TY - JOUR
T1 - The External Genitalia Score (EGS)
T2 - 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
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.
KW - anogenital distances
KW - atypical genitalia
KW - external genitalia score
KW - external masculinization score
UR - http://www.scopus.com/inward/record.url?scp=85080846050&partnerID=8YFLogxK
U2 - https://doi.org/10.1210/clinem/dgz142
DO - https://doi.org/10.1210/clinem/dgz142
M3 - Article
C2 - 31665438
SN - 0021-972X
VL - 105
JO - Journal of clinical endocrinology and metabolism
JF - Journal of clinical endocrinology and metabolism
IS - 3
M1 - dgz142
ER -