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Legg-Calve-Perthes Disease in Emergency Medicine

  • Author: Jessica Hernandez, MD; Chief Editor: Trevor John Mills, MD, MPH  more...
 
Updated: Sep 23, 2015
 

Background

Legg-Calvé-Perthes disease is the eponym given to idiopathic osteonecrosis of the femoral head. It was described approximately 100 years ago as a unique disease entity affecting the pediatric population.[1] Legg-Calvé-Perthes disease can lead to hip deformities and severe degenerative arthritis.

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Pathophysiology

The pathophysiology and temporal sequence of events in Legg-Calvé-Perthes disease remains unclear; however, the following scenario is generally accepted:

  1. The blood supply to the femoral head is interrupted.[2]
  2. Bone infarction and necrosis affects the articular cartilage, subchondral bone, and the bony epiphysis.[3]
  3. Revascularization occurs and new bone ossification starts. In some cases, patients may have normal bone growth and development.
  4. With progression of the disease, bone resorption, delayed bone formation, and subchondral fracture occurs. This microdamage is usually the result of normal physical activity, not direct trauma.
  5. This may result in deformities in the femoral head, epiphyseal growth plate, and possible lesions in the metaphysis.[3]
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Epidemiology

One in 1200 children younger than 15 years is affected by Legg-Calvé-Perthes disease. Legg-Calvé-Perthes disease most commonly is seen in persons aged 3-12 years, with a median age of 7 years.The disease is familial approximately 10% of the time.[4]  

Legg-Calvé-Perthes disease is a self-limited disease if not treated. Outcome widely varies. In 15-20% of patients with Legg-Calvé-Perthes disease, involvement is bilateral.

Whites are affected more frequently than persons of other races. Males are affected 4-5 times more often than females.

In a study of girls with Legg-Calvé-Perthes disease, of the 451 patients who presented at a single large urban children's hospital from 1990-2014 with a diagnosis of Legg-Calvé-Perthes disease, 82 (18.2%) were female. The average age at presentation for girls was 6.58 years.[5]

 

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Contributor Information and Disclosures
Author

Jessica Hernandez, MD Attending Physician, Department of Emergency Medicine, Einstein Healthcare Network

Jessica Hernandez, MD is a member of the following medical societies: American College of Emergency Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Chun-Der Li, MD Fellow in Neuroradiology, Hospital of the University of Pennsylvania

Chun-Der Li, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, Radiological Society of North America, Society of Radiologists in Ultrasound, Society for Imaging Informatics in Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Trevor John Mills, MD, MPH Chief of Emergency Medicine, Veterans Affairs Northern California Health Care System; Professor of Emergency Medicine, Department of Emergency Medicine, University of California, Davis, School of Medicine

Trevor John Mills, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians

Disclosure: Nothing to disclose.

Additional Contributors

Eric M Kardon, MD, FACEP Attending Emergency Physician, Georgia Emergency Medicine Specialists; Physician, Division of Emergency Medicine, Athens Regional Medical Center

Eric M Kardon, MD, FACEP is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Medical Association of Georgia

Disclosure: Nothing to disclose.

Acknowledgements

Geofrey Nochimson, MD Consulting Staff, Department of Emergency Medicine, Sentara Careplex Hospital

Geofrey Nochimson, MD is a member of the following medical societies: American College of Emergency Physicians

Disclosure: Nothing to disclose.

Tom Scaletta, MD President, Smart-ER (http://smart-er.net); Chair, Department of Emergency Medicine, Edward Hospital; Past-President, American Academy of Emergency Medicine

Tom Scaletta, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

References
  1. Brand RA. Legg-Calvé-Perthes syndrome (LCPS): an up-to-date critical review Charles W. Goff, MD CORR 1962;22:93-107. Clin Orthop Relat Res. 2012 Sep. 470(9):2628-35. [Medline].

  2. Kim HK. Legg-Calve-Perthes disease. J Am Acad Orthop Surg. 2010 Nov. 18(11):676-86. [Medline].

  3. Kim HK. Pathophysiology and new strategies for the treatment of Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 2012 Apr 4. 94(7):659-69. [Medline].

  4. Marx J, Hockberger R, Walls R. Musculoskeletal Disorders. Marx JA, ed. Rosen's Emergency Medicine: Concepts and Clinical Practice. 7th Ed. Philadelphia, Pa: Mosby Elsevier; 2010. 174.

  5. Georgiadis AG, Seeley MA, Yellin JL, Sankar WN. The presentation of Legg-Calvé-Perthes disease in females. J Child Orthop. 2015 Aug. 9 (4):243-7. [Medline].

  6. 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. [Medline].

  7. Boss JH, Misselevich I. Osteonecrosis of the femoral head of laboratory animals: the lessons learned from a comparative study of osteonecrosis in man and experimental animals. Vet Pathol. 2003 Jul. 40(4):345-54. [Medline].

  8. Herring JA, Kim HT, Browne R. Legg-Calve-Perthes disease. Part I: Classification of radiographs with use of the modified lateral pillar and Stulberg classifications. J Bone Joint Surg Am. 2004 Oct. 86-A(10):2103-20. [Medline].

  9. Hyman JE, Trupia EP, Wright ML, Matsumoto H, Jo CH, Mulpuri K, et al. Interobserver and intraobserver reliability of the modified Waldenström classification system for staging of Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 2015 Apr 15. 97 (8):643-50. [Medline].

  10. 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. [Medline].

  11. de Sanctis N. Magnetic resonance imaging in Legg-Calvè-Perthes disease: review of literature. J Pediatr Orthop. 2011 Sep. 31(2 Suppl):S163-7. [Medline].

  12. Kaniklides C, Lonnerholm T, Moberg A. Legg-Calve-Perthes disease. Comparison of conventional radiography, MR imaging, bone scintigraphy and arthrography. Acta Radiol. 1995 Jul. 36(4):434-9. [Medline].

  13. Milla SS, Coley BD, Karmazyn B, Dempsey-Robertson ME, et al. ACR Appropriateness Criteria® limping child--ages 0 to 5 years. J Am Coll Radiol. 2012 Aug. 9(8):545-53. [Medline].

  14. Kim HK, Wiesman KD, Kulkarni V, Burgess J, Chen E, Brabham C, et al. Perfusion MRI in Early Stage of Legg-Calvé-Perthes Disease to Predict Lateral Pillar Involvement: A Preliminary Study. J Bone Joint Surg Am. 2014 Jul 16. 96 (14):1152-1160. [Medline].

  15. Sankar WN, Thomas S, Castañeda P, Hong T, Shore BJ, Kim HK, et al. Feasibility and safety of perfusion MRI for Legg-Calvé-Perthes disease. J Pediatr Orthop. 2014 Oct-Nov. 34 (7):679-82. [Medline].

  16. Milla SS, Coley BD, Karmazyn B, Dempsey-Robertson ME, Dillman JR, Dory CE, et al. ACR Appropriateness Criteria® limping child--ages 0 to 5 years. J Am Coll Radiol. 2012 Aug. 9 (8):545-53. [Medline].

  17. Kamegaya M, Saisu T, Ochiai N, Hisamitsu J, Moriya H. A paired study of Perthes' disease comparing conservative and surgical treatment. J Bone Joint Surg Br. 2004 Nov. 86(8):1176-81. [Medline].

  18. Sharma S, Shewale S, Sibinski M, Sherlock DA. Legg-Calve-Perthes disease affecting children less than eight years of age: a paired outcome study. Int Orthop. 2009 Feb. 33(1):231-5. [Medline].

  19. 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 Oct. 90(10):1364-71. [Medline].

  20. Nakamura J, Kamegaya M, Saisu T, Kakizaki J, Hagiwara S, Ohtori S, et al. Outcome of patients with Legg-Calvé-Perthes onset before 6 years of age. J Pediatr Orthop. 2015 Mar. 35 (2):144-50. [Medline].

 
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Legg-Calvé-Perthes disease. Image shows subchondral sclerosis and radiolucency in the left femoral head (stage II disease). The femoral head is slightly smaller on the left than the right.
Legg-Calvé-Perthes disease. The left subchondral radiolucency is more readily demonstrated on a frog-leg view and represents subchondral fracture.
Legg-Calvé-Perthes disease. Image shows left femoral subchondral sclerosis and radiolucency.
Legg-Calvé-Perthes disease. Image shows left femoral subchondral sclerosis and radiolucency.
Legg-Calvé-Perthes disease. Image shows flattening and early fragmentation of the left femoral head with the presence of femoral neck cysts. The femoral head is obviously smaller on the left than on the right.
Legg-Calvé-Perthes disease. Image shows loss of structural integrity of the right femoral head. Also note lateral extrusion of the right femoral head.
Legg-Calvé-Perthes disease. Coronal T1-weighted image demonstrates irregularity and distortion of the right femoral head in this 7-year-old child with known Legg-Calvé-Perthes disease. Image courtesy of Radiopaedia.org, contributed by Dr. Roberto Schubert, published at http://radiopaedia.org/cases/legg-calve-perthes-disease-4.
 
 
 
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