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Persistent müllerian duct syndrome. A case report
1Department of Obstetrics and Gynecology and of Surgery, University of Kocaeli, School of Medicine, Turkey.
Background:
Persistent müllerian duct syndrome is a rare form of male pseudohermaphroditism in which well-developed müllerian structures are present in an otherwise normal male with XY chromosomes. The syndrome was first described 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 because of having a palpable right-sided, irreducible inguinal hernia. Two testicles with surface nodularity and a bicornuate uterus with rudimentary fallopian 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 be helpful for diagnosing this condition before surgery. All patients with this 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.
