The majority of patients with DSD will be found to carry an XY karyotype and be assigned male gender. From a phenotypical standpoint, most will present with proximal hypospadias ± cryptorchidism. In this review article, the authors present the current status of reconstruction of the male genitalia in this setting. The report addresses the following topics: surgical input in the evaluation of the newborn with an undervirilized external genitalia, including gender assignment considerations; controversies surrounding timing and indication for hypospadias surgery in proximal cases as well as use of testosterone; surgical techniques and decision-making process for one- vs. two-stage repairs; complications of hypospadias surgery based on technique used for repair; and long-term follow-up. The high complication rates observed in the treatment of proximal hypospadias attest to its challenging nature. Concentration of experience, tracking carefully identified patient-centered outcomes and long-term follow-up of this patient population are recommended.
Keywords: Ambiguous genitalia; Hypospadias; Intersex; Male; Surgery; XY DSD.
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