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Self- and proxy-reported outcomes after surgery in people with disorders/differences of sex development (DSD) in Europe (dsd-LIFE).

Authors
  • Rapp, Marion1
  • Duranteau, Lise2
  • van de Grift, Tim C3
  • Schober, Justine4
  • Hirschberg, Angelica L5
  • Krege, Susanne6
  • Nordenstrom, Anna7
  • Roehle, Robert8
  • Thyen, Ute9
  • Bouvattier, Claire10
  • Kreukels, Baudewijntje P C11
  • Nordenskjold, Agneta12
  • 1 Klinik fur Kinder- und Jugendmedizin, Universitat zu Lubeck, Ratzeburger Allee 160, Lubeck, 23538, Germany. Electronic address: [email protected] , (Germany)
  • 2 Adolescent and Young Adult Gynecology Unit, Centre de Reference des Maladies Rares du Développement Génital, Hôpital Bicêtre, AP.HP Paris Saclay University, 78 rue du General Leclerc, Le Kremlin Bicêtre, 94270, France. Electronic address: [email protected] , (France)
  • 3 Department of Plastic, Reconstructive and Hand Surgery, Amsterdam University Medical Centers, location VUmc, PO Box 7057, Amsterdam. 1007, the Netherlands; Department of Medical Psychology, Amsterdam University Medical Centers, location VUmc, PO Box 7057, Amsterdam, 1007, the Netherlands. Electronic address: [email protected] , (Netherlands)
  • 4 UPMC Hamot, Department of Urology, 201 State St. Erie, PA, 16550, USA. Electronic address: [email protected]
  • 5 Department of Women's and Children's Health, Karolinska Institutet, Department of Gynaecology and Reproductive Medicine, Karolinska University Hospital, Stockholm, 171 76, Sweden. Electronic address: [email protected] , (Sweden)
  • 6 Kliniken Essen-Mitte, Klinik fur Urologie, Kinderurologie und urologische Onkologie, Evangelische Huyssens Stiftung/Knappschaft GmbH, Essen, 45136, Germany. Electronic address: [email protected] , (Germany)
  • 7 Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, 171 76, Sweden. Electronic address: [email protected] , (Sweden)
  • 8 Charité - Universitätsmedizin Berlin, Coordinating Center for Clinical Studies, Berlin, 13353, Germany. Electronic address: [email protected] , (Germany)
  • 9 Klinik fur Kinder- und Jugendmedizin, Universitat zu Lubeck, Ratzeburger Allee 160, Lubeck, 23538, Germany. Electronic address: [email protected] , (Germany)
  • 10 Endocrinologie pediatrique, Centre de Reference des Maladies Rares du Développement Génital, Hôpitaux Universitaires Paris Saclay (Bicêtre), 78 rue du General Leclerc, Le Kremlin Bicetre, 94270, France. Electronic address: [email protected] , (France)
  • 11 Department of Medical Psychology, Amsterdam University Medical Centers, location VUmc, PO Box 7057, Amsterdam, 1007, the Netherlands. Electronic address: [email protected] , (Netherlands)
  • 12 Department of Women's and Children's Health and Centre for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden; Pediatric Surgery, Astrid Lindgren Children Hospital, Karolinska University Hospital, Stockholm, Sweden. Electronic address: [email protected] , (Sweden)
Type
Published Article
Journal
Journal of pediatric urology
Publication Date
Jun 01, 2021
Volume
17
Issue
3
Pages
353–365
Identifiers
DOI: 10.1016/j.jpurol.2020.12.007
PMID: 33358555
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Surgery is performed in many individuals with disorders/differences of sex development (DSD). Irreversibility of some surgical procedures, lack of information about the procedures, and lack of follow-up care for physical and psychological outcomes, lead to wish for more knowledge from both surgeons and patients. After the consensus conference in 2006, multidisciplinary care is provided to a higher degree with psychological support and more restricted surgical procedures. Outcome studies after genital surgery often lack of patient's perspective. To describe surgical procedures in relation to diagnosis, to evaluate the outcomes of surgery through genital examination, and through patient's and observer's satisfaction with the anatomical and functional result after genital surgery. In a cross-sectional clinical study performed in six European countries in 2014/15, we have included 500 participants where surgery was performed, from a total of 1040 adolescents (≥16years) and adults with a DSD. Diagnoses included Turner syndrome (n = 301), mixed gonadal dysgenesis (45,XO/46,XY; n = 45), Klinefelter syndrome (n = 218), XYY (n = 1), 46, XY DSD (n = 222) and 46, XX DSD (n = 253). Study protocol included clinical report files, an optional gynecological or urological examination, patient reported outcomes including received surgical interventions, satisfaction with appearance and function after surgery, and impact of the surgical procedure on life. Five hundred participants had received genital or breast surgery, with the highest rate in 46, XY DSD and the lowest in Turner syndrome. Altogether; 240 participants had feminizing surgery, 112 had masculinizing surgery, and 217 underwent gonadectomy. Physicians evaluated anatomical appearance at genital examination as poor in less than 10%. Dissatisfaction with anatomical appearance was reported by 22% of the participants, dissatisfaction with function by 20%. Being (very) dissatisfied with anatomical appearance and function was reported by 13% of the study participants. Most participants reported no impact, or positive impact, of the surgical procedures on their lives, but 29% experienced a negative effect of gonadectomy on their life. There might be a selection bias and/or a recall bias for participating in our studies. Due to poor data quality about surgical procedures performed in the past, we also relied on participants memory about surgical procedures in their past. Ideally, patient reported outcomes should be evaluated both before and after surgical procedures. A vast majority are satisfied with appearance and function, but still genital or breast surgery have a long-lasting effect on patient's life. Self-reported satisfaction is usually lower than the observer's evaluation regarding both appearance and function. Copyright © 2020 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

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