Mullerian malformations are not uncommon. Overall incidence ranges widely between 1 in 200 women and 4 in 100 women
(Chan et al. in Hum Reprod Update 17:761–771, 2011; Grimbizis et al. in Hum Reprod 28:2032–2044, 2013). Other way,
these observations suggest presently an increased number of Mullerian abnormalities are diagnosed with more details, following the use of newer diagnostic modalities. Most classifications that are available have limitations. Diagnosis was based
on imaging studies that had low diagnostic accuracy. It was focused mainly on the anomalies of the uterus. Less is known
about the anomalies of the cervix or the vagina in isolation or in combination with the utero-cervical and vaginal malformations. Improved diagnostic modalities and incorporation of assisted reproductive technology have improved the outcome
further. Therefore, a more expanded classification needs to be introduced with wider criteria for the clinicians. This will
make clinicians’ approach for the management more simple.
Keywords : Mullerian malformations , Clinical presentations , Diagnosis , Reconstructive surgery , Menstrual , Sexual and reproductive functions
Development of the Mullerian system is a complex process
that occurs over a period between 5 and 15 weeks of embryonic life. Development and progressive diferentiation of the
two Mullerian ducts (mesodermal) are under the infuence of
many factors (molecular, genetic, chromosomal, hormonal and environmental). Paramesonephric ducts developments
are in continuum with the development of urogenital sinus
(urinary bladder), urorectal septum, ureteric buds, cloacal
membrane and the endodermal cloaca. Maldevelopment of
the Mullerian system thus leads to the non-function or dysfunction of other organs too. Successful development of the
Mullerian system is essential for normal menstrual function, sexual function and finally the reproductive function.
A wide range of malformations have been observed affecting the genital organs either in isolation or in combination
with other organs. Interestingly in the present days, major
changes have been observed in all the areas of management
of Mullerian abnormalities. Microarray technology can provide insight into the genomic expression of developmental control. Newer diagnostic modalities provide details of
structural information for any type of Mullerian malformation. More and more reconstructive and organ-sparing surgery is now possible to restore the menstrual as well as the
sexual function. Endoscopy is used as an alternative method
of open surgery with all its advantages. Presently, there is
remarkable development in the field of reproductive endocrinology and assisted reproductive technology. Combined
together, a signifcant change is observed in the management
outcome of patients with Mullerian malformations. There is a need to update our understanding in the management of
this problem based on current evidences [1, 2].
Conflict of interest The author declares that he has no conflict of interest.
Ethical Standards All procedures followed were in accordance with the ethical standards of the responsible committee on the human participants (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008.
Informed Consent Informed consent was obtained from the eligible
women. Additional informed consent was obtained from all individual
participants for whom identifying information is included in this study.
1. Chan YY, Jayaprakasan K, Zamora J, et al. The prevalence of congenital uterine anomalies in unselected and high-risk populations: a systematic review. Hum Reprod Update. 2011;17:761–71.
2. Grimbizis GF, Gordts G, Di Spiezio SA, et al. The ESHRE/ESGE consensus on the classifcation of female genital tract congenital malformations. Hum Reprod. 2013;28:2032–44.
3. Acien P. Embryological observations on the female genital tract. Hum Reprod. 1992;7:437–45.
4. Simpson JL. Genetics of the female reproductive ducts. Am J Med Genet. 1999;89:224–39. \
5. Sotirios HS, Karen AC, Li T-C. The pattern of pregnancy loss in women with congenital uterine anomalies and recurrent miscarriage. Reprod Biomed Online. 2010;20:416–22.
6. Kaufman RH, Binder GL, Gray PM Jr, et al. Upper genital tract changes associated with exposure in utero to diethylstilbestrol. Am J Obstet Gynecol. 1977;128:51–9.
7. American Fertility Society. The AFS classifcation of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Mullerian anomalies and intrauterine adhesions. Fertil Steril. 1988;49:944–55.
8. Grimbizis GF, Gordts G, Di Spiezio SA, et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital malformations. Gynecol Surg. 2013;10:199–212.
9. Grimbizis GF, Di Spiezio Sardo A, Saravelos SH, et al. The Thessaloniki ESHRE/ESGE consensus on diagnosis of female genital anomalies. Hum Reprod. 2016;31(1):2–7.
10. Rackow BW, Arici A. Reproductive performance of women with Mullerian anomalies. Curr Opin Obstet Gynecol. 2007;19:229–37.
11. Jafe SB, Loucopoulos A, Jewelewich R. Cytogenetics of mullerian agenesis. A case report. J Reprod Med. 1992;37:242–6.
12. Goodman FR, Bacchelli C, Brady AF, et al. Novel HOXA 13 mutation and the phenotypic spectrum of hand-foot-genital syndrome. Am Jr Hum Genet. 2000;67:197–202.
13. Brännström M, Johannesson L, Bokström H, et al. Live birth after uterus transplantation. Lancet. 2015;385(9968):607–16.
14. Communal PH, Chevret-Measson GF, Raudrant D. Sexuality after sigmoid colpopoiesis in patients with Mayer–Rokitansky–Kuster– Hauser syndrome. Fertil Steril. 2003;80:600–6.
15. Chakravorty BN, Konar H, Roychoudhury NN. Pregnancies after reconstructive surgery for congenital cervicovaginal atresia. Am J Obstet Gynecol. 2000;183:421–3.
16. Konar H, De Banerjee M, Mukherjee S. Diagnostic Dielemma. Jr. ISOPARB, 2007.
17. Valle RF, Ekpo GE. Hysteroscopic metroplasty for the septate uterus: review and meta-analysis. J Minim Invasive Gynecol. 2013;20:22–42.
18. Glacomucci E, Bellavia E, Sandri F, et al. Term delivery rate after
hysteroscopic metroplasty in patients with recurrent spontaneous
abortion and T-shaped, arcuate and septate uterus. Gynecol Obstet
Invest. 2011;71:183–8.