-0969-3
- Ахапкина Е.С., Батырова З.К., Уварова Е.В., Наджарян А.Г. Клинико-анамнестические и эндокринологические особенности девочек с синдромом Майера–Рокитанского–Кюстера–Хаузера. Репродуктивное здоровье детей и подростков. 2019; 15(4): 33-40. https://dx.doi.org/10.24411/1816-2134-2019-14004 [Akhapkina E.S., Batyrova Z.K., Uvarova E.V., Nadzharyan A.G. Clinical-anamnestic and endocrinological features of girls with Mayer–Rokitansky–Küster–Hauser syndrome. Pediatric and Adolescent Reproductive Health. 2019; 15(4): 33-40 (in Russian). https://dx.doi.org/10.24411/1816-2134-2019-14004].
Received 02.02.2026
Accepted 07.07.2026
About the Authors
Dmitry A. Severinov, PhD, Associate Professor at the Department of Pediatric Surgery and Pediatrics, Institute of Continuing Education, Kursk State Medical University, Ministry of Health of Russia, 305041, Russia, Kursk, Karl Marx str., 3, +7(920)262-15-55, dmitriy.severinov.93@mail.ru, https://orcid.org/0000-0003-4460-1353Lyudmila Yu. Zakutaeva, Teaching Assistant at the Department of Pediatric Surgery and Pediatrics, Institute of Continuing Education, Kursk State Medical University,
Ministry of Health of Russia, 305041, Russia, Kursk, Karl Marx str., 3, https://orcid.org/0000-0002-7204-1851
Maria I. Statina, Teaching Assistant at the Department of Pediatric Surgery and Pediatrics, Institute of Continuing Education, Kursk State Medical University,
Ministry of Health of Russia, 305041, Russia, Kursk, Karl Marx str., 3. https://orcid.org/0000-0001-9918-9306
Tatiana V. Skirda, student at the Pediatric Faculty, Kursk State Medical University, Ministry of Health of Russia, 305041, Russia, Kursk, Karl Marx str., 3,
https://orcid.org/0009-0005-8698-3793
Corresponding author: Dmitry A. Severinov, dmitriy.severinov.93@mail.ru
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