{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T18:09:01Z","timestamp":1791828541143,"version":"4.3.4"},"reference-count":33,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2022,8,8]],"date-time":"2022-08-08T00:00:00Z","timestamp":1659916800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"European Union Seventh Framework Programme","award":["305373 [FP7\/2007-2013]"],"award-info":[{"award-number":["305373 [FP7\/2007-2013]"]}]},{"name":"Deutsche Forschungsgemeinschaft","award":["305373 [FP7\/2007-2013]"],"award-info":[{"award-number":["305373 [FP7\/2007-2013]"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Representatives for congenital adrenal hyperplasia (CAH) continue to desire early feminizing surgery in girls with 46,XX-CAH. The aim of this analysis, which included 174 46,XX- individuals with salt-wasting (SW) or simple-virilizing (SV) CAH, a female gender identity, and an age &gt; 16 years participating in a multicenter cross-sectional clinical evaluation study (dsd-LIFE), was to evaluate the long-term results of surgery and patient-reported outcomes (PRO). The gynecological examination (n = 84) revealed some shortcomings concerning surgical feminization. A clitoris was absent in 9.5% of cases, while a clitoral hood was missing in 36.7% of cases. Though all women had large labia, they didn\u2019t look normal in 22.6% of cases. Small labia were absent in 23.8% of cases. There was no introitus vaginae, and the urethra and vagina had no separate opening in 5.1% of cases. A mucosal lining was missing in 15.4% of cases. Furthermore, 86.2% of the women had scars at the region of their external genitalia. A vaginal stenosis was described in 16.5% of cases, and a meatal stenosis was described in 2.6% of cases. Additionally, PRO data showed a very-\/high satisfaction rate of 21.3%\/40.2% with cosmesis and 23.8%\/38.1% with functionality, while 3.3%\/10.7% showed a very-\/low satisfaction with cosmesis as well as 5.6%\/10.3% with functionality. The remaining women\u201424.6% and 23.8%\u2014were indifferent. Satisfaction concerning sex life was very-\/high in 9.6%\/27.7%. In 12.0%\/16.9% it was very-\/low. Furthermore, 33.7% had no opinion. Furthermore, 27.0%\/31.6% of the women reported that clitoriplasty, but not clitoridectomy, had a very-\/positive influence on their lives, while 1.3%\/8.9% felt it to be very-\/negative, and 28.4% were indifferent. Vaginoplasty had a very-\/positive influence in 25.7%\/33.8% and a very-\/negative effect in 3.6%\/6.8%. 29.7% had no opinion. Additionally, 75.7% of the women preferred feminizing surgery during infancy\/childhood, especially concerning clitoreduction. In conclusion, though the majority of the participants (76%) preferred early feminizing surgery and 60% described a positive effect on their lives, about 10% felt it to have been negative. About 15% of the women suffered from insufficient cosmesis and functionality after surgery. Sex life was even described as poor in nearly 30%. Therefore, the decision about early genital surgery in 46,XX-CAH girls should be considered carefully. Parents should get detailed information about possible complications of surgery and should receive support to understand that postponing surgery does not inevitably cause harm for their child. Importantly, genital surgery when performed in children should only be performed in expert centers with a specialized team including surgeons who are trained in feminizing surgery.<\/jats:p>","DOI":"10.3390\/jcm11154629","type":"journal-article","created":{"date-parts":[[2022,8,8]],"date-time":"2022-08-08T22:33:11Z","timestamp":1659997991000},"page":"4629","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Long-Term Results of Surgical Treatment and Patient-Reported Outcomes in Congenital Adrenal Hyperplasia\u2014A Multicenter European Registry Study"],"prefix":"10.3390","volume":"11","author":[{"given":"Susanne","family":"Krege","sequence":"first","affiliation":[{"name":"Department of Urology, Pediatric Urology and Urooncology, Kliniken Essen Mitte, 45136 Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5622-6987","authenticated-orcid":false,"given":"Henrik","family":"Falhammar","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Karolinska University Hospital, 171 77 Stockholm, Sweden"},{"name":"Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 76 Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hildegard","family":"Lax","sequence":"additional","affiliation":[{"name":"Institute of Medical Informatics, Biometry and Epidemiology, University of Essen, 45147 Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8130-6524","authenticated-orcid":false,"given":"Robert","family":"Roehle","sequence":"additional","affiliation":[{"name":"Institute of Biometry and Clinical Epidemiology, Charite-University Medicine Berlin, 10117 Berlin, Germany"},{"name":"Institute of Health, Charite-University Medicine Berlin, 10117 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0181-0403","authenticated-orcid":false,"given":"Hedi","family":"Claahsen-van der Grinten","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology, Radboud University Medical Center, 6525 GA Nijmegen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Kortmann","sequence":"additional","affiliation":[{"name":"Department of Pediatric Urology, Radboud University Medical Center, 6525 GA Nijmegen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9434-248X","authenticated-orcid":false,"given":"Lise","family":"Duranteau","sequence":"additional","affiliation":[{"name":"Department of Medical Gynaecology and Reference Centre for Rare Diseases of Genital Development, Bicetre Hospital, APHP Paris Saclay University, 94270 Le Kremlin Bicetre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agneta","family":"Nordenskj\u00f6ld","sequence":"additional","affiliation":[{"name":"Department of Women\u2019s and Children\u2019s Health, Center of Molecular Medicine, Karolinska Institutet, 17176 Stockholm, Sweden"},{"name":"Department of Pediatric Surgery, Astrid Lindgren Children\u2019s Hospital, Karolinska University Hospital, 17176 Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1210\/endrev\/bnab016","article-title":"Congenital adrenal hyperplasia\u2014Current insights in pathophysiology, diagnostics and management","volume":"43","author":"Speiser","year":"2022","journal-title":"Endocr. 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