title: Clinical aspects and molecular genetics of persistent müllerian duct syndrome associated with transverse testicular ectopia: report of three cases.
abstract: Persistent müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism, characterized by the presence of a uterus and fallopian tubes owing to failure of müllerian duct regression in genotypically normal males. The association between a persistent müllerian duct and transverse testicular ectopia (TTE) is even more uncommon. PMDS with TTE is a very rare pathological association, often discovered during repair for inguinal hernia or cryptorchidism. We report 3 cases of Chinese patients with PMDS associated with TTE. Hysterectomy was performed, with resection of the underdeveloped fallopian tubes. Both gonads were placed into subdartos pouches in each scrotum by the transseptal approach. PMDS with TTE is a rarely encountered form of male pseudohermaphroditism usually unexpectedly found at surgery for cryptorchidism or inguinal hernia. Surgical treatment should avoid damage of fertile testes and vasa deferens.
authors.affiliation: Department of Urology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China.
authors.forename: Xiaobing
authors.initials: X
authors.lastname: Ju
authors.forename: ZhongXing
authors.initials: Z
authors.lastname: Li
authors.forename: Chao
authors.initials: C
authors.lastname: Zhang
authors.forename: Chao
authors.initials: C
authors.lastname: Qin
authors.forename: Pengfei
authors.initials: P
authors.lastname: Shao
authors.forename: Jie
authors.initials: J
authors.lastname: Li
authors.forename: Pu
authors.initials: P
authors.lastname: Li
authors.forename: Qiang
authors.initials: Q
authors.lastname: Cao
authors.forename: Wei
authors.initials: W
authors.lastname: Zhang
authors.forename: Zengjun
authors.initials: Z
authors.lastname: Wang
authors.forename: Changjun
authors.initials: C
authors.lastname: Yin
mesh_terms: D000328:Adult; D002828:Choristoma / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D003456:Cryptorchidism / Q000209:etiology* / Q000601:surgery; D058490:Disorder of Sex Development, 46,XY / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D006552:Hernia, Inguinal / Q000209:etiology / Q000601:surgery; D059685:Herniorrhaphy; D006801:Humans; D007044:Hysterectomy; D007223:Infant; D007621:Karyotyping; D008297:Male; D056126:Orchiopexy; D058994:Salpingectomy; D013737:Testis / Q000002:abnormalities* / Q000601:surgery; D016896:Treatment Outcome