Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia

Abstract

AA Al-Bassam, Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia. 1997; 17(2): 226-227 Persistent müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism, characterized by the presence of a uterus and fallopian tubes due to failure of müllerian duct regression in genotypically normal males.1,2 More than 150 cases have been recorded, most of them in adults. The association between the persistent müllerian duct and transverse testicular ectopia is even more uncommon. In 1886, von Cenhossek described the first case of transverse testicular ectopia and in 1895, Jordan described the syndrome of transverse testicular ectopia with persistent müllerian ducts. 3 Case ReportA 10-month-old boy presented with a left inguinal hernia and absence of the right testis since birth. Examination of the groins showed a normal phallus, left inguinal hernia, empty right hemiscrotum, impalpable right testis and normal left testis.Urinalysis, complete blood count and serum electrolytes were normal. At exploration through the left inguinal incision, two spermatic cords were found separated by a rudimentary uterus, thick-walled vagina and ligamentous structure corresponding to the broad ligament. The two vasa deferentia were adherent to the thickened vagina. The left testis was lying in the left hemiscrotum, but were pulled up into the operative field during dissection. The right testis ended at the left mid-inguinal canal ( Figure 1). The two testes were biopsied, the two cord structures were carefully separated from the persisting müllerian structures and limited resection of the rudimentary uterus and vagina was done. The associated inguinal hernia was repaired and bilateral orchidopexy with transposition of the right testis to the right hemiscrotum through a suprapubic subcutaneous tunnel was performed.The postoperative course was uncomplicated. The karyotype confirmed a male gender 46XY. Renal ultrasound was normal. DiscussionNormally, the testis is located in the scrotum at birth. Ectopic testes have been reported at different sites, including the superficial inguinal pouch, suprapubic, femoral and perineal areas, and at the base of the penis. Migration of the testis to the opposite side, where both testes pass through the same inguinal canal, is known as transverse testicular ectopia (TTE). Over 100 cases of TTE have been reported in the literature. 5,6 Persistent müllerian duct syndrome is rare, characterized by the presence of well-developed or rudimentary uterus, cervix, vagina and fallopian tubes in a normal 46XY male.The presence of PMDS with TTE is even more uncommon. In most cases, the PMDS is discovered during surgery for the inguinal hernia or cryptorchidism or by the presence of transverse testicular ectopia. Our patient was found to have PMDS with TTE during surgery for herniotomy in a child with contralateral undescended testis. PMDS represents only a small number of male pseudohermaphroditism. The external genitalia in these patients are normal in shape and size wit...