Background: Congenital adrenal hyperplasia in females leads to virilization of external genitalia and persistent urogenital sinus. There are controversies regarding the timing and outcomes of surgery. Deferring surgeries beyond childhood is difficult to implement in conservative societies, and may result in stigmatization and distress to individuals with disorders of sexual differentiation and their families.
Methods: Thirty girls with virilization due to congenital adrenal hyperplasia were admitted for single-stage feminizing genitoplasty, between 2011 and 2014. We prospectively studied the concerns and input of the families represented by the mothers. After comprehensive counselling, the mothers completed a questionnaire to clarify their priorities and concerns related to surgery.
Results: Surgeries were performed at a mean age of 22 months. Most cases ranged between Prader's degrees III and IV. Egyptian families believe that early surgical reconstruction is in the best interest of their girls. They are marginally more concerned about functional outcomes and future child bearing than external appearance and cosmetic outcomes.
Conclusions: Social difficulties noticeably add challenges to the management plan within conservative societies. Early genital reconstructive surgery, when reasonably indicated, needs to remain a viable option. Comprehensive psychosocial support within a multidisciplinary approach is needed to defer feminizing genitoplasty in selected cases to adolescence.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1159/000442200 | DOI Listing |
Front Surg
December 2024
Department of Burn and Plastic Surgery, West China Hospital, Sichuan University, Chengdu, China.
Background: Ovotesticular disorder of sex development is a rare form of disorder of sex development that manifests as ovotestis in individuals. The precise diagnosis and the choice of surgical procedures are still in conflict condition due to the rarity of the disease, diverse clinical presentations, and the lack of evidence-based medical studies on postoperative outcomes.
Case Presentation: We present a 46, XX ovotesticular disorder of sex development case, aged 19, with Prader stage IV virilization who underwent feminizing genitoplasty surgery.
J Pediatr Surg
November 2024
Department of Pediatric Surgery, Fattouma Bourguiba University Hospital, Monastir, Tunisia; Research Laboratory LR12SP13, Faculty of Medicine of Monastir, Tunisia.
Background: Disorders of Sexual Development can cause significant psychological distress for parents, particularly in traditional societies where such conditions are stigmatized.
Aim: This study aims to investigate the psychoaffective impact of sexual development disorders caused by congenital adrenal hyperplasia in children with 46, XX karyotype, in order to determine the predictive factors for the onset of anxiety and depression among parents and the coping strategies employed by parents to adapt to their child's condition.
Materials And Methods: This is a retrospective, cross-sectional, descriptive, and analytical study involving parents of children treated for sexual development disorders related to congenital adrenal hyperplasia, with a 46, XX karyotype, who underwent feminizing genitoplasty during the period from January 1, 2005 and December 31, 2021.
J Paediatr Child Health
November 2024
Department of Surgery, School of Clinical Medicine, The University of Hong Kong, Queen Mary Hospital, Pokfulam, Hong Kong.
Aim: To investigate the long-term quality of life and surgical outcome of female patients with congenital adrenal hyperplasia (CAH).
Methods: Questionnaires for health-related quality of life (HRQoL) outcome (WHO-5, SF-36), lower urinary tract symptoms (ICIQ-FLUTS) and sexual outcome (ICIQ-FLUTSsex) were administered to adult CAH patients. Paediatric CAH patients and their parents were invited to complete WHO-5, PedsQL-4.
Introduction: Congenital adrenal hyperplasia (CAH) is characterized by a broad spectrum of symptoms. This study aims to describe genotype-phenotype correlations, clinical manifestations at diagnosis, and the frequency of feminizing surgery in childhood.
Methods: A nationwide retrospective cohort study of patients diagnosed with CAH, aged ≤18, between 1943 and 2018.
Endocrine
October 2024
Department of Women's and Children's Health, Center of Molecular Medicine, Karolinska Institutet, 17176, Stockholm, Sweden.
Purpose: To study what adult women with congenital adrenal hyperplasia (CAH) thought about the timing of genital surgery.
Methods: As part of a larger follow-up study performed between the years 2002-2005 there were questionnaires concerning genital surgery, type of surgery, their thoughts about timing of genital surgery and experience of information about surgery. Early surgery was defined as ≤4 years of age and late ≥10 years.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!