ESPE2019 Rapid Free Communications Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology (6 abstracts)
Contemporary Surgical Approach in CAH 46XX – Results from the I-DSD/I-CAH Registries
Doris Hebenstreit 1 , Faisal Ahmed , on behalf of the contributing centres within the I-DSD registry and I-CAH registry 2 , Alexander Springer 3 , Christoph Krall 4 , Nils Krone 5 , Niels Birkebaek 6 , Tatjana Milenkovic 7 , Birgit Koehler 8 , Christa Flueck 9 , Ruth Krone 10 , Antonio Balsamo 11 , Rodolfo Rey 12 , Carlo Acerini 13 , Alya Guven 14 , Tulay Guran 15 , Feyza Darendeliler 16 , Sabah Alvi 17 , Marta Korbonits 18 , Walter Bonfig 19 , Eduardo Correa Costa 20 , Richard Ross 21 , Violeta Iotova 22 , Daniel Konrad 23,24 , Jillian Bryce 2 , Hedi Claahsen van der Grinten 25 & Liat de Vries 26
1Department of Urology, Hanusch Krankenhaus, Vienna, Austria. 2Developmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow, Glasgow, United Kingdom. 3Department of Pediatric Surgery, Medical University Vienna, Vienna, Austria. 4Section of Medical Statistics, Medical University Vienna, Vienna, Austria. 5Department of Oncology&Metabolism, University of Sheffield, Sheffield, United Kingdom. 6Department of Pediatrics, Aarhus University Hospital, Aarhus N, Denmark. 7Department of Endocrinology, Mother and Child Health Care Institute of Serbia "Dr Vukan Cupic", Belgrade, Serbia. 8Klinik für Padiatrische Endokrinologie und Diabetologie, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany. 9Pediatric Endocrinology, Diabetology and Metabolism, Department of Pediatrics, University Hospital Inselspital, University of Bern, 3010 Bern, Switzerland; Department of BioMedical Research, University Hospital Inselspital, University of Bern, Bern, Switzerland. 10Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom. 11Department of Medical and Surgical Sciences, Pediatric Unit, S.Orsola-Malpighi University Hospital, Bologna,, Italy. 12Centro de Investigaciones Endocrinológicas "Dr. César Bergadá" (CEDIE) CONICET – FEI – División de Endocrinología, Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina. 13Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom. 14Istanbul Medeniyet University, Goztepe Education and Research Hospital, Pediatric Endocrinology Clinic, Istanbul, Turkey. 15Marmara University, Department of Pediatric Endocrinology and Diabetes, Pendik, Istanbul, Turkey. 16Istanbul Faculty of Medicine, Department of Pediatrics, Pediatric Endocrinology Unit, Istanbul University, Istanbul,, Turkey. 17Paediatric Endocrinology, Leeds Children's Hospital, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom. 18Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London, United Kingdom. 19Department of Pediatrics, Technical University München, Munich, Germany. 20Pediatric Surgery and Urology Department, Porto Alegre Clinics Hospital, Porto Alegre, Brazil. 21University of Sheffield, Sheffield, United Kingdom. 22Department of Pediatrics, Medical University-Varna, Varna, Bulgaria. 23Division of Pediatric Endocrinology and Diabetology, University Children's Hospital, Zurich, Switzerland. 24Children's Research Center, University Children's Hospital, Zurich, Switzerland. 25Department of Pediatric Endocrinology, Radoud University Medical centre, Nijmegen, Netherlands. 26The Jesse and Sara Lea Shafer Institute of Endocrinology and Diabetes, Schneider Children's Medical Center of Israel, Petah Tikva, Israel
Introduction: Congenital adrenal hyperplasia (CAH) is the most common genetic condition in the spectrum of differences of sex development (DSD). Surgery in DSD is a controversial topic and there is no consensus if to perform surgery, how to perform surgery, and when to perform surgery. The current study was designed to evaluate the current practice in CAH related surgical practice in girls.
Patients and Methods: All cases that had been classified in the I-DSD and I-CAH registries as 46XX CAH born prior to 2017 were identified (555 participants in 41 centres, born 1953-2016). Centres were approached to obtain additional information in each eligible case on factors that influenced the option of performing surgery, timing and type of surgery. 330 participants were suitable for analysis.
Results: 208 (63%) presented within the first month of life; 306 (93%) had 21-hydroxylase deficiency. 326 (99%) cases were raised as girls after birth. At latest assessment, 329 (99.6%) cases had a female sex assignment. Genital surgery has been performed in 251 (76%). Clitoral surgery been performed in 231 (92%), vaginal surgery in 204 (81%) and a combination of clitoral and vaginal surgery had been performed in 186 (74%). Of the 251 who had surgery, 18 (7%) had vaginal but no clitoral surgery whilst 42 (17%) had clitoral but no vaginal surgery. Mean age at first surgery was 2.5 years (0-15), with clitoral surgery and vaginal surgery at 2.6 years (range) and 3.2 years (range), respectively. In a logistic regression model it could be shown that there was a negative trend for surgery over time. Moreover, there was a significant trend towards surgery before 24 months of life over time. However, there were significant geographic differences in the probability/incidence of genital surgery: Europe 64%, Asia 97%, South America 89%. The Chicago Consensus Statement on DSD (comparison of data before and after 2006) did not have any significant influence on the timing or probability of surgery. Last but not least, age at first assessment showed to have a mild negative but non-significant effect on the probability of surgery.
Discussion: This is the largest international study to analyse the current surgical approach towards CAH. There are geographic and sociocultural differences. There is a trend towards avoiding/postponing surgery, especially in Europe. Moreover, there is a significant trend towards surgery before 24 months of life.
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