The External Genitalia Score (EGS): A European Multicenter Validation Study

Author:

van der Straaten Saskia1ORCID,Springer Alexander2,Zecic Aleksandra3,Hebenstreit Doris4,Tonnhofer Ursula2,Gawlik Aneta5,Baumert Malgorzata6,Szeliga Kamila5,Debulpaep Sara7,Desloovere An7,Tack Lloyd1,Smets Koen3,Wasniewska Malgorzata8,Corica Domenico8,Calafiore Mariarosa8,Ljubicic Marie Lindhardt9,Busch Alexander Siegfried9,Juul Anders9ORCID,Nordenström Anna10,Sigurdsson Jon10,Flück Christa E11,Haamberg Tanja11,Graf Stefanie11,Hannema Sabine E1213,Wolffenbuttel Katja P14,Hiort Olaf15,Ahmed S Faisal16,Cools Martine1

Affiliation:

1. Ghent University Hospital, Department of Pediatrics, Division of Pediatric Endocrinology and Ghent University, Department of Internal Medicine and Pediatrics, Ghent, Belgium

2. Medical University of Vienna, Department of Pediatric Surgery, Vienna, Austria

3. Ghent University Hospital, Department of Neonatology, Ghent, Belgium

4. Medical University Innsbruck, Department of Urology

5. Medical University of Silesia, Department of Pediatrics, Pediatric Endocrinology, Katowice, Poland

6. Medical University of Silesia, Department of Neonatology, Katowice, Poland

7. Ghent University Hospital, Department of Pediatrics, Ghent, Belgium

8. University Hospital of Messina, Department of Human Pathology of Adulthood and Childhood, Messina, Italy

9. Copenhagen University Hospital - Rigshospitalet, Department of Growth and Reproduction, Copenhagen, Denmark

10. Karolinska University Hospital Stockholm, Department of Women’s and Children’s Health Karolinska Institutet, Stockholm, Sweden

11. Bern University Children’s Hospital Inselspital, Department of Pediatrics, Division of Pediatric Endocrinology, Diabetology and Metabolism and Department of BioMedical Research, University of Bern, Bern, Switzerland

12. Erasmus MC - Sophia Children’s Hospital, University Medical Center Rotterdam, Department of Pediatric Endocrinology, GD Rotterdam, the Netherlands

13. Leiden University Medical Centre, Department of Paediatrics, RC Leiden, The Netherlands

14. Erasmus MC - Sophia Children’s Hospital, University Medical Center Rotterdam, Department of Urology and Paediatric Urology, Rotterdam, the Netherlands

15. University of Lübeck, Division of Paediatric Endocrinology and Diabetes, Department of Paediatrics, Lübeck, Germany

16. Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK

Abstract

Abstract Context Standardized description of external genitalia is needed in the assessment of children with atypical genitalia. Objectives To validate the External Genitalia Score (EGS), to present reference values for preterm and term babies up to 24 months and correlate obtained scores with anogenital distances (AGDs). Design, Setting A European multicenter (n = 8) validation study was conducted from July 2016 to July 2018. Patients and Methods EGS is based on the external masculinization score but uses a gradual scale from female to male (range, 0–12) and terminology appropriate for both sexes. The reliability of EGS and AGDs was determined by the interclass correlation coefficient (ICC). Cross-sectional data were obtained in 686 term babies (0–24 months) and 181 preterm babies, and 111 babies with atypical genitalia. Results The ICC of EGS in typical and atypical genitalia is excellent and good, respectively. Median EGS (10th to 90th centile) in males < 28 weeks gestation is 10 (8.6–11.5); in males 28–32 weeks 11.5 (9.2–12); in males 33–36 weeks 11.5 (10.5–12) and in full-term males 12 (10.5–12). In all female babies, EGS is 0 (0-0). The mean (SD) lower/upper AGD ratio (AGDl/u) is 0.45 (0.1), with significant difference between AGDl/u in males 0.49 (0.1) and females 0.39 (0.1) and intermediate values in differences of sex development (DSDs) 0.43 (0.1). The AGDl/u correlates with EGS in males with typical genitalia and in atypical genitalia. Conclusions EGS is a reliable and valid tool to describe external genitalia in premature and term babies up to 24 months. EGS correlates with AGDl/u in males. It facilitates standardized assessment, clinical decision-making and multicenter research.

Funder

European Cooperation in Science and Technology

Medical University of Silesia

COPENHAGEN Minipuberty Study

Danish Environmental Protection Agency

Aase and Ejnar Danielsens Foundation

Swiss National Science Foundation

Publisher

The Endocrine Society

Subject

Biochemistry (medical),Clinical Biochemistry,Endocrinology,Biochemistry,Endocrinology, Diabetes and Metabolism

Reference29 articles.

1. Caring for individuals with a difference of sex development (DSD): a Consensus Statement;Cools;Nat Rev Endocrinol.,2018

2. Global disorders of sex development update since 2006: perceptions, approach and care;Lee;Horm Res Paediatr.,2016

3. Early assessment of ambiguous genitalia;Ogilvy-Stuart;Arch Dis Child.,2004

4. Consensus statement on management of intersex disorders;Hughes;Arch Dis Child.,2006

5. Epidemiology and initial management of ambiguous genitalia at birth in Germany;Thyen;Horm Res.,2006

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