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Review
. 2022 Mar 15;17(1):125.
doi: 10.1186/s13023-022-02275-z.

Legg-Calvé-Perthes disease overview

Affiliations
Review

Legg-Calvé-Perthes disease overview

Armando O Rodríguez-Olivas et al. Orphanet J Rare Dis. .

Abstract

Background: Legg-Calvé-Perthes Disease (LCPD) is a necrosis of the femoral head which affects the range of motion of the hips. Its incidence is variable, ranging from 0.4/100,000 to 29.0/ 100,000 children. Although LCPD was first described in the beginning of the past century, limited is known about its etiology. Our objective is to describe the main areas of interest in Legg-Calve-Perthes disease.

Methods: A review of the literature regarding LCPD etiology was performed, considering the following inclusion criteria: Studies reporting clinical or preclinical results. The research group carried out a filtered search on the PubMed and Science Direct databases. To maximize the suitability of the search results, we combined the terms ''Perthes disease" OR "LCPD" OR "children avascular femoral head necrosis" with "diagnostic" OR "treatment" OR "etiology" as either key words or MeSH terms.

Results: In this article been described some areas of interest in LCPD, we include topics such as: history, incidence, pathogenesis, diagnosis, treatment and possible etiology, since LCPD has an unknown etiology.

Conclusions: This review suggests that LCPD has a multifactorial etiology where environmental, metabolic and genetic agents could be involved.

Keywords: Biochemical factors; Diagnosis; Environmental factors; Genetic factors; LCPD; Treatment.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Flow diagram
Fig. 2
Fig. 2
AP radiography. In the AP X-ray the deformity of the hip and femoral head characteristic of LCPD is demonstrable, A healthy control, B LCPD patient. Courtesy of INR-LGII genetics laboratory 2017
Fig. 3
Fig. 3
Dotted lines divide the femoral head into medial, central and lateral pillars. The gray dashed line indicates approximately the middle of the lateral pillar. Its dashed outline is the necrosed area. The zig-zag line represents the subchondral fracture size (fs). a Healthy femoral head. b Cavity with 25% of total area lost; its discontinuous outline is a necrosed area and there is a subchondral fissure (fs). c Loss of ~ 50% of total area, increased necrosed area, increased size of fs and loss of lateral abutment height of < 50%. d Loss of > 50% of total area, increased fs and loss of lateral abutment height of ~ 50%. d Total cavity with loss of nearly 100% of area, maximum subchondral damage and damage of > 50% of lateral abutment
Fig. 4
Fig. 4
Loss of the hip junction axis. The deformity in the femoral head, as well as the shortening of the affected extremity will cause the loss of the hip junction axis, due to mechanical damage, causing the characteristic symptoms of LCPD
Fig. 5
Fig. 5
Study algorithm for patients with Legg–Calvé–Perthes disease

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