perthes disease lcpd
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Dr. ANOOP G.C. Junior Resident in orthopedics
Legg Calve Perthes Disease
Osteochondritis deformans coxa juveniles
Osteochondrosis of hip joint
Childhood Aseptic Necrosis of Femoral Head
Osteochondritis dessicans of Hip
Leggs stress fracture
PERTHES DISEASE : is a self-limiting form of osteochondrosis of the femoral capital epiphysis
of unknown etiology that develops in children commonly between the ages of 4 12 years
caused by impaired circulation in the femoral head
necrosis of the femoral epiphysis and its replacement by new bone
resulting in deformation of the femoral head.
HISTORY Described first by Waldenstrom in 1909
who mistakenly ascribed it to tuberculosis.
In 1910 was independently described by
Arthur Legg , U. S. A - February
Jacques Calve , France - July
George Perthes ,Germany - October
Hence name Legg Calve Perthes Disease
In 1922 Waldenstrom gave the correct
interpretation and described the stages .
LEGG CALVE PERTHES
ETIOLOGICAL FACTORS - that play a role in development of illness
Increased intra-articular pressure
Attention deficit Disorders
FEMORAL HEAD - vascular supply
EXTRINSIC OR EXTRAOSSEOUS ANATOMY (Crock & Chung)
1. Extra capsular ring base of neck - Formed by Medial circumflex femoral (Major)and Lateral
circumflex femoral arteries - Branches ascend in 4 groups as Ascending cervical(Extra
capsular) or Retinacular(intra capsular) arteries
- Lateral group most important. - The Lateral group pierce the capsule and enter epiphysis to
become Lateral epiphyseal arteries
2. Intra capsular ring base of head - incomplete in 57% males and 31% females. - Formed by branches of ascending cervical & retinacular
arteries. - They contribute to lateral epiphyseal arteries
3. Artery of Ligamentum teres Dr.Anoop G.C.,JR,Orthopaedics,GMCK
FEMORAL HEAD - vascular supply
INTRINSIC OR INTRAOSSEOUS ANATOMY
(Trueta & Harrison)
1. Lateral epiphyseal Arteries
- Formed by the Lateral group of Ascending cervical or Retinacular arteries
- Supplies 2/3 rd of femoral head
- Exclusive supply of suprolateral head
2. Medial epiphyseal or Artery of Ligamentum teres
- Negligible to of epipysis
3. Metaphyseal arteries
FEMORAL HEAD - vascular supply
ADULT Dr.Anoop G.C.,JR,Orthopaedics,GMCK
- TRUETAS HYPOTHESIS Infants and children
TRUETAS HYPOTHESIS In adults :cartilage growth plate disappears
& the metaphyseal arteries enter the epiphysis and thus the adult pattern of blood supply by
Metaphyseal arteries occurs .
Hence the disease doesnot occur in adults & rare after 12 year.
Supply by ligamentum teres occurs earlier in blacks - disease is rare in blacks
FEMORAL HEAD - The Blood Supply of the Immature Femoral Head
Metaphyseal or no
other vessels cross
is not developed
Avascular necrosis of femoral head
results from intraepiphyseal
compression of blood supply to the
ossification center and not due to
external compression of vessel.
Vascular : General reduction in blood flow with a significant reduction in medial circumflex artery
Normally venous drainage occurs through medial circumflex vein
In Perthes disease increased venous pressure in affected neck associated venous congestion in metaphysis
Increased intra-articular pressure : Animal experiments have shown that an
ischemia similar to that in Perthes disease can be generated by increasing the intra-articular pressure.
However, the condition of transient synovitis of the hip does not appear to be a precursor stage of Perthes disease as the increased pressure resulting from the effusion in transient synovitis does not lead to vessel closure
Intraosseous pressure: The measurement of intraosseous pressure in Perthes
patients has shown that the venous drainage in the femoral head is impaired, causing an increase in intraosseous pressure.
Coagulation disorder : Study have found a coagulation disorder in 75%
children with Perthes disease.
In most cases the disorder was thrombophilia.
protein C or protein S deficiency, elevated serum lipoprotein, Factor-V Leiden mutation, Anticardiolipin antibodies were also noted.
ETIOLOGICAL FACTORS Growth hormones :
While earlier studies found reduced levels of the growth hormone.
Recent studies have not shown any difference from control groups in respect of hormone status
Children with Perthes disease are shorter, on average, than their peers of the same age & show a retarded skeletal age (cartilaginous dysplasia).
The maturation disorder occurs between the ages of 3 and 5 years.
Both the trunk and extremities lag behind in terms of growth.
But pick up to attain normal skeletal maturity by adulthood.
Studies in the UK have shown that Perthes disease is more common in the lower social status.
The authors suggest a poorer diet during pregnancy as one possible explanation for this phenomenon.
A recent study did not confirm this theory
Studies have shown that first degree relatives of children with Perthes disease are 35 times more likely to suffer from the condition than the normal population.
Even second- and third-degree relatives show a fourfold increased risk.
Attention deficit Hyperkinetic Disorders : more prone to trauma
Synovitis : predisposed due to increased intra articular pressure
Trauma in the predisposed child ppts AVN OF FEMORAL HEAD AND development of Perthes disease
the lateral epiphyseal artery which courses
through a narrow passage is susceptible to damage
Congenital abnormalities Hemivertebrae Deafnes Imperforate anus Pyloric stenosis Epilepsy
Cong heart disease
By Waldenstrom in 1922
based on microscopic and gross pathology
Paul_Petter_Waldenstrm Dr.Anoop G.C.,JR,Orthopaedics,GMCK
Stage 1 : Incipient or synovitis stage
Lasts for 1-3 week
synovium is swollen edematous and hyperemic
joint fluid is increased
Inflammatory cell are notably absent
Stages Stage 2 : Avascular necrosis
Lasts for 6 month to 1year
Significant necrosis of bone
trabeculae are crushed into minute fragments.
Absent/pyknotic nuclei in the osteocytes
No evidence of bone regeneration
Degenerative changes in the basal layer of
Thickened peripheral cartilagenois cells
Gross contour of femoral head is unchanged
Stage 3 : Fragmentation or Regeneration
Lasts for 2- 3 year.
Dead bone infested with vascular connective tissue was actively resorbed by osteoclasts and replaced by newly formed immature bone.
Loss of epiphyseal height due to collapse of bony trabeculae and resorption of fragmented necrotic bone
Stage 4 : Healed or Residual Stage
Normal bone starts replacing necrotic bone.
Ossific nucleus is deformed assuming mushroom contour
Femoral head enlarges, flattens and subluxate.
ONSET : 18 months - skele