Guidance for shared decision-making regarding orchiectomy in individuals with differences of sex development due to 17-β-hydroxysteroid dehydrogenase type 3 deficiency.

ABSTRACT: 17β-hydroxysteroid dehydrogenase type 3 deficiency is a 46,XY difference of sex development (DSD) that may present in childhood with inguinal testes or at puberty following virilization. We present four individuals, assigned female at birth, to highlight complexities and considerations surrounding orchiectomy. We reviewed the literature and created a "FACT sheet" to guide shared decision-making for patients, parents, and providers. "Ruth" presented at 16 months with inguinal herniae and underwent orchiectomy, based on parental preference. "Erica" presented at 13 years with voice deepening; she and her parents chose pubertal suppression and eventual orchiectomy. "Riley" presented at 18 months with inguinal herniae; after pubertal suppression and estrogen replacement, orchiectomy at age 13 y

SUBMITTER: Yu LX 

PROVIDER: S-EPMC11882584 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature