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Legg-Calve-Perthes Disease

  • Benjamin Joseph
Chapter

Abstract

Legg-Calve-Perthes disease (LCPD) is a self-limiting disorder of childhood characterised by interruption of blood supply to the capital femoral epiphysis, which can result in deformation of the femoral head during a protracted period of revascularization. This, in turn, can lead to development of premature degenerative arthritis in early adult life. The primary aim of treatment of LCPD should be to preserve femoral head sphericity. Since the prime factor leading to deformation is epiphyseal extrusion, the cornerstone of management involves ensuring “containment” of the femoral epiphysis within the acetabulum and preserving range of hip motion. Most importantly, if containment is to succeed in preventing femoral head deformation treatment should be instituted early in the course of the disease. Although there is little disagreement on these principles, the choice of the method of containment remains controversial with paucity of high quality evidence to support any particular approach. The goal of this chapter is to provide a comprehensive review of the most up-to-date evidence on the diagnosis and management of LCPD in children, while incorporating the author’s philosophy.

Keywords

Legg-Calve-Perthes disease Avascular necrosis Containment Femoral head sphericity Femoral osteotomy Pelvic osteotomy Trochanteric overgrowth 

References

  1. 1.
    Catterall A. The natural history of Perthes’ disease. J Bone Joint Surg. 1971;53(1):37–53.CrossRefGoogle Scholar
  2. 2.
    Snyder CH. A sling for use in Legg-Perthes disease. J Bone Joint Surg Am. 1947;29(2):524–6.PubMedGoogle Scholar
  3. 3.
    Meyer J. Treatment of Legg-Calve-Perthes disease. Assessment of therapeutic results with particular reference to the value of traction in bed. Acta Orthop Scand. 1966;(Suppl 86):9–111.Google Scholar
  4. 4.
    Zadek I, Berkett GD. The effect of drilling the neck of the femur in Legg-Perthes’ disease. N Y State J Med. 1948;48(3):273.PubMedGoogle Scholar
  5. 5.
    Bertrand P. Technic of intra-epiphysial graft in therapy of coxa plana. Rev Chir Orthop. 1954;40(1):116–20.PubMedGoogle Scholar
  6. 6.
    Axer A. Subtrochanteric osteotomy in the treatment of Perthes’ disease: a preliminary report. J Bone Joint Surg. 1965;47:489–99.CrossRefGoogle Scholar
  7. 7.
    Ibrahim T, Little DG. The pathogenesis and treatment of Legg-Calve-Perthes disease. JBJS Rev. 2016;4(7)  https://doi.org/10.2106/JBJS.RVW.15.00063.CrossRefGoogle Scholar
  8. 8.
    Cheng TL, Murphy CM, Cantrill LC, Mikulec K, Carpenter C, Schindeler A, et al. Local delivery of recombinant human bone morphogenetic proteins and bisphosphonate via sucrose acetate isobutyrate can prevent femoral head collapse in Legg-Calve-Perthes disease: a pilot study in pigs. Int Orthop. 2014;38(7):1527–33.PubMedPubMedCentralCrossRefGoogle Scholar
  9. 9.
    Young ML, Little DG, Kim HK. Evidence for using bisphosphonate to treat Legg-Calve-Perthes disease. Clin Orthop Relat Res. 2012;470(9):2462–75.PubMedPubMedCentralCrossRefGoogle Scholar
  10. 10.
    de Camargo FP, de Godoy RM Jr, Tovo R. Angiography in Perthes’ disease. Clin Orthop Relat Res. 1984;(191):216–20.Google Scholar
  11. 11.
    Atsumi T, Yamano K, Muraki M, Yoshihara S, Kajihara T. The blood supply of the lateral epiphyseal arteries in Perthes’ disease. J Bone Joint Surg. 2000;82(3):392–8.CrossRefGoogle Scholar
  12. 12.
    Catterall A, Pringle J, Byers PD, Fulford GE, Kemp HB, Dolman CL, et al. A review of the morphology of Perthes’ disease. J Bone Joint Surg. 1982;64(3):269–75.CrossRefGoogle Scholar
  13. 13.
    Kim HK, Herring JA. Pathophysiology, classifications, and natural history of Perthes disease. Orthop Clin North Am. 2011;42(3):285–95.. vPubMedCrossRefGoogle Scholar
  14. 14.
    Joseph B, Varghese G, Mulpuri K, Narasimha Rao K, Nair NS. Natural evolution of Perthes disease: a study of 610 children under 12 years of age at disease onset. J Pediatr Orthop. 2003;23(5):590–600.PubMedCrossRefGoogle Scholar
  15. 15.
    Neal DC, O’Brien JC, Burgess J, Jo C, Kim HK, International Perthes Study G. Quantitative assessment of synovitis in Legg-Calve-Perthes disease using gadolinium-enhanced MRI. J Pediatr Orthop B. 2015;24(2):89–94.PubMedCrossRefGoogle Scholar
  16. 16.
    Joseph B, Pydisetty RK. Chondrolysis and the stiff hip in Perthes’ disease: an immunological study. J Pediatr Orthop. 1996;16(1):15–9.PubMedCrossRefGoogle Scholar
  17. 17.
    Joseph B. Serum immunoglobulin in Perthes’ disease. J Bone Joint Surg. 1991;73(3):509–10.CrossRefGoogle Scholar
  18. 18.
    Kamiya N, Yamaguchi R, Adapala NS, Chen E, Neal D, Jack O, et al. Legg-Calve-Perthes disease produces chronic hip synovitis and elevation of interleukin-6 in the synovial fluid. J Bone Miner Res. 2015;30(6):1009–13.PubMedCrossRefGoogle Scholar
  19. 19.
    Kamegaya M, Moriya H, Tsuchiya K, Akita T, Ogata S, Someya M. Arthrography of early Perthes’ disease. Swelling of the ligamentum teres as a cause of subluxation. J Bone Joint Surg. 1989;71(3):413–7.CrossRefGoogle Scholar
  20. 20.
    Joseph B. Morphological changes in the acetabulum in Perthes’ disease. J Bone Joint Surg. 1989;71(5):756–63.CrossRefGoogle Scholar
  21. 21.
    Green NE, Beauchamp RD, Griffin PP. Epiphyseal extrusion as a prognostic index in Legg-Calve-Perthes disease. J Bone Joint Surg Am. 1981;63(6):900–5.PubMedCrossRefGoogle Scholar
  22. 22.
    Waldenstrom H. The definite form of the coxa plana. Acta Radiol. 2016;57(7):e79–94.PubMedCrossRefGoogle Scholar
  23. 23.
    Joseph B, Nair NS, Narasimha Rao K, Mulpuri K, Varghese G. Optimal timing for containment surgery for Perthes disease. J Pediatr Orthop. 2003;23(5):601–6.PubMedPubMedCentralCrossRefGoogle Scholar
  24. 24.
    Hyman JE, Trupia EP, Wright ML, Matsumoto H, Jo CH, Mulpuri K, et al. Interobserver and intraobserver reliability of the modified Waldenstrom classification system for staging of Legg-Calve-Perthes disease. J Bone Joint Surg Am. 2015;97(8):643–50.PubMedCrossRefGoogle Scholar
  25. 25.
    Joseph B. Natural history of early onset and late-onset Legg-Calve-Perthes disease. J Pediatr Orthop. 2011;31(2 Suppl):S152–5.PubMedCrossRefGoogle Scholar
  26. 26.
    Herring JA, Neustadt JB, Williams JJ, Early JS, Browne RH. The lateral pillar classification of Legg-Calve-Perthes disease. J Pediatr Orthop. 1992;12(2):143–50.PubMedCrossRefGoogle Scholar
  27. 27.
    Herring JA, Kim HT, Browne R. Legg-Calve-Perthes disease. Part II: Prospective multicenter study of the effect of treatment on outcome. J Bone Joint Surg Am. 2004;86-A(10):2121–34.CrossRefGoogle Scholar
  28. 28.
    Joseph B, Mulpuri K, Varghese G. Perthes’ disease in the adolescent. J Bone Joint Surg. 2001;83(5):715–20.CrossRefGoogle Scholar
  29. 29.
    Shah H, Siddesh ND, Joseph B. To what extent does remodeling of the proximal femur and the acetabulum occur between disease healing and skeletal maturity in Perthes disease? A radiological study. J Pediatr Orthop. 2008;28(7):711–6.PubMedCrossRefGoogle Scholar
  30. 30.
    Stulberg SD, Cooperman DR, Wallensten R. The natural history of Legg-Calve-Perthes disease. J Bone Joint Surg Am. 1981;63(7):1095–108.PubMedCrossRefGoogle Scholar
  31. 31.
    Wiig O, Terjesen T, Svenningsen S. Inter-observer reliability of the Stulberg classification in the assessment of Perthes disease. J Child Orthop. 2007;1(2):101–5.PubMedPubMedCentralCrossRefGoogle Scholar
  32. 32.
    Herring JA, Kim HT, Browne R. Legg-Calve-Perthes disease. Part I: Classification of radiographs with use of the modified lateral pillar and Stulberg classifications. J Bone Joint Surg Am. 2004;86-A(10):2103–20.CrossRefGoogle Scholar
  33. 33.
    Barker DJ, Hall AJ. The epidemiology of Perthes’ disease. Clin Orthop Relat Res. 1986;(209):89–94.Google Scholar
  34. 34.
    Joseph B, Chacko V, Rao BS, Hall AJ. The epidemiology of Perthes’ disease in south India. Int J Epidemiol. 1988;17(3):603–7.PubMedCrossRefGoogle Scholar
  35. 35.
    Hall AJ, Barker DJ. Perthes’ disease in Yorkshire. J Bone Joint Surg. 1989;71(2):229–33.CrossRefGoogle Scholar
  36. 36.
    Kealey WD, Moore AJ, Cook S, Cosgrove AP. Deprivation, urbanisation and Perthes’ disease in Northern Ireland. J Bone Joint Surg. 2000;82(2):167–71.CrossRefGoogle Scholar
  37. 37.
    Perry DC, Bruce CE, Pope D, Dangerfield P, Platt MJ, Hall AJ. Perthes’ disease: deprivation and decline. Arch Dis Child. 2011;96(12):1124–8.PubMedCrossRefGoogle Scholar
  38. 38.
    Mullan CJ, Thompson LJ, Cosgrove AP. The declining incidence of Legg-Calve-Perthes’ disease in Northern Ireland: an epidemiological study. J Pediatr Orthop. 2017;37(3):e178–82.PubMedCrossRefGoogle Scholar
  39. 39.
    Sharma S, Sibinski M, Sherlock DA. A profile of Perthes’ disease in Greater Glasgow: is there an association with deprivation? J Bone Joint Surg. 2005;87(11):1536–40.CrossRefGoogle Scholar
  40. 40.
    Pillai A, Atiya S, Costigan PS. The incidence of Perthes’ disease in Southwest Scotland. J Bone Joint Surg. 2005;87(11):1531–5.CrossRefGoogle Scholar
  41. 41.
    Perry DC, Bruce CE, Pope D, Dangerfield P, Platt MJ, Hall AJ. Legg-Calve-Perthes disease in the UK: geographic and temporal trends in incidence reflecting differences in degree of deprivation in childhood. Arthritis Rheum. 2012;64(5):1673–9.PubMedCrossRefGoogle Scholar
  42. 42.
    Perry DC. Unravelling the enigma of Perthes disease. Ann R Coll Surg Engl. 2013;95(5):311–6.PubMedPubMedCentralCrossRefGoogle Scholar
  43. 43.
    Daniel AB, Shah H, Kamath A, Guddettu V, Joseph B. Environmental tobacco and wood smoke increase the risk of Legg-Calve-Perthes disease. Clin Orthop Relat Res. 2012;470(9):2369–75.PubMedCrossRefGoogle Scholar
  44. 44.
    Gordon JE, Schoenecker PL, Osland JD, Dobbs MB, Szymanski DA, Luhmann SJ. Smoking and socio-economic status in the etiology and severity of Legg-Calve-Perthes’ disease. J Pediatr Orthop B. 2004;13(6):367–70.PubMedCrossRefGoogle Scholar
  45. 45.
    Garcia Mata S, Ardanaz Aicua E, Hidalgo Ovejero A, Martinez Grande M. Legg-Calve-Perthes disease and passive smoking. J Pediatr Orthop. 2000;20(3):326–30.PubMedGoogle Scholar
  46. 46.
    Bahmanyar S, Montgomery SM, Weiss RJ, Ekbom A. Maternal smoking during pregnancy, other prenatal and perinatal factors, and the risk of Legg-Calve-Perthes disease. Pediatrics. 2008;122(2):e459–64.PubMedCrossRefGoogle Scholar
  47. 47.
    Glueck CJ, Freiberg RA, Crawford A, Gruppo R, Roy D, Tracy T, et al. Secondhand smoke, hypofibrinolysis, and Legg-Perthes disease. Clin Orthop Relat Res. 1998;(352):159–67.CrossRefGoogle Scholar
  48. 48.
    Woratanarat P, Thaveeratitharm C, Woratanarat T, Angsanuntsukh C, Attia J, Thakkinstian A. Meta-analysis of hypercoagulability genetic polymorphisms in Perthes disease. J Orthop Res. 2014;32(1):1–7.PubMedCrossRefGoogle Scholar
  49. 49.
    Perry DC, Hall AJ. The epidemiology and etiology of Perthes disease. Orthop Clin North Am. 2011;42(3):279–83.. vPubMedCrossRefGoogle Scholar
  50. 50.
    Kenet G, Ezra E, Wientroub S, Steinberg DM, Rosenberg N, Waldman D, et al. Perthes’ disease and the search for genetic associations: collagen mutations, Gaucher’s disease and thrombophilia. J Bone Joint Surg. 2008;90(11):1507–11.CrossRefGoogle Scholar
  51. 51.
    Perry DC, Skellorn PJ, Bruce CE. The lognormal age of onset distribution in Perthes’ disease: an analysis from a large well-defined cohort. Bone Joint J. 2016;98-B(5):710–4.PubMedCrossRefGoogle Scholar
  52. 52.
    Loder RT, Schwartz EM, Hensinger RN. Behavioral characteristics of children with Legg-Calve-Perthes disease. J Pediatr Orthop. 1993;13(5):598–601.PubMedCrossRefGoogle Scholar
  53. 53.
    Du J, Lu A, Dempsey M, Herring JA, Kim HK. MR perfusion index as a quantitative method of evaluating epiphyseal perfusion in Legg-Calve-Perthes disease and correlation with short-term radiographic outcome: a preliminary study. J Pediatr Orthop. 2013;33(7):707–13.PubMedCrossRefGoogle Scholar
  54. 54.
    Kim HK, Wiesman KD, Kulkarni V, Burgess J, Chen E, Brabham C, et al. Perfusion MRI in early stage of Legg-Calve-Perthes disease to predict lateral pillar involvement: a preliminary study. J Bone Joint Surg Am. 2014;96(14):1152–60.PubMedCrossRefGoogle Scholar
  55. 55.
    Sankar WN, Thomas S, Castaneda P, Hong T, Shore BJ, Kim HK, et al. Feasibility and safety of perfusion MRI for Legg-Calve-Perthes disease. J Pediatr Orthop. 2014;34(7):679–82.PubMedCrossRefGoogle Scholar
  56. 56.
    Mintowt-Czyz W, Tayton K. Indication for weight relief and containment in the treatment of Perthes’ disease. Acta Orthop Scand. 1983;54(3):439–45.PubMedCrossRefGoogle Scholar
  57. 57.
    Kim HK. Pathophysiology and new strategies for the treatment of Legg-Calve-Perthes disease. J Bone Joint Surg Am. 2012;94(7):659–69.PubMedCrossRefGoogle Scholar
  58. 58.
    Muirhead-Allwood W, Catterall A. The treatment of Perthes’ disease. The results of a trial of management. J Bone Joint Surg. 1982;64(3):282–5.CrossRefGoogle Scholar
  59. 59.
    Rich MM, Schoenecker PL. Management of Legg-Calve-Perthes disease using an A-frame orthosis and hip range of motion: a 25-year experience. J Pediatr Orthop. 2013;33(2):112–9.PubMedCrossRefGoogle Scholar
  60. 60.
    Bobechko WP. The Toronto brace for Legg-Perthes disease. Clin Orthop Relat Res. 1974;(102):115–7.CrossRefGoogle Scholar
  61. 61.
    Martinez AG, Weinstein SL, Dietz FR. The weight-bearing abduction brace for the treatment of Legg-Perthes disease. J Bone Joint Surg Am. 1992;74(1):12–21.PubMedCrossRefGoogle Scholar
  62. 62.
    Kamegaya M. Comparative study of Perthes’ disease treated by various ambulatory orthoses. Nihon Seikeigeka Gakkai Zasshi. 1987;61(7):917–32.PubMedGoogle Scholar
  63. 63.
    Axer A, Gershuni DH, Hendel D, Mirovski Y. Indications for femoral osteotomy in Legg-Calve-Perthes disease. Clin Orthop Relat Res. 1980;(150):78–87.Google Scholar
  64. 64.
    Hoikka V, Lindholm TS, Poussa M. Intertrochanteric varus osteotomy in Legg-Calve-Perthes disease: a report of 112 hips. J Pediatr Orthop. 1986;6(5):600–4.PubMedCrossRefGoogle Scholar
  65. 65.
    Kitakoji T, Hattori T, Iwata H. Femoral varus osteotomy in Legg-Calve-Perthes disease: points at operation to prevent residual problems. J Pediatr Orthop. 1999;19(1):76–81.PubMedGoogle Scholar
  66. 66.
    Copeliovitch L. Femoral varus osteotomy in Legg-Calve-Perthes disease. J Pediatr Orthop. 2011;31(2 Suppl):S189–91.PubMedCrossRefGoogle Scholar
  67. 67.
    Joseph B, Srinivas G, Thomas R. Management of Perthes disease of late onset in southern India. The evaluation of a surgical method. J Bone Joint Surg. 1996;78(4):625–30.CrossRefGoogle Scholar
  68. 68.
    Shah H, Siddesh ND, Joseph B, Nair SN. Effect of prophylactic trochanteric epiphyseodesis in older children with Perthes’ disease. J Pediatr Orthop. 2009;29(8):889–95.PubMedCrossRefGoogle Scholar
  69. 69.
    Shim JS, Kim HT, Mubarak SJ, Wenger DR. Genu valgum in children with coxa vara resulting from hip disease. J Pediatr Orthop. 1997;17(2):225–9.PubMedCrossRefGoogle Scholar
  70. 70.
    Tercier S, Shah H, Siddesh ND, Joseph B. Does proximal femoral varus osteotomy in Legg-Calve-Perthes disease predispose to angular mal-alignment of the knee? A clinical and radiographic study at skeletal maturity. J Child Orthop. 2013;7(3):205–11.PubMedPubMedCentralCrossRefGoogle Scholar
  71. 71.
    Salter RB. Legg-Perthes disease: the scientific basis for the methods of treatment and their indications. Clin Orthop Relat Res. 1980;(150):8–11.Google Scholar
  72. 72.
    Kumar D, Bache CE, O’Hara JN. Interlocking triple pelvic osteotomy in severe Legg-Calve-Perthes disease. J Pediatr Orthop. 2002;22(4):464–70.PubMedGoogle Scholar
  73. 73.
    Kruse RW, Guille JT, Bowen JR. Shelf arthroplasty in patients who have Legg-Calve-Perthes disease. A study of long-term results. J Bone Joint Surg Am. 1991;73(9):1338–47.PubMedCrossRefGoogle Scholar
  74. 74.
    Willett K, Hudson I, Catterall A. Lateral shelf acetabuloplasty: an operation for older children with Perthes’ disease. J Pediatr Orthop. 1992;12(5):563–8.PubMedCrossRefGoogle Scholar
  75. 75.
    Daly K, Bruce C, Catterall A. Lateral shelf acetabuloplasty in Perthes’ disease. A review of the end of growth. J Bone Joint Surg. 1999;81(3):380–4.CrossRefGoogle Scholar
  76. 76.
    Carsi B, Judd J, Clarke NM. Shelf acetabuloplasty for containment in the early stages of Legg-Calve-Perthes disease. J Pediatr Orthop. 2015;35(2):151–6.PubMedCrossRefGoogle Scholar
  77. 77.
    Saran N, Varghese R, Mulpuri K. Do femoral or salter innominate osteotomies improve femoral head sphericity in Legg-Calve-Perthes disease? A meta-analysis. Clin Orthop Relat Res. 2012;470(9):2383–93.PubMedPubMedCentralCrossRefGoogle Scholar
  78. 78.
    Joseph B, Rao N, Mulpuri K, Varghese G, Nair S. How does a femoral varus osteotomy alter the natural evolution of Perthes’ disease? J Pediatr Orthop B. 2005;14(1):10–5.PubMedCrossRefGoogle Scholar
  79. 79.
    Terjesen T, Wiig O, Svenningsen S. Varus femoral osteotomy improves sphericity of the femoral head in older children with severe form of Legg-Calve-Perthes disease. Clin Orthop Relat Res. 2012;470(9):2394–401.PubMedCrossRefGoogle Scholar
  80. 80.
    Lim KS, Shim JS. Outcomes of combined shelf acetabuloplasty with femoral varus osteotomy in severe Legg-Calve-Perthes (LCP) disease: advanced containment method for severe LCP disease. Clin Orthop Surg. 2015;7(4):497–504.PubMedPubMedCentralCrossRefGoogle Scholar
  81. 81.
    Olney BW, Asher MA. Combined innominate and femoral osteotomy for the treatment of severe Legg-Calve-Perthes disease. J Pediatr Orthop. 1985;5(6):645–51.PubMedCrossRefGoogle Scholar
  82. 82.
    Javid M, Wedge JH. Radiographic results of combined Salter innominate and femoral osteotomy in Legg-Calve-Perthes disease in older children. J Child Orthop. 2009;3(3):229–34.PubMedPubMedCentralCrossRefGoogle Scholar
  83. 83.
    Kamegaya M, Morita M, Saisu T, Kakizaki J, Oikawa Y, Segawa Y. Single versus combined procedures for severely involved Legg-Calve-Perthes disease. J Pediatr Orthop. 2018;38(6):312–9.PubMedCrossRefGoogle Scholar
  84. 84.
    Mosow N, Vettorazzi E, Breyer S, Ridderbusch K, Stucker R, Rupprecht M. Outcome after combined pelvic and femoral osteotomies in patients with Legg-Calve-Perthes disease. J Bone Joint Surg Am. 2017;99(3):207–13.PubMedCrossRefGoogle Scholar
  85. 85.
    Joseph B, Price CT. Principles of containment treatment aimed at preventing femoral head deformation in Perthes disease. Orthop Clin North Am. 2011;42(3):317–27.. viCrossRefPubMedGoogle Scholar
  86. 86.
    Wenger DR, Hosalkar HS. Principles of treating the sequelae of Perthes disease. Orthop Clin North Am. 2011;42(3):365–72.. viiPubMedCrossRefGoogle Scholar
  87. 87.
    Yoo WJ, Choi IH, Chung CY, Cho TJ, Kim HY. Valgus femoral osteotomy for hinge abduction in Perthes’ disease. Decision-making and outcomes. J Bone Joint Surg. 2004;86(5):726–30.CrossRefGoogle Scholar
  88. 88.
    Choi IH, Yoo WJ, Cho TJ, Moon HJ. Principles of treatment in late stages of Perthes disease. Orthop Clin North Am. 2011;42(3):341–8.. viPubMedCrossRefGoogle Scholar
  89. 89.
    Bankes MJ, Catterall A, Hashemi-Nejad A. Valgus extension osteotomy for ‘hinge abduction’ in Perthes’ disease. Results at maturity and factors influencing the radiological outcome. J Bone Joint Surg. 2000;82(4):548–54.CrossRefGoogle Scholar
  90. 90.
    Siebenrock KA, Anwander H, Zurmuhle CA, Tannast M, Slongo T, Steppacher SD. Head reduction osteotomy with additional containment surgery improves sphericity and containment and reduces pain in Legg-Calve ́-Perthes Disease. Clin ORthop Relat Res. 2015;473:1274–83.CrossRefGoogle Scholar
  91. 91.
    Ziebarth K, Slongo T, Siebenrock KA. Residual Perthes deformity and surgical reduction of the size of the femoral head. Oper Tech Orthop. 2013;23:134–9.CrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Benjamin Joseph
    • 1
  1. 1.Aster MedcityKochiIndia

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