Thesis (Ph.D.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Introduction: Müllerian ducts regress in males under the effect of anti-Müllerian hormone (AMH), produced by pre-Sertoli cells. Müllerian duct remnants (MDRs) persist due to (a) AMH deficiency, (b) receptor insensitivity or (C) gonadal dysgenesis (either 45XO/46XY, 46XY or 46XX). Cases may present clinically with proximal hypospadias and/or testicular maldescent of various combinations. MDRs may be missed on ultrasonography thus escape early detection, present as an intraoperative surprise, post-urethroplasty complications or malignant degeneration. Methods: Non-randomized prospective clinical trial (case series). 20 patient with MDRs were collected prospectively as candidates for laparoscopy. Except one case, all patients were assigned to be raised as males following karyotyping, hormonal essay and HCG stimulation test through a multidisciplinary decision by the DSD MDT involving both the endocrine and surgical teams. The surgical management for MDRs followed one of two approaches; either laparoscopic longitudinal splitting/division or near total excision. We reviewed the selection criteria for each approach and the decision-making to reach a technique suitable for each case depending on the individual morphological and histopathological findings of every case

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