Section I - The Disease
Pathophysiology, Classifications, and Natural History of Perthes Disease

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Pathophysiology

It is generally accepted that a disruption of blood supply to the femoral head is a key pathogenic event in Legg-Calvé-Perthes disease (LCPD). Necropsy and biopsy studies of patients with LCPD show evidence of tissue necrosis consistent with ischemic injury.1, 2 Various imaging studies also show evidence of disruption of blood flow to the affected femoral head.3, 4, 5, 6, 7 Furthermore, the disruption of the blood supply to the femoral head in large animal models (porcine and canine models)

Classifications

The classifications for LCPD can be divided into the one that defines the stage of the disease and the ones used to prognosticate outcome. Waldenström’s radiographic classification defines 4 radiographic stages of LCPD during the active phase of the disease, termed the initial stage, fragmentation stage, reossification stage, and residual stage, according to the characteristic radiographic features of each stage (Table 1). The duration of each stage is variable from one patient to another. What

Natural history

Long-term studies on the natural history of the disease are few in number and have limitations due to a small sample size, loss of follow-up, and inclusion of patients treated with various nonoperative treatments that may have influenced their outcome. In general, long-term studies with an average follow-up of less than 40 years show that the majority of the patients are active and asymptomatic even with a deformed femoral head. A study of a cohort from Iowa by Gower and Johnston69 with an

Summary

LCPD remains a controversial condition in pediatric orthopedics. Several key questions relating to its pathophysiology, classifications, and natural history remain partially answered. Experimental studies reveal that mechanical and biological factors contribute to the pathophysiology of the femoral head deformity following ischemic necrosis. Further research is needed to define the mechanisms responsible for the pathologic healing process and how to control the process effectively. This

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