Aetiology of Legg-Calvé-Perthes disease: A systematic review

Abstract

BACKGROUNDLegg-Calvé-Perthes disease (LCPD) is a clinical condition affecting the femoral head of children during their growth. Its prevalence is set to be between 0.4/100000 to 29.0/100000 children less than 15 years of age with a peak of incidence in children aged from 4 years to 8 years. LCPD aetiology has been widely studied, but it is still poorly understood.AIMTo analyse the available literature to document the up-to-date evidence on LCPD aetiology.METHODSA systematic review of the literature was performed regarding LCPD aetiology, using the following inclusion criteria: studies of any level of evidence, reporting clinical or preclinical results and dealing with the aetiology or pathogenesis of LCPD. Two reviewers searched the PubMed and Science Direct databases from their date of inception to the 20th of May 2018 in accordance with the Preferred Reporting Items for Systemic Reviews and Meta-Analyses guidelines. To achieve the maximum sensitivity of the search strategy, we combined the terms: ‘‘Perthes disease OR LCPD OR children avascular femoral head necrosis” with “pathology OR aetiology OR biomechanics OR genetics” as either key words or MeSH terms.RESULTSWe include 64 articles in this review. The available evidence on LCPD aetiology is still debated. Several hypotheses have been researched, but none of them was found decisive. While emerging evidence showed the role of environmental risk factors and evidence from twin studies did not support a major role for genetic factors, a congenital or acquired predisposition cannot be excluded in disease pathogenesis. One of the most supported theories involved mechanical induced ischemia that evolved into avascular necrosis of the femoral head in sensible patients.CONCLUSIONThe literature available on the aetiology of LCPD presents major limitations in terms of great heterogeneity and a lack of high-profile studies. Although a lot of studies focused on the genetic, biomechanical and radiological background of the disease, there is a lack of consensus on one or multiple major actors of the etiopathogenesis. More studies are needed to understand the complex and multifactorial genesis of the avascular necrosis characterizing the disease.

Impingement-free hip range of motion after osteochondroplasty and relative neck lengthening in adults with healed Perthes disease

“…The etiology of LCPD is still debated as the literature available shows major limitations in terms of great heterogeneity and a lack of high-profile studies. In the systematic review done by Pavone et al [2] which include 64 articles, they reported that in the role of environmental risk factors genetic factors, a congenital or acquired predisposition cannot be excluded in disease pathogenesis. One of the most supported theories involved mechanical induced ischemia that evolved into avascular necrosis of the femoral head in sensible patients [2].…”

Does early and aggressive management of significant extrusion of the femoral head affect the outcome of Perthes’ disease with the age of onset younger than 7 years?

“…genetic mutation [26][27][28][29]. Whole exome sequencing with detailed genome analysis in such children might help to differentiate Perthes disease with localized manifestation of skeletal dysplasia [30].…”

What are the newest outcomes about Legg-Calve-Perthes disease etiology after 110 years over its discovering ? – literature review

“…It is underlined in the literature that some disturbances during prenatal life may have an effect on the unborn skeletal system and be the baseline for developing LCPD [1,2]. However, nowadays only one factor which is tobacco has been confirmed as a factor increasing the possibility of LCPD occurrence [3].…”