medigraphic.com
Federación Mexicana de Ginecología y Obstetricia, A.C.

2015, Number 03

<< Back

Ginecol Obstet Mex 2015; 83 (03)

Mayer-Rokitansky-Küster-Hauser Syndrome: Two cases report

Ávila-Vergara MA, León-Álvarez DA, López-Villegas MN, Quintero-Medrano SM, Angulo-Bueno GF, Vadillo-Ortega F

Language: Spanish
References: 13
Page: 199-205
PDF size: 348.71 Kb.


ABSTRACT

Agenesia of the Müllerian ducts is a low-frequency congenital disease but with devastating effects on women’s reproductive health. In this paper we present two cases of women affected by Mayer-Rokitansky- Küster-Hauser syndrome (MRKH). First case was a 17-year-old woman with aplasia of the upper vagina and absence of uterus. No other defects were found and was classified as type 1-MRKH. Second case was 18-year-old woman with absence of uterus, escoliosis and polycystic ovary syndrome, classified as type II-MRKH. Patients were seen at the Hospital with primary amenorrhea and fully developed secondary sexual characteristics. A clinical follow-up protocol, including the use of high-resolution image studies was used for diagnosis. Diagnostic procedures and current medical approaches to the treatment of MRKH are discussed, including psychological advisory, surgical procedures and new tissue-engineering techniques.


REFERENCES

  1. ACOG Müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol 2013;121:1134-1137.

  2. Morcel K, Camboreioux L. Mayer-Rokitansky- Küster-Hauser (MRKH) syndrome. Orphanet J Rare Disease 2007;2:13 doi:10.1186/1750-1172-2-13.

  3. 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;10:57.

  4. Fiaschetti V, Tagliery A, Gisonel 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. Radiology Case. 2012; 6:17-24.

  5. Strubbe EH,Willemsen WN, Lemmens JA, Thijn CJ, Rolland R. Mayer-Rokitansky-Kuster-Hauser syndrome: distinction between two forms based on excretory urographic, sonographic, and laparoscopic findings. Am J Roentgenol 1993;160:331-334.

  6. Bombard DS, Mousa SA. Mayer-Rokitansky-Kuster- Hauser syndrome: complications, diagnosis, and possible treatment options: a review. Gynecol Endocrinol 2014;30:618-623.

  7. Nodale C, Ceccarelli S, Giulano M, Cammarota M, D´Amici S, Vescarelli E, et al. Gene expression profile of patients with Mayer- Rokitansky-Küster-Hauser syndrome: new insights into the potential role of developmental pathways. PLoS One 2014;9:e91010.

  8. Mok-Lin EY, Wolfberg A, Hollinquist H, Laufer MR. Endometriosis in a patient with Mayer- Rokitansky-Küster-Hauser syndrome and complete uterine agenesis: Evidence to support the theory of coelomic metaplasia.

  9. Callens N,De Cuypere G, De Sutter P, Monstrey S, Weyers S, Hoebeke P, Cools M. An update on surgical and non-surgical treatments for vaginal hypoplasia. Hum Reprod Update 2014;20:775-801.

  10. Raya-Rivera AM, Esquilano D,Fierro-Pastrana R, López- Bayghen E, Valencia P, Ordorica-Flores R, et al. Tissueengineered autologous vaginal organs in patients: a pilot cohort study. Lancet 2014;384:329-336.

  11. Brännström M, Johannesson L, Bokström H, Kvarnström N, Mölne J, Dahm-Kahler P, et al. Livebirth after uterus transplantation. Lancet 2014 (en prensa). Online: http:// dx.doi.org/10.1016/S0140-6736(14)61728-1

  12. Mattingly RF, Thompson JD.Te Linde´s Operative Gynecology. Sixth edition. Philadelphia: Lippincott Company;1985.p 355-359.

  13. Vecchietti G. Creation of an artificial vagina in Rokitansky- Küster-Hauser syndrome. Attual Ostet Ginecol 1965;11:131-147.




2020     |     www.medigraphic.com