Department of Paediatric Surgery | Cairo University Specialized Paediatric Hospital (CUSPH) & Cairo University Children's Hospital (CUCH) (email)
Scottish Surgical Paediatric Society
May 2015
Mahmoud Marei; Jimmy Lam; Claire Clark; Fraser Munro
Review of Remnant Müllerian Structures in Male DSD, The Edinburgh Experience.
Introduction: Müllerian duct regression occurs in males, between the 8th and 11th weeks of intra-uterine life. It is under control of AMH which is produced by the pre-Sertoli cells. Müllerian structures could persist due to lack of AMH, or a defect of its receptors, as well as in the dysgenetic types of male DSD. The cases may present by proximal hypospadias, and/or testicular maldescent of various combinations and severity.
Methods: Five cases of remnant Müllerian structures were managed at our centre in the last four years. The presentations met and operations performed in these complex cases were reviewed, to help devising a protocol and clear indications of surgical removal.
Results: In this series three cases were discovered during groin conditions, one during an inguinal hernia repair, one during a groin orchidopexy for a palpable undescended testis, and one during a laparoscopic orchidopexy for an impalpable testis. Two cases were discovered during laparoscopy, as an investigation for genital ambiguity in the neonatal period, to enable allocating the sex of rearing.
Conclusion: The indications of removal of a Müllerian structures in this series were to enable tension-free orchidopexy, as they anchor the testis to a higher position, and as a preparation for proximal hypospadias repair to avoid their filling with urine due to outflow resistance following urethroplasty, hence stasis and infection. It was possible to remove the Müllerian structures in all cases laparoscopically.