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

Szymański, Konrad M.; Whittam, Benjamin; Kaefer, Martin; Frady, Heather; Cain, Mark P.; Rink, Richard C.

doi:10.1016/j.jpurol.2018.07.010

Cited by 10 publications

(4 citation statements)

References 13 publications

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“…Disorders/differences of sex development (DSD) are rare congenital conditions that affect sex differentiation and may result in ambiguous genitalia (Hughes et al, 2006;Lee et al, 2016;Szymanski et al, 2018a;Szymanski et al, 2018b;Thyen et al, 2006). DSD is typified by a discordance in genetic chromosomal sex, gonadal sex, and/or the appearance of internal/external reproductive organs (Hughes et al, 2006;Wisniewski et al, 2012).…”

Section: Introductionmentioning

confidence: 99%

Illness Uncertainty Longitudinally Predicts Distress Among Caregivers of Children Born With DSD

Roberts

Sharkey

Bakula

et al. 2020

Journal of Pediatric Psychology

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Objective A subset of parents of children with disorders/differences of sex development (DSD) including ambiguous genitalia experience clinically elevated levels of anxious and depressive symptoms. Research indicates that uncertainty about their child’s DSD is associated with parent psychosocial distress; however, previous studies have been cross-sectional or correlational in nature. The current study is the first to examine the longitudinal trajectory of the relationship between caregiver-perceived uncertainty about their child’s DSD and caregiver anxious and depressive symptoms across the first 12 months following genital surgery in young children, or if surgery was not performed, the first 12 months following study entry. Methods One hundred and thirteen caregivers (Mage = 32.12; 57.5% mothers; 72.6% Caucasian) of children (N = 70; Mage = 9.81 months; 65.7% female) with DSD were recruited from 12 DSD specialty clinics in the United States. Caregivers completed psychosocial measures at baseline, 6 and 12 months following genitoplasty, or study entry if parents elected not to have surgery for their child. Results Caregiver illness uncertainty and both anxious and depressive symptoms were highest at baseline and decreased over time (ps < .05). Caregiver illness uncertainty predicted symptoms of anxious and depressive symptoms across all time points (ps < .05). Conclusions Caregivers’ perceptions of uncertainty about their child’s DSD are highest soon after diagnosis, and uncertainty continues to predict both anxious and depressive symptoms across time. Thus, the initial diagnostic period is a critical time for psychological assessment and intervention, with parent illness uncertainty being an important clinical target.

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Section: Introductionmentioning

confidence: 99%

Illness Uncertainty Longitudinally Predicts Distress Among Caregivers of Children Born With DSD

Roberts

Sharkey

Bakula

et al. 2020

Journal of Pediatric Psychology

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“…On the opposite side of the spectrum, a number of advocacy groups, question the benefit of early surgery and the ethical issues involving parents and physicians rights making decisions for the children. The recommendation is that the decision has to be made by a transdisciplinary team with transparency, presenting all pros and cons of surgery versus observation without withholding any information from families (1,(4)(5)(6)12).…”

Section: Discussionmentioning

confidence: 99%

“…Most families opt for early surgery, considering the positive implications for children's psychosocial development, relieving parents distress and restoring "normal" external genital configuration. Szymanski et al evaluated parental decisional regret after FG, they reported that 20.5% of parents, reported some regret, in contrast no parent would have chosen again delayed surgery (1,(4)(5)(6).…”

Section: Introductionmentioning

confidence: 99%

Complete corporeal preservation clitoroplasty: new insights into feminizing genitoplasty

2021

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Introduction: 46,XX Congenital adrenal hyperplasia (CAH) remains the fi rst cause of genital virilization and current surgical techniques aim to restore female aspect of genitalia while preserving dorsal neurovascular bundle but not at the expense of not preserving erectile tissue. We aim to report our experience with a new surgical technique for clitoroplasty, completely preserving corporeal bodies, neurovascular bundles without dismembering the clitoris, in four patients with over a year follow up. Materials and Methods: After IRB approval four patients with 46,XX CAH and Prader 5 and 3 external genitalia, underwent feminizing genitoplasty. Complete preservation of erectile tissue was accomplished without a need to dissect dorsal neurovascular bundle. Glans size allowed no need for glanular reduction and there was no need to dismember the corporeal bodies.Results: Four patients 12 to 24-months-old underwent complete corporeal preservation clitoroplasty (CCPC), mean age was 18.5 months, mean follow up was 10.25 months. Vaginoplasty was performed in all patients with partial urogenital mobilization (PUM) and Urogenital Sinus fl ap (UF), only one severely virilized patient required a parasagittal pre-rectal approach to mobilize the vagina. We had no complications until last follow up. Conclusion: To our knowledge, we are introducing the concept of CCPC without the need of disassembling the corporeal bodies, neurovascular bundle and glans. It stands as a new alternative for feminizing genitoplasty with complete preservation of erectile tissue and no dissection of neurovascular bundle. Although there is still lacking longterm follow-up, it represents a new step in conservative reconfi guration of the external virilized female genitalia.

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“…It would be detrimental to people's health if an endocrine disease like CAH would be managed like other diagnoses under the DSD umbrella because females present with atypical genitalia. Given its unique pathophysiology [1] and psychosocial impact, [3,13] it would be prudent to consider CAH separate from DSD in order to provide individualized multidisciplinary care.…”

Section: Discussionmentioning

confidence: 99%

Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex

Szymański

Rink

Whittam

et al. 2021

Journal of Pediatric Urology

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

Cited by 10 publications

References 13 publications

Illness Uncertainty Longitudinally Predicts Distress Among Caregivers of Children Born With DSD

Illness Uncertainty Longitudinally Predicts Distress Among Caregivers of Children Born With DSD

Complete corporeal preservation clitoroplasty: new insights into feminizing genitoplasty

Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex

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