ESPE Yearbook of Paediatric Endocrinology

Brief Summary: This qualitative study investigated parental communication with their daughters regarding variation in clitoral size related to severity of classical congenital adrenal hyprplasia (CAH). The authors conducted semi-structured in-person interviews with 24 parents of children with a specific genital difference, without direct exploration of parental values regarding the clitoris or the application of adequate psychosocial care.

Comment: CAH due to 21-hydroxylase deficiency (21-OHD) requires life-long medical management of adrenal insufficiency and adrenal androgen excess. 46,XX children with 21-OHD have altered genital morphology, including clitoral enlargement, due to high fetal androgen secretion in utero, and this condition is therefore recognized by most medical experts to be a ‘difference of sex development’ (DSD). Childhood clitoral surgery has been questioned owing to concerns about negative long-term effects on clitoral sensitivity and sexual function (1). As reported in previous studies, the parents interviewed here worried about their child’s sexual future, and expressed considerable hesitancy about educating the child and facilitating a growing awareness of their development at birth. (2) (3).

This study focused on clitoral variation as a means of exploring parents’ communication and barriers in the management of children’s atypical genital appearance. Parents were led by professionals to an “Obvious Choice” regarding accepting or avoiding early childhood clitoral surgery, after which most parents perceived their child’s genitals to be “Still Different”. The impact of their child’s genital difference remained a psychological and practical “Parental Burden,” and most parents alluded to a belief that they should prevent their child from knowing about her genital difference because “Ignorance is Bliss.” Almost all participants referenced their belief in benevolent ignorance and the burden of responsibility caused by feeling able to maintain the child’s lack of awareness, poor knowledge, and pre-sexual life temporary state of ignorance. This approach is controversial given that adults with experience have repeatedly interpreted parental inability to talk about their bodily difference as a sign of unspeakable shame (4). Exclusion of CAH from DSD is controversial, although this would not eliminate the ongoing bioethical debate on surgical alteration of the clitoris in childhood (5).

An important dimension of multi-professional care is facilitating patient self-knowledge. This study suggests that DSD services may not be fully aware of the lack of information available to children if the responsibility for education falls on their parents. Health professionals share responsibility for the child well-being via partnership and direct support of parents. These findings suggest that neither childhood genital surgery, nor its absence, singularly eradicates parental perception of difference or removes parental concerns. Healthcare services must support parental ability to engage in essential communication with their children on topics such as clitoral size variation related to neonatal CAH.

References: 1. Crouch NS, Liao LM, Woodhouse CR, Conway GS, Creighton SM. Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia. J Urol. 2008; 179(2): 634-8.2. Lundberg T, Lindstrom A, Roen K, Hegarty P. From Knowing Nothing to Knowing What, How and Now: Parents’ Experiences of Caring for their Children with Congenital Adrenal Hyperplasia. J Pediatr Psychol. 2017; 42(5): 520-9.3. Thornton M, Harcourt D, Deave T, Kiff J, Williamson H. "Have We Done Enough?" A Cross-condition Exploration of the Experiences of Parents Caring for A Child with an Appearance-affecting Condition or Injury. Dev Neurorehabil. 2021; 24(6): 418-28.4. Alderson J, Madill A, Balen A. Fear of devaluation: understanding the experience of intersexed women with androgen insensitivity syndrome. Br J Health Psychol. 2004; 9(Pt 1): 81-100.5. Gonzalez R, Ludwikowski BM. Is It Beneficial to Patients to Include Congenital Adrenal Hyperplasia (CAH) Among the Disorders of Sex Development (DSD)? Front Pediatr. 2018; 6: 344.