The limping child often grabs our attention and makes us ponder many conditions, like Toddler’s Fracture, Septic Arthritis, SCFE, Osteomyelitis, and Growing Pains. One additional condition that shouldn’t be overlooked is one with an interesting name: Legg Calve Perthes Disease.
Legg Calve Perthes Disease: Basics
- Legg Calve Perthes Disease is a Juvenile form of Idiopathic Osteonecrosis of the Femoral Head. [Kim, 2012]
- Incidence = 0.4 – 29 per 100,000 children (<15 years of age) [Loder, 2011]
- Large range due to discrepancy in definitions and variability between populations.
- Male:Female = 4:1
- Described in 1909-1910 by Legg (in US), Calve (in France), and Perthes (in Germany).
- Each postulated a different etiology, but described the same findings. [Wenger, 2011]
- Today ideas continue to range from traumatic to abnormal osteogenesis to inflammatory to hypercoagulable states to genetic.
- Some have found associations with high impact sports also. [Georgiadis, 2015]
- The exact etiology, pathogenesis, and epidemiology is still debated. [Cook, 2014; Kim, 2012]
- While the exact cause is debated, studies do show that the immature femoral head is mechanically weakened following ischemic necrosis. [Kim, 2012]
- There is an imbalance of bone resorption and new bone formation.
- The relationship between the femoral head and acetabulum becomes distorted leading to the acetabular rim impinging on the femoral head. [Wenger, 2011]
- Mechanical weight bearing on the hip leads to further:
- Deformity
- Hinge abduction
- Incongruent joint
- Premature arthritis
- There are several classification systems – which also points toward persistent controversies. [Kim, 2012; Kuo, 2011; Wenger, 2011]
Legg Calve Perthes Disease: Presentation
- Age at presentation: [Cook, 2015; Loder, 2011]
- Ranges from 3 – 10 years.
- Average age = 6.5 years.
- Children from Indian subcontinent present at average age of 9.5 years.
- Unlike septic arthritis, it is more common in older children.
- Presentation can be similar to other hip pathology and is often on a spectrum of disease.
- Hip pain
- Don’t forget about “Knee” or “Thigh” pain as being referred pain from the hip!
- Limp and antalgic gait
- Limited range of motion of hip
- Findings that favor Legg Calve Perthes over others (i.e., septic arthritis or synovitis): [Cook, 2015]
- Onset: weeks – months and may have intermittent episodes reported
- “Recurrent transient synovitis” should raise your concern.
- Lack of fever
- Able to bear weight and may even have normal gait
- May have a “lurch” or “stiff hip gait”
- Helps patient avoid using hip flexors while walking.
- Mild to moderate pain
- May not even have pain during your exam.
- NSAIDs often help with pain.
- “Hip strain” in child? Keep high index of suspicion for other pathology!
- Pain on flexion and internal rotation.
- Transient synovitis often has pain at end of range of motion arc.
- Septic arthritis has severe pain throughout the range of motion arc.
Legg Calve Perthes Disease: Evaluation
- Generally, the best initial testing is with plain radiographs.
- Obtain:
- AP Pelvis
- Better than isolated hip film
- Provides information about sacroiliac joints and pelvis
- Allows comparison to other hip as well
- Bilateral frog-leg laterals (AP pelvis with the hips flexed and abducted)
- May have normal film on initial presentation.
- Look for subtle findings:
- Mild flattening of femoral head compared to other side
- Loss of epiphyseal height
- Sclerosis
- Crescent sign – lucency just under the joint line of the femoral head
Legg Calve Perthes Disease: Management
- Objectives are to: [Kim, 2012]
- Maintain containment of the femoral head in the acetabulum
- Maintain good hip range of motion
- Current treatment strategies yield only modest results.
- Non-operative strategies (ex, bracing, casting) – challenging for patients to use
- Operative strategies is generally successful, but do have cases of failure. [Nguyen, 2012; Wenger, 2011]
- Not clear that the objectives actually yield improvement in outcomes.
Moral of the Morsel
- There are a number of conditions on the Differential of Limp or Hip/Knee pain.
- Don’t overlook Legg Calve Perthes Disease just because the patient is able to walk.
- Think twice before diagnosing “Recurrent Transient Synovitis” or “Hip Strain.”
- Consider it highly if there is prolonged history of pain or episodic history of pain.
- Most will be managed as outpatient, but early diagnosis and consultation may help maintain hip functionality.
References
Divi SN, Bielski RJ.
Legg-Calvé-Perthes Disease. Pediatr Ann. 2016 Apr 1;45(4):e144-9. PMID:
27064472.
[PubMed] [Read by QxMD]
Legg-Calve-Perthes disease (LCPD) is the insidious onset of idiopathic avascular necrosis of the hip in the pediatric population. The disease encompasses a wide spectrum of pathology, from mild with no long-term sequelae to severe with permanent degenerative change of the hip joint. A pediatric patient with hip pathology may initially only present with knee or thigh pain, thus obligating the clinician to maintain awareness about the hip during ex […]
Georgiadis AG1, Seeley MA, Yellin JL, Sankar WN.
The presentation of Legg-Calvé-Perthes disease in females. J Child Orthop. 2015 Aug;9(4):243-7. PMID:
26210773.
[PubMed] [Read by QxMD]
Legg-Calvé-Perthes disease (LCPD) is uncommon in girls. The presentation of LCPD in female patients has been reported as later in onset and associated with certain high-impact activities. Our aim is to characterize the presentation of female LCPD at a large center, with particular attention to the clinical and radiographic features of late-onset disease (>ten years of age). We perceived an increasing burden of late-onset disease with adult-li […]
Hailer YD1, Haag AC, Nilsson O.
Legg-Calvé-perthes disease: quality of life, physical activity, and behavior pattern. J Pediatr Orthop. 2014 Jul-Aug;34(5):514-21. PMID:
24787306.
[PubMed] [Read by QxMD]
Legg-Calvé-Perthes disease (LCPD) is a disease in children leading to deformation of the femoral head and can be a promoter for early dysfunction of the hip and early osteoarthritis of the hip. The study of health-related quality of life, physical activity, and behavior patterns in patients with LCPD can reveal its consequences later in life and also contribute to a better understanding of the etiology of the disease. […]
Nguyen NA1, Klein G, Dogbey G, McCourt JB, Mehlman CT.
Operative versus nonoperative treatments for Legg-Calvé-Perthes disease: a meta-analysis. J Pediatr Orthop. 2012 Oct-Nov;32(7):697-705. PMID:
22955534.
[PubMed] [Read by QxMD]
Legg-Calvé-Perthes disease (LCPD) is an idiopathic avascular necrosis of the femoral head primarily affecting children of ages 4 to 12 years. There is no clear consensus on nonoperative or operative treatment protocols for pediatric patients presenting with LCPD. This study uses meta-analysis and a binary logistic regression model to analyze the radiographic outcomes of these treatment modalities in pediatric patients. […]
Kim HK1.
Pathophysiology and new strategies for the treatment of Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 2012 Apr 4;94(7):659-69. PMID:
22488623.
[PubMed] [Read by QxMD]
Legg-Calvé-Perthes disease is a juvenile form of idiopathic osteonecrosis of the femoral head that can lead to permanent femoral head deformity and premature osteoarthritis. According to two recent multicenter, prospective cohort studies, current nonoperative and operative treatments have modest success rates of producing a good outcome with a spherical femoral head in older children with Legg-Calvé-Perthes disease. Experimental studies have re […]
Loder RT1, Skopelja EN2.
The epidemiology and demographics of legg-calvé-perthes’ disease. ISRN Orthop. 2011 Sep 5;2011:504393. PMID:
24977062.
[PubMed] [Read by QxMD]
The etiology of Legg-Calvé-Perthes’ disease (LCPD) is unknown. There are many insights however from epidemiologic/demographic information. A systematic medical literature review regarding LCPD was performed. The incidence ranges from 0.4/100,000 to 29.0/100,000 children <15 years of age. There is significant variability in incidence within racial groups and is frequently higher in lower socioeconomic classes. The typical age at presentation r […]
Wenger DR1, Pandya NK.
A brief history of Legg-Calvé-Perthes disease. J Pediatr Orthop. 2011 Sep;31(2 Suppl):S130-6. PMID:
21857426.
[PubMed] [Read by QxMD]
Legg-Calvé-Perthes (LCP) disease has an extensive history that has provided an ongoing intellectual challenge for the orthopaedic community. Debate around etiology and treatment of LCP disease continues even after its initial description in the early 1900 s. In order for modern day clinicians to have a full understanding of the condition, one must be a scholar of its development. The purpose of our review will be to discuss the scientific commun […]
Kuo KN1, Wu KW, Smith PA, Shih SF, Altiok H.
Classification of Legg-Calvé-Perthes disease. J Pediatr Orthop. 2011 Sep;31(2 Suppl):S168-73. PMID:
21857433.
[PubMed] [Read by QxMD]
Although the etiology of Perthes disease remains unknown 100 years after its first description, there are many articles that describe the disease course, final outcome, and results of treatment. A system of classification of the extent and severity of the disease is essential to understanding variability of Perthes, and along with the age of the patient when first affected, is useful in predicting long-term outcomes. Published reports of treatmen […]
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