title: Persistent müllerian duct syndrome.
abstract: Persistent müllerian syndrome is rare. A case of phenotypically normal male with persistent müllerian structures that consisted of a bicornuate uterus, fallopian tubes, and upper third of the vagina is reported. These unusual structures were found in association with bilateral cryptorchidism and a right inguinal hernia, and were diagnosed while repairing the hernia in the neonatal period. The uterus and fallopian tubes were removed via laparotomy when the child was eighteen months old; at the same time a bilateral orchiopexy was performed. To avoid damage to the vas deferens, which lay in closely to the müllerian structures and could not be separated from the vaginal wall, a small segment of the upper third of the vagina was retained. The testicles, although normal on pathologic examination, have shown poor response to hormonal stimulation with human chorionic gonadotropin. Long-term follow-up for these patients is necessary because they have an increased risk of testicular tumors developing.
authors.affiliation: Department of Surgery, LeBonheur Children's Hospital, Memphis, Tennessee.
authors.forename: E T
authors.initials: ET
authors.lastname: Fernandes
authors.forename: R S
authors.initials: RS
authors.lastname: Hollabaugh
authors.forename: J A
authors.initials: JA
authors.lastname: Young
authors.forename: S R
authors.initials: SR
authors.lastname: Wilroy
authors.forename: E A
authors.initials: EA
authors.lastname: Schriock
mesh_terms: D003456:Cryptorchidism / Q000150:complications / Q000601:surgery; D006552:Hernia, Inguinal / Q000150:complications / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009095:Mullerian Ducts / Q000002:abnormalities* / Q000601:surgery; D013577:Syndrome