Skip to main content
SpringerLink
Log in

Hip Dysplasia Is More Severe in Charcot-Marie-Tooth Disease Than in Developmental Dysplasia of the Hip

  • Symposium: 2013 Hip Society Proceedings
  • Published:
Clinical Orthopaedics and Related Research®

Abstract

Background

Patients with Charcot-Marie-Tooth disease may develop hip dysplasia. Hip geometry in these patients has not been well described in the literature.

Questions/purposes

We compared the hip morphometry in Charcot-Marie-Tooth hip dysplasia (CMTHD) and developmental dysplasia of the hip (DDH) in terms of extent of (1) acetabular dysplasia and subluxation, (2) acetabular anteversion and osseous support, (3) coxa valga and femoral version, and (4) osteoarthritis.

Methods

Fourteen patients with CMTHD (19 hips; mean age, 23 years) presenting for periacetabular osteotomy were matched to 45 patients with DDH (45 hips; mean age, 21 years) based on age, sex, and BMI. We assessed acetabular dysplasia and subluxation using lateral center-edge angle (LCEA), anterior center-edge angle (ACEA), and acetabular roof angle of Tönnis (TA) on plain pelvic radiographs and acetabular volume, area of femoral head covered by acetabulum, and percentage of femoral head covered by acetabulum on three-dimensional CT reconstruction models. Acetabular version and bony support, femoral version, and neck-shaft angle were measured on two-dimensional axial CT scans. Hip osteoarthritis was graded radiographically according to Tönnis criteria.

Results

Acetabular dysplasia was more severe in CMTHD, as measured by smaller LCEA (p < 0.001), ACEA (p < 0.001), and acetabular volume (p = 0.0178) and larger TA (p = 0.025). Hip subluxation was more pronounced in CMTHD, as demonstrated by lower area of femoral head covered by acetabulum (p = 0.034) and percentage of femoral head covered by acetabulum (p = 0.007). CMTHD was associated with higher acetabular anteversion (p < 0.001), lower anterior (p < 0.001) and posterior (p = 0.072) osseous support, and more severe coxa valga (p < 0.001). More (p = 0.006) arthritic hips were found in CMTHD.

Conclusions

The extent of acetabular dysplasia, hip subluxation, acetabular anteversion, coxa valga, and hip osteoarthritis was more severe in CMTHD. These findings are important in choosing the appropriate surgical strategy for patients affected by CMTHD.

Level of Evidence

Level IV, diagnostic study. See Instructions for Authors for a complete description of levels of evidence.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1A–B
Fig. 2
Fig. 3A–B
Fig. 4A–C

Similar content being viewed by others

References

  1. Albers CE, Steppacher SD, Ganz R, Tannast M, Siebenrock KA. Impingement adversely affects 10-year survivorship after periacetabular osteotomy for DDH. Clin Orthop Relat Res. 2013;471:1602–1614.

    Article  PubMed  Google Scholar 

  2. Anda S, Svenningsen S, Dale LG, Benum P. The acetabular sector angle of the adult hip determined by computed tomography. Acta Radiol Diagn (Stockh). 1986;27:443–447.

    CAS  PubMed  Google Scholar 

  3. Anda S, Terjesen T, Kvistad KA. Computed tomography measurements of the acetabulum in adult dysplastic hips: which level is appropriate? Skeletal Radiol. 1991;20:267–271.

    Article  CAS  PubMed  Google Scholar 

  4. Anda S, Terjesen T, Kvistad KA, Svenningsen S. Acetabular angles and femoral anteversion in dysplastic hips in adults: CT investigation. J Comput Assist Tomogr. 1991;15:115–120.

    Article  CAS  PubMed  Google Scholar 

  5. Bamford NS, White KK, Robinett SA, Otto RK, Gospe SM Jr. Neuromuscular hip dysplasia in Charcot-Marie-Tooth disease type 1A. Dev Med Child Neurol. 2009;51:408–411.

    Article  PubMed  Google Scholar 

  6. Buller LT, Rosneck J, Monaco FM, Butler R, Smith T, Barsoum WK. Relationship between proximal femoral and acetabular alignment in normal hip joints using 3-dimensional computed tomography. Am J Sports Med. 2012;40:367–375.

    Article  PubMed  Google Scholar 

  7. Chan G, Bowen JR, Kumar SJ. Evaluation and treatment of hip dysplasia in Charcot-Marie-Tooth disease. Orthop Clin North Am. 2006;37:203–209, vii.

    Google Scholar 

  8. Clohisy JC, Barrett SE, Gordon JE, Delgado ED, Schoenecker PL. Periacetabular osteotomy for the treatment of severe acetabular dysplasia. J Bone Joint Surg Am. 2005;87:254–259.

    Article  PubMed  Google Scholar 

  9. Clohisy JC, Schutz AL, St John L, Schoenecker PL, Wright RW. Periacetabular osteotomy: a systematic literature review. Clin Orthop Relat Res. 2009;467:2041–2052.

    Article  PubMed  Google Scholar 

  10. Dandachli W, Kannan V, Richards R, Shah Z, Hall-Craggs M, Witt J. Analysis of cover of the femoral head in normal and dysplastic hips: new CT-based technique. J Bone Joint Surg Br. 2008;90:1428–1434.

    Article  CAS  PubMed  Google Scholar 

  11. Fujii M, Nakashima Y, Yamamoto T, Mawatari T, Motomura G, Matsushita A, Matsuda S, Jingushi S, Iwamoto Y. Acetabular retroversion in developmental dysplasia of the hip. J Bone Joint Surg Am. 2010;92:895–903.

    Article  PubMed  Google Scholar 

  12. Ito H, Matsuno T, Hirayama T, Tanino H, Yamanaka Y, Minami A. Three-dimensional computed tomography analysis of non-osteoarthritic adult acetabular dysplasia. Skeletal Radiol. 2009;38:131–139.

    Article  PubMed  Google Scholar 

  13. Kim WY, Hutchinson CE, Andrew JG, Allen PD. The relationship between acetabular retroversion and osteoarthritis of the hip. J Bone Joint Surg Br. 2006;88:727–729.

    Article  CAS  PubMed  Google Scholar 

  14. Kohnlein W, Ganz R, Impellizzeri FM, Leunig M. Acetabular morphology: implications for joint-preserving surgery. Clin Orthop Relat Res. 2009;467:682–691.

    Article  PubMed  Google Scholar 

  15. Krajewski KM, Lewis RA, Fuerst DR, Turansky C, Hinderer SR, Garbern J, Kamholz J, Shy ME. Neurological dysfunction and axonal degeneration in Charcot-Marie-Tooth disease type 1A. Brain. 2000;123(pt 7):1516–1527.

    Article  PubMed  Google Scholar 

  16. Kumar SJ, Marks HG, Bowen JR, MacEwen GD. Hip dysplasia associated with Charcot-Marie-Tooth disease in the older child and adolescent. J Pediatr Orthop. 1985;5:511–514.

    Article  CAS  PubMed  Google Scholar 

  17. Kuruvilla A, Costa JL, Wright RB, Yoder DM, Andriacchi TP. Characterization of gait parameters in patients with Charcot-Marie-Tooth disease. Neurol India. 2000;48:49–55.

    CAS  PubMed  Google Scholar 

  18. Lequesne M, de Sèze S. [False profile of the pelvis: a new radiographic incidence for the study of the hip: its use in dysplasias and different coxopathies] [in French]. Rev Rhum Mal Osteoartic. 1961;28:643–652.

    CAS  PubMed  Google Scholar 

  19. Lorensen WE, Cline HE. Marching cubes: a high resolution 3D surface construction algorithm. ACM SIGGRAPH Computer Graphics. 1987;21:163–169. 45

  20. Mandel DM, Loder RT, Hensinger RN. The predictive value of computed tomography in the treatment of developmental dysplasia of the hip. J Pediatr Orthop. 1998;18:794–798.

    CAS  PubMed  Google Scholar 

  21. Matheney T, Kim YJ, Zurakowski D, Matero C, Millis M. Intermediate to long-term results following the Bernese periacetabular osteotomy and predictors of clinical outcome. J Bone Joint Surg Am. 2009;91:2113–2123.

    Article  PubMed  Google Scholar 

  22. McGann R, Gurd A. The association between Charcot-Marie-Tooth disease and developmental dysplasia of the hip. Orthopedics. 2002;25:337–339.

    PubMed  Google Scholar 

  23. Murphy SB, Kijewski PK, Millis MB, Harless A. Acetabular dysplasia in the adolescent and young adult. Clin Orthop Relat Res. 1990;261:214–223.

    PubMed  Google Scholar 

  24. Nakahara I, Takao M, Sakai T, Nishii T, Yoshikawa H, Sugano N. Gender differences in 3D morphology and bony impingement of human hips. J Orthop Res. 2011;29:333–339.

    Article  PubMed  Google Scholar 

  25. Nakamura S, Yorikawa J, Otsuka K, Takeshita K, Harasawa A, Matsushita T. Evaluation of acetabular dysplasia using a top view of the hip on three-dimensional CT. J Orthop Sci. 2000;5:533–539.

    Article  CAS  PubMed  Google Scholar 

  26. Nelitz M, Guenther KP, Gunkel S, Puhl W. Reliability of radiological measurements in the assessment of hip dysplasia in adults. Br J Radiol. 1999;72:331–334.

    CAS  PubMed  Google Scholar 

  27. Newman CJ, Walsh M, O’Sullivan R, Jenkinson A, Bennett D, Lynch B, O’Brien T. The characteristics of gait in Charcot-Marie-Tooth disease types I and II. Gait Posture. 2007;26:120–127.

    Article  PubMed  Google Scholar 

  28. Ninomiya S. Rotational acetabular osteotomy for the severely dysplastic hip in the adolescent and adult. Clin Orthop Relat Res. 1989;247:127–137.

    PubMed  Google Scholar 

  29. Pailthorpe CA, Benson MK. Hip dysplasia in hereditary motor and sensory neuropathies. J Bone Joint Surgery Br. 1992;74:538–540.

    CAS  Google Scholar 

  30. Perreira AC, Hunter JC, Laird T, Jamali AA. Multilevel measurement of acetabular version using 3-D CT-generated models: implications for hip preservation surgery. Clin Orthop Relat Res. 2011;469:552–561.

    Article  PubMed  Google Scholar 

  31. Schneider CA, Rasband WS, Eliceiri KW. NIH Image to ImageJ: 25 years of image analysis. Nat Methods. 2012;9:671–675.

    Article  CAS  PubMed  Google Scholar 

  32. Siebenrock KA, Leunig M, Ganz R. Periacetabular osteotomy: the Bernese experience. Instr Course Lect. 2001;50:239–245.

    CAS  PubMed  Google Scholar 

  33. Steppacher SD, Tannast M, Ganz R, Siebenrock KA. Mean 20-year followup of Bernese periacetabular osteotomy. Clin Orthop Relat Res. 2008;466:1633–1644.

    Article  PubMed  Google Scholar 

  34. Tallroth K, Lepisto J. Computed tomography measurement of acetabular dimensions: normal values for correction of dysplasia. Acta Orthop. 2006;77:598–602.

    Article  PubMed  Google Scholar 

  35. Terjesen T, Gunderson RB. Reliability of radiographic parameters in adults with hip dysplasia. Skeletal Radiol. 2012;41:811–816.

    Article  PubMed  Google Scholar 

  36. Thawrani D, Sucato DJ, Podeszwa DA, DeLaRocha A. Complications associated with the Bernese periacetabular osteotomy for hip dysplasia in adolescents. J Bone Joint Surg Am. 2010;92:1707–1714.

    Article  PubMed  Google Scholar 

  37. Tönnis D. General radiograph of the hip joint. In: Tönnis D, ed. Congenital Dysplasia, Dislocation of the Hip. New York, NY: Springer; 1987:100–142.

    Chapter  Google Scholar 

  38. Tönnis D, Heinecke A. Acetabular and femoral anteversion: relationship with osteoarthritis of the hip. J Bone Joint Surg Am. 1999;81:1747–1770.

    PubMed  Google Scholar 

  39. Trumble SJ, Mayo KA, Mast JW. The periacetabular osteotomy: minimum 2 year followup in more than 100 hips. Clin Orthop Relat Res. 1999;363:54–63.

    Article  PubMed  Google Scholar 

  40. Ushiyama T, Tanaka C, Kawasaski T, Matsusue Y. Hip dysplasia in Charcot-Marie-Tooth disease: report of a family. J Orthop Sci. 2003;8:610–612.

    Article  PubMed  Google Scholar 

  41. van Erve RH, Driessen AP. Developmental hip dysplasia in hereditary motor and sensory neuropathy type 1. J Pediatr Orthop. 1999;19:92–96.

    PubMed  Google Scholar 

  42. Walker JL, Nelson KR, Heavilon JA, Stevens DB, Lubicky JP, Ogden JA, VandenBrink KA. Hip abnormalities in children with Charcot-Marie-Tooth disease. J Pediatr Orthop. 1994;14:54–59.

    Article  CAS  PubMed  Google Scholar 

  43. Weaver AA, Gilmartin TD, Anz AW, Stubbs AJ, Stitzel JD. A method to measure acetabular metrics from three dimensional computed tomography pelvis reconstructions—biomed 2009. Biomed Sci Instrum. 2009;45:155–160.

    PubMed  Google Scholar 

  44. Weiner LS, Kelley MA, Ulin RI, Wallach D. Development of the acetabulum and hip: computed tomography analysis of the axial plane. J Pediatr Orthop. 1993;13:421–425.

    Article  CAS  PubMed  Google Scholar 

  45. Wiberg G. Studies on dysplastic acetabula and congenital subluxation of the hip joint: with special reference to the complication of osteo-arthritis. Acta Chir Scand. 1939;83(suppl 58):5–135.

    Google Scholar 

  46. Yagerman SE, Cross MB, Green DW, Scher DM. Pediatric orthopedic conditions in Charcot-Marie-Tooth disease: a literature review. Curr Opin Pediatr. 2012;24:50–56.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

We acknowledge the help of Kerri Murray and Gloria Boyen in gathering the data for this study.

Author information

Authors and Affiliations

  1. Department of Orthopedic Surgery, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA

    Eduardo N. Novais MD & Patrick M. Carry BA

  2. Department of Radiology, Boston Children’s Hospital, Boston, MA, USA

    Sara D. Bixby MD

  3. Northeastern University, Boston, MA, USA

    John Rennick MSc

  4. Department of Orthopedic Surgery, Boston Children’s Hospital, 300 Longwood Avenue, Hunnewell 213, Boston, MA, 02115-5724, USA

    Young-Jo Kim MD, PhD & Michael B. Millis MD

Authors
  1. Eduardo N. Novais MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sara D. Bixby MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. John Rennick MSc
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Patrick M. Carry BA
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Young-Jo Kim MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Michael B. Millis MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Michael B. Millis MD.

Additional information

Each author certifies that he or she, or a member of his or her immediate family, has no commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc) that might pose a conflict of interest in connection with the submitted article.

All ICMJE Conflict of Interest Forms for authors and Clinical Orthopaedics and Related Research editors and board members are on file with the publication and can be viewed on request.

Each author certifies that his or her institution approved the human protocol for this investigation, that all investigations were conducted in conformity with ethical principles of research, and that informed consent for participation in the study was obtained.

This work was performed at Boston Children’s Hospital, Boston, MA, USA.

About this article

Cite this article

Novais, E.N., Bixby, S.D., Rennick, J. et al. Hip Dysplasia Is More Severe in Charcot-Marie-Tooth Disease Than in Developmental Dysplasia of the Hip. Clin Orthop Relat Res 472, 665–673 (2014). https://doi.org/10.1007/s11999-013-3127-z

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11999-013-3127-z

Keywords

Search

Navigation