Persistent müllerian duct syndrome.
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.
Fernandes
E T
ET
Department of Surgery, LeBonheur Children's Hospital, Memphis, Tennessee.
Hollabaugh
R S
RS
Young
J A
JA
Wilroy
S R
SR
Schriock
E A
EA
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