Persistent mullerian duct syndrome - A case report

BACKGROUND: Persistent mullerian duct syndrome is a rare form of male pseud ohermaphroditism in which well-developed mullerian structures are present i n an otherwise normal male with XY chromosomes. The syndrome was first desc ribed by Nilson in 1939, and almost 100 cases have been reported. CASE: A 22-year-old man presented with mild, right-sided inguinal pain and heaviness in his scrotum. He underwent right-sided inguinal exploration bec ause of having a palpable right-sided, irreducible inguinal hernia. Two tes ticles with surface nodularity and a bicornuate uterus with rudimentary fal lopian tubes were detected and removed as one specimen, and the hernia was repaired. CONCLUSION: Management of this syndrome is difficult because of the limited number of cases. If the diagnosis can be made before surgery, karyotyping can be useful to decide on orchiopexy or orchioectomy. In suspected cases, laparoscopy and ultrasonographic evaluation of all crytorchidic cases may b e helpful for diagnosing this condition before surgery. All patients with t his syndrome have a male phenotype; therefore, it is essential to preserve secondary sex characteristics. Androgen replacement therapy should be given to patients who have undergone gonadectomy and to those with low levels of testosterone.