Surgical management of 46,XY differences in sex development

The surgical management of differences in sex development (DSD) has evolved over the decades with advances in diagnostic methods rendering clarity to the etiopathogenesis, surgical anatomy, natural history, and principles of treatment of various forms of 46,XY DSD. Cumulative insights into intermediate and long-term results of surgical cosmesis, functional outcomes, and gender dysphoria have tempered the surgical strategies with a global moratorium on elective interventions till the age of consent. Besides the pros and cons of early versus late surgery for 46,XY DSD, the narrative elaborates on the indications, surgical principles, timing of surgery, salient details of procedures, and their outcomes. Masculinizing procedures include gonadal surgery, penile reconstruction, surgery for urogenital sinus (UGS) and/or Müllerian remnants, and breast reduction. Likewise, feminizing procedures encompass clitorophallic reduction, surgery for UGS and/or vaginoplasty, gonadectomy, and breast augmentation. Also included are algorithms for surgical management in broad categories of 46,XY DSD and illustrative cases.