Individualized care for patients with intersex (differences of sex development): part 4/5.Considering the Ifs, Whens, and Whats regarding sexual-reproductive system surgery. Issue 3 (June 2021)

• Record Type: Journal Article
• Authors: Bangalore Krishna, Kanthi; Kogan, Barry A.; Mazur, Tom; Hoebeke, Piet; Bogaert, Guy; Lee, Peter A.
• Is Part Of: Journal of pediatric urology. Volume 17:Issue 3(2021)
• Page Start: 338
• Page End: 345
• Publication Date: 2021-06
• Subjects: Differences of sex development -- Complete androgen insensitivity syndrome -- Congenital adrenal hyperplasia -- Gender assignment -- Genital surgery -- Vaginoplasty; AIS androgen insensitivity syndrome -- CAH congenital adrenal hyperplasia -- DSD differences of sex development; Pediatric urology -- Periodicals; Urologic Diseases -- Periodicals; Urogenital Diseases -- Periodicals; Urologic Surgical Procedures -- Periodicals; Child; Infant; Urologie pédiatrique -- Périodiques; Appareil urinaire -- Maladies -- Périodiques; Pédiatrie; Urologie; Pediatric urology; Périodique électronique (Descripteur de forme); Ressource Internet (Descripteur de forme); Electronic journals; Periodicals; Electronic journals; 618.926
• DOI: 10.1016/j.jpurol.2021.02.011
• Languages: English
• ISSNs: 1477-5131

Abstract

Summary: Issues and concerns regarding surgery of the sexual-reproductive anatomy during infancy and early childhood are discussed using four actual examples. A case of a 46, XX infant with 21 hydroxylase deficiency congenital adrenal hyperplasia (CAH) with atypical (ambiguous) genitalia is discussed regarding timing and potential harms and benefits of surgery. We present the perspective of balancing the child's rights to bodily autonomy and right to an open future versus parents' decision making authority regarding what they perceive as their child's future best interests. The second case is a newborn with complete androgen insensitivity syndrome and we discuss the harms, benefits and timing of gonadectomy. The third case examines the physical and psychological impact of penile shaft hypospadias, raising the question of whether surgery is justified to prevent what may or may not be considered a permanent disability. The fourth case involves an adult woman with classic CAH, born with a urogenital sinus and clitoromegaly, who never had genital surgery and is now requesting vaginoplasty, but not clitoral reduction. The primary message of this article, as the previous articles in this series, is to encourage patient-family centered care that individualizes treatment guided by shared decision making.