What about my daughter's future? Parental concerns when considering female genital restoration surgery in girls with congenital adrenal hyperplasia

Abstract

The parental decision-making process regarding female genital restoration surgery (FGRS) for girls with congenital adrenal hyperplasia (CAH) is controversial and poorly understood. The aim of the study aim was to evaluate parental concerns related to their child's future and parental plans about disclosure prior to FGRS. The authors performed an online survey of consecutive parents presenting at a tertiary referral center for consultation regarding FGRS for their daughter with CAH before 3 years of age (2016-2018). Twenty issues initially identified by three families and six clinicians were rated on a 6-point Likert scale of importance ('not at all' to 'extremely'). Sixteen consecutive families participated (Prader 3/4/5: 43.8%/43.8%/12.5%). Fourteen girls (87.5%) subsequently underwent FGRS at a median age of 8 months. Most issues (19/20, 95.0%) were ranked 'quite a bit' to 'extremely' important (Table). Top issues were not surgical: Normal physical/mental development, adrenal crisis and side-effects of medications. Surgery-related and self-image concerns followed in importance. Least prioritized issues were multiple genital exams ('quite a bit' important) and thechild not being involved in the decision to proceed with FGRS ('somewhat' important). On average, no issues were considered 'not at all' or 'a little' important. Disclosure of FGRS to their daughter was the 15th prioritized issues. Almost all families (93.8%, 1 unsure) planned to disclose the surgery to their daughter, although many were unsure when and how to do it (33.3% and 37.5%, respectively). Initial efforts to understand the complex process of parental decision-making regarding FGRS in the context of CAH, a complex, multifactorial disease, are presented. Parents of infant girls with CAH simultaneously weighmultiple life-threatening concerns with a decision about FGRS. While issues of genital ambiguity and surgery are important, they are not overriding concerns for parents of girls with CAH. Parents report significant uncertainty about appropriate timing and approach to disclosing FGRS to their daughters. Unfortunately, best practice guidelines for this process are lacking. The findings are not based on actual history of disclosurebut on parents' anticipated behavior. Further data are need from parents, children, and women with CAH about successful disclosure. Being a single-center series, these data may not correspond to the wider CAH community. Parental decision-making regarding FGRS is multifactorial. Even when considering FGRS, parents' largest concerns remain focused on the life-threatening and developmental effects of CAH and side-effects of its medical treatment. The disclosure process deserves further attention.