Pediatric Radiology

, Volume 43, Issue 12, pp 1615–1621 | Cite as

Validation of automatic bone age determination in children with congenital adrenal hyperplasia

  • David D. Martin
  • Katharina Heil
  • Conrad Heckmann
  • Angelika Zierl
  • Jürgen Schaefer
  • Michael B. Ranke
  • Gerhard Binder
Original Article



Determination of bone age is routinely used for following up substitution therapy in congenital adrenal hyperplasia (CAH) but today is a procedure with significant subjectivity.


The aim was to test the performance of automatic bone age rating by the BoneXpert software package in all radiographs of children with CAH seen at our clinic from 1975 to 2006.

Materials and methods

Eight hundred and ninety-two left-hand radiographs from 100 children aged 0 to 17 years were presented to a human rater and BoneXpert for bone age rating. Images where ratings differed by more than 1.5 years were each rerated by four human raters.


Rerating was necessary in 20 images and the rerating result was closer to the BoneXpert result than to the original manual rating in 18/20 (90 %). Bone age rating precision based on the smoothness of longitudinal curves comprising a total of 327 data triplets spanning less than 1.7 years showed BoneXpert to be more precise (P<0.001).


BoneXpert performs reliable bone age ratings in children with CAH.


Congenital adrenal hyperplasia Skeletal maturation Bone age rating Glucocorticoid substitution 


Conflicts of interest



  1. 1.
    White PC, Speiser PW (2000) Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr Rev 21:245–291PubMedCrossRefGoogle Scholar
  2. 2.
    Koletzko B (2007) Kinder-und Jugendmedizin, Heidelberg: Springer Medizin Verlag, HeidelbergCrossRefGoogle Scholar
  3. 3.
    Pang SY, Wallace MA, Hofman L et al (1998) Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Pediatrics 81:866–874Google Scholar
  4. 4.
    David M, Sempe M, Blanc M et al (1994) Final height in 69 patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Arch Pediatr 1:363–367PubMedGoogle Scholar
  5. 5.
    Ghali I, David M, David L (1978) Linear growth and pubertal development in treated congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Obstet Gynecol Surv 33:120–122CrossRefGoogle Scholar
  6. 6.
    Jääskeläinen J, Voutilainen R (1997) Growth of patients with 21-hydroxylase deficiency: an analysis of the factors influencing adult height. Pediatr Res 41:30–33PubMedCrossRefGoogle Scholar
  7. 7.
    Muirhead S, Sellers EAC, Guyda H (2002) Indicators of adult height outcome in classical 21-hydroxylase deficiency congenital adrenal hyperplasia. J Pediatr 141:247–252PubMedCrossRefGoogle Scholar
  8. 8.
    Van der Kamp HJ, Otten BJ, Buitenweg N et al (2002) Longitudinal analysis of growth and puberty in 21-hydroxylase deficiency patients. Arch Dis Child 87:139–144PubMedCrossRefGoogle Scholar
  9. 9.
    Bonfig W, Bechtold S, Schmidt H et al (2007) Reduced final height outcome in congenital adrenal hyperplasia under prednisone treatment: deceleration of growth velocity during puberty. J Clin Endocrinol Metabol 92:1635–1639CrossRefGoogle Scholar
  10. 10.
    Eugster EA, DiMeglio LA, Wright JC et al (2001) Height outcome in congenital adrenal hyperplasia caused by 21-hydroxylase deficiency: a meta-analysis. J Pediatr 138:26–32PubMedCrossRefGoogle Scholar
  11. 11.
    Hargitai G, Solyom J, Battelino T et al (2000) Growth patterns and final height in congenital adrenal hyperplasia due to classical 21-hydroxylase deficiency. Horm Res 55:161–171CrossRefGoogle Scholar
  12. 12.
    Lin-Su K, Vogiatzi MG, Marshall I et al (2005) Treatment with growth hormone and luteinizing hormone releasing hormone analog improves final adult height in children with congenital adrenal hyperplasia. J Clin Endocrinol Metabol 90:3318–3325CrossRefGoogle Scholar
  13. 13.
    Martin DD, Neuhof J, Jenni OG et al (2010) Automatic determination of left-and right-hand bone age in the First Zurich Longitudinal Study. Horm Res Paediatr 74:50–55PubMedCrossRefGoogle Scholar
  14. 14.
    Thodberg HH, Jenni OG, Caflisch J et al (2009) Prediction of adult height based on automated determination of bone age. J Clin Endocrinol Metab 94:4868–4874PubMedCrossRefGoogle Scholar
  15. 15.
    Thodberg HH, Neuhof J, Ranke MB et al (2010) Validation of bone age methods by their ability to predict adult height. Horm Res Paediatr 74:15–22PubMedCrossRefGoogle Scholar
  16. 16.
    van Rijn RR, Lequin MH, Thodberg HH (2009) Automatic determination of Greulich and Pyle bone age in healthy Dutch children. Pediatr Radiol 39:591–597PubMedCrossRefGoogle Scholar
  17. 17.
    Martin DD, Deusch D, Schweizer R et al (2009) Clinical application of automated Greulich-Pyle bone age determination in children with short stature. Pediatr Radiol 39:598–607PubMedCrossRefGoogle Scholar
  18. 18.
    Martin DD, Meister K, Schweizer R et al (2008) Validation of automatic bone age rating in children with precocious and early puberty. J Pediatr Endocrinol Metab. 2011;24(11-12):1009–1014Google Scholar
  19. 19.
    Thodberg HH, Kreiborg S, Juul A et al (2009) The BoneXpert method for automated determination of skeletal maturity. IEEE Trans Med Imaging 28:52–66PubMedCrossRefGoogle Scholar
  20. 20.
    Berst MJ, Dolan L, Bogdanowicz MM et al (2001) Effect of knowledge of chronologic age on the variability of pediatric bone age determined using the Greulich and Pyle standards. AJR Am J Roentgonol 176:507–510CrossRefGoogle Scholar
  21. 21.
    King DG, Steventon DM, O’Sullivan MP et al (1994) Reproducibility of bone ages when performed by radiology registrars: an audit of Tanner and Whitehouse II versus Greulich and Pyle methods. Br J Radiol 67:848–851PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • David D. Martin
    • 1
  • Katharina Heil
    • 1
  • Conrad Heckmann
    • 1
  • Angelika Zierl
    • 2
  • Jürgen Schaefer
    • 2
  • Michael B. Ranke
    • 1
  • Gerhard Binder
    • 1
  1. 1.Pediatric Endocrinology and DiabetologyUniversity Children’s HospitalTuebingenGermany
  2. 2.Department of Diagnostic RadiologyUniversity of TuebingenTuebingenGermany

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