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

Saskia Van Der Straaten; Alexander Springer; Aleksandra Zecic; Doris Hebenstreit; Ursula Tonnhofer; Aneta Gawlik; Malgorzata Baumert; Kamila Szeliga; Sara Debulpaep; An Desloovere; Lloyd Tack; Koen Smets; Malgorzata Wasniewska; Domenico Corica; Mariarosa Calafiore; Marie Lindhardt Ljubicic; Alexander Siegfried Busch; Anders Juul; Anna Nordenström; Jon Sigurdsson; Christa E. Flück; Tanja Haamberg; Stefanie Graf; Sabine E. Hannema; Katja P. Wolffenbuttel; Olaf Hiort; S. Faisal Ahmed; Martine Cools

doi:10.1210/clinem/dgz142

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

Saskia Van Der Straaten, Alexander Springer, Aleksandra Zecic, Doris Hebenstreit, Ursula Tonnhofer, Aneta Gawlik, Malgorzata Baumert, Kamila Szeliga, Sara Debulpaep, An Desloovere, Lloyd Tack, Koen Smets, Malgorzata Wasniewska, Domenico Corica, Mariarosa Calafiore, Marie Lindhardt Ljubicic, Alexander Siegfried Busch, Anders Juul, Anna Nordenström, Jon SigurdssonChrista E. Flück, Tanja Haamberg, Stefanie Graf, Sabine E. Hannema, Katja P. Wolffenbuttel, Olaf Hiort, S. Faisal Ahmed, Martine Cools^*

^*Corresponding author for this work

Department of Pediatric and Adolescent Medicine

95 Citations (Scopus)

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.

Original language	English
Article number	dgz142
Journal	Journal of Clinical Endocrinology and Metabolism
Volume	105
Issue number	3
ISSN	0021-972X
DOIs	https://doi.org/10.1210/clinem/dgz142
Publication status	Published - 08.01.2020

Funding

Financial Support: This article is based upon work from COST Action DSDnet, supported by COST (European Cooperation in Science and Technology), grant BM1303. M.C. holds a senior clinical investigator grant from the Research Foundation Flanders, A.G., K.S., and M.B. are supported by statutory work of Medical University of Silesia (number KNW-1–011/N/8/K). M.L.L, A.S.B. and A.J. are supported by COPENHAGEN Minipuberty Study (R146-A5644) and The Danish Environmental Protection Agency and Aase and Ejnar Danielsens Foundation (10-00101874). C.E.F. is supported by the Swiss National Science Foundation (grant number 320030-146127).

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being
SDG 5 Gender Equality
SDG 10 Reduced Inequalities

Research Areas and Centers

Academic Focus: Center for Brain, Behavior and Metabolism (CBBM)

DFG Research Classification Scheme

2.22-17 Endocrinology, Diabetology, Metabolism
2.22-20 Pediatric and Adolescent Medicine

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10.1210/clinem/dgz142

Cite this

Van Der Straaten, S., Springer, A., Zecic, A., Hebenstreit, D., Tonnhofer, U., Gawlik, A., Baumert, M., Szeliga, K., Debulpaep, S., Desloovere, A., Tack, L., Smets, K., Wasniewska, M., Corica, D., Calafiore, M., Ljubicic, M. L., Busch, A. S., Juul, A., Nordenström, A., ... Cools, M. (2020). The External Genitalia Score (EGS): A European Multicenter Validation Study. Journal of Clinical Endocrinology and Metabolism, 105(3), Article dgz142. https://doi.org/10.1210/clinem/dgz142

@article{95fabbd71ac04024b939e2a77e234a6e,

title = "The External Genitalia Score (EGS): A European Multicenter Validation Study",

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.",

author = "\{Van Der Straaten\}, Saskia and Alexander Springer and Aleksandra Zecic and Doris Hebenstreit and Ursula Tonnhofer and Aneta Gawlik and Malgorzata Baumert and Kamila Szeliga and Sara Debulpaep and An Desloovere and Lloyd Tack and Koen Smets and Malgorzata Wasniewska and Domenico Corica and Mariarosa Calafiore and Ljubicic, \{Marie Lindhardt\} and Busch, \{Alexander Siegfried\} and Anders Juul and Anna Nordenstr{\"o}m and Jon Sigurdsson and Fl{\"u}ck, \{Christa E.\} and Tanja Haamberg and Stefanie Graf and Hannema, \{Sabine E.\} and Wolffenbuttel, \{Katja P.\} and Olaf Hiort and Ahmed, \{S. Faisal\} and Martine Cools",

note = "Funding Information: Financial Support: This article is based upon work from COST Action DSDnet, supported by COST (European Cooperation in Science and Technology), grant BM1303. M.C. holds a senior clinical investigator grant from the Research Foundation Flanders, A.G., K.S., and M.B. are supported by statutory work of Medical University of Silesia (number KNW-1–011/N/8/K). M.L.L, A.S.B. and A.J. are supported by COPENHAGEN Minipuberty Study (R146-A5644) and The Danish Environmental Protection Agency and Aase and Ejnar Danielsens Foundation (10-00101874). C.E.F. is supported by the Swiss National Science Foundation (grant number 320030-146127). Publisher Copyright: {\textcopyright} 2019 Endocrine Society 2019. Copyright: Copyright 2020 Elsevier B.V., All rights reserved.",

year = "2020",

month = jan,

day = "8",

doi = "10.1210/clinem/dgz142",

language = "English",

volume = "105",

journal = "Journal of Clinical Endocrinology and Metabolism",

issn = "0021-972X",

publisher = "Endocrine Society",

number = "3",

}

Van Der Straaten, S, Springer, A, Zecic, A, Hebenstreit, D, Tonnhofer, U, Gawlik, A, Baumert, M, Szeliga, K, Debulpaep, S, Desloovere, A, Tack, L, Smets, K, Wasniewska, M, Corica, D, Calafiore, M, Ljubicic, ML, Busch, AS, Juul, A, Nordenström, A, Sigurdsson, J, Flück, CE, Haamberg, T, Graf, S, Hannema, SE, Wolffenbuttel, KP, Hiort, O, Ahmed, SF & Cools, M 2020, 'The External Genitalia Score (EGS): A European Multicenter Validation Study', Journal of Clinical Endocrinology and Metabolism, vol. 105, no. 3, dgz142. https://doi.org/10.1210/clinem/dgz142

TY - JOUR

T1 - The External Genitalia Score (EGS): A European Multicenter Validation Study

AU - Van Der Straaten, Saskia

AU - Springer, Alexander

AU - Zecic, Aleksandra

AU - Hebenstreit, Doris

AU - Tonnhofer, Ursula

AU - Gawlik, Aneta

AU - Baumert, Malgorzata

AU - Szeliga, Kamila

AU - Debulpaep, Sara

AU - Desloovere, An

AU - Tack, Lloyd

AU - Smets, Koen

AU - Wasniewska, Malgorzata

AU - Corica, Domenico

AU - Calafiore, Mariarosa

AU - Ljubicic, Marie Lindhardt

AU - Busch, Alexander Siegfried

AU - Juul, Anders

AU - Nordenström, Anna

AU - Sigurdsson, Jon

AU - Flück, Christa E.

AU - Haamberg, Tanja

AU - Graf, Stefanie

AU - Hannema, Sabine E.

AU - Wolffenbuttel, Katja P.

AU - Hiort, Olaf

AU - Ahmed, S. Faisal

AU - Cools, Martine

N1 - Funding Information: Financial Support: This article is based upon work from COST Action DSDnet, supported by COST (European Cooperation in Science and Technology), grant BM1303. M.C. holds a senior clinical investigator grant from the Research Foundation Flanders, A.G., K.S., and M.B. are supported by statutory work of Medical University of Silesia (number KNW-1–011/N/8/K). M.L.L, A.S.B. and A.J. are supported by COPENHAGEN Minipuberty Study (R146-A5644) and The Danish Environmental Protection Agency and Aase and Ejnar Danielsens Foundation (10-00101874). C.E.F. is supported by the Swiss National Science Foundation (grant number 320030-146127). Publisher Copyright: © 2019 Endocrine Society 2019. Copyright: Copyright 2020 Elsevier B.V., All rights reserved.

PY - 2020/1/8

Y1 - 2020/1/8

N2 - 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.

AB - 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.

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UR - https://www.mendeley.com/catalogue/d8bb952c-1eda-385b-854e-482a6500b8c0/

U2 - 10.1210/clinem/dgz142

DO - 10.1210/clinem/dgz142

M3 - Journal articles

C2 - 31665438

AN - SCOPUS:85080846050

SN - 0021-972X

VL - 105

JO - Journal of Clinical Endocrinology and Metabolism

JF - Journal of Clinical Endocrinology and Metabolism

IS - 3

M1 - dgz142

ER -

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

Abstract

Funding

UN SDGs

Research Areas and Centers

DFG Research Classification Scheme

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