Persistent Mullerian duct syndrome

Abstract

We report an 18-month old male presenting with a right-sided inguinal hernia and undescended testes. At herniotomy, a uterus and two fallopian tubes were found in the pelvic peritoneum adjacent to the two gonads which received their blood supply partly along the müllerian duct remnants. The gonads were testes by histological examination. Bilateral orchiopexy was performed without removal of the müllerian structures for fear of jeopardizing the testicular blood supply. The diagnosis of persistent müllerian duct syndrome was confirmed postoperatively by genetic and hormonal investigations.

Persistent mullerian duct syndrome: A 24-year experience

“…Various innovative techniques have been described to preserve vas creation of neoseminal vesicle from the retained part of cervix and vagina. 7 Some workers favour bilateral orchidopexy with retention of all mullerian duct structures to avoid any chance of damage to vas deferens in an attempt to preserve fertility 5,13,16,21,31,[34][35][36]. Others favour radical measures i.e., removal of all mullerian duct structures along with testes and vas to prevent any chance of malignancy, as these patients are infertile in majority.…”