Early Genital Surgery in Disorders/Differences of Sex Development: Patients’ Perspectives

Abstract

Controversy continues over a proposed moratorium on elective genital surgery in childhood for disorders/differences of sex development (DSD). Empirical evidence on patient preference is needed to inform decision-making. We conducted a multicentre survey by cross-sectional questionnaire in 14 specialized clinics in six European countries. The sample comprised 459 individuals (≥ 16 years) with a DSD diagnosis, including individuals with congenital adrenal hyperplasia (CAH) (n = 192), XY DSD with prenatal androgen effect (A) (n = 150), and without (nA) (n = 117). Main outcome measures were level of agreement with given statements regarding genital surgery, including clitoris reduction, vaginoplasty, and hypospadias repair. A total of 66% of individuals with CAH and 60% of those with XY DSD-A thought that infancy or childhood were the appropriate age for genital surgery. Females with XY DSD were divided on this issue and tended to prefer vaginoplasty at a later age (XY DSD-A 39%, XY DSD-nA 32%). A total of 47% of males preferred early hypospadias surgery. Only 12% (CAH), 11% (XY DSD-A), and 21% (XY DSD-nA) thought they would have been better off without any surgery in childhood or adolescence. Individuals who had early genital surgery were more likely to approve of it. Outcome data failed to support a general moratorium on early elective genital surgery. Participant perspectives varied considerably by diagnostic category, gender, history of surgery, and contact with support groups. Case-by-case decision-making is better suited to grasping the ethical complexity of the issues at stake.Trial registration: German Clinical Trials Register DRKS00006072.

The Timing of Genital Surgery in Somatic Intersexuality: Surveys of Patients’ Preferences

“…Ten pertinent surveys with data from individuals with somatic intersexuality were retained (Table 1), all published in the English language: three from the USA [37,39,45]; four from European countries [25,[40][41][42]; and one each from Brazil [43], China [44], and Malaysia [36]. All 10 surveys were based on samples of clinic patients, most of whom had previously undergone genital surgery.…”

“…Similarly, in the Brazilian study [43], the subgroup of patients first operated in adolescence or adulthood would have preferred earlier surgery. On the other hand, each study included a minority of patients with different preferences or uncertainty, especially patients with XY complete androgen insensitivity (CAIS) [44] and patients with XY intersexuality without prenatal androgen effects (including CAIS) [42].…”

“…In regard to the timing of genital surgery, the clear majority of patients in seven of nine studies directly inquiring about timing preferences favored early surgery (mostly at infancy or childhood age) over later ages ( p < 0.05) [25, 36, 39-42, 45]. In the Chinese study [44], in which late clinical presentation and late surgery was prevalent, 74% of patients with surgery in adolescence and 78% of patients with surgery in adulthood thought the surgery was too late.…”

“…The latter large-sample six-country European study [42] allows a comparison of three subgroups: (A) XX CAH (almost all female-identified), (B) XY-DSD with prenatal androgen effects (most either male- or female-identified), and (C) XY-DSD without prenatal androgen effect (almost all female-identified and none male-identified). Group A showed the strongest preference for early surgery, Group B a similar, although less clear-cut preference pattern, but the majority of Group C patients favored somewhat later surgery.…”

“…Reference selection was limited to articles in English, Spanish, French, and German. This search led to the identification of three articles reporting data from surveys of parents only [33][34][35], four articles including surveys of both parents and patients [25,[36][37][38], and four articles including surveys of patients only [39][40][41][42]. Two additional articles on surveys of patients only [43,44] were accidentally identified while studying article texts during an independent search for articles on behavioral outcomes of genital surgery in individuals with intersexuality, and a further article on a patients-only survey [45] was mailed by its principal investigator to this author.…”