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Legg Calve Perthes Disease

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Other Names

  • Coxa plana
  • Legg-Perthes
  • Legg Calve
  • Perthes disease
  • Idiopathic osteonecrosis of the capital femoral epiphysis of the femoral head
  • Idiopathic avascular necrosis of the capital femoral epiphysis of the femoral head
  • Osteochondrosis of the femoral head

Background

  • This page refers to avascular necrosis of the capital femoral epiphysis of the femoral head, more commonly referred to as Legg-Calve-Perthes Disease (LCPD), a disease seen in skeletally immature children

History

  • Described by Arthur Legg, Jacques Calve, and Georg Perthes in 1910[1]

Epidemiology

  • Between the ages of 3-12, mostly between 5-7 years old
  • Males to female ratio of 3.1-5.0:1[2]
  • Incidence estimates range from 0.4 to 29 per 100,000 children under the age of 15[3]

Pathophysiology

  • General
  • Interruption of vascular supply theory
    • Unclear whether it is an arterial infarction or venous congestion[4]
    • Systemic coagulopathy may play a role[5]
  • Trauma
    • Acute Trauma to immature hip may ben accessory etiologic factor[6]
    • Repetitive mechanical stress may also be implicated
  • Proposed four phase model
    • 1) Necrosis
      • Disruption of the blood supply causing infarction, bone softening and death of the femoral capital epiphysis
    • 2) Fragmentation
      • Resorption of infarcted bone.
    • 3) Reossification
      • Osteoblastic activity and reforms the femoral epiphysis
    • 4) Remodeling
      • New femoral head reshapes during growth and response to conservative treatment will usually show healing in 2-4 years [7]

Risk Factors

  • Coagulation disturbance
    • Increased coagulability including inherited coagulopathies (ie, Factor V Leiden), thrombophilias, and hypofibrinolysis
    • 2012 meta-analysis: found factor V leiden, Prothrombin II increased risk, but not MTHFR polymorphism[8]
  • Inflammatory markers
    • Increased interleukin-6 (IL-6) polymorphism (G-174C/G-597A)
  • Genetics
    • Collagen type II gene COL2A1 mutation[9]
    • Proapoptotic factor Bcl-2-associated X protein (Bax)[10]
    • Twin-twin studies have identified a genetic relationship[11]
  • HIV (Up to 5% of HIV patients have avascular necrosis of the hip)
  • Secondhand smoke exposure[12]
    • Maternal smoking[13]
  • Low socioeconomic status (SES)
    • Generally low SES[14]
    • In rural areas[15]
  • Microsomia[16]
    • Birth weight < 2.5 kg in boys
  • Anthropometrics
    • Short stature
    • Obesity[17]
  • Caucasian and Asian ethnicity [18]
  • Psychiatric
  • Mechanical stress[20]
  • Developmental
    • Delay in endochondral ossification in the proximal capital femoral epiphysis[21]
  • Other
    • Increased Vascular endothelial growth factor (VEGF) and hypoxia-inducible factor (HIF-1) in rat models[22]
    • Altered Insulin growth factor 1 (IGF-1) expression in rat models[23]

Differential Diagnosis


Clinical Features

  • History
    • Limp of acute or insidious onset from between 1 to 3 months
    • If present, pain localized to the hip with or without referred pain to the knee, thigh, or abdomen
      • May worsen with activity
    • No systemic symptoms (ie, fever, chills, weight loss, migratory joint pains) should be found
  • Physical Exam Physical Exam Hip
    • Decreased internal rotation and abduction of the hip
    • Pain on rotation referred to the anteromedial thigh and/or knee
    • Atrophy of thigh & buttock muscles
  • Special Tests

Evaluation

Legg-Calve-Perth of the left hip[25]

Radiographs

  • Standard Radiographs Hip
    • AP Pelvis, Frog leg views
  • Early Findings
    • Widening of joint space (epiphyseal cartilage hypertrophy)
    • Changes in the epiphysis (smaller, appears denser)
  • Late Findings
    • Flattening of the femoral head, fragmentation, healing (sclerosis)

MRI

  • Bone Scan and MRI may be used if X-ray are unequivocal
    • Bone scans show decreased perfusion to the femoral head
    • MRI may show marrow changes [26]

Classification

Lateral Pillar or Herring Classification

  • Clinical utility: Prognostication
    • Wiig et al found: 70% of lateral pillar A hips, 51% of lateral pillar B hips, and 30% of C hips had Stulberg I or II outcomes[27]
  • Group A: lateral pillar is at full height with no density changes
    • Consistently good prognosis.
  • Group B: lateral pillar maintains greater than 50% height
    • Poor outcome if the bone age is greater than 6.
  • Group C: Less than 50% of the lateral pillar height is maintained
    • All patients will experience a poor outcome. [28]

Stulberg classification

  • Gold standard for rating residual femoral head deformity and joint congruence on radiograph
    • Recent studies show poor interobserver and intraobserver reliability
  • I: Normal, congruent Hip
    • Arthritis does not develop
  • II: Spherical head, concentric in acetabulum on AP, frog leg lateral
    • Shortened femoral neck, abnormally steep acetabulum
  • III: Ovoid, mushroom or umbrella-shaped femoral head; not flat
    • Mild-to-moderate arthritis in adulthood
  • IV: Flat head and acetabulum, congruent joint
  • V: Flat femoral head, normal femoral neck and acetabulum with incongruent joint
    • Severe arthritis before 50 years of age

Management

  • Goal: maintain the sphericity of the femoral head and the congruency of the femur-acetabulum relationship

Prognosis

  • Wiig et al found younger patients have better outcomes[27]
    • 57% of patients <6 years were found to be Stulberg I or II
    • Only 38% of patients >6 years were found to be Stulberg I or II
  • Lateral Pillar Classification (degree of femoral head involvement: A [least] to C [most])
    • >8 years old and patients in lateral pillar group B or B/C have better outcomes with surgery
    • <8 years old and patients in group B do well regardless of treatment choice
    • Patients in lateral pillar C group do poorly regardless of age or treatment method
    • Group C patients experience poor outcomes regardless of treatment choice
  • Recovery
    • 50% of patients almost fully recover with no long-term sequelae (need citation)
  • Pain and Disability
    • 50% of patients develop pain, disability, degenerative joint disease and in their 40-50s leading to hip replacement in 60s-70s.
  • Gender
    • Female patients have worse outcomes if onset occurs >8 years of age [26]

Nonoperative

  • Indications
    • Children with bone age less than 6 or lateral pillar A involvement
  • Activity restriction and protective weight-bearing until ossification is complete
  • NSAIDs can be prescribed for comfort
  • Treatment Options: Bracing, No treatment, Range of Motion
    • Herring et al found no difference between these three treatment choices [29]

Operative

  • Femoral or Pelvic Osteotomy
    • Indications: children older than 8 years
    • Lateral pillar B and B/C have improved outcomes with surgery compared to A and C
    • Studies suggest early surgery before femoral head deformity develops
  • Valgus or Shelf Osteotomies
    • Indications: children with hinge abduction
    • Improves abductor mechanism
  • Hip Arthroscopy
    • May be used for treating mechanical symptoms and impingement[30]

Rehab and Return to Play

Rehabilitation

  • A care team comprised of a pediatric orthopedic surgeon, orthopedic nurse, and physical therapist may best benefit the patient during rehab
  • Physical therapy may be used, however, literature does not support the use of orthotics, braces or casts
  • NSAIDs may be used for symptomatic pain management [26]

Return to Play

  • Needs to be updated

Complications

  • Coxa magna (widening of the femoral head)
  • Coxa plana (flattening of the femoral head)
  • Leg length discrepancy
  • Acetabular dysplasia
  • Hip incongruency
  • Lateral hip subluxation or extrusion
  • Pediatric arthritis [31]
  • Chronic pain
  • Disability
  • Osteoarthritis of the Hip or Degenerative joint disease
  • Need for Total Hip Arthroplasty
    • Occurs in approximately 5% of cases[32]

See Also


References

  1. Legg A. An obscure affliction of the hip joint. Boston Med and Surg J. 1910;162:202–204.
  2. Loder RT, Skopelja EN. The epidemiology and demographics of legg-calvé-perthes' disease. ISRN Orthop 2011; 2011: 504393
  3. Perry DC, Hall AJ. The epidemiology and etiology of Perthes disease. Orthop Clin North Am 2011; 42: 279-283, v
  4. Inoue A, Freeman MA, Vernon-Roberts B, et al. The pathogenesis of Perthes’ disease. J Bone Joint Surg Br. 1976;58-B:453–461.
  5. Ebong WW. Avascular necrosis of the femoral head associated with haemoglobinopathy. Trop Geogr Med. 1977;29:19–23.
  6. Douglas G, Rang M. The role of trauma in the pathogenesis of the osteochondroses. Clin Orthop Relat Res. 1981;28–32.
  7. Dustmann HO. [Etiology and pathogenesis of epiphyseal necrosis in childhood as exemplified with the hip]. Z Orthop Ihre Grenzgeb. 1996 Sep-Oct;134(5):407-12.
  8. Woratanarat P, Thaveeratitharm C, Woratanarat T, Angsanuntsukh C, Attia J, Thakkinstian A. Metaanalysis of hypercoagulability genetic polymorphisms in Perthes disease. J Orthop Res 2014; 32: 1-7
  9. Su P, Li R, Liu S, Zhou Y, Wang X, Patil N, Mow CS, Mason JC, Huang D, Wang Y. Age at onsetdependent presentations of premature hip osteoarthritis, avascular necrosis of the femoral head, or Legg- Calvé-Perthes disease in a single family, consequent upon a p.Gly1170Ser mutation of COL2A1. Arthritis Rheum 2008; 58: 1701-1706
  10. Srzentić S, Nikčević G, Spasovski D, Baščarević Z, Živković Z, Terzic-Šupić Z, Matanović D, Djordjević V, Pavlović S, Spasovski V. Predictive genetic markers of coagulation, inflammation and apoptosis in Perthes disease—Serbian experience. Eur J Pediatr 2015; 174: 1085-1092
  11. Lappin K, Kealey D, Cosgrove A, Graham K. Does low birthweight predispose to Perthes' disease? Perthes' disease in twins. J Pediatr Orthop B 2003; 12: 307-310
  12. Bahmanyar S, Montgomery SM, Weiss RJ, Ekbom A. Maternal smoking during pregnancy, other prenatal and perinatal factors, and the risk of Legg-Calvé-Perthes disease. Pediatrics 2008; 122: e459-e464
  13. Perry DC, Thomson C, Pope D, Bruce CE, Platt MJ. A case control study to determine the association between Perthes' disease and the recalled use of tobacco during pregnancy, and biological markers of current tobacco smoke exposure. Bone Joint J 2017; 99-B: 1102-1108
  14. Perry DC, Bruce CE, Pope D, Dangerfield P, Platt MJ, Hall AJ. Legg-Calvé-Perthes disease in the UK: geographic and temporal trends in incidence reflecting differences in degree of deprivation in childhood. Arthritis Rheum 2012; 64: 1673-1679
  15. Kealey WD, Moore AJ, Cook S, Cosgrove AP. Deprivation, urbanisation and Perthes' disease in Northern Ireland. J Bone Joint Surg Br 2000; 82: 167-171
  16. Metcalfe D, Van Dijck S, Parsons N, Christensen K, Perry DC. A Twin Study of Perthes Disease. Pediatrics 2016; 137: e20153542
  17. Neal DC, Alford TH, Moualeu A, Jo CH, Herring JA, Kim HK. Prevalence of Obesity in Patients With Legg-Calvé-Perthes Disease. J Am Acad Orthop Surg 2016; 24: 660-665
  18. Loder RT, Skopelja EN. The epidemiology and demographics of legg-calvé-perthes' disease. ISRN Orthop. 2011;2011:504393.
  19. Loder RT, Schwartz EM, Hensinger RN. Behavioral characteristics of children with Legg-Calvé-Perthes disease. J Pediatr Orthop 1993; 13: 598-601
  20. Pavone, Vito, et al. "Aetiology of Legg-Calvé-Perthes disease: A systematic review." World journal of orthopedics 10.3 (2019): 145.
  21. Kitoh H, Kitakoji T, Katoh M, Takamine Y. Delayed ossification of the proximal capital femoral epiphysis in Legg-Calvé-Perthes' disease. J Bone Joint Surg Br 2003; 85: 121-124
  22. Zhang W, Yuan Z, Pei X, Ma R. In vivo and in vitro characteristic of HIF-1α and relative genes in ischemic femoral head necrosis. Int J Clin Exp Pathol 2015; 8: 7210-7216
  23. Neidel J, Zander D, Hackenbroch MH. Low plasma levels of insulin-like growth factor I in Perthes' disease. A controlled study of 59 consecutive children. Acta Orthop Scand 1992; 63: 393-398
  24. Dobbe AM, Gibbons PJ. Common paediatric conditions of the lower limb. J Paediatr Child Health. 2017 Nov;53(11):1077-1085.
  25. Case courtesy of Dr Mohammad Osama Hussein Yonso, Radiopaedia.org, rID: 22448
  26. 26.0 26.1 26.2 Mills S, Burroughs KE. Legg Calve Perthes Disease (Calves Disease) [Updated 2020 Jul 13]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-.
  27. 27.0 27.1 Wiig O, Terjesen T, Svenningsen S. Prognostic factors and outcome of treatment in Perthes’ disease: a prospective study of 368 patients with five-year follow-up. J Bone Joint Surg Br. 2008;90:1364–1371.
  28. Leroux J, Abu Amara S, Lechevallier J. Legg-Calvé-Perthes disease. Orthop Traumatol Surg Res. 2018 Feb;104(1S):S107-S112.
  29. Herring JA. Legg-Calvé-Perthes disease at 100: a review of evidence-based treatment. J Pediatr Orthop. 2011 Sep;31(2 Suppl):S137-40. doi: 10.1097/BPO.0b013e318223b52d. PMID: 21857427.
  30. Nguyen NA, 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.
  31. Heesakkers N, van Kempen R, Feith R, Hendriks J, Schreurs W. The long-term prognosis of Legg-Calvé-Perthes disease: a historical prospective study with a median follow-up of forty one years. Int Orthop. 2015 May;39(5):859-63.
  32. Larson AN, Sucato DJ, Herring JA, Adolfsen SE, Kelly DM, Martus JE, Lovejoy JF, Browne R, Delarocha A. A prospective multicenter study of Legg-Calvé-Perthes disease: functional and radiographic outcomes of nonoperative treatment at a mean follow-up of twenty years. J Bone Joint Surg Am 2012; 94: 584-592
Created by:
John Kiel on 30 June 2019 20:53:31
Last edited:
5 October 2022 13:10:49
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