perthes disease lcpd

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  • Dr. ANOOP G.C. Junior Resident in orthopedics

    MCH Kozhikkode

    Legg Calve Perthes Disease

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • SYNONYMS

    Coxa plana

    Osteochondritis deformans coxa juveniles

    Pseudocoxalgia

    Osteochondrosis of hip joint

    Childhood Aseptic Necrosis of Femoral Head

    Osteochondritis dessicans of Hip

    Leggs stress fracture

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • DEFINITION

    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.

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • 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 .

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • LEGG CALVE PERTHES

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • ETIOLOGICAL FACTORS - that play a role in development of illness

    Vascular supply

    Increased intra-articular pressure

    Intraosseous pressure

    Coagulation disorder

    Growth hormones

    Skeletal Growth

    Social conditions

    Genetic factors

    Attention deficit Disorders

    TRAUMA

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • 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

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • 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

    Foveolar

    Retinacular

    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

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • FEMORAL HEAD - The Blood Supply of the Immature Femoral Head

    CHILD

    Metaphyseal or no

    other vessels cross

    epiphyseal plate

    Artery of

    Ligamentum teres

    is not developed

    Solely dependent

    on Lateral

    epiphyeal arteries

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • CAFFEYS HYPOTHESIS

    Avascular necrosis of femoral head

    results from intraepiphyseal

    compression of blood supply to the

    ossification center and not due to

    external compression of vessel.

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • ETIOLOGICAL FACTORS

    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

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • ETIOLOGICAL FACTORS

    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

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • ETIOLOGICAL FACTORS

    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.

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • 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

    Skeletal Growth:

    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.

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • ETIOLOGICAL FACTORS

    Social conditions:

    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

    Genetic factors:

    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.

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • ETIOLOGICAL FACTORS

    Attention deficit Hyperkinetic Disorders : more prone to trauma

    Synovitis : predisposed due to increased intra articular pressure

    TRAUMA :

    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

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • HERIDITARY ASSOCIATIONS

    Congenital abnormalities Hemivertebrae Deafnes Imperforate anus Pyloric stenosis Epilepsy

    Cong heart disease

    Short tibia

    Undescended testis

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • PATHOLOGY

    By Waldenstrom in 1922

    4 stages

    based on microscopic and gross pathology

    Paul_Petter_Waldenstrm Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • Stages

    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

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • 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

    articular cartilage

    Thickened peripheral cartilagenois cells

    Gross contour of femoral head is unchanged

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • Stages

    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

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • Stages

    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.

    Dr.Anoop G.C.,JR,Orthopaedics,GMCK

  • EPIDEMIOLOGY

    ONSET : 18 months - skele

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