Systematic Review
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Orthop. Mar 18, 2019; 10(3): 145-165
Published online Mar 18, 2019. doi: 10.5312/wjo.v10.i3.145
Aetiology of Legg-Calvé-Perthes disease: A systematic review
Vito Pavone, Emanuele Chisari, Andrea Vescio, Claudio Lizzio, Giuseppe Sessa, Gianluca Testa
Vito Pavone, Emanuele Chisari, Andrea Vescio, Claudio Lizzio, Giuseppe Sessa, Gianluca Testa, Department of General Surgery and Medical Surgical Specialties, Section of Orthopaedics and Traumatology, University Hospital Policlinico-Vittorio Emanuele, University of Catania, Catania 95100, Italy
Author contributions: Pavone V, Chisari E and Testa G contributed equally to the work; Chisari E conceptualized and designed the review together with Vescio A and Testa G carried out the analysis; Lizzio C and Sessa G drafted the initial manuscript; All authors reviewed and approved the final manuscript as submitted.
Conflict-of-interest statement: All authors have no conflicts of interest to report.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Vito Pavone, MD, PhD, Associate Professor, Department of General Surgery and Medical Surgical Specialties, Section of Orthopaedics and Traumatology, University Hospital Policlinico-Vittorio Emanuele, University of Catania, Via Plebiscito 628, Catania 95100, Italy. vpavone@unict.it
Telephone: +3-909-53782273 Fax: +3-909-53782700
Received: November 2, 2018
Peer-review started: November 2, 2018
First decision: November 29, 2018
Revised: December 6, 2018
Accepted: January 10, 2019
Article in press: January 10, 2019
Published online: March 18, 2019
Abstract
BACKGROUND

Legg-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.

AIM

To analyse the available literature to document the up-to-date evidence on LCPD aetiology.

METHODS

A 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.

RESULTS

We 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.

CONCLUSION

The 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.

Keywords: Legg-Calvé-Perthes disease, Aetiology, Pathogenesis, Genetics, Risk factors

Core tip: Legg-Calvé-Perthes disease is a complex disease affecting the epiphysis of the femoral head in the paediatric population. Historically considered an osteochondrosis, it is now being referred to as an idiopathic avascular necrosis of the femoral head in the paediatric population. Despite the aetiology of the disease having been widely researched, it is still not fully understood. The major hypothesis relies on a multifactorial genesis involving mechanical, genetic and systemic conditions. Further studies are necessary to understand the complex and multifactorial genesis of the avascular necrosis characterizing the disease.