Urethral stricture in a 46,XX male with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency: A literature review and case report

Abstract

Disorders of sex development in 46,XX male individuals are rare and present complex surgical challenges, particularly regarding urethral stricture following urethral surgery. We present a 46,XX individual raised male who underwent early urethral surgery and developed a urethral stricture in adulthood. Surgical repair was not feasible due to anatomy and comorbidities. A systematic review on the management of urethral strictures in this patient population was conducted. Only one case of successful buccal mucosa graft urethroplasty in a 46,XX male was found. This case underscores the scarcity of data and challenges in managing urethral strictures in virilized 46,XX individuals.