A Case Report of Coital Trauma to the Genital Tract in a Patient with Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: Diagnostic Imaging and Surgical Management
-
Ghazaleh Salehabadi
,
Nedasadat Rezaei
,
Somayeh Noei Teymoordash
,
Ramin Ebrahimi
,
Peyman Famili
,
Manizhe Ataee Kachuee
Abstract
Mülle rian duct anomalies (MDAs) are congenital malforma tions resulting from abnormal development of the Müllerian ducts. Among these anomalies, MayerRokit ansky -Küster-Hauser (MRKH) syndrome, characterized by uterine and vaginal agenesis or aplasia, is a severe form often presenting with primary amenorrhea. This
case involves a 25-year-old woman with vaginal bleeding , diagnosed by MRI as having MRKH syndrome with cervicovaginal atresia and unde scended ovaries. The patient unde rwent hysteroscopy and improved post-surgery. MRKH syndrome requires a multidisciplinary appr oach f or manag ement, including surgical in tervention, psychologic al support, and fertility counseling , highligh ting the import ance of early diagnosis and personalized care.
[1] Strassmann EO. Fertility and unification of double uterus. Fertil Steril. 1966 Mar-Apr;17(2):165-76.
https://doi.org/10.1016/S0015-0282(16)35882-4
[2] Propst AM, Hill JA 3rd. Anatomic factors associated with recurrent pregnancy loss. Semin Reprod Med.
2000;18(4):341-50. https://doi.org/10.1055/s-2000-13723
[3] Santana González L, Artibani M, Ahmed AA. Studying Müllerian duct anomalies - from cataloguing
phenotypes to discovering causation. Dis Model Mech. 2021 Jun 1;14(6):dmm047977. https://doi.org/10.1242/dmm.047977
[4] KO’Flynn O’Brien KL, Bhatia V, Homafar M, Gong YY, Winsten MT, Gerber J, et al. The Prevalence of Müllerian
Anomalies in Women with a Diagnosed Renal Anomaly. J Pediatr Adolesc Gynecol. 2021 Apr;34(2):154-160.
https://doi.org/10.1016/j.jpag.2020.11.015
[5] Cunha GR, Robboy SJ, Kurita T, Isaacson D, Shen J, Cao M, et al. Development of the human female reproductive
tract. Differentiation. 2018 Sep-Oct;103:46-65. https://doi.org/10.1016/j.diff.2018.09.001
[6] Rey R, Josso N, Racine C. Sexual Differentiation. In: Feingold KR, et al., editors. Endotext. South Dartmouth
(MA): MDText.com, Inc.; 2000.
[7] Sultan C, Paris F, Jeandel C, Lumbroso S, Galifer RB. Ambiguous genitalia in the newborn. Semin
Reprod Med. 2002 Aug;20(3):181-8. https://doi.org/10.1055/s-2002-35382
[8] Chandler TM, Machan LS, Cooperberg PL, Harris AC, Chang SD. Mullerian duct anomalies: from diagnosis to
intervention. Br J Radiol. 2009 Dec;82(984):1034-42. https://doi.org/10.1259/bjr/99354802
[9] Sugi MD, Penna R, Jha P, Pōder L, Behr SC, Courtier J, et al. Müllerian Duct Anomalies: Role in Fertility and
Pregnancy. Radiographics. 2021 Oct;41(6):1857-75. https://doi.org/10.1148/rg.2021210022
[10] Oppelt PG, Lermann J, Strick R, Dittrich R, Strissel P, Rettig I, et al. Malformations in a cohort of 284 women
with Mayer-Rokitansky-Küster-Hauser syndrome(MRKH). Reprod Biol Endocrinol. 2012 Aug 20;10:57.
https://doi.org/10.1186/1477-7827-10-57
[11] Timmreck LS, Reindollar RH. Contemporary issues in primary amenorrhea. Obstet Gynecol Clin North Am.
2003 Jun;30(2):287-302. https://doi.org/10.1016/S0889-8545(03)00027-5
[12] Bermejo C, Martínez Ten P, Cantarero R, Diaz D, Pérez Pedregosa J, Barrón E, et al. Three-dimensional
ultrasound in the diagnosis of Müllerian duct anomalies and concordance with magnetic resonance imaging.
Ultrasound Obstet Gynecol. 2010 May;35(5):593-601. https://doi.org/10.1002/uog.7551
[13] Hassan MA, Lavery SA, Trew GH. Congenital uterine anomalies and their impact on fertility. Womens
Health (Lond). 2010 May;6(3):443-61. https://doi.org/10.2217/WHE.10.19
[14] Ribeiro SC, Tormena RA, Peterson TV, Gonzáles Mde O, Serrano PG, Almeida JA, et al. Müllerian duct anomalies:
review of current management. Sao Paulo Med J. 2009 May;127(2):92-6. https://doi.org/10.1590/S1516-
31802009000200007
[15] Knebelmann B, Boussin L, Guerrier D, Legeai L, Kahn A, Josso N, Picard JY. Anti-Müllerian hormone Bruxelles:
a nonsense mutation associated with the persistent Müllerian duct syndrome. Proc Natl Acad Sci U S A.
1991 May 1;88(9):3767-71. https://doi.org/10.1073/pnas.88.9.3767
[16] Robbins JB, Broadwell C, Chow LC, Parry JP, Sadowski EA. Müllerian duct anomalies: embryological
development, classification, and MRI assessment. J Magn Reson Imaging. 2015 Jan;41(1):1-12. https://doi.
org/10.1002/jmri.24771
[17] Committee on Adolescent Health Care. ACOG Committee Opinion No. 728: Müllerian Agenesis:
Diagnosis, Management, And Treatment. Obstet Gynecol. 2018 Jan;131(1):e35-e42.
https://doi.org/10.1097/AOG.0000000000002458
[18] Deldar Pesikhani M, Ghanbari Z, Borna S, Pourali L, Chegini H, Lotfi Z. Uterine Conserving Surgery in Patient
With Cervicovaginal Agenesis: A Case Report. Acta Med Iran. 2020;58(6):291-3. https://doi.org/10.18502/acta.
v58i6.4057
[19] Jaha G, AlMutairi B. Mullerian ducts anomaly of 2 divergent uterine horns with cervicovaginal hypoplasia:
Is it considered unclassified or under the spectrum of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome?
Radiol Case Rep. 2023 Oct 4;18(12):4393-9. https://doi.org/10.1016/j.radcr.2023.09.016
[20] Coleman AD, Arbuckle JL. Advanced Imaging for the Diagnosis and Treatment of Coexistent Renal and
Müllerian Abnormalities. Curr Urol Rep. 2018 Sep 6;19(11):89. https://doi.org/10.1007/s11934-018-0840-x
[21] Behr SC, Courtier JL, Qayyum A. Imaging of müllerian duct anomalies. Radiographics. 2012 Oct;32(6):E233-50.
https://doi.org/10.1148/rg.326125515
[22] Herlin MK, Petersen MB, Brännström M. MayerRokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update. Orphanet J Rare Dis. 2020 Aug 20;15(1):214. https://doi.org/10.1186/s13023-020-
01491-9
[23] Reichman DE, Laufer MR. Mayer-Rokitansky-KüsterHauser syndrome: fertility counseling and treatment.
Fertil Steril. 2010 Oct;94(5):1941-3. https://doi.org/10.1016/j.fertnstert.2010.01.036
[24] Chmel R, Cekal M, Pastor Z, Chmel R Jr, Paulasova P, Havlovicova M, et al. Assisted Reproductive Techniques
and Pregnancy Results in Women with MayerRokitansky-Küster-Hauser Syndrome Undergoing Uterus Transplantation: the Czech Experience. J Pediatr Adolesc Gynecol. 2020 Aug;33(4):410-4. https://doi.org/10.1016/j.jpag.2020.03.006
[25] Kang S, Peng W, Zhu Y, Lu S, Zhou M, Lin W, et al. Recent progress in understanding 2019 novel coronavirus
(SARS-CoV-2) associated with human respiratory disease: detection, mechanisms and treatment. Int J
Antimicrob Agents. 2020 May;55(5):105950. https://doi.org/10.1016/j.ijantimicag.2020.105950
https://doi.org/10.1016/S0015-0282(16)35882-4
[2] Propst AM, Hill JA 3rd. Anatomic factors associated with recurrent pregnancy loss. Semin Reprod Med.
2000;18(4):341-50. https://doi.org/10.1055/s-2000-13723
[3] Santana González L, Artibani M, Ahmed AA. Studying Müllerian duct anomalies - from cataloguing
phenotypes to discovering causation. Dis Model Mech. 2021 Jun 1;14(6):dmm047977. https://doi.org/10.1242/dmm.047977
[4] KO’Flynn O’Brien KL, Bhatia V, Homafar M, Gong YY, Winsten MT, Gerber J, et al. The Prevalence of Müllerian
Anomalies in Women with a Diagnosed Renal Anomaly. J Pediatr Adolesc Gynecol. 2021 Apr;34(2):154-160.
https://doi.org/10.1016/j.jpag.2020.11.015
[5] Cunha GR, Robboy SJ, Kurita T, Isaacson D, Shen J, Cao M, et al. Development of the human female reproductive
tract. Differentiation. 2018 Sep-Oct;103:46-65. https://doi.org/10.1016/j.diff.2018.09.001
[6] Rey R, Josso N, Racine C. Sexual Differentiation. In: Feingold KR, et al., editors. Endotext. South Dartmouth
(MA): MDText.com, Inc.; 2000.
[7] Sultan C, Paris F, Jeandel C, Lumbroso S, Galifer RB. Ambiguous genitalia in the newborn. Semin
Reprod Med. 2002 Aug;20(3):181-8. https://doi.org/10.1055/s-2002-35382
[8] Chandler TM, Machan LS, Cooperberg PL, Harris AC, Chang SD. Mullerian duct anomalies: from diagnosis to
intervention. Br J Radiol. 2009 Dec;82(984):1034-42. https://doi.org/10.1259/bjr/99354802
[9] Sugi MD, Penna R, Jha P, Pōder L, Behr SC, Courtier J, et al. Müllerian Duct Anomalies: Role in Fertility and
Pregnancy. Radiographics. 2021 Oct;41(6):1857-75. https://doi.org/10.1148/rg.2021210022
[10] Oppelt PG, Lermann J, Strick R, Dittrich R, Strissel P, Rettig I, et al. Malformations in a cohort of 284 women
with Mayer-Rokitansky-Küster-Hauser syndrome(MRKH). Reprod Biol Endocrinol. 2012 Aug 20;10:57.
https://doi.org/10.1186/1477-7827-10-57
[11] Timmreck LS, Reindollar RH. Contemporary issues in primary amenorrhea. Obstet Gynecol Clin North Am.
2003 Jun;30(2):287-302. https://doi.org/10.1016/S0889-8545(03)00027-5
[12] Bermejo C, Martínez Ten P, Cantarero R, Diaz D, Pérez Pedregosa J, Barrón E, et al. Three-dimensional
ultrasound in the diagnosis of Müllerian duct anomalies and concordance with magnetic resonance imaging.
Ultrasound Obstet Gynecol. 2010 May;35(5):593-601. https://doi.org/10.1002/uog.7551
[13] Hassan MA, Lavery SA, Trew GH. Congenital uterine anomalies and their impact on fertility. Womens
Health (Lond). 2010 May;6(3):443-61. https://doi.org/10.2217/WHE.10.19
[14] Ribeiro SC, Tormena RA, Peterson TV, Gonzáles Mde O, Serrano PG, Almeida JA, et al. Müllerian duct anomalies:
review of current management. Sao Paulo Med J. 2009 May;127(2):92-6. https://doi.org/10.1590/S1516-
31802009000200007
[15] Knebelmann B, Boussin L, Guerrier D, Legeai L, Kahn A, Josso N, Picard JY. Anti-Müllerian hormone Bruxelles:
a nonsense mutation associated with the persistent Müllerian duct syndrome. Proc Natl Acad Sci U S A.
1991 May 1;88(9):3767-71. https://doi.org/10.1073/pnas.88.9.3767
[16] Robbins JB, Broadwell C, Chow LC, Parry JP, Sadowski EA. Müllerian duct anomalies: embryological
development, classification, and MRI assessment. J Magn Reson Imaging. 2015 Jan;41(1):1-12. https://doi.
org/10.1002/jmri.24771
[17] Committee on Adolescent Health Care. ACOG Committee Opinion No. 728: Müllerian Agenesis:
Diagnosis, Management, And Treatment. Obstet Gynecol. 2018 Jan;131(1):e35-e42.
https://doi.org/10.1097/AOG.0000000000002458
[18] Deldar Pesikhani M, Ghanbari Z, Borna S, Pourali L, Chegini H, Lotfi Z. Uterine Conserving Surgery in Patient
With Cervicovaginal Agenesis: A Case Report. Acta Med Iran. 2020;58(6):291-3. https://doi.org/10.18502/acta.
v58i6.4057
[19] Jaha G, AlMutairi B. Mullerian ducts anomaly of 2 divergent uterine horns with cervicovaginal hypoplasia:
Is it considered unclassified or under the spectrum of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome?
Radiol Case Rep. 2023 Oct 4;18(12):4393-9. https://doi.org/10.1016/j.radcr.2023.09.016
[20] Coleman AD, Arbuckle JL. Advanced Imaging for the Diagnosis and Treatment of Coexistent Renal and
Müllerian Abnormalities. Curr Urol Rep. 2018 Sep 6;19(11):89. https://doi.org/10.1007/s11934-018-0840-x
[21] Behr SC, Courtier JL, Qayyum A. Imaging of müllerian duct anomalies. Radiographics. 2012 Oct;32(6):E233-50.
https://doi.org/10.1148/rg.326125515
[22] Herlin MK, Petersen MB, Brännström M. MayerRokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update. Orphanet J Rare Dis. 2020 Aug 20;15(1):214. https://doi.org/10.1186/s13023-020-
01491-9
[23] Reichman DE, Laufer MR. Mayer-Rokitansky-KüsterHauser syndrome: fertility counseling and treatment.
Fertil Steril. 2010 Oct;94(5):1941-3. https://doi.org/10.1016/j.fertnstert.2010.01.036
[24] Chmel R, Cekal M, Pastor Z, Chmel R Jr, Paulasova P, Havlovicova M, et al. Assisted Reproductive Techniques
and Pregnancy Results in Women with MayerRokitansky-Küster-Hauser Syndrome Undergoing Uterus Transplantation: the Czech Experience. J Pediatr Adolesc Gynecol. 2020 Aug;33(4):410-4. https://doi.org/10.1016/j.jpag.2020.03.006
[25] Kang S, Peng W, Zhu Y, Lu S, Zhou M, Lin W, et al. Recent progress in understanding 2019 novel coronavirus
(SARS-CoV-2) associated with human respiratory disease: detection, mechanisms and treatment. Int J
Antimicrob Agents. 2020 May;55(5):105950. https://doi.org/10.1016/j.ijantimicag.2020.105950
| Files | ||
| Issue | Vol 10 No 6 (2025): November- December | |
| Section | Case Report(s) | |
| Keywords | ||
| Müllerian duct anomaly Mayer-rokitansky-küsterhauser syndrome (MRKH syndrome) Vaginal atresia | ||
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How to Cite
1.
Salehabadi G, Rezaei N, Noei Teymoordash S, Ebrahimi R, Famili P, Ataee Kachuee M. A Case Report of Coital Trauma to the Genital Tract in a Patient with Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: Diagnostic Imaging and Surgical Management. CRCP. 2026;10(6):241-246.
