BAPS papersResults from a pediatric surgical centre justify early intervention in disorders of sex development
Section snippets
Methods
The records of children born between 1997 and 2003 (aged 5-10 years at time of study) who were treated for a DSD at the RCH were retrospectively evaluated. Diagnoses, date of birth, and dates of surgical intervention were noted. Those patients who had not undergone surgery for ambiguous genitalia were excluded. At the time of their review appointments, the study was explained to children and their parents. After obtaining informed consent, children were examined and genitalia scored on a
Results
After review of medical records, 54 children were identified as being eligible for participation in the study. Ten of these patients were lost to follow-up. Of 44 patients, 41 participated in 1 or more component of the study. Diagnoses of female participants included 16 congenital adrenal hyperplasia, 2 ovotesticular DSD, and 1 XX virilisation of unclear cause, and diagnoses of males included 5 mixed gonadal dysgenesis, 3 partial androgen insensitivity syndrome, 3 ovotesticular DSD, 1 5α
Discussion
This study found that acceptable cosmetic results can be achieved by early genital surgery, as 100% of females and 90% of males had satisfactory or better outcomes. Also, when practised in concert with appropriate psychological care as part of a holistic management plan, early intervention results in minimal impairment in quality of life or gender development.
The finding that most patients in our cohort at the RCH had acceptable cosmetic results agrees with other reports in the literature that
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Cited by (51)
Individualized care for patients with intersex (differences of sex development): part 4/5.Considering the Ifs, Whens, and Whats regarding sexual-reproductive system surgery
2021, Journal of Pediatric UrologyCitation Excerpt :A cross-sectional cohort analysis illustrates the multiple factors involved in sexual function outcomes by including those who had normal genitalia (non-classical individuals). This group plus others with clinical signs of hyperandrogenism had higher distress regarding sexual function compared to women without clinical signs of hyperandrogenism, highlighting the importance of early diagnosis and therapy initiation in all patients with CAH including those with non-classical CAH [35,36]. How efficacious surgical external genital construction is in overall good quality of life depends not only the quality of surgery but other factors, some of which are alluded to above.
Congenital adrenal hyperplasia: Does repair after two years of age have a worse outcome?
2020, Journal of Pediatric UrologyAnalysis of Presentations and Outcomes of Care of Children with Disorders of Sexual Development in a Nigerian Hospital
2019, Journal of Pediatric and Adolescent GynecologyCitation Excerpt :There is an ongoing debate on the ideal time for reconstructive surgery in children born with DSD. Most centers in Europe continue to offer early surgical intervention as part of a holistic treatment plan.22–24 Some of the reasons for early surgery include the maternal estrogenic effect on infant tissues, and reduced risk of urinary tract infections.
Prospective assessment of cosmesis before and after genital surgery
2017, Journal of Pediatric UrologyCitation Excerpt :Most studies have included only cosmetic and functional outcomes rated by a physician [7,8,10–12], and satisfaction ratings may differ between patients and physicians. Studies that include cosmetic ratings by affected adults are scarce [9,12], and fewer exist among adolescents [7] or children [11], with most being small, retrospective and single center [7,9,10,12]. The present prospective, observational, multi-center study aimed to: 1) describe the frequency of sex assignment and types of surgery performed in a cohort of patients followed with moderate-to-severe genital ambiguity at DSD centers in the USA; and 2) prospectively determine cosmesis ratings by parents and surgeons before and after genital surgery.
Quality of Life in Children and Young People With Congenital Adrenal Hyperplasia—UK Nationwide Multicenter Assessment
2024, Journal of Clinical Endocrinology and Metabolism
Presented at the 55th Annual Congress of the British Association of Paediatric Surgeons, Salamanca, Spain, July 2-5, 2008.
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Presenting author.