175 / 330
175
19–22 APRIL, 2017, BARCELONA, SPAIN
13:52–13:55
S16-4 (PP)
PROXY- AND SELF-REPORTED OUTCOME AFTER
SURGERY IN DSD PATIENTS
Marion RAPP
1
, Lise DURANTEAU
2
, Robert RÖHLE
3
, Tim VAN DE GRIFT
4
, Angelica
Linden HIRSCHBERG
5
, Susanne KREGE
6
, Justine SCHOBER
7
, Claire BOUVATTIER
2
,
Peggy COHEN-KETTENIS
4
, Birgit KÖHLER
3
, Anna NORDENSTRÖM
8
, Ute THYEN
9
and Agneta NORDENSKJÖLD
8
1) University of Lübeck, Department of Pediatrics, Lübeck, GERMANY - 2) HUPS (Bicêtre), Le Kremlin-Bicêtre,
FRANCE - 3) Charité, Berlin, GERMANY - 4) VU Medical Center, Department of Medical Psychology, Hv Amsterdam,
NETHERLANDS - 5) Karolinska Universitetssjukhuset, Department for Gynaecology and Obstetrics, Solna, SWEDEN
- 6) Klinikum Essen Mitte, Department of Urology, Essen, GERMANY - 7) UPMC Hamot, Department of Urology, Erie,
USA - 8) Karolinska Universitetssjukhuset, Solna, SWEDEN - 9) University of Lübeck, Department of Pedatrics, Lübeck,
GERMANY
PURPOSE
Outcome studies of genital surgery in disorders of sex development (DSD) often lack the
patient’s perspective. We describe observer’s and patient’s satisfaction with the anatomical and
functional result after genital surgery in a large European cohort.
MATERIAL AND METHODS
1040 adolescents (>15 yrs.) and adults with DSD took part in a cross-sectional multicentre clinical
evaluation study in six European countries in 2014/15. Diagnoses were Turner syndrome (n=301),
45,XO/46,XY (n=45), Klinefelter syndrome (n=218), XYY (n=1), 46,XY DSD (n =219) and 46,XX
DSD (n=256). Study protocol included a clinical report file, an optional gynaecological/urological
examination and a patient reported outcome including received surgical interventions, and opinions
about the anatomical and functional outcome.
RESULTS
500 of 1040 participants were subjected to genital surgery with the highest rate in 46,XY DSD
and the lowest in Turner syndrome. 303 participants got a feminising and 125 a masculinising
surgery and overall 217 underwent gonadectomy. Of those who answered the patient reported
outcome 13 % were (very) dissatisfied with anatomical appearance and function. Participant’s view
correlated positively with the examiner’s view in case of anatomical appearance. Regarding specific
interventions clitoridectomy had a very negative effect on participant’s life and one third experienced
a negative one of gonadectomy.
CONCLUSIONS
Irreversible removal of organs was regarded most negatively. Physicians should be aware of its
possible long-lasting negative effect on patient’s life. Patient reported outcome should be evaluated
whenever possible to gain as much understanding of the influence of genital surgery in DSD on
future life as possible.