Bilateral Legg-Calve-Perthes Disease and Kienbock’s Disease in a Child With Factor V Leiden Thrombophilia

Baltzer, Heather; Riester, Scott M.; Moran, Steven L.

doi:10.1177/1558944715627274

Cited by 9 publications

(9 citation statements)

References 15 publications

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“…None of the children with bilateral disease complained of pain in the wrist or ankle suggestive of a more generalized osteochondritis syndrome. 15,16…”

Section: Resultsmentioning

confidence: 99%

Radiologic Outcomes of Bilateral and Unilateral Perthes Disease: A Comparative Cohort Study

Singh

Guddattu

Shah

2021

Journal of Pediatric Orthopaedics

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Introduction: This study was undertaken to compare the radiologic outcomes of bilateral and unilateral Perthes disease and also to evaluate the outcome of synchronous and metachronous bilateral Perthes disease. Methods: Of 353 children with Perthes disease followed up from presentation to healing during the last 10 years, 37 had bilateral involvement (11 synchronous and 26 metachronous onset). The radiologic outcomes of each hip of children with bilateral disease were compared with outcomes of 148 children with unilateral disease who were matched for age, sex, and treatment. Children with unilateral or bilateral diseases were treated with a proximal femoral varus derotation osteotomy if they fulfilled the criteria for surgery. The primary outcome measure was the shape of the femoral head at healing assessed by the Sphericity Deviation Score (SDS). Results: The children with bilateral disease were younger than those with unilateral disease (6.2 vs. 7.03 y; P < 0.001), and they had a longer duration of the disease. All other characteristics of bilateral and unilateral cases were similar. The SDS values of unilateral and bilateral disease were comparable, as were the SDS of synchronous and metachronous bilateral disease. The effect of early surgery on the evolution of the disease in bilateral cases was similar to that reported in unilateral disease. The age of onset of the disease alone influenced the SDS in bilateral cases. Conclusion:The age at onset of the bilateral disease is lower, the duration of the disease longer than that of unilateral disease, but the disease outcome is similar.

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“…None of the children with bilateral disease complained of pain in the wrist or ankle suggestive of a more generalized osteochondritis syndrome. 15,16…”

Section: Resultsmentioning

confidence: 99%

Radiologic Outcomes of Bilateral and Unilateral Perthes Disease: A Comparative Cohort Study

Singh

Guddattu

Shah

2021

Journal of Pediatric Orthopaedics

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“…Factor V Leiden is a mutation that is inherited in an autosomal and dominant manner that presents incomplete dominance and results in a variant of factor V that cannot be so easily inactivated by protein C (9). Since this mutation has been found in patients with LCPD in different studies, it could be involved in the disease (22)(23)(24)(25). However, other studies have not found this mutation or alterations in factor V in patients with LCPD (26,27), thus raising the question of whether factor V is actually involved in the etiology of LCDP.…”

Section: Discussionmentioning

confidence: 99%

Increased coagulation factors V, VIII, IX, and homocysteine in Mexican patients with Legg-Calvé-Perthes disease

Hernández-Zamora

Rodríguez-Olivas

Rosales-Cruz

et al. 2022

Preprint

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Background: Legg-Calvé-Perthes disease (LCPD) is a pediatric disorder that occurs due to the avascular necrosis of the femoral head and affects the range of motion of the hip in various degrees. Its etiology is still unknown, although it has been associated with coagulation abnormalities. In Mexico, there are no reports describing hypercoagulability markers in patients with LCPD. Therefore, our objective is to determine coagulation factors, Von Willebrand factor (VWF), and Homocysteine (Hcy) in patients with LCPD and controls.Results: Were tested 25 patients with LCPD and 50 controls, matched by sex and age. The results showed significant differences in patients whit respect to controls: Prothrombin time↘, FV↗, FVIII↗ FIX↗, and Hcy↗. These results indicate that there may be hypercoagulable states in patients, which can cause thrombotic events.Conclusions: This is the first report in Mexico with a clinical and research focus regarding patients with LCPD. The increase in FV activity, FVIII, FIX, and Hcy concentration support the hypothesis that microthrombi formation in small-caliber vessels could be causing avascularity and femoral necrosis, which are traits of LCPD.

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“…Thus, even though the topic was extensively studied, recent evidence of hypercoagulability is inconclusive. Baltzer et al[ 103 ] reported a case of a child affected by Kienbock’s disease and factor V thrombophilia who developed LCPD. The patient’s hypercoagulability state may link with the bilateral manifestation of Perthes disease.…”

Section: Discussionmentioning

confidence: 99%

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

Pavone

Chisari

Vescio

et al. 2019

WJO

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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.

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Bilateral Legg-Calve-Perthes Disease and Kienbock’s Disease in a Child With Factor V Leiden Thrombophilia

Cited by 9 publications

References 15 publications

Radiologic Outcomes of Bilateral and Unilateral Perthes Disease: A Comparative Cohort Study

Radiologic Outcomes of Bilateral and Unilateral Perthes Disease: A Comparative Cohort Study

Increased coagulation factors V, VIII, IX, and homocysteine in Mexican patients with Legg-Calvé-Perthes disease

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

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