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Synonyms

Morbus Legg-Calvé-Perthes; LCP; Legg-Calvé-Perthes' disease; LCPD; Juvenile/idiopathic avascular necrosis of the head of the femur; Osteochondropathia deformans coxae juvenilis; Ischemic necrosis of the hip; Coxa plana; Osteochondritis coxae juvenilis

Definition and Characteristics

Osteochondritis was independently identified by AT. Legg, J. Calvé, and G. Perthes' at the beginning of the nineteenth century [1], and is associated with avascular necrosis of the capital femoral epiphysis and often the most proximal part of the metaphysis, related to an intermittent vascular occlusion. The necrosis of the femoral head develops in sequential radiologic stages (I subchondral fracture, II fragmentation, III re-ossification, and IV healing), and heals at the latest after closure of the epiphyseal plate, with a residual deformity of the proximal femur and acetabulum.

Prevalence

Occurs in children mainly between the age of 2 and 14 years with an incidence of 0.05–0.11‰, a boy-to-girl...

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References

  1. Catterall A (1982) Legg-Calvé-Perthes' disease. Churchill Livingstone

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  2. Dezateux C, Roposch A (2005) The puzzles of Perthes' disease: definitive studies of causal factors are needed. J Bone Joint Surg Br 87(11):1463–1464

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  3. Miyamoto Y, Matsuda T, Kitoh H, Haga N, Ohashi H, Nishimura G, Ikegawa SA (2007) recurrent mutation in type II collagen gene causes Legg-Calvé-Perthes disease in a Japanese family. Hum Genet 121(5):625–629

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  4. Wiig O, Terjesen T, Svenningsen S, Lie SA (2006) The epidemiology and aetiology of Perthes' disease in Norway. A nationwide study of 425 patients. J Bone Joint Surg Br 88(9):1217–1223

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  5. Eijer H (2006) Towards a better understanding of the aetiology of Legg–Calvé–Perthes' disease: Acetabular retroversion may cause abnormal loading of dorsal femoral head–neck junction with restricted blood supply to the femoral epiphysis. Med Hypotheses 68(5):995–997

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© 2009 Springer-Verlag GmbH Berlin Heidelberg

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Eijer, H. (2009). Perthes' Disease. In: Lang, F. (eds) Encyclopedia of Molecular Mechanisms of Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-29676-8_1028

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