Skip to main content

The Elbow and Forearm: Congenital and Developmental Conditions

  • Chapter
  • First Online:
  • 3833 Accesses

Abstract

This chapter presents important normal anatomy and variants, including the supracondylar process and elbow fat pads. It then discusses a wide variety of congenital and developmental anomalies that affect the elbow, radius, and ulna. Several classification schemes of congenital developmental anomalies are presented. The more common entities, such as radial longitudinal deficiency, ulna dysplasia, elbow synostosis, and radial head dislocation, are presented. The chapter also discusses transverse deficiency, phocomelia, ulnar longitudinal deficiency, and Poland deformity. The associated syndromes are briefly discussed. Lastly, Madelung deformity is presented.

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Freyschmidt J, Brossmann J, Wiens J, Sternberg A. Koehler/Zimmer’s borderlands of normal and early pathological findings in skeletal radiography. 5th ed. Stuttgart: Thieme; 2003.

    Google Scholar 

  2. Keats TL, Anderson MW. Atlas of normal roentgen variants that may simulate disease. 8th ed. Philadelphia: Mosby; 2006.

    Google Scholar 

  3. Swanson AB. Congenital limb defects classification and treatment. Clin Symp. 1981;33(3):1–32. PubMed PMID: 7344809, Epub 1981/01/01. eng.

    CAS  PubMed  Google Scholar 

  4. Oberg KC, Feenstra JM, Manske PR, Tonkin MA. Developmental biology and classification of congenital anomalies of the hand and upper extremity. J Hand Surg Am. 2010;35(12):2066–76. PubMed PMID: 21134615, Epub 2010/12/08. eng.

    Article  PubMed  Google Scholar 

  5. Scotland TR, Galway HR. A long-term review of children with congenital and acquired upper limb deficiency. J Bone Joint Surg Br. 1983;65(3):346–9. PubMed PMID: 6841409, Epub 1983/05/01. eng.

    CAS  PubMed  Google Scholar 

  6. Kelikian H. Congenital deformities of the hand and forearm. Philadelphia: WB Saunders; 1974.

    Google Scholar 

  7. Poznanski A. The hand in radiologic diagnosis. In: EJ P, editor. Philadelphia: WB Saunders and Company; 1984.

    Google Scholar 

  8. Goldfarb CA, Manske PR, Busa R, Mills J, Carter P, Ezaki M. Upper-extremity phocomelia reexamined: a longitudinal dysplasia. J Bone Joint Surg Am. 2005;87(12):2639–48. PubMed PMID: 16322613, Epub 2005/12/03. eng.

    Article  PubMed  Google Scholar 

  9. Goldfarb CA, Wall L, Manske PR. Radial longitudinal deficiency: the incidence of associated medical and musculoskeletal conditions. J Hand Surg Am. 2006;31(7):1176–82. PubMed PMID: 16945723, Epub 2006/09/02. eng.

    Article  PubMed  Google Scholar 

  10. Quan L, Smith DW. The VATER association. Vertebral defects, anal atresia, T-E fistula with esophageal atresia, radial and renal dysplasia: a spectrum of associated defects. J Pediatr. 1973;82(1):104–7. PubMed PMID: 4681850. Epub 1973/01/01. eng.

    Article  CAS  PubMed  Google Scholar 

  11. Bednar MS, James MA, Light TR. Congenital longitudinal deficiency. J Hand Surg Am. 2009;34(9):1739– 47. PubMed PMID: 19896016, Epub 2009/11/10. eng.

    Article  PubMed  Google Scholar 

  12. Maschke SD, Seitz W, Lawton J. Radial longitudinal deficiency. J Am Acad Orthop Surg. 2007;15(1):41– 52. PubMed PMID: 17213381, Epub 2007/01/11. eng.

    PubMed  Google Scholar 

  13. James MA, McCarroll Jr HR, Manske PR. The spectrum of radial longitudinal deficiency: a modified classification. J Hand Surg Am. 1999;24(6):1145–55. PubMed PMID: 10584934, Epub 1999/12/10. eng.

    Article  CAS  PubMed  Google Scholar 

  14. Schmidt CC, Neufeld SK. Ulnar ray deficiency. Hand Clin. 1998;14(1):65–76. PubMed PMID: 9526157, Epub 1998/04/04. eng.

    CAS  PubMed  Google Scholar 

  15. Ogden JA, Watson HK, Bohne W. Ulnar dysmelia. J Bone Joint Surg Am. 1976;58(4):467–75. PubMed PMID: 818089, Epub 1976/06/01. eng.

    CAS  PubMed  Google Scholar 

  16. Azouz EM, Oudjhane K. Disorders of the upper extremity in children. Magn Reson Imaging Clin N Am. 1998;6(3):677–95. PubMed PMID: 9654591, Epub 1998/07/09. eng.

    CAS  PubMed  Google Scholar 

  17. Makhoul IR, Goldstein I, Smolkin T, Avrahami R, Sujov P. Congenital limb deficiencies in newborn infants: prevalence, characteristics and prenatal diagnosis. Prenat Diagn. 2003;23(3):198–200. PubMed PMID: 12627419, Epub 2003/03/11. eng.

    Article  CAS  PubMed  Google Scholar 

  18. McIntyre JD, Brooks A, Benson MK. Humeroradial synostosis and the multiple synostosis syndrome: case report. J Pediatr Orthop B. 2003;12(3):192–6. PubMed PMID: 12703033, Epub 2003/04/19. eng.

    PubMed  Google Scholar 

  19. Sachar K, Akelman E, Ehrlich MG. Radioulnar synostosis. Hand Clin. 1994;10(3):399–404. PubMed PMID: 7962146, Epub 1994/08/01. eng.

    CAS  PubMed  Google Scholar 

  20. Yammine K, Salon A, Pouliquen JC. Congenital radioulnar synostosis. Study of a series of 37 children and adolescents. Chir Main. 1998;17(4):300–8.

    CAS  PubMed  Google Scholar 

  21. Shinohara T, Horii E, Tatebe M, Yamamoto M, Okui N, Hirata H. Painful snapping elbow in patients with congenital radioulnar synostosis: report of two cases. J Hand Surg Am. 2010;35(8):1336–9. PubMed PMID: 20684930, Epub 2010/08/06. eng.

    Article  PubMed  Google Scholar 

  22. Wang E, Wenger DR, Zhang L, Zhao Q, Ji S, Li J. The mechanism of acute elbow flexion contracture in children with congenital proximal radioulnar synostosis. J Pediatr Orthop. 2010;30(3):277–81. PubMed PMID: 20357595. Epub 2010/04/02. eng.

    Article  PubMed  Google Scholar 

  23. Elliott AM, Kibria L, Reed MH. The developmental spectrum of proximal radioulnar synostosis. Skelet Radiol. 2010;39(1):49–54. PubMed PMID: 19669136, Epub 2009/08/12. eng.

    Article  Google Scholar 

  24. Cleary JE, Omer Jr GE. Congenital proximal radio-ulnar synostosis. Natural history and functional assessment. J Bone Joint Surg. 1985;67(4):539–45. PubMed PMID: 3980498, Epub 1985/04/01. eng.

    CAS  PubMed  Google Scholar 

  25. Lachman RS. Taybi and Lachman’s radiology of syndromes, metabolic disorders and skeletal dysplasias. 5th ed. Philadelphia: Mosby; 2007.

    Google Scholar 

  26. Sachar K, Mih AD. Congenital radial head dislocations. Hand Clin. 1998;14(1):39–47. PubMed PMID: 9526155, Epub 1998/04/04. eng.

    CAS  PubMed  Google Scholar 

  27. Cockshott WP, Omololu A. Familial congenital posterior dislocation of both radial heads. J Bone Joint Surg Br. 1958;40(3):483–6. PubMed PMID: 13575461, Epub 1958/08/01. eng.

    PubMed  Google Scholar 

  28. Mardam-Bey T, Ger E. Congenital radial head dislocation. J Hand Surg Am. 1979;4(4):316–20. PubMed PMID: 469206, Epub 1979/07/01. eng.

    Article  CAS  PubMed  Google Scholar 

  29. Miura T. Congenital dislocation of the radial head. J Hand Surg Br. 1990;15(4):477–81. PubMed PMID: 2269842, Epub 1990/11/01. eng.

    Article  CAS  PubMed  Google Scholar 

  30. Wiley JJ, Loehr J, McIntyre W. Isolated dislocation of the radial head. Orthop Rev. 1991;20(11):973–6. PubMed PMID: 1749662, Epub 1991/11/01. eng.

    CAS  PubMed  Google Scholar 

  31. Kim HT, Park BG, Suh JT, Yoo CI. Chronic radial head dislocation in children, part 2: results of open treatment and factors affecting final outcome. J Pediatr Orthop. 2002;22(5):591–7. PubMed PMID: 12198459. Epub 2002/08/29. eng.

    PubMed  Google Scholar 

  32. Song KS, Ramnani K, Cho CH. Long term follow-up of open realignment procedure for congenital dislocation of the radial head. J Hand Surg Eur. 2011;36(2):161–2. PubMed PMID: 21285239, Epub 2011/02/03. eng.

    Article  Google Scholar 

  33. Anderson BL, Lomasney LM, Demos TC, Bielski RJ. Radiologic case study. Madelung’s deformity of the wrist. Orthopedics. 1997;20(6):576, 69-71. PubMed PMID: 9195641. Epub 1997/06/01. eng.

    CAS  PubMed  Google Scholar 

  34. Schmidt-Rohlfing B, Schwobel B, Pauschert R, Niethard FU. Madelung deformity: clinical features, therapy and results. J Pediatr Orthop B. 2001;10(4):344–8. PubMed PMID: 11727381, Epub 2001/12/01. eng.

    CAS  PubMed  Google Scholar 

  35. Stehling C, Langer M, Nassenstein I, Bachmann R, Heindel W, Vieth V. High resolution 3.0 Tesla MR imaging findings in patients with bilateral Madelung’s deformity. Surg Radiol Anat. 2009;31(7):551–7.

    Article  PubMed  Google Scholar 

  36. Vickers D, Nielsen G. Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion. J Hand Surg Br. 1992;17(4):401–7. PubMed PMID: 1402266, Epub 1992/08/01. eng.

    Article  CAS  PubMed  Google Scholar 

  37. Aucourt J, Budzik JF, Manouvrier-Hanu S, Mezel A, Cotten A, Boutry N. Congenital malformations of the hand and forearm in children: what radiologists should know. Semin Musculoskelet Radiol. 2012;16(2):146–58. PubMed PMID: 22648430, Epub 2012/06/01. eng.

    Article  PubMed  Google Scholar 

  38. Langer Jr LO. Mesomelic dwarfism of the hypoplastic ulna, fibula, mandible type. Radiology. 1967;89(4):654–60. PubMed PMID: 6059604, Epub 1967/10/01. eng.

    Article  PubMed  Google Scholar 

  39. de Billy B, Gastaud F, Repetto M, Chataigner H, Clavert JM, Aubert D. Treatment of Madelung’s deformity by lengthening and relaxation of the distal extremity of the radius by Ilizarov’s technique. Eur J Pediatr Surg. 1997;7(5):296–8. PubMed PMID: 9402490, Epub 1997/12/24. eng.

    Article  PubMed  Google Scholar 

  40. Zebala LP, Manske PR, Goldfarb CA. Madelung’s deformity: a spectrum of presentation. J Hand Surg Am. 2007;32(9):1393–401. PubMed PMID: 17996774, Epub 2007/11/13. eng.

    Article  PubMed  Google Scholar 

  41. Felman AH, Kirkpatrick Jr JA. Madelung’s deformity: observations in 17 patients. Radiology. 1969;93(5):1037–42. PubMed PMID: 5350668, Epub 1969/11/01. eng.

    Article  CAS  PubMed  Google Scholar 

  42. McCarroll Jr HR, James MA, Newmeyer 3rd WL, Manske PR. Madelung’s deformity: diagnostic thresholds of radiographic measurements. J Hand Surg Am. 2010;35(5):807–12. PubMed PMID: 20347533, Epub 2010/03/30. eng.

    Article  PubMed  Google Scholar 

  43. Cook PA, Yu JS, Wiand W, Lubbers L, Coleman CR, Cook 2nd AJ, et al. Madelung deformity in skeletally immature patients: morphologic assessment using radiography, CT, and MRI. J Comput Assist Tomogr. 1996;20(4):505–11. PubMed PMID: 8708046. Epub 1996/07/01. eng.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Rebecca Stein-Wexler MD .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Stein-Wexler, R. (2015). The Elbow and Forearm: Congenital and Developmental Conditions. In: Stein-Wexler, R., Wootton-Gorges, S., Ozonoff, M. (eds) Pediatric Orthopedic Imaging. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-45381-6_7

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-45381-6_7

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-45380-9

  • Online ISBN: 978-3-642-45381-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics