Journal article

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

  • van der Straaten S Ghent University Hospital, Department of Pediatrics, Division of Pediatric Endocrinology and Ghent University, Department of Internal Medicine and Pediatrics, Ghent, Belgium.
  • Springer A Medical University of Vienna, Department of Pediatric Surgery, Vienna, Austria.
  • Zecic A Ghent University Hospital, Department of Neonatology, Ghent, Belgium.
  • Hebenstreit D Medical University Innsbruck, Department of Urology.
  • Tonnhofer U Medical University of Vienna, Department of Pediatric Surgery, Vienna, Austria.
  • Gawlik A Medical University of Silesia, Department of Pediatrics, Pediatric Endocrinology, Katowice, Poland.
  • Baumert M Medical University of Silesia, Department of Neonatology, Katowice, Poland.
  • Szeliga K Medical University of Silesia, Department of Pediatrics, Pediatric Endocrinology, Katowice, Poland.
  • Debulpaep S Ghent University Hospital, Department of Pediatrics, Ghent, Belgium.
  • Desloovere A Ghent University Hospital, Department of Pediatrics, Ghent, Belgium.
  • Tack L Ghent University Hospital, Department of Pediatrics, Division of Pediatric Endocrinology and Ghent University, Department of Internal Medicine and Pediatrics, Ghent, Belgium.
  • Smets K Ghent University Hospital, Department of Neonatology, Ghent, Belgium.
  • Wasniewska M University Hospital of Messina, Department of Human Pathology of Adulthood and Childhood, Messina, Italy.
  • Corica D University Hospital of Messina, Department of Human Pathology of Adulthood and Childhood, Messina, Italy.
  • Calafiore M University Hospital of Messina, Department of Human Pathology of Adulthood and Childhood, Messina, Italy.
  • Ljubicic ML Copenhagen University Hospital - Rigshospitalet, Department of Growth and Reproduction, Copenhagen, Denmark.
  • Busch AS Copenhagen University Hospital - Rigshospitalet, Department of Growth and Reproduction, Copenhagen, Denmark.
  • Juul A Copenhagen University Hospital - Rigshospitalet, Department of Growth and Reproduction, Copenhagen, Denmark.
  • Nordenström A Karolinska University Hospital Stockholm, Department of Women's and Children's Health Karolinska Institutet, Stockholm, Sweden.
  • Sigurdsson J Karolinska University Hospital Stockholm, Department of Women's and Children's Health Karolinska Institutet, Stockholm, Sweden.
  • Flück CE 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.
  • Haamberg T 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.
  • Graf S 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.
  • Hannema SE Erasmus MC - Sophia Children's Hospital, University Medical Center Rotterdam, Department of Pediatric Endocrinology, GD Rotterdam, the Netherlands.
  • Wolffenbuttel KP Erasmus MC - Sophia Children's Hospital, University Medical Center Rotterdam, Department of Urology and Paediatric Urology, Rotterdam, the Netherlands.
  • Hiort O University of Lübeck, Division of Paediatric Endocrinology and Diabetes, Department of Paediatrics, Lübeck, Germany.
  • Ahmed SF Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK.
  • Cools M Ghent University Hospital, Department of Pediatrics, Division of Pediatric Endocrinology and Ghent University, Department of Internal Medicine and Pediatrics, Ghent, Belgium.
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  • 2019-10-31
Published in:
  • The Journal of clinical endocrinology and metabolism. - 2020
English 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.
Language
  • English
Open access status
green
Identifiers
Persistent URL
https://sonar.ch/global/documents/187167
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