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the paramesonephric duct or mullerian duct first appears in the human embryo at the 10 mm stage and begins to regress in the male due to mullerian inhibiting substance ( mis ) ( 1 ) .
apart from various disorders of regression , there is also a well documented syndrome known as the pmds , caused by the failure of regression of the mullerian duct structures .
this is a rare form of male pseudohermaphroditism , characterized by the presence of a uterus and fallopian tubes , in phenotypically and genotypically normal males .
von cenhossek in 1886 described the first case of tte and in 1895 jordan described the syndrome of tte with pmds ( 2 ) .
a 38 year old married man with two children presented with a left inguinal hernia ( lih ) and absence of right testis since birth .
examination of the groins showed normal phallus , lih , empty right hemiscrotum , impalpable right testis and normal left testis .
patient was advised lih repair and imaging for undescended right testis . since patient was not willing for the imaging evaluation of the right testis and insisted more on the hernia repair to be done first , he was booked for a lichtenstein s repair .
resected mullerian duct derivatives of pmds patient at exploration through the left inguinal incision , an irregular mass was identified within the indirect hernia sac .
the sac contained two spermatic cords , separated by a rudimentary uterus , with one cornua leading to an ovary like structure ( fig.1 ) .
the entire mass was removed carefully without damaging the left spermatic cord and sent for hpe .
we were surprised about these findings and initially had no clues , until we searched the literature and found about pmds .
the hpe report was confirmative , showing the presence of normal rudimentary uterus , testis ( not ovary , as we thought ) showing germ cell atrophy ( sertoli cell only ) , normal epididymis and spermatic cord ( vas deferens ) .
during normal embryogenesis , differentiation of an indifferent gonad into a testis or ovary depends on the sex chromosomes .
mis and testosterone are the two principal hormones produced by the testis , the former causes the regression of mullerian ducts , and the latter induces the development of internal male genital structures .
contrastingly , female development is an autonomous process , and does not require the presence of either gonad or hormone .
the male pseudohermaphrodite has male gonads and karyotype , but varying degrees of virilisation of the internal and external genital tracts .
pmds represents a small fraction of this broad spectrum of male pseudohermaphroditism , characterized by the presence of well developed or rudimentary uterus , cervix , vagina and fallopian tube in normal 46xy male .
ectopic testes have been reported at various sites including superficial inguinal pouch , suprapubic , femoral , perineal region and the base of penis ( 3 ) .
migration of the testis to the opposite side where both testes pass through the same inguinal canal is known as tte , 100 cases of which have been reported ( 4 ) . in most cases the pmds is discovered during surgery for inguinal hernia .
routine pelvic ultrasound was recommended by adamsbaum et al , in bilaterally cryptorchid patients and in patients with inguinal hernia of unusually hard consistency ( 5 ) .
recently , with the advent of laparoscopic exploration of udt , the diagnosis of tte was possible . in a case of pmds with tte ,
the optimal surgical approach includes tissue biopsies , hernoitomy or hernioplasty , orchidopexy and excision of mullerian duct remnants .
some authors believe that excision of mullerian duct structures and scrotal orchidopexy are not possible without sacrificing the vas deferens . in our patient
it was possible to dissect the vas deferens from the rudimentary uterus and remove the mullerian duct structures without risking the vas deferens .
this approach is reported by many authors as it preserves the fertility of the individual ( 2,5,6 ) .
there are three anatomic variants : in the most common male type , one testis is usually foundwithin the scrotum ; the uterus and ipsilateral fallopian tube are either in the inguinal canal or can be brought into it by gentle traction in the presenting testis . in some cases , the contralateral testis and the tube are also in the hernia sac ; transverse testicular ectopia can also occur .
the least common form , or female type , is characterized by bilateral cryptorchidism with testis embedded in the broad ligaments in an ovarian position with respect to the uterus , which is fixed in the pelvis ( 7 ) . in conclusion ,
removal of uterus and its derivatives can be performed provided , care is taken not to damage the vas deferens , if there is some spermatogenesis .
hence , surgeons dealing with inguinal hernia should suspect pmds in the case of adult bilateral cryptorchidism .
persistent mullerian duct syndrome ( pmds ) with transverse testicular ectopia ( tte ) is a rare syndrome . 
 we report a case of pmds associated with tte in a 38 year old male discovered during surgery for a left inguinal hernia . 
 hernioplasty with excision of the persistent mullerian duct structures was done and the excised structures sent for histopathologic examination ( hpe ) which revealed pmds .