Persistan müllerian kanal sendromu, transvers testiküler ektopi ve hipospadias birlikteliği

Persistan müllerian kanal sendromu PMKS , erkek fenotipinde tuba uterina, uterus ve vajenin 1/3 üst kısmı gibi müllerian artıkların bulunduğu bir sendromdur. Erkek psödohermafroditizmin nadir bir şeklidir. Bu sendrom nadiren transvers testiküler ektopi TTE ile birlikte olabilir. PMDS ve TTE birlikteliği çok nadir bir patolojidir. Hastalar genellikle normal erkek görünümünde, tek ya da iki taraflı inmemiş testisle birliktedir. Bu yazıda PMKS, TTE ve hipospadiası bulunan 5 yaşında hasta sunulmaktadır

Anahtar Kelimeler:

Persistan müllerian kanal, transvers testiküler ektopi, hipospadias

The togetherness of persistent mullerian duct syndrome, transverse testicular ectopia and hypospadias

Persistent Müllerian duct syndrome PMDS is characterized by the presence of müllerian duct structures such as fallopian tubes, uterus and 1/3 upper part of vagina in the phenotypic male. It is a rare form of male pseudohermaphroditism. This syndrome is rarely associated with transverse testicular ectopia TTE . PMDS and TTE is extremely rare. Patients are normally virilised, although uni- or bilateral cryptorchidism may be present. In this paper we report a PMDS associated with TTE and hypospadias in 5 years old boy

Keywords:

Persistent mullerian duct, Transverse testicular ectopia, hypospadias,

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