Differences/disorders of sex development (DSD) are a heterogeneous group of congenital conditions in which the development of chromosomal, gonadal, or anatomical sex is atypical. Patients usually present during the newborn period but occasionally some cases remain unrecognized until later in infancy or even adolescence. Genital appearance, psychosocial support, sociocultural background, gender identity development, and genetic and biochemical analysis in addition to ethical and legal implications need to be considered when deciding on the appropriate treatment strategy. Surgeons are important members of the multidisciplinary expert teams involved in the initial approach and long-term follow-up. Surgical care of DSD patients is one of the main challenges. Recommendations regarding the opportunity and timing of surgical procedures are still under discussion. Surgical procedures are aimed to reduce urologic problems, prevent the risk of gonadal germ-cell cancer, and facilitate sexual function and reproduction. Providing its excellent visualization, access to pelvic structures and less postoperative adhesion MIS has been an important tool in the diagnosis and treatment of DSD. The role of MIS will be summarized in: 1) Gonadal biopsy / gonadectomy 2) Treatment of urogenital sinus/vaginoplasty 3) Vaginal Replacement 4) Resection / treatment of Mullerian structures.
Keywords: 45XX DSD; 46xyDSD; MIS gonadal biopsies; MIS vaginal replacement; PMDS; germ cell tumors in DSD; ovotestes; streak gonad; urogenital sinus.
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