Perthe's Disease

Orthopedics

Clinicals - History

Fact Explanation
Age of presentation - 4 to 12 years This is the commonest age of presentation: between the ages of 4 to 12 years. With the mean age of onset being 7 years of age. More common among boys, with a male to female ratio of 5:1. The most significant prognostic factor is the age of onset; with a later onset (>8years) having a poorer prognosis. It is also relatively more common among Caucasians and Asians and African populations show lower prevalence. The reason is thought to be due to anatomical differences in the blood supply to the head of the femur. Other risk factors are trauma during childhood and lower socio-economic class. Age of presentation - 4 to 12 years
This is the commonest age of presentation: between the ages of 4 to 12 years. With the mean age of onset being 7 years of age. More common among boys, with a male to female ratio of 5:1. The most significant prognostic factor is the age of onset; with a later onset (>8years) having a poorer prognosis. It is also relatively more common among Caucasians and Asians and African populations show lower prevalence. The reason is thought to be due to anatomical differences in the blood supply to the head of the femur. Other risk factors are trauma during childhood and lower socio-economic class.
Limping Likely to be the first symptom noticed by the care-giver. Usually not incapacitating. Occurs due the pain that is caused by avascular necrosis of the femoral head. May rarely present as bilateral disease. Limping
Likely to be the first symptom noticed by the care-giver. Usually not incapacitating. Occurs due the pain that is caused by avascular necrosis of the femoral head. May rarely present as bilateral disease.
Groin pain Usually noticed during the child's active play. The child will not be able to recount an instance where he/she got hurt. Though the pain originates in the hip it can also be felt in the groin and knee due to a phenomenon known as referred pain. Groin pain
Usually noticed during the child's active play. The child will not be able to recount an instance where he/she got hurt. Though the pain originates in the hip it can also be felt in the groin and knee due to a phenomenon known as referred pain.
Hip pain Occurs due to the ischemic necrosis of the femoral head. Children suffering from this disease have reduced blood flow and a reduction in the velocity of blood which is independent of vessel calibre. Hip pain
Occurs due to the ischemic necrosis of the femoral head. Children suffering from this disease have reduced blood flow and a reduction in the velocity of blood which is independent of vessel calibre.
Anterior thigh pain Occurs as result of referred pain from the hip. Anterior thigh pain
Occurs as result of referred pain from the hip.

Clinicals - Examination

Fact Explanation
Reduced range of motion at the hip Occurs as a result of muscle spasm and pain. Markedly reduced range of motion especially in abduction and internal rotation. However when children are examined under anesthesia which oblivates pain, a normal range of motion is found. During re-ossification the range of movement will improve. Reduced range of motion at the hip
Occurs as a result of muscle spasm and pain. Markedly reduced range of motion especially in abduction and internal rotation. However when children are examined under anesthesia which oblivates pain, a normal range of motion is found. During re-ossification the range of movement will improve.
Leg length discrepency Due to collapse of the femoral head after it undergoes avascular necrosis. Leg length discrepency
Due to collapse of the femoral head after it undergoes avascular necrosis.
Reduced muscle bulk of thigh, calf and buttocks Occurs due to disuse atrophy due to the pain and reduced range of movement at the hip, may have Trendelenberg's (waddling) gait. Children with long-standing unresolved disease may even develop adduction contractures. Reduced muscle bulk of thigh, calf and buttocks
Occurs due to disuse atrophy due to the pain and reduced range of movement at the hip, may have Trendelenberg's (waddling) gait. Children with long-standing unresolved disease may even develop adduction contractures.
Irritability of the hip Gentle rotation of the affected hip with the leg extended often demonstrates restriction to the rolling maneuver. Irritability of the hip
Gentle rotation of the affected hip with the leg extended often demonstrates restriction to the rolling maneuver.

Investigations - Diagnosis

Fact Explanation
X-Ray of the pelvis Anteroposterior view and Frog's leg lateral view should be sought. Can be used to determine the radiographic stage of the disease.
Four clinical stages have been described( by Waldenstrom) during the active phase of the disease: stage of increased radiodensity, the stage of fragmentation, the stage of reossification, and the healed stage.
X-Ray of the pelvis
Anteroposterior view and Frog's leg lateral view should be sought. Can be used to determine the radiographic stage of the disease.
Four clinical stages have been described( by Waldenstrom) during the active phase of the disease: stage of increased radiodensity, the stage of fragmentation, the stage of reossification, and the healed stage.
Bone scintigraphy Can detect changes in bone perfusion in early stages of the disease. Changes can be seen X-ray changes and are also useful in assessing re-perfusion. Bone scintigraphy
Can detect changes in bone perfusion in early stages of the disease. Changes can be seen X-ray changes and are also useful in assessing re-perfusion.
Magnetic resonance imaging (MRI) Gadolinium enhanced MRI may be used to quantify the extent of avascularity at the initial stage of the disease, while it may also be useful in understanding the degree of revascularisation As such this may be used as prognostic indicator of possible deformity. Magnetic resonance imaging (MRI)
Gadolinium enhanced MRI may be used to quantify the extent of avascularity at the initial stage of the disease, while it may also be useful in understanding the degree of revascularisation As such this may be used as prognostic indicator of possible deformity.

Management - Supportive

Fact Explanation
Bed rest with Bucks traction This form treatment is recommended if passive hip abduction of 30 degrees or more can be achieved. Goals of treatment is to splint the limb to reduce pain while containing the hip joint. Bed rest with Bucks traction
This form treatment is recommended if passive hip abduction of 30 degrees or more can be achieved. Goals of treatment is to splint the limb to reduce pain while containing the hip joint.
Ischial leg brace Allows for ambulatory containment treatment of the patient. Ischial leg brace
Allows for ambulatory containment treatment of the patient.
Snyder Sling Good results can be obtained if treatment is instituted early. Principles are minimal weight bearing and immobilization of the joint. A sling can be used in uni-lateral disease. Snyder Sling
Good results can be obtained if treatment is instituted early. Principles are minimal weight bearing and immobilization of the joint. A sling can be used in uni-lateral disease.
Containment approach Assumes that the most important aspect in treatment of Pethes disease is prevention of deformity of the femoral head. With containment the femoral head is protected within the acetabulum. Containment position is defined as abduction and internal rotation of the extremity until the femoral epiphysis is well inside Perkins line. Several orthotic devices such as Newington brace, Toronto brace, Scottish rite brace, Broomstick plaster and Birmingham splint can be used in this method. Containment approach
Assumes that the most important aspect in treatment of Pethes disease is prevention of deformity of the femoral head. With containment the femoral head is protected within the acetabulum. Containment position is defined as abduction and internal rotation of the extremity until the femoral epiphysis is well inside Perkins line. Several orthotic devices such as Newington brace, Toronto brace, Scottish rite brace, Broomstick plaster and Birmingham splint can be used in this method.

Management - Specific

Fact Explanation
Innominate osteotomy Used to correct deficiencies in the alignment between the head of the femur and the acetabulum. The bones of the pelvis are reshaped or partially removed in order to realign the load-bearing surfaces of the joint. Innominate osteotomy
Used to correct deficiencies in the alignment between the head of the femur and the acetabulum. The bones of the pelvis are reshaped or partially removed in order to realign the load-bearing surfaces of the joint.
Femoral osteotomy The femoral head is reshaped in order to maintain alignment at the hip joint. Femoral osteotomy
The femoral head is reshaped in order to maintain alignment at the hip joint.

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  1. BERRY Daniel J and LIEBERMAN Jay R. Surgery of the Hip. 1st edition. 2011. Elsevier.
  2. BERRY Daniel J and LIEBERMAN Jay R. Surgery of the Hip. 1st edition. 2011. Elsevier.
  3. CARNEY BT, MINTER CL. Nonsurgical treatment to regain hip abduction motion in Perthes disease: a retrospective review. South Med J. 2004 May;97(5):485-8.[viewed 17 March 2014]. Available from: http://www.ncbi.nlm.nih.gov/pubmed/15180025
  4. EVANS Donald L. Legg-Calve-Perthes’ Disease: A Study of Late Results. Journal of Bone and Joint Surgery (Br). May 1958 VOL. 40B, NO. 2 [viewed 17 March 2104]. Available from: http://boneandjoint.org.uk/highwire/filestream/20004/field_highwire_article_pdf/0/168.full-text.pdf
  5. KARIMI Mohammad Taghi and MCGARRY Tony. A Comparison of the Effectiveness of Surgical and Nonsurgical Treatment of Legg-Calve-Perthes Disease: A Review of the Literature. Advances in Orthopedics Volume 2012 (2012), 7 pages. [viewed 17 March 2014]. Available from: http://dx.doi.org/10.1155/2012/490806
  6. KIM Harry KW. Legg-Calvé-Perthes Disease. J Am Acad Orthop Surg November 2010 ; 18:676-686. [viewed on 17 March 2104.] Available from: doi: 10.2106/JBJS.J.01725.
  7. Legg-Calve'-Perthes Disease. National Osteonecrosis Foundation, Johns Hopkins University, 2000 [viewed 17 March 2014]. Available from: http://nonf.org/perthesbrochure/perthes-brochure.htm
  8. MAZDA K, PENNECOT GF, ZELLER R, TAUSSIG G. Perthes’ disease after the age of twelve years: Role of remaining growth. Journal of Bone and Joint Surgery (Br). 1999 vol-81-B:696-9 [viewed 17 March 2014]. Available from: http://www.boneandjoint.org.uk/highwire/filestream/16775/field_highwire_article_pdf/0/696.full-text.pdf
  9. O'HARA JP et al. Long-term follow-up of Perthes' disease treated nonoperatively. Clin Orthop Relat Res. 1977 Jun;(125):49-56. [viewed on 17 March 2014]. Available from: http://www.ncbi.nlm.nih.gov/pubmed/880779
  10. PERRY Daniel C, GREEN Daniel J, BRUCE Colin E et al. Abnormalities of Vascular Structure and Function in Children With Perthes Disease. PEDIATRICS, July 1, 2012 Vol. 130 No. 1 e126 -e131 [viewed 17 March 2014]. Available from: 10.1542/peds.2011-3269
  11. Pediatrics Clerkship. The University of Chicago. 2103 [viewed on 17 March 2014]. Available from: http://pedclerk.bsd.uchicago.edu/page/legg-calve-perthes-disease
  12. ROWE SM, JUNG ST, LEE Lee, BAE BH et al. The incidence of Perthes’ disease in Korea, a focus on differences among races. Journal of Bone Joint Surgery (Br) December 2005 vol. 87-B no. 12 1666-1668 [viewed 17 March 2014]. Available from : doi: 10.1302/0301-620X.87B12.16808
  13. SNYDER Clarence H. A sling for use in Legg-Perthes Disease. J Bone Joint Surg Am, 1947 Apr 01;29(2):524-526 [viewed 17 March 2014]. Available from : http://jbjs.org/article.aspx?articleid=10451
  14. STANITSKI Carl L. Hip range of motion in Perthes’ disease: comparison of pre-operative and intra-operative values. J Child Orthop. Mar 2007; 1(1): 33–35.[viewed 17 March 2104] Available from doi: 10.1007/s11832-007-0009-5