Endocrine Abstracts

Background: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency results in cortisol and aldosterone deficiency and increased prenatal androgens resulting in varying degrees of virilization of external genitalia in females. Early genital surgery was until recent decades common practice. Unsatisfactory long-term outcomes and human rights perspectives have resulted in a shift in praxis towards postponed surgery to an age when the girl can participate in the decision making process herself. We study the psychological impact for the affected families of postponing surgery.

Methods: Parents to children with CAH, age 4-17 years, answered validated questionnaires on parental protectiveness (PPS), perceived stigma, future concerns (Parent self-report PSR), decisional regret and quality of life for both parents and children. Participants were 42 parents to 27 children. They were divided into parent of ‘girl no surgery’ (n = 19), and ‘girl early surgery’ (n = 4). Parents of boys with CAH (n =19) were included to control for the situation of having a child with CAH per se.

Results: No differences were seen between the groups regarding parental protectiveness, and the large majority >80% did not meet the clinical cut-off. More than 50% of parents to girls with no surgery reported moderately high concerns regarding future genital appearance, limitation in sexual and social relationships. Decisional regret varied among the parents to girls with no surgery. The parents of boys with CAH were significantly more worried that their child would die due to their condition compared to those with a daughter.

Conclusions: Parents to girls who did not have early genital surgery reported moderately high concerns related to the genital virilisation but did not report increased parental protectiveness. Future studies on psychological effects for the girls themselves throughout childhood and their perception and views on their own decision-making process on genital surgery at a later age is warranted.