Disorder characterized by unilateral or bilateral aseptic necrosis of the emphysis of the head of the femur (caput femoris). This is s a consquence of insufficient blood supply to the femur, and the tip of the bone dies over a period of 1 to 3 weeks. New blood supply causes new bone cells to appear in the region over the next 6 to 12 months. New bone then replaces the old bone within 2 to 3 years. The disease thus progresses from necrosis through revascularization, mottling, and fragmentation of the epiphysis to reossification and flattening of the head of the femur.
There is moderate pain in the hip, with stiffness and restricted movement of the hip and a slight limp of involved leg. The limp becomes worse as result of the atrophy and shortening of the femur.
The sporadic type is unilateral in most instances and prevalent in in boys; sudden or gradual onset between 3 and 12 years (most commonly 5-10) of age. The hereditary type is transmitted as an autosomal dominant trait, and there is equal susceptibility of the sexes and a greater proportion of bilateral involvement.
The condition was first described by Karel Maydl (1853-1903) in 1897.
- Karel Maydl in:
- Wiener klinische Rundschau, 1897, 11: 153.
- A. T. Legg:
The cause of atrophy in joint disease.
American Journal of Orthopedic Surgery, 1908-1909, 6: 84-90. An obscure affection of the hip-joint.
Boston Medical and Surgical Journal, 1910, 162: 202-204.
- H. Waldenström:
Der obere tuberkulöse Cullumherd.
Zeitschrift für Orthopädische Chirurgie, Stuttgart, 1909, 24: 487-512.
- J. Calvé:
Sur une forme particulière de pseudo-coxalgie greffée sur des déformations caractéristiques de l’extrémité supérieure du fémur.
Revue de chirurgie, Paris, 1910, 42: 54-84.
- G. Perthes:
Über Arthritis deformans juvenilis.
Deutsche Zeitschrift für Chirurgie, Leipzig, 1910, 107: 111-159.