Legg-Calve-Perthes disease - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Legg-Calvé-Perthes disease is characterized by a phenotype of short stature, delayed bone age, and hyperactivity.
Although several etiologies have been proposed, a multifactorial involvement is most likely.
Typically unilateral, though bilateral involvement is present in 10% of cases.
Clinical features include a limping gait and hip pain frequently radiating into the thigh, knees, groin, or buttocks. Pain worsens during activities.
Treatment is age-dependent. Options include mobilization and monitoring, nonsurgical or surgical containment, or salvage procedures.

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History & Exam
- Key Factors
- Other Factors
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Diagnostics Tests
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Treatment Options
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Definition

Epidemiology

Etiology

Pathophysiology

Images

AP radiograph of a patient with Perthes disease
Frog lateral radiograph of a patient with Perthes disease
Surgical containment
Postoperative radiograph at 4 weeks
Postoperative radiograph at 3 months
Stulberg classification and prognosis of future hip arthritis
$X-ray of stage 2 showing a subchondral fracture line (yellow arrow) and fragmentation (red arrow)$
X-ray of stage 2 showing a subchondral fracture line (yellow arrow) and fragmentation (red arrow)
X-ray of stage 3 showing reossification
MRI scans of the same patient taken 8 years apart showing Perthes disease of the left femoral epiphysis and proportional increase in cartilage thickness compared to the right side. Later scan (image on right) starting to show uptake
MRI showing partial collapse of the left femoral head with areas of necrosis
Catterall classification: Group I shows anterior head involvement (hatched area), no sequestrum, no collapse of the epiphysis. Group II shows the anterior head involvement with a clearly demarcated sequestrum. Group III shows that only a small part of the epiphysis is not involved. Group IV shows a total head involvement
Original Herring classification: Group A, no involvement of the lateral pillar which retains the original height with no density changes; Group B, lateral pillar shows lucency and a loss of height, not exceeding 50% of the original; Group C, characterizes the lateral pillar with increased lucency and collapse of over 50% of the original height

Citations

Key Articles

Stulberg SD, Cooperman DR, Wallensten R. The natural history of Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 1981 Sep;63(7):1095-108.[Abstract]
Catterall A. The natural history of Perthes' disease. J Bone Joint Surg Br. 1971 Feb;53(1):37-53.[Abstract]
Salter RB, Thompson GH. Legg-Calvé-Perthes disease. The prognostic significance of the subchondral fracture and a two-group classification of the femoral head involvement. J Bone Joint Surg Am. 1984 Apr;66(4):479-89.[Abstract]
Herring JA, Neustadt JB, Williams JJ, et al. The lateral pillar classification of Legg-Calvé-Perthes disease. J Pediatr Orthop. 1992 Mar-Apr;12(2):143-50.[Abstract]
Canale ST, D'Anca AF, Cotler JM, et al. Innominate osteotomy in Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 1972 Jan;54(1):25-40.[Abstract]

Referenced Articles

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28. Szepesi K, Pósán E, Hársfalvi J, et al. The most severe forms of Perthes' disease associated with the homozygous Factor V Leiden mutation. J Bone Joint Surg Br. 2004 Apr;86(3):426-9.[Abstract]
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31. Perry DC, Green DJ, Bruce CE, et al. Abnormalities of vascular structure and function in children with Perthes disease. Pediatrics. 2012 Jul;130(1):e126-31.[Abstract]
32. Kallio P, Ryoppy S, Kunnamo I. Transient synovitis and Perthes' disease. Is there an aetiological connection? J Bone Joint Surg Br. 1986 Nov;68(5):808-11.[Abstract]
33. Mukamel M, Litmanovitch M, Yosipovich Z, et al. Legg-Calvé-Perthes disease following transient synovitis. How often? Clin Pediatr (Phila). 1985 Nov;24(11):629-31.[Abstract]
34. Kamiya N, Yamaguchi R, Adapala NS, et al. Legg-Calvé-Perthes disease produces chronic hip synovitis and elevation of interleukin-6 in the synovial fluid. J Bone Miner Res. 2015 Jun;30(6):1009-13.[Abstract]
35. Kealey DW, Lappin KJ, Leslie H, et al. Endocrine profile and physical stature of children with Perthes disease. J Pediatr Orthop. Mar-Apr 2004;24(2):161-6.[Abstract]
36. Harrison MH, Turner MH, Jacobs P. Skeletal immaturity in Perthes' disease. J Bone Joint Surg Br. 1976 Feb;58(1):37-40.[Abstract]
37. Burwell RG, Vernon CL, Dangerfield PH, et al. Raised somatomedin activity in the serum of young boys with Perthes' disease revealed by bioassay. A disease of growth transition? Clin Orthop Rel Res. 1986 Aug;(209):129-38.[Abstract]
38. Tanaka H, Tanura K, Takano K, et al. Serum somatomedin A in Perthes' disease. Acta Orthop Scand. 1984 Apr;55(2):135-40.[Abstract]
39. Joseph B. Serum immunoglobulin in Perthes' disease. J Bone Joint Surg Br. 1991 May;73(3):509-10.[Abstract][Full Text]
40. Kitsugi T, Kasahara Y, Seto Y, et al. Normal somatomedin-C activity measured by radioimmunoassay in Perthes' disease. Clin Orthop Relat Res. 1989 Jul;(244):217-21.[Abstract]
41. Katz JF. Protein-bound iodine in Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 1955 Jul;37-A(4):842-6.[Abstract]
42. Westhoff B, Krauspe R, Kalke AE, et al. Urinary excretion of deoxypyridinoline in Perthes' disease: a prospective, controlled comparative study in 83 children. J Bone Joint Surg Br. 2006 Jul;88(7):967-71.[Abstract]
43. Weiner DS, O'Dell HW. Legg-Calvé-Perthes disease. Observations on skeletal maturation. Clin Orthop Relat Res. 1970 Jan-Feb;68:44-9.
44. Garcia Mata S, Ardanaz Aicua E, Hidalgo Overjero A, et al. Legg-Calvé-Perthes disease and passive smoking. J Pediatr Orthop. 2000 May-Jun;20(3):326-30.[Abstract]
45. Gordon JE, Schoenecker PL, Osland JD, et al. Smoking and socio-economic status in the etiology and severity of Legg-Calvé-Perthes' disease. J Pediatr Orthop B. 2004 Nov;13(6):367-70.[Abstract]
46. Bahmanyar S, Montgomery SM, Weiss RJ, et al. Maternal smoking during pregnancy and other prenatal and perinatal factors and the risk of Legg-Calvé-Perthes disease. Pediatrics. 2008 Aug;122(2):e459-64.[Abstract]
47. Adams JC, Hamblen D. Outline of orthopaedics. 12th ed. Edinburgh, UK: Churchill Livingstone; 1995:312-6.
48. Joseph B. Morphological changes in the acetabulum in Perthes' disease. J Bone Joint Surg Br. 1989 Nov;71(5):756-63.[Abstract][Full Text]
49. Stulberg SD, Cooperman DR, Wallensten R. The natural history of Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 1981 Sep;63(7):1095-108.[Abstract]
50. Barker DJ, Hall AJ. The epidemiology of Perthes' disease. Clin Orthop Relat Res. 1986 Aug;(209):89-94.[Abstract]
51. Wiig O, Terjesen T, Svenningsen S, et al. The epidemiology and etiology of Perthes' disease in Norway. A nationwide survey of 425 patients. J Bone Joint Surg Br. 2006 Sep;88(9):1217-23.[Abstract]
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53. Purry NA. The incidence of Perthes disease in three population groups in the Eastern Cape region of South Africa. J Bone Joint Surg Br. 1982;64(3):286-8.[Abstract]
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55. Sharma S, Sibinski M, Sherlock DA. A profile of Perthes' disease in Greater Glasgow: is there an association with deprivation? J Bone Joint Surg Br. 2005 Nov;87(11):1536-40.[Abstract]
56. Perry DC, Bruce CE, Pope D, et al. Perthes' disease of the hip: socioeconomic inequalities and the urban environment. Arch Dis Child. 2012 Dec;97(12):1053-7.[Abstract]
57. Perry DC, Bruce CE, Pope D, et al. Comorbidities in Perthes' disease: a case control study using the General Practice Research database. J Bone Joint Surg Br. 2012 Dec;94(12):1684-9.[Abstract]
58. Shaw ED, Beals RK. The hip joint in Trisomy 21. A study of its structure and associated disease. Clin Orthop. 1992;278:101-107.[Abstract]
59. Oshima M, Yoshihasi Y, Ito K, et al. Initial stage of Legg-Calve Perthes' disease: comparison of three-phase scintigraphy and SPECT with MR imaging. Eur J Radiol. 1992 Sep;15(2):107-12.[Abstract]
60. Bos CF, Bloem JL, Bloem RM. Sequential magnetic resonance imaging in Perthes' disease. J Bone Joint Surg Br. 1991 Mar;73(2):219-24.[Abstract][Full Text]
61. Du J, Lu A, Dempsey M, et al. 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 Oct-Nov;33(7):707-13.[Abstract]
62. Kim HK, Wiesman KD, Kulkarni V, et al. Perfusion MRI in early stage of Legg-Calvé-Perthes disease to predict lateral pillar involvement: a preliminary study. J Bone Joint Surg Am. 2014 Jul 16;96(14):1152-60.[Abstract]
63. Catterall A. The natural history of Perthes' disease. J Bone Joint Surg Br. 1971 Feb;53(1):37-53.[Abstract]
64. Huhnstock S, Svenningsen S, Merckoll E, et al. Radiographic classifications in Perthes disease. Acta Orthop. 2017 Oct;88(5):522-9.[Abstract][Full Text]
65. Salter RB, Thompson GH. Legg-Calvé-Perthes disease. The prognostic significance of the subchondral fracture and a two-group classification of the femoral head involvement. J Bone Joint Surg Am. 1984 Apr;66(4):479-89.[Abstract]
66. Herring JA, Neustadt JB, Williams JJ, et al. The lateral pillar classification of Legg-Calvé-Perthes disease. J Pediatr Orthop. 1992 Mar-Apr;12(2):143-50.[Abstract]
67. 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 Oct;86(10):2103-20.[Abstract]
68. Canale ST, D'Anca AF, Cotler JM, et al. Innominate osteotomy in Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 1972 Jan;54(1):25-40.[Abstract]
69. Sinigaglia R, Bundy A, Okoro T, et al. Is conservative treatment really effective for Legg-Calve-Perthes disease? A critical review of the literature. Chirurgia Narzadow Ruchu i Ortopedia Polska. 2007 Nov-Dec;72(6):439-43.
70. Nguyen NA, Klein G, Dogbey G, et al. Operative versus nonoperative treatments for Legg-Calvé-Perthes disease: a meta-analysis. J Pediatr Orthop. 2012 Oct-Nov;32(7):697-705.[Abstract]
71. Saran N, Varghese R, Mulpuri K. Do femoral or salter innominate osteotomies improve femoral head sphericity in Legg-Calvé-Perthes disease? A meta-analysis. Clin Orthop Relat Res. 2012 Sep;470(9):2383-93.[Abstract]
72. Rich MM, Schoenecker PL. Management of Legg-Calvé-Perthes disease using an A-frame orthosis and hip range of motion: a 25-year experience. J Pediatr Orthop. 2013 Mar;33(2):112-9.[Abstract]
73. Wiig O, Terjesen T, Svenningsen S. Prognostic factors and outcome of treatment in Perthes' disease: a prospective study of 368 patients with five-year follow-up. J Bone Joint Surg Br. 2008 Oct;90(10):1364-71.[Abstract]
74. Jasiewicz B, Zarzycka M, Zarzycki D, et al. Salter osteotomy in Perthes' disease. Late radiological results. Ortop Traumatol Rehabil. 2004 Nov-Dec;6(6):733-9.[Abstract]
75. Hsu JE, Baldwin KD, Tannast M, et al. What is the evidence supporting the prevention of osteoarthritis and improved femoral coverage after shelf procedure for Legg-Calvé-Perthes disease? Clin Orthop Relat Res. 2012 Sep;470(9):2421-30.[Abstract]
76. Kadhim M, Holmes L Jr, Bowen JR. The role of shelf acetabuloplasty in early and late stages of Perthes disease: a meta-analysis of observational studies. J Child Orthop. 2012 Oct;6(5):379-90.
77. Hanna SA, Sarraf KM, Ramachandran M, et al. Systematic review of the outcome of total hip arthroplasty in patients with sequelae of Legg-Calvé-Perthes disease. Arch Orthop Trauma Surg. 2017 Aug;137(8):1149-54.[Abstract]
78. Nakamura J, Kamegaya M, Saisu T, et al. Outcome of patients with Legg-Calvé-Perthes onset before 6 years of age. J Pediatr Orthop. 2015 Mar;35(2):144-50.[Abstract]
79. Wiig O, Huhnstock S, Terjesen T, et al. The outcome and prognostic factors in children with bilateral Perthes' disease: a prospective study of 40 children with follow-up over five years. Bone Joint J. 2016 Apr;98-B(4):569-75.[Abstract]
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