Mayer‑Rokitansky‑Kuster‑Hauser Syndrome in an 18‑year‑old Female: The Radiodiagnostic Perspective
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Abstract
Müllerian duct anomalies are a group of congenital uterine disorders that arise from an arrest in development, incomplete fusion or incomplete resorption of the mesonephric ducts. They are usually asymptomatic but diagnosed incidentally. An 18‑year‑old girl presented at the gynaecology clinic with a history of primary amenorrhea and failure to develop secondary sexual characteristics. She
is the only daughter of a widow. According to the mother, her pregnancy and birth histories were normal, and delivery was by spontaneous vaginal delivery. The patient had satisfactory developmental milestones and had completed secondary school at the time of presentation. She is of average physical stature with rudimentary breasts (tanner Stage 2), lacking axillary and pubic hairs. A pelvic
examination revealed a 1.5 cm blind‑ending vaginal pouch. A radiologic evaluation was done using ultrasonography and magnetic resonance imaging showed the absence of the uterus and the ovaries. The patient and the mother were counselled on management options, including the future fertility options. The mother vehemently rejected the option of surgery and has not been seen since after that.
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References
Capuñay C, Sueldo CE, Baronio JM. Congenital Uterine Anomalies. Available from: https://radiologykey.com/congenital‑uterine‑anomalies‑2/. [Last accessed on 2021 Apr 01].
Saber M, Farooq S. Mayer‑Rokitansky‑Küster‑Hauser syndrome.Radiopadea. Available from: https://radiopaedia.org/articles/mayer‑rokitansky‑kuster‑hauser‑syndrome. [Last accessed on 2021 Apr 20].
Azoury RS, Jones HW Jr. Cytogenetic findings in patients with congenital absence of the vagina. Am J Obstet Gynecol 1966;94:178‑80.
Bousfiha N, Errarhay S, Saadi H, Ouldim K, Bouchikhi C, Banani A. Gonadal dysgenesis 46, XX associated with Mayer‑Rokitansky‑Kuster‑Hauser syndrome: One case report. Obstet Gynecol Int 2010;2010:847370.
Yakasai IA, Daneji SM, Natasha A, Yunus EM. Late presentation of Mayer‑Rokitansky‑Kuster‑Hauser syndrome in the tropics‑Northern Nigeria. BJMMR 2015;5:1077‑82.
Aittomäki K, Eroila H, Kajanoja P. A population‑based study of the incidence of Müllerian aplasia in Finland. Fertil Steril 2001;76:624‑5.
Müllerian Agenesis. Available from: https://en.wikipedia.org/ wiki/Müllerian_agenesis. [Last accessed on 2021 Apr 26].
Fiaschetti V, Taglieri A, Gisone V, Coco I, Simonetti G. Mayer‑Rokitansky‑Kuster‑Hauser 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;6:17‑24.
Morcel K, Camborieux L, Programme de Recherches sur les Aplasies Müllériennes; Guerrier D. Mayer‑Rokitansky-Küster‑Hauser (MRKH) syndrome. Orphanet J Rare Dis 2007;2:13.
Okonofua F, Odunsi K, editor. Contemporary Obstetrics Gynaecology for Developing Countries. Cham, Switzerland: WHARC; 2003.
Decherney AH, Nathan L, Goodwin TM, Laufer N, editors. Current Obstetrics and Gynecology. 10th ed. USA: Mc Graw Hill; 2007. p. 549.
Karine M, Laure C. Review Mayer Rokitansky-Küster-Hauser (MRKH) syndrome. Orphanet J Rare Dis 2007;2:13.
Griffin JE, Edwards C, Madden JD, Harrod MJ, Wilson JD. Congenital absence of the vagina. The Mayer‑Rokitansky‑Kuster‑Hauser syndrome. Ann Intern Med 1976;85:224‑36.
Fligner J R. Congenital atresia of the vagina. Surg Gynecol Obs 1987;165:387-91.
Folch M, Pigem I, Konje JC. Müllerian agenesis: Etiology, diagnosis, and management. Obstet Gynecol Surv 2000;55:644‑9.
Uterine Anomaly. Available from: https://www.columbiadoctors. org/condition/uterine‑anomaly. [Last accessed on 2021 Apr 21].
Normal and Variant Pelvic Anatomy on MRI. Available from: https://radiologykey.com/normal-and-variant-pelvic-anatomy-on-mri/. [Last accessed on 2021 Apr 01].
Rathee M, Singhal SR, Malik S, Gupta G. Resilient customized hollow vaginal stent for the treatment of vaginal agenesis in Mayer‑Rokitansky‑Kuster‑Hauser syndrome. Niger J Surg 2020;26:88‑91.