{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T21:57:01Z","timestamp":1774303021772,"version":"3.50.1"},"reference-count":20,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,3,25]],"date-time":"2024-03-25T00:00:00Z","timestamp":1711324800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,3,25]],"date-time":"2024-03-25T00:00:00Z","timestamp":1711324800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Med Case Reports"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Introduction<\/jats:title>\n                <jats:p>Menstruation is a developmental milestone and usually marks healthy and normal pubertal changes in females. Menarche refers to the onset of first menstruation in a female. The causes of primary amenorrhea include outflow tract abnormalities, resistant endometrium, primary ovarian insufficiency, and disorders of the hypothalamus, pituitary, or other endocrine glands. A rare variant of mullerian agenesis, which warrants an individualized approach to management, is presented here.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case report<\/jats:title>\n                <jats:p>We present here the case of a 25-year-old Indian female with pain in the lower abdomen and primary amenorrhea. After a thorough history, clinical examination, imaging, and diagnostic laparoscopy, two small uteri, a blind upper half vagina, bilateral polycystic ovaries, and a blind transverse connection between the two uteri\u2014a horseshoe band cervix\u2014were detected, which confirmed the diagnosis of mullerian agenesis. There was evidence of adenomyosis in the mullerian duct element. This is a rare form of M\u00fcllerian abnormality with an unusual presentation.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>Mullerian agenesis is the most common cause of primary amenorrhea with well-developed secondary sexual characteristics. There are various forms of mullerian agenesis. Most of the cases are managed by a multidisciplinary team. Rare variants warrant an individualized approach to management.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13256-024-04438-x","type":"journal-article","created":{"date-parts":[[2024,3,25]],"date-time":"2024-03-25T02:32:08Z","timestamp":1711333928000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["A rare variant of mullerian agenesis: a\u00a0case report and review of the literature"],"prefix":"10.1186","volume":"18","author":[{"given":"Shriya Devendra","family":"Tayade","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nadia","family":"Mehdi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1539-6162","authenticated-orcid":false,"given":"Rajani","family":"Dube","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vaishnavi","family":"Bose","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashna","family":"Ameer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zainabsadat Tabatabaei","family":"Hakim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arnaud","family":"Wattiez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,3,25]]},"reference":[{"key":"4438_CR1","doi-asserted-by":"publisher","unstructured":"ACOG Committee Opinion No. 651. Menstruation in girls and adolescents: using the menstrual cycle as a vital sign. Obstet Gynecol 2015;126(6), e143\u2013e146. https:\/\/doi.org\/10.1097\/AOG.0000000000001215","DOI":"10.1097\/AOG.0000000000001215"},{"key":"4438_CR2","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1016\/0002-9378(81)90029-6","volume":"140","author":"RH Reindollar","year":"1981","unstructured":"Reindollar RH, Byrd JR, McDonough PG. Delayed sexual development: a study of 252 patients. Am J Obstet Gynecol. 1981;140:371\u201380.","journal-title":"Am J Obstet Gynecol"},{"issue":"9","key":"4438_CR3","first-page":"590","volume":"96","author":"DA Klein","year":"2017","unstructured":"Klein DA, Emerick JE, Sylvester JE, Vogt KS. Disorders of puberty: an approach to diagnosis and management. Am Fam Physician. 2017;96(9):590\u20139.","journal-title":"Am Fam Physician"},{"key":"4438_CR4","unstructured":"Welt CK, Barbieri RL. Causes of primary amenorrhea. https:\/\/www.uptodate.com Accessed 20 Nov 2022."},{"key":"4438_CR5","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1111\/cge.12883","volume":"91","author":"L Fontana","year":"2017","unstructured":"Fontana L, Gentilin B, Fedele L, et\u00a0al. Genetics of Mayer\u2013Rokitansky\u2013Kuster\u2013Hauser (MRKH) syndrome. Clin Genet. 2017;91:233\u201346.","journal-title":"Clin Genet"},{"issue":"5","key":"4438_CR6","doi-asserted-by":"publisher","first-page":"1413","DOI":"10.1210\/jc.2017-00131","volume":"102","author":"CM Gordon","year":"2017","unstructured":"Gordon CM, Ackerman KE, Berga SL, et\u00a0al. Functional hypothalamic amenorrhea: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2017;102(5):1413\u201339.","journal-title":"J Clin Endocrinol Metab"},{"issue":"11","key":"4438_CR7","first-page":"781","volume":"87","author":"DA Klein","year":"2013","unstructured":"Klein DA, Poth MA. Amenorrhea: an approach to diagnosis and management. Am Fam Physician. 2013;87(11):781\u20138.","journal-title":"Am Fam Physician"},{"key":"4438_CR8","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1186\/1477-7827-10-57","volume":"10","author":"PG Oppelt","year":"2012","unstructured":"Oppelt PG, Lermann J, Strick R, et\u00a0al. Malformations in a cohort of 284 women with Mayer-Rokitansky-K\u00fcster-Hauser syndrome (MRKH). Reprod Biol Endocrinol. 2012;10:57.","journal-title":"Reprod Biol Endocrinol."},{"issue":"3","key":"4438_CR9","doi-asserted-by":"publisher","first-page":"686","DOI":"10.1016\/j.fertnstert.2011.12.038","volume":"97","author":"DK Edmonds","year":"2012","unstructured":"Edmonds DK, Rose GL, Lipton MG, et\u00a0al. Mayer\u2013Rokitansky\u2013Kuster\u2013Hauser syndrome: a review of 245 consecutive cases managed by a multidisciplinary approach with vaginal dilators. Fertil Steril. 2012;97(3):686\u201390.","journal-title":"Fertil Steril"},{"key":"4438_CR10","doi-asserted-by":"crossref","unstructured":"ACOG Practice Bulletin no. 194: polycystic ovary syndrome. Obstet Gynecol. 2018;131(6):e157-e171.","DOI":"10.1097\/AOG.0000000000002656"},{"key":"4438_CR11","doi-asserted-by":"publisher","first-page":"1238","DOI":"10.1016\/j.fertnstert.2021.09.025","volume":"116","author":"M Samantha","year":"2021","unstructured":"Samantha M, Pfeifer SM, Attaran M, et\u00a0al. ASRM mullerian anomalies classification 2021. Fertil Steril. 2021;116:1238\u201352.","journal-title":"Fertil Steril"},{"key":"4438_CR12","doi-asserted-by":"publisher","unstructured":"ACOG Committee Opinion No. 728, Mullerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 2018;131(1):e35\u2013e42. https:\/\/doi.org\/10.1097\/AOG.0000000000002458","DOI":"10.1097\/AOG.0000000000002458"},{"issue":"7","key":"4438_CR13","doi-asserted-by":"publisher","first-page":"865","DOI":"10.2147\/IJWH.S75637","volume":"2","author":"L Londra","year":"2015","unstructured":"Londra L, Chuong FS, Kolp L. Mayer\u2013Rokitansky\u2013Kuster\u2013Hauser syndrome: a review. Int J Womens Health. 2015;2(7):865\u201370. https:\/\/doi.org\/10.2147\/IJWH.S75637.","journal-title":"Int J Womens Health"},{"issue":"4","key":"4438_CR14","first-page":"17","volume":"6","author":"V Fiaschetti","year":"2012","unstructured":"Fiaschetti V, Taglieri A, Gisone V, et\u00a0al. Mayer\u2013Rokitansky\u2013Kuster\u2013Hauser 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\u201324.","journal-title":"J Radiol Case Rep"},{"issue":"7","key":"4438_CR15","doi-asserted-by":"publisher","first-page":"1621","DOI":"10.1007\/s00330-014-3156-3","volume":"24","author":"H Preibsch","year":"2014","unstructured":"Preibsch H, Rall K, Wietek BM, et\u00a0al. Clinical value of magnetic resonance imaging in patients with Mayer\u2013Rokitansky\u2013Kuster\u2013Hauser (MRKH) syndrome: diagnosis of associated malformations, uterine rudiments and intrauterine endometrium. Eur Radiol. 2014;24(7):1621\u20137.","journal-title":"Eur Radiol"},{"key":"4438_CR16","doi-asserted-by":"publisher","unstructured":"Boruah DK, Sanyal S, Gogoi BB, et\u00a0al. Spectrum of MRI appearance of Mayer\u2013Rokitansky\u2013Kuster\u2013Hauser (MRKH) syndrome in primary amenorrhea patients. J Clin Diagn Res. 2017;11(7):TC30-TC35. https:\/\/doi.org\/10.7860\/JCDR\/2017\/29016.10317.","DOI":"10.7860\/JCDR\/2017\/29016.10317"},{"key":"4438_CR17","doi-asserted-by":"publisher","first-page":"79","DOI":"10.4103\/sjmms.sjmms_204_21","volume":"10","author":"M Ahmed","year":"2022","unstructured":"Ahmed M, Rage KA, Saeed S. Utero-ovarian inguinal hernia in a young female with Mayer\u2013Rokitansky\u2013K\u00fcster\u2013Hauser syndrome type 2. Saudi J Med Med Sci. 2022;10:79\u201380.","journal-title":"Saudi J Med Med Sci."},{"key":"4438_CR18","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/628717","volume":"2013","author":"A Pizzo","year":"2013","unstructured":"Pizzo A, Lagan\u00e0 AS, Sturlese E, et\u00a0al. Mayer\u2013Rokitansky\u2013Kuster\u2013Hauser syndrome: embryology, genetics and clinical and surgical treatment. ISRN Obstet Gynecol. 2013;2013: 628717. https:\/\/doi.org\/10.1155\/2013\/628717.","journal-title":"ISRN Obstet Gynecol"},{"key":"4438_CR19","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1111\/tog.12729","volume":"23","author":"BP Jones","year":"2021","unstructured":"Jones BP, Ranaei-Zamani N, Vali S, et\u00a0al. Options for acquiring motherhood in absolute uterine factor infertility; adoption, surrogacy and uterine transplantation. Obstet Gynaecol. 2021;23:138\u201347. https:\/\/doi.org\/10.1111\/tog.12729.","journal-title":"Obstet Gynaecol"},{"key":"4438_CR20","doi-asserted-by":"publisher","first-page":"120","DOI":"10.3390\/ijms25010120","volume":"25","author":"R Dube","year":"2024","unstructured":"Dube R, Kar SS, Jhancy M, George BT. Molecular basis of m\u00fcllerian agenesis causing congenital uterine factor infertility\u2014a systematic review. Int J Mol Sci. 2024;25:120. https:\/\/doi.org\/10.3390\/ijms25010120.","journal-title":"Int J Mol Sci"}],"container-title":["Journal of Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-024-04438-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13256-024-04438-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-024-04438-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,25]],"date-time":"2024-03-25T02:33:09Z","timestamp":1711333989000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmedicalcasereports.biomedcentral.com\/articles\/10.1186\/s13256-024-04438-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,25]]},"references-count":20,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["4438"],"URL":"https:\/\/doi.org\/10.1186\/s13256-024-04438-x","relation":{},"ISSN":["1752-1947"],"issn-type":[{"value":"1752-1947","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,25]]},"assertion":[{"value":"7 January 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"5 February 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 March 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All procedures performed in this study were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki Declaration and its later amendments.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"126"}}