The female internal sex organs develop from the paramesonephric (Mullerian) duct. In male embryos, the regression of the Mullerian duct is caused by the anti-Mullerian hormone (AMH), which plays an important role in the process of testicular descent. The physiological remnant of the Mullerian duct in males is the appendix testis (AT). In our previous study, we presented evidence for the decreas...

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome comprises the combined hypoplasia of the vagina and the uterus. In recent years, variable anomalies of the development of the Mullerian duct [as classified by the American Fertility Society (AFS)] have been operated on by the laparoscopic approach. We describe here a laparoscopic extirpation of an aplastic ectopic uterus in a patient found to h...

A 10-month-old male infant presented with bilateral inguinal hernia and left un-descended testis. During right herniotomy, both gonads were found on same side with mullerian duct structures. On naked eye examination, both gonads were normal looking. Excision of mullerian duct remnant and fixation of ectopic testis was made. Histopathological examination revealed that gonads were testicles. Pres...

Herlyn-Werner-Wunderlich syndrome also known as uterus didelphys with obstructing hemivaginal septum and ipsilateral renal agenesis is a rare syndrome with only a few hundred reported cases described since 1922. Uterus Didelphys is a type of lateral fusion disorder of mullerian ducts. Mullerian duct anomalies have an incidence of 2–3%, while obstructing hemivaginal septum and ipsilateral renal ...

Persistent Mullerian duct syndrome is a rare form of internal male pseudohermaphroditism, in which Mullerian duct derivatives (uterus and fallopian tubes) are present in a genotypic (46XY) and phenotypic male. Over 150 cases have been reported, mainly from outside the African setting. This article presents an unexpected case encountered in an African setting. Handicaps in the management were un...

In the case reported, diagnosed with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, the presence of normal ovaries proved to be challenging to confirm due to unusual high positioned (ectopic) ovaries. MRKH syndrome is a rare pathological condition characterized by a spectrum of the Mullerian duct abnormalities resulting in congenital aplasia of the uterus and of the upper part (2/3) of the vag...

Mayer Rokitansky Kuster Houser syndrome (MRKH syndrome) is characterized by Mullerian duct structures agenesis, vaginal atresia being the commonest variant. It can be associated with renal, skeletal, spine and other malformations. Patient with Mayer Rokitansky syndrome has a varied presentation from newborn period to adolescence. Thorough investigations are required for classification of the sy...

Abnormalities of embryogenesis of mullerian duct system resulting in congenital anomalies of female genital tract are relatively common [1]. The prevalence of congenital uterine anomalies in the general population is 6.7 % [2]. In women with a history of repeated pregnancy loss, the rate of mullerian anomalies increases to 3–25 % [2, 3]. Unicornuate uterus accounts for 5 % of all mullerian anom...