{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T15:21:06Z","timestamp":1790004066394,"version":"4.0.1"},"reference-count":25,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2024,11,10]],"date-time":"2024-11-10T00:00:00Z","timestamp":1731196800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Women with an aplastic uterus (ESHRE\/ESGE classification) or M\u00fcllerian agenesis (ASRM MAC 2021) might present with functional uterine remnants. Our study aimed to report the clinical course of symptomatic uterine rudiments in adolescents and adults with Mayer\u2013Rokitansky\u2013K\u00fcster\u2013Hauser syndrome (MRKHS). Methods: This study involved 20 patients with MRKHS who, between 2012 and 2023, underwent surgery for symptomatic uterine horns at the mean age of 25.2 years in the Division of Gynaecology, Gynaecological and Obstetric Clinical Hospital, Poznan University of Medical Sciences, Poland. The records of the patients were retrospectively analysed. Results: The volume of the uterine horns ranged from 0.8 to 58.3 cm3, and the volume of the endometrial cavity within the horns ranged from 0.03 to 12 cm3, with no significant difference between adolescents and adults (p = 0.36). In five patients (25%), MRKHS was identified during the diagnosis of recurrent pelvic pain at the age of 12.6\u201314.8 years. In 19 patients, uterine rudiments were removed: unilaterally in 3 patients (16%), bilaterally in 16 patients (84%), and laparoscopically in 89% of cases. In one patient, the horn was preserved (horno-neovaginal anastomosis). Histopathology confirmed the presence of the endometrium in uterine rudiments ipsilateral to the pain location in 75% of cases. Four patients (20%) were diagnosed with endometriosis. Conclusions: Recurrent pelvic pain in patients with MRKHS should prompt the diagnosis of functional uterine rudiments. The resection of symptomatic uterine horns can result in the complete resolution of pain. Patients with endometriosis are at risk of pain recurrence. In some patients strongly desirous of menstruation, horno-neovaginal anastomosis can be cautiously attempted.<\/jats:p>","DOI":"10.3390\/jcm13226767","type":"journal-article","created":{"date-parts":[[2024,11,11]],"date-time":"2024-11-11T11:34:11Z","timestamp":1731324851000},"page":"6767","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Symptomatic Uterine Rudiments in Adolescents and Adults with Mayer\u2013Rokitansky\u2013K\u00fcster\u2013Hauser Syndrome (MRKHS): Management and Outcomes"],"prefix":"10.3390","volume":"13","author":[{"given":"Maria","family":"Buda","sequence":"first","affiliation":[{"name":"Gynaecological and Obstetric Clinical Hospital of Poznan University of Medical Sciences, Polna 33, 60-535 Poznan, Poland"},{"name":"Physiology Department, Poznan University of Medical Sciences, 60-781 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-6344-0204","authenticated-orcid":false,"given":"Weronika","family":"Zaj\u0105czkowska","sequence":"additional","affiliation":[{"name":"Division of Gynaecology, Poznan University of Medical Sciences, Polna 33, 60-535 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaudyna","family":"Madziar","sequence":"additional","affiliation":[{"name":"Division of Gynaecology, Poznan University of Medical Sciences, Polna 33, 60-535 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Witold","family":"K\u0119dzia","sequence":"additional","affiliation":[{"name":"Division of Gynaecology, Poznan University of Medical Sciences, Polna 33, 60-535 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0218-6841","authenticated-orcid":false,"given":"Karina","family":"Kapczuk","sequence":"additional","affiliation":[{"name":"Division of Gynaecology, Poznan University of Medical Sciences, Polna 33, 60-535 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,11,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1093\/humrep\/dei381","article-title":"Clinical aspects of Mayer-Rokitansky-Kuester-Hauser syndrome: Recommendations for clinical diagnosis and staging","volume":"21","author":"Oppelt","year":"2006","journal-title":"Hum. Reprod."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1016\/j.jgyn.2008.07.002","article-title":"Le syndrome de Mayer-Rokitansky-K\u00fcster-Hauser (MRKH): Clinique et g\u00e9n\u00e9tique [The Mayer-Rokitansky-K\u00fcster-Hauser (MRKH) syndrome: Clinical description and genetics]","volume":"37","author":"Morcel","year":"2008","journal-title":"J. Gynecol. Obstet. Biol. Reprod."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Romano, F., Carlucci, S., Stabile, G., Mirenda, G., Mirandola, M., Mangino, F.P., Romano, A., and Ricci, G. (2021). The Rare, Unexpected Condition of a Twisted Leiomyoma in Mayer-Rokitansky-K\u00fcster-Hauser (MRKH) Syndrome: Etiopathogenesis, Diagnosis and Management. Our Experience and Narrative Review of the Literature. Int. J. Environ. Res. Public Health, 18.","DOI":"10.3390\/ijerph18115895"},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Herlin, M.K. (2024). Genetics of Mayer-Rokitansky-K\u00fcster-Hauser (MRKH) syndrome: Advancements and implications. Front. Endocrinol., 15.","DOI":"10.3389\/fendo.2024.1368990"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1083","DOI":"10.1159\/000464116","article-title":"Decidualization is Impaired in Endometrial Stromal Cells from Uterine Rudiments in Mayer-Rokitansky-K\u00fcster-Hauser Syndrome","volume":"41","author":"Brucker","year":"2017","journal-title":"Cell Physiol. Biochem."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1392","DOI":"10.1016\/j.fertnstert.2012.12.002","article-title":"Uterine rudiments in patients with Mayer-Rokitansky-Kuster-Hauser syndrome consist of typical uterine tissue types with predominantly basalis-like endometrium","volume":"99","author":"Rall","year":"2013","journal-title":"Fertil. Steril."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1007\/s10397-013-0800-x","article-title":"The ESHRE-ESGE consensus on the classification of female genital tract congenital anomalies","volume":"10","author":"Grimbizis","year":"2013","journal-title":"Gynecol. Surg."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1016\/j.fertnstert.2021.09.025","article-title":"ASRM m\u00fcllerian anomalies classification 2021","volume":"116","author":"Pfeifer","year":"2021","journal-title":"Fertil. Steril."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"e86","DOI":"10.1016\/j.fertnstert.2011.06.006","article-title":"Presurgical management of dysmenorrhea and endometriosis in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome","volume":"96","author":"Elliott","year":"2011","journal-title":"Fertil. Steril."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1093\/humrep\/dev264","article-title":"The Thessaloniki ESHRE\/ESGE consensus on diagnosis of female genital anomalies","volume":"31","author":"Grimbizis","year":"2016","journal-title":"Hum. Reprod."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Oppelt, P.G., Lermann, J., Strick, R., Dittrich, R., Strissel, P., Rettig, I., Schulze, C., Renner, S.P., Beckmann, M.W., and Brucker, S. (2012). Malformations in a cohort of 284 women with Mayer-Rokitansky-K\u00fcster-Hauser syndrome (MRKH). Reprod. Biol. Endocrinol., 10.","DOI":"10.1186\/1477-7827-10-57"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1621","DOI":"10.1007\/s00330-014-3156-3","article-title":"Clinical value of magnetic resonance imaging in patients with Mayer-Rokitansky-K\u00fcster-Hauser (MRKH) syndrome: Diagnosis of associated malformations, uterine rudiments and intrauterine endometrium","volume":"24","author":"Preibsch","year":"2014","journal-title":"Eur. Radiol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/j.jpag.2012.11.014","article-title":"Uterine remnants and pelvic pain in females with Mayer-Rokitansky-K\u00fcster-Hauser syndrome","volume":"26","author":"Marsh","year":"2013","journal-title":"J. Pediatr. Adolesc. Gynecol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"43","DOI":"10.4137\/JCM.S12611","article-title":"Functioning endometrium and endometrioma in a patient with mayer-rokitanski-kuster-hauser syndrome","volume":"5","author":"Kawano","year":"2014","journal-title":"Jpn. Clin. Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.jpag.2018.11.006","article-title":"M\u00fcllerian Aplasia with Severe Hematometra: A Case Report of Diagnosis and Management in a Low Resource Setting","volume":"32","author":"Howard","year":"2019","journal-title":"J. Pediatr. Adolesc. Gynecol."},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Lih Yuan, T., Sulaiman, N., Nur Azurah, A.G., Maarof, M., Rabiatul, A.R., and Yazid, M.D. (2022). Oestrogen-induced epithelial- mesenchymal transition (EMT) in endometriosis: Aetiology of vaginal agenesis in Mayer-Rokitansky-K\u00fcster- Hauser (MRKH) syndrome. Front. Physiol., 13.","DOI":"10.3389\/fphys.2022.937988"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"101655","DOI":"10.1016\/j.jogoh.2019.101655","article-title":"Postoperative evaluation of chronic pain in patients with Mayer-Rokitansky-K\u00fcster-Hauser (MRKH) syndrome and uterine horn remnant: Experience of a tertiary referring gynaecological department","volume":"49","author":"Dabi","year":"2020","journal-title":"J. Gynecol. Obstet. Hum. Reprod."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"(1997). Revised American Society for Reproductive Medicine classification of endometriosis: 1996. Fertil. Steril., 67, 817\u2013821.","DOI":"10.1016\/S0015-0282(97)81391-X"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/j.jpag.2018.05.002","article-title":"Age of Menarche in a Longitudinal US Cohort","volume":"31","author":"Biro","year":"2018","journal-title":"J. Pediatr. Adolesc. Gynecol."},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Committee on Adolescent Health Care (2018). ACOG Committee Opinion No. 728: M\u00fcllerian Agenesis: Diagnosis, Management, And Treatment. Obstet. Gynecol., 131, e35\u2013e42.","DOI":"10.1097\/AOG.0000000000002458"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1016\/j.jmig.2023.05.002","article-title":"Rokitansky Syndrome or Cervicovaginal Atresia?","volume":"30","author":"Fedele","year":"2023","journal-title":"J. Minim. Invasive Gynecol."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Di Michele, S., Bramante, S., Angioni, S., Bernassola, M., De Vita, T., Iaccarino, D.A., Giannoni, L., and Rosati, M. (2024). Superficial Peritoneal Endometriosis Vaporization Using a CO2 Laser: A Long-Term Single-Center Experience. J. Clin. Med., 13.","DOI":"10.20944\/preprints202402.1716.v1"},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Pietzsch, M., Sch\u00f6nfisch, B., H\u00f6ller, A., Koch, A., Staebler, A., Dreser, K., Bettecken, K., Schaak, L., Brucker, S.Y., and Rall, K. (2024). A Cohort of 469 Mayer\u2013Rokitansky\u2013K\u00fcster\u2013Hauser Syndrome Patients\u2014Associated Malformations, Syndromes, and Heterogeneity of the Phenotype. J. Clin. Med., 13.","DOI":"10.3390\/jcm13020607"},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"Steinmacher, S., B\u00f6sm\u00fcller, H., Granai, M., Koch, A., Brucker, S.Y., and Rall, K.K. (2022). Endometriosis in Patients with Mayer-Rokitansky-K\u00fcster-Hauser-Syndrome-Histological Evaluation of Uterus Remnants and Peritoneal Lesions and Comparison to Samples from Endometriosis Patients without Mullerian Anomaly. J. Clin. Med., 11.","DOI":"10.3390\/jcm11216458"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"e1821","DOI":"10.1002\/rcs.1821","article-title":"Outcomes in patients undergoing robotic reconstructive uterovaginal anastomosis of congenital cervical and vaginal atresia","volume":"13","author":"Zhang","year":"2017","journal-title":"Int. J. Med. Robot."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/22\/6767\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:29:49Z","timestamp":1760113789000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/22\/6767"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11,10]]},"references-count":25,"journal-issue":{"issue":"22","published-online":{"date-parts":[[2024,11]]}},"alternative-id":["jcm13226767"],"URL":"https:\/\/doi.org\/10.3390\/jcm13226767","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,11,10]]}}}