Case report: Persistent mullerian duct syndrome

Persistent mullerian duct syndrome also named as hernia uteri inguinale is a form of internal male pseudohermafroditsm and may result from the failure of synthesis or release of AMH, defect of AMH receptors or the defect in the timing of the release of AMH. This syndrome is an inherited disorder, it is usually transmitted as a recessive autosomal trait or X linked trait. Patients affected with PMDS are phenotypic males with either bilateral cryptoorchidism or unilateral testicular ectopia associated with inguinal hernia on the contralateral side. The presence of uterus and tubes is usually discovered at surgery. Three month old male child operated for cryptoorchidism and found out to be PMDS after the examination of the operation material is reported. This is a comprehensive review of the literature with evaluation of the clinical features.

Olgu sunumu: Persistent müller kanalý sendromu (PMKS)

Herni uteri inguinal e olarak da bilinen Persistan Müller Ka¬nal Sendromu (PMKS) internal erkek pseudohcrmafroditizmi- nin bir formudur ve AMH (Antimüllerian hormon) yetmezliði, AMH reseptör defekti veya sekresyonunda zamanlama defek- tine baðlý olarak geliþir. Bu sendrom familyal olup muhteme¬len otozomal resessif veya X’ e baðlý resesif geçiþlidir. PMKS’li hastalar fenotipik erkeklerdir. Bilateral kriptoorþitizm veya unilateral testiküler ektopi ile iliþkili kontrlateral inguinal herni mevcuttur. Utenis ve tubalarýn varlýðý genelde cerrahi sýrasýnda farkedilir. Kriptoorþitizm ön tanýsýyla opere edilen iiç aylýk erkek çocukta operasyon materyalinin incelenmesi sonucu PMKS tesbit edildi. Literatür bilgileri eþliðinde olguyu araþtýrmak ve birlikte tartýþmak amacý ile sunuldu.