van der Straaten, Saskia; Springer, Alexander; Zecic, Aleksandra; Hebenstreit, Doris; Tonnhofer, Ursula; Gawlik, Aneta; Baumert, Malgorzata; Szeliga, Kamila; Debulpaep, Sara; Desloovere, An; Tack, Lloyd; Smets, Koen; Wasniewska, Malgorzata; Corica, Domenico; Calafiore, Mariarosa; Ljubicic, Marie Lindhardt; Busch, Alexander Siegfried; Juul, Anders; Nordenström, Anna; Sigurdsson, Jon; ... (2020). The External Genitalia Score (EGS): A European multicenter validation study. The journal of clinical endocrinology and metabolism, 105(3) Oxford University Press 10.1210/clinem/dgz142
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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 multicentre (n=8) validation study was conducted from 07/2016 until 07/2018.
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 AGD's were determined by the interclass correlation coefficient (ICC). Cross-sectional data were obtained in 686 term (0-24 months), and 181 preterm babies and 111 babies with atypical genitalia.
RESULTS
ICC of EGS in typical and atypical genitalia is excellent and good. Median EGS (10th - 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) 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 in-between values in DSD 0.43 (0.1). 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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