Mayer–Rokitansky–Küster–Hauser syndrome
https://doi.org/10.17749/2313-7347/ob.gyn.rep.2023.419
Abstract
The Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is an instructive story not only from a historical but also a gnoseological perspective. The mechanisms of embryogenesis as well as multiple aspects for correction of this syndrome remain enigmatic and unsolved. Here, we attempted to organize and detail striking issues related to MRKH syndrome.
About the Authors
R. T. AdamyanRussian Federation
Ruben T. Adamyan – MD, Dr Sci Med, Professor, Department of Plastic, Reconstructive and Maxillofacial Surgery; Professor, Department of Oncology, Radiotherapy and Plastic Surgery
1 Leninskie Gory, Moscow 119991
62 Str. Zemlyanoi Val, Moscow 109004
A. K. Blbulyan
Armenia
Armen K. Blbulyan – MD, Dr Sci Med, Deputy Director for Science
6/2 Markaryan Str., Yerevan 0078
R. A. Abrahamyan
Armenia
Razmik A. Abrahamyan – MD, Dr Sci Med, Professor, Director
6/2 Markaryan Str., Yerevan 0078
L. R. Abrahamyan
Armenia
Lusine R. Abrahamyan – MD, Dr Sci Med, Professor, Head of the Department of Obstetrics and Gynecology No. 2
2 Koryun Str., Yerevan 0025
G. R. Abrahamyan
Armenia
Gohar R. Abrahamyan – MD, Dr Sci Med, Associate Professor, Department of Obstetrics and Gynecology No. 2
2 Koryun Str., Yerevan 0025
G. K. Ghardyan
Armenia
Gegham K. Ghardyan – MD, Assistant, Department of Pathological Anatomy and Clinical Morphology; Clinical Resident, Department of Obstetrics and Gynecology No. 2
2 Koryun Str., Yerevan 0025
D. A. Blbulyan
Russian Federation
David A. Blbulyan – MD, Clinical Resident, Department of Obstetrics, Gynecology and Perinatal Medicine
62 Str. Zemlyanoi Val, Moscow 109004
References
1. Geodakyan V.A. Evolutionary chromosomes and evolutionary sexual dimorphism. [Evolyucionnye hromosomy i evolyucionnyj polovoj dimorfizm]. Izvestiya Akademii Nauk. Seriya Biologicheskaya. 2000;(2):133–48. (In Russ.).
2. Kobayashi A., Behringer R.R. Developmental genetics of the female reproductive tract in mammals. Nat Rev Genet. 2003;4(12):969–80. https://doi.org/10.1038/nrg1225.
3. Realdus Columbus M. De re Anatomica libri XV. Paris: Andream Wechelum, 1562.
4. Küster H. Uterus bipartitus solidus rudimentarius cum vagina solida. Z Geburtshilfe Gynäkol. 1910;67:692–718.
5. Marsh C.A., Will M.A., Smorgick N. et al. Uterine remnants and pelvic pain in females with Mayer-Rokitansky-Kuster-Hauser syndrome. J Pediatr Adolesc Gynecol. 2013;26(3):199–202. https://doi.org/10.1016/j.jpag.2012.11.014.
6. Banister J.B., McIndoe A.H. Congenital absence of the vagina, treated by means of an indwelling skin-graft. Proc R Soc Med. 1938;31(9):1055–6.
7. Baldwin J. XIV. The formation of an artificial vagina by intestinal transplantation. Ann Surg. 1904;40(3):398–403.
8. Davydov S.N. Colpopoiesis from the peritoneum of the utero-rectal space. [Kol'popoez iz bryushiny matochno-pryamokishechnogo prostranstva]. Akusherstvo i ginekologiya. 1969;45(12):55–7. (In Russ.).
9. Vecchietti G. Neovagina nella sindrome di Rokitansky–Kuster–Hauser. Attual Ostet Ginecol. 1965;11:131–47.
10. Frank R.T. The formation of an artificial vagina without operation. Am J Obstet Gynecol. 1938;35:1053–5.
11. Heller-Boersma J.G., Schmidt U.H., Edmonds D.K. Psychological distress in women with uterovaginal agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome, MRKH). Psychosomatics. 2009;50(3):277–81. https://doi. org/10.1176/appi.psy.50.3.277.
12. McCraw J.B., Massey F.M., Shanklin K.D., Horton C.E. Vaginal reconstruction with gracilis myocutaneous flaps. Plast Reconstr Surg. 1976;58(2):176–83. https://doi.org/10.1097/00006534-197608000-00006.
13. Tobin G.R., Day T.G. Vaginal and pelvic reconstruction with distally based rectus abdominis myocutaneous flaps. Plast Reconstr Surg. 1988;81(1):62–70. https://doi.org/10.1097/00006534-198801000-00012.
14. Johnson N., Lilford R.J., Batchelor A. The free-flap vaginoplasty; a new surgical procedure for the treatment of vaginal agenesis. Br J Obstet Gynaecol. 1991;98(2):184–8. https://doi.org/10.1111/j.1471-0528.1991.tb13366.x.
15. Wee J.T.K., Joseph V.T. A new technique of vaginal reconstruction using neurovascular pudendal thigh flap: a preliminary report. Plast Reconstr Surg. 1989;83(4):701–9. https://doi.org/10.1097/00006534-198904000-00018.
16. Wamalwa A.O., Khainga S.O. Island singapore flap vaginoplasty of two adult cases of Mayer–Rokitansky–Küster–Hauser syndrome type I. Ann Afr Surg. 2019;16(1):40–2. http://doi.org/10.4314/aas.v16i1.10.
17. Selçuk C.T., Evsen M.S., Özalp B., Durgun M. Reconstruction of vaginal agenesis with pudendal thigh flaps thinned with liposuction. J Plast Reconstr Aesthet Surg. 2013;66(9):e246–50. https://doi.org/10.1016/j.bjps.2013.04.006.
18. Joseph V.T. Pudendal-thigh flap vaginoplasty in the reconstruction of genital anomalies. J Pediatr Surg. 1997;32(1):62–5. https://doi.org/10.1016/s0022-3468(97)90095-6.
19. Ohmaru Y., Sakata K., Hashiguchi S.-I. et al. A new modified Pudendal Thigh Flap of vaginoplasty including reconstruction of vaginal vestibule. Case Reports Plast Surg Hand Surg. 2017;4(1):21–6. http://doi.org/10.108/23320885.2017.1296363.
20. Giraldo F., Solano A., Mora M.J., Smith V. Hair growth in the vagina after reconstruction with pudendal thigh flaps in congenital vaginal agenesis. Plast Reconstr Surg. 1998;102(3):924–5. https://doi. org/10.1097/00006534-199809030-00066.
21. Tham N.L.Y., Pan W.R., Rozen W.M. et al. The pudendal thigh flap for vaginal reconstruction: Optimising flap survival. J Plast Reconstr Aesthet Surg. 2010;63(5):826–31. https://doi.org/10.1016/j.bjps.2009.02.060.
Review
For citations:
Adamyan R.T., Blbulyan A.K., Abrahamyan R.A., Abrahamyan L.R., Abrahamyan G.R., Ghardyan G.K., Blbulyan D.A. Mayer–Rokitansky–Küster–Hauser syndrome. Obstetrics, Gynecology and Reproduction. 2023;17(5):654-662. (In Russ.) https://doi.org/10.17749/2313-7347/ob.gyn.rep.2023.419

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.