Decision-making in pediatric persistent Mullerian duct syndrome

  • Ameer Al-Hadidi
  • Ismael Nassar
  • Wael Amro
  • Ahmad Shaltaf
  • Nathan Novotny
Keywords: cryptorchidism, Mullerian duct syndrome, male pseudohermaphroditism, undescended testicle

Abstract

We are reporting a case of an 18-month old male who presented with bilateral cryptorchidism. The patient underwent an explorative laparoscopy in which two gonads were identified in close proximity to the uterus and fallopian tubes. Biopsy of the gonads confirmed testicular tissue. Genetic analysis demonstrated a 46, XY male. Male external genitalia were appropriate for age with no evidence of female structures. Persistent Mullerian duct syndrome is extremely rare, with approximately 260 cases reported in the literature. Best practice for the extent of surgical management is still evolving as we gather data on long-term outcome

Keywords: cryptorchidism, Mullerian duct syndrome, male pseudohermaphroditism, undescended testicle

Published
2018-02-20
Section
Articles

Journal Identifiers


eISSN: 1687-4137
print ISSN: 1687-4137