pmds is a rare form of male pseudo - hermaphroditism characterized by the presence of mullerian duct structures in an otherwise phenotypically , as well as genotypically , normal man .
it is characterized by the persistence of the uterus , fallopian tubes and upper vagina in otherwise normally virilized boys . despite the normal male genotype ( 46 xy ) and the subsequent normal development of fetal testes , mllerian structures do not regress either due to absence of mllerian inhibiting substance ( mis ) or lack of response to it .
the persistence of a large uterus - like paramesonephric duct in a man is in itself clinically unusual , but when it forms a part of the contents of a hernial sac , it must be considered a rarity .
we report the case of a 70 year old man with unilateral cryptorchidism on the right side and left obstructed inguinal hernia containing uterus and fallopian tube ( that is , hernia uteri inguinalis ; type i male form of pmds ) coincidentally detected during an operation for an obstructed left inguinal hernia with right cryptorchidism .
a 70 year old man presented to our hospital with a painful left - sided inguinal swelling of one day duration . the patient gave history of asymptomatic left inguinal swelling from past 20 years and absence of the right testis since birth .
he had been married for fifty years and was having 4 children , the youngest one being 38 year old female .
general physical examination revealed a man of sub - average built with well developed secondary sexual characters . his urethra and penis
were fully developed with a poorly developed right hemi - scrotum and no palpable right testis in the scrotum or inguinal canal .
there was a non - reducible , tender swelling measuring approximately 10    8  cm in the left inguinal region with absent cough impulse .
exploration of the inguinal canal revealed an indirect inguinal hernia containing a globular structure resembling uterus , fallopian tubes with an atrophic right testis embedded in the broad ligament and attached to pelvis with a thick fibrous band [ figs . 1 and 2 ] .
total excision of the uterus with fallopian tubes and atrophic right testis was performed and the operation was completed with left inguinal hernioplasty [ figs . 3 and 4 ] .
grossly , the specimens removed were identified as a uterus with patent endometrial and endocervical linings and two fallopian tubes .
the right testis measuring 2    1    1  cm , was atrophic and embedded in the right broad ligament .
the specimen was sent for histopathological examination which revealed uterine muscular tissue with its cavity lined by endometrial tissue and congested fallopian tubes .
male pseudo - hermaphroditism is a condition in which the gonads are testes but the internal genitalia are not completely virilized .
pmds is a rare form of internal male pseudo - hermaphroditism in which mullerian duct derivatives are seen in men .
the exact cause of pmds is not known , however it is thought to result from a defect of the synthesis or release of mif , or from defects in the mif receptor .
defects in the mif gene lead to the persistence of a uterus and fallopian tube in males .
it is likely that remnant mullerian structures lead to cryptorchidism by hindering the normal testicular descent mechanism .
patients with pmds usually have normal development of external genitalia and secondary sexual characteristics . the typical patient with pmds
has unilateral or bilateral cryptorchidism and is assigned to the male sex at birth without hesitation , as they have normal male genotypes and phenotypes .
the male form is encountered in 8090% of cases , characterized by unilateral cryptorchidism with contralateral inguinal hernia , and can be one of the two types : the first type is hernia uteri inguinalis , which is characterized by one descended testis and herniation of the ipsilateral corner of uterus and fallopian tube into the inguinal canal .
the second type is crossed testicular ectopia , which is characterized by herniation of both testes and the entire uterus with both fallopian tubes .
clinically , the persistence of a uterus and fallopian tubes leads to either cryptorchidism or inguinal hernia depending on whether or not mullerian derivatives can be mobilized during testicular descent .
if the uterus and fallopian tube are mobile , they may descend into the inguinal canal during testicular descent .
pmds is usually coincidently detected during surgical operation , as in our patient 's case .
however pre - operative ultrasonography , computerized tomography and mri allow possible pre - operative diagnosis .
an element of deciding on intervention is largely based on reducing risk of malignancy , while maintaining maximum reproductive function . in pmds patients with undescended testes ,
the rate of testicular cancer is about 12% which is comparable to the rate seen in undescended testes not associated with pmds .
several authors have asserted that the mullerian structures should not be removed as there is minimal risk associated with their retention , and excision of the mullerian structures risks damaging primary blood supply to the pmds testis via the internal spermatic and deferential arteries .
however most recent reports have demonstrated rare malignancies including adenocarcinomas of the mullerian duct associated with retained mullerian structures .
pmds is a rare form of male pseudo - hermaphroditism characterized by the presence of mullerian duct structures in an otherwise phenotypically , as well as genotypically , normal man .
since patients are phenotypically male , the diagnosis is usually not suspected until surgery is performed for cryptorchidism or hernia repair .
hernia uteri inguinalis is type i of the male form of pmds , characterized by one descended testis and the herniation of the ipsilateral corner of the uterus and fallopian tube into the inguinal canal . in order to prevent further complications such as infertility and malignant change
, the surgeon should be aware of pmds while dealing with patients who present with unilateral or bilateral cryptorchidism . in summary , in cases of unilateral or bilateral cryptorchidism associated with hernia , as in our patient 's case ,
a written informed consent was obtained from the patient for publication of the case report and various images which may be used with the same .
the patient being a major gave a written consent of the same after assuring him that name and hospital mrd no of the patient will not appear in the case report .
all the authors have contributed either as being part of surgical and anaesthesia team or helping in the photography , paper writing , submission etc . afak yusuf sherwani , abdul qayoom shah , abdul majeed wani , farooq ahmad sofi and ashfaq amin wani were in the surgical team .
bashir a chalkoo , bashir a khan and ab hamid sherwani in the anaesthesia team .
wasim lone , mehmood a sheikh and raj reshi sharma did photography and paper writing .
 introductionpersistent mullerian duct syndrome is a rare form of male pseudo - hermaphroditism characterized by the presence of mullerian duct structures in an otherwise phenotypically , as well as genotypically , normal man ; only a few cases have been reported in the worldwide literature . 
 a great variety of organs have been found in indirect inguinal hernial sacs.presentation of casewe report a case of 70 year old man , father of 4 children with unilateral cryptorchidism on the right side and left - sided obstructed inguinal hernia containing uterus and fallopian tube ( that is , hernia uteri inguinalis ; type i male form of persistent mullerian duct syndrome ) coincidentally detected during an operation for an obstructed left inguinal hernia.discussionpmds is usually coincidently detected during surgical operation , as was in our case . 
 however pre - operative ultrasonography , computerized tomography and mri allow possible pre - operative diagnosis.3conclusionin cases of unilateral or bilateral cryptorchidism associated with hernia , as in our patient 's case , the possibility of pmds should be kept in mind . 
Introduction
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