Perthes disease

Clinical Cases 22.10.2002
Scan Image
Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 6 years, male
Authors: N.Ramesh,N El Saeity,M O'Dowd,E Colhoun
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AI Report

Clinical History

Left hip pain and limping.

Imaging Findings

The patient presented with pain in the left hip and limping. Plain radiographs of the pelvis, AP and frog lateral views, were performed. Follow-up films were performed after 3 months. Follow-up films showed good healing of the left femoral epiphysis with a mild coxa magna deformity of the femoral neck.

Discussion

The initial radiographs showed fragmentation of the left femoral epiphysis, which appears dense as compared with the right. The diagnosis of Perthes disease was made.

The aetiology of Perthes disease is idiopathic. It represents multiple vascular occlusive episodes that involve the femoral head, causing changes of avascular necrosis. It is a long ongoing process of vascular change and repair and is different from avascular necrosis secondary to trauma. It usually affects Caucasian boys (4-5 times more often than females), in the age group of 3-12 years, occurring bilaterally in 15% of cases and when bilateral is usually asymmetrical. When symmetric involvement is seen, hyporthyroidism and epiphyseal dysplasia should be excluded. The onset is earlier in females and the prognosis worse. There is increased incidence of associated congenital anomalies, congenital heart disease, pyloric stenosis and undescended testes.

The plain radiograph of the pelvis including both hips is still the best way to detect and monitor changes and progress of the disease. There are four stages described in the disease. In stage 1, the initial or the devascularisation stage, the affected femoral head is smaller and there is apparent widening of the joint space. The epiphysis appears denser than in the normal hip. Stage 2 involves collapse and fragmentation of the epiphysis. Stage 3, reossification, is marked by new bone formation as healing commences and in stage 4, the remodelling and healing phase, the radiograph will show residual deformity in the femoral neck and head, with the coxa magna appearing wider and shorter than normal. Every affected child goes through each phase. The whole process can extend over months to years. Despite advances in modern imaging, plain films are still the best way to evaluate and follow up Perthes disease. MR imaging, arthrography and radionuclide examinations are alternative investigations for evaluation of the disease.MR imaging is extremely sensitive in detection of areas of bone infarction and extent,early in the disease process.In the early stages there are areas of low signal intensity within the femoral head on T1-weighted images and normal or increase intensity on T2.Gradient echo images and T2-weighted images with fat saturation may show changes of the acetabular and femoral head cartilage.MR is sensitive in imaging the loss of containment of the femoral head within the acetabulum.MR is shown to correlate well with the outcome.

The prognosis is worse when the age of onset is over 9 years and is also dependent on the shape of the femoral head at maturity. A non-spherical head has a worse prognosis. The disease process is prolonged and outcome poor if a major part of the physis is involved. Most patients develop osteoarthritis after the 6th decade.

Differential Diagnosis List

Perthes disease

Final Diagnosis

Perthes disease

Liscense

Figures

Pelvic X-ray - frog lateral view

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Pelvic X-ray - frog lateral view

Pelvic X-ray - AP view

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Pelvic X-ray - AP view