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Legg Calve Perthes Dr.Aftab Qadir

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Osteochondritis

Legg Calve PerthesDr.Aftab Qadir

Osteochondritis

Osteochondritis is a disease of epiphyses, beginning as necrosis and followed by healing

Osteochondritis of the femoral capital epiphysisSynonym:Coxa plana,WaldenstromCharacterized by idiopathic avascular osteonecrosis of the capital femoral epiphysis of the femoral headLegg Calve Perthes

Epidemiology

Disorder of the hip in young children

Usually ages 4-8 years

Boys:Girls= 4:1

Usually unilateral

ETIOLOGY Idiopathic

Common in low socioeconomic group

Parents of the effected children are often elderly

Low birth weight babies

Associated congenital anomalies

Pathology

Ischemia

Epiphysis necrosis

Growth arrest

Dense necrotic bone

Replaced by Vital bone

Clinical Presentation

Patients usually limp while walkingKnee pain, constant pain in groin or thighLimited range of movementHip stiffness, which limits motion in the hipAtrophy of muscles in the upper thighShortening of the leg or unequal length of legs

Four phases of Legg-Calve Perthes Disease Waldenstrom stages:1) Initial stage2) Fragmentation stage3) Re ossification stage4) Healed stage

Initial Stage Early radiographic signs:Failure of femoral ossific nucleus to growWidening of medial joint spaceIrregular physeal plateBlurry/ radiolucent metaphysis

Fragmentation Stage

Bony epiphysis begins to fragmentAreas of increased lucency

Re ossification StageNormal bone density returnsAlterations in shape of femoral head and neck evident

Healed StageLeft with residual deformity from disease and repair process

Perthes' disease. A series of radiographs showing the stages of healing. (A) The initial radiograph shows a flattened, sclerotic femoral head. (B) An osteotomy is performed. (C, D) Later films show resorption of the sclerotic dead bone and its replacement with vital bone, resulting in a mushroom shaped femoral head.

Imaging

Plane radiography (AP pelvis and Frog leg lateral)UltrasoundArthrographyCT RadionuclideMRI

Early radiographic signs of LCPD

Small femoral epiphysisSclerosis of the femoral head with sequestration and collapsewidening of the joint space

The flattened appearance on the affected side, compared to the smooth curve of the femoral head on the normal side.

Late signs of LCPD on radiographs

A radiolucent crescent line representing a subchondral fractureFemoral head fragmentation and femoral neck cysts and coxa plana

Coxa magna, or remodeling of the femoral head, which becomes wider and flatter, similar in appearance to a mushroom

The left femoral neck is broadened, the metaphysis sclerotic with focal areas of lucency, the growth plate irregular and the femoral head flattened and sclerotic. It is uncovered laterally. The joint space appears widened.

ultrasoundAssess hip joint for effusions.Assessment of epiphyseal cortexArticular cartilage assessment

Normal anatomy of the anterior hip joint capsule. fce = femoral capital epiphysis; fm = femoral metaphysis; between cursors = both layers of joint capsule (hyperechoeic to muscle); I = iliopsoas muscle; small arrows = echogenic interface between joint capsule layers.

Normal right hip compared to the left hip with an effusion. Hyperaemia of the soft tissues is evident on the left side. The machine settings are kept the same for comparison.

Transverse image of both hips at the level of the neck of femur. Fluid distends the joint capsule on the left side (as marked by calipers).

Perthes disease in a 4 year old boy with fragmentation of the femur epiphysisPerthes disease with irregular flattened epiphysis and joint effusion

ArthrographyDoes not diagnose LCPD but determine:Size and shape of the articular cartilagePresence or absence of congruity

(A) Gross Perthes' disease of the femoral head. (B) The outline of the cartilaginous epiphysis is somewhat dome shaped with slight flattening and broadening laterally. The lateral aspect of the femoral head is uncovered but in the neutral position is in congruity. (C) In abduction there is pooling of contrast agent medially within the joint.

COMPUTED TOMOGRAPHYEarly signs of LCPD on CT scans include:Bone collapseCurvilinear zones of sclerosisSubtle changes in bone trabecular pattern

Late signs of the disease on CT scans include:Central or peripheral areas of decreased attenuationIntraosseous cystsCoronal reconstructions can show subchondral fractures, or collapse of the articular surface.

Legg-Calv-Perthes disease. Axial nonenhanced CT scan through the hip joints shows the loss of structural integrity of the right femoral head.

Legg-Calv-Perthes disease. Nonenhanced axial CT section through the hip joints obtained at a different level in the same patient. The scan shows the loss of structural integrity of the right femoral head, with acetabular subchondral sclerosis

Legg-Calv-Perthes disease. Coronal reconstruction shows flattening, sclerosis, and early fragmentation of the right femoral head

Radionuclide scanTechnetium scan images of bone depend in part on blood flow to boneAvascular areas are seen as scan defects(photopenic void)

Magnified pinhole views from a bone scan show decreased accumulation of radiopharmaceutical in the lateral aspect of the left femoral head (arrow), caused by disruption of the blood supply to the femoral head. The normal right femoral head is shown for comparison.

MRIIrregular foci of low signal intensity or linear segments. an intra-articular effusionsmall, laterally displaced ossification nucleus femoral head deformity.

Legg-Calv-Perthes disease. Axial T1-weighted MRIs through the femoral heads show low signal intensity in the left femoral head.

Legg-Calv-Perthes disease. Coronal T2-weighted MRIs show irregularity and flattening of cortical margins of the left femoral epiphysis. A mild joint effusion

Differential Diagnosisrule out infectious etiology(septic arthritis, toxic synovitis)

Others:Sickle Cell anemiaOsteomyelitis Traumatic AVNNeoplasm Medication-steroid

Few Cases

The leftcapitalfemoral epiphysis is seen collapsed and sclerotic with low signal in all sequences and irregular contour as well as metaphyseal cystic lesion. Thickening of the articular cartilage is also noted with subsequent widening of the joint space. Bilateral hip joint effusions is seen, more on the left side. Relative loss of girth of left gluteal and thigh muscles with no abnormal signal within.

Thank You

also known as Ischemic Necrosis of the Hip or Avascular Necrosis of the Hip.LCPD represents idiopathic avascular necrosis of the femoral headIn adults, the corresponding condition is termed Chandler disease. *The disease is bilateral in 10-20% of patientsBilateral disease is even more common in boys (M : F = 7 : 1)

The age of onset is earlier in girls and the prognosis worse.If symmetry is present, hypothyroidism or multiple epiphyseal dysplasia should be excluded.

*There is an increased incidence of associated congenital anomalies, including congenital heart disease, pyloric stenosis, hernia, renal anomalies and undescended testes.

at presentation, show skeletal growth retardation in the hands.

*The femoral head is supplied with blood from the medial circumflex femoral and lateral circumflex femoral arteries, which are branches of the profunda femoris artery.Following ischaemia, the ossific nucleus of the epiphysis necroses, causing growth arrest. The overlying cartilage, which is supplied by synovial fluid, survives and thickens especially in the nonweight- bearing regions, medially and laterally. Dense, necrotic bone is slowly absorbs and is replaced by vital bone.

*Following ischemia, the ossific nucleus of the epiphysis necroses, causing growth arrest. The overlying cartilage, which is supplied by synovial fluid, survives and thickens especially in the non weight- bearing regions, medially and laterally. Dense, necrotic bone is slowly absorbs and is replaced by vital bone.

*results in a subchondral fracture or a compaction fracture with continued loading of the hip in the early stages of Legg-Calv-Perthes disease.*A drawing representing a normal femoral head and an infarcted femoral head in an early stage of revascularization. Ischemic necrosis produces extensive cell death in the deep layer of the articular cartilage. This is the growth cartilage responsible for the circumferential growth of the secondary center of ossification. The ischemic damage produces a growth arrest of the secondary center, which may not be restored symmetrically during the healing process and produces growth disturbance of the secondary center. Revascularization of the infarcted femoral head is associated with a predominance of resorptive activity, as shown in the drawing*There are four phases of Legg-Calve Perthes Disease which are as follows:1. Increased density of femoral head possibly leading to fractures 2. Bone undergoes fragmentation and reabsorption 3. Growth of new bone 4. Reshaping of new bone

*Increase bone density*Progression of Perthes' DiseaseNecrosis - Fragmentation - Healing - Remodelling

**Catterall (1971) has grouped Perthes' disease according to the degree of epiphyseal involvement as assessed radiologically.

*Asequestrum is a piece of deadbonethat has become separated during the process of necrosis from normal bone

Prognosis depends on the degree of radiological involvement.*Key= view films sequentially over course of disease

*This is especially useful in children with-Perthes disease-slipped capital femoral epiphysis

* Lateral displacement of the femoral head. A subcortical fissure in the femoral ossific nucleus. This sign is seen early in the disease but is transient. It is best seen in the ` frog' lateral view Reduction in size of the ossific nucleus of the epiphysis. This is found in some 50% of cases and is due to growth retardation. The medial joint space then seems wider. Increase in density of the femoral ossific nucleus. This is due to trabecular compression, dystrophic calcification in debris and creeping substitution repair, with laying down of new bone on the pre-existing trabeculation Metaphyseal broadening and irregularity. The neck may end up shortened

*Coxa[L.]1.hip.2.hip joint.coxamagnabroadeningoftheheadandneckofthefemur.coxaplanaosteochondrosisofthecapitularepiphysisofthefemur.coxavalgadeformityofthehipwithincreaseintheangleofinclinationbetweentheneckandshaftofthe femur.coxavaradeformityofthehipwithdecreaseintheangleof inclinationbetweentheneckandshaftofthefemur.

*X-ray of stage 2 showing a subchondral fracture line (yellow arrow) and fragmentation (red arrow)*crescent sign that is associated with avascular necrosis (AVN)A Conventional radiograph of the right femur in the frog-leg position shows subchondral area of radiolucency(arrows)in the anterolateral aspect of the proximal femoral head*Legg-Calve-Perthes Disease both femoral epiphyses show extensive destruction, the acetabula are deformed**Ultrasonography is useful in establishing the diagnosis of transient synovitis of the hip and the onset of LCPD.

scanning in the sagittal plane from the front of the patient. If there is at least 3 mm of fluid depth, a difference between the two sides of 2 mm, and convexity of the capsule, then a decision can be made to aspirate fluid, so excluding infection and relieving pain

*Anarthrogramis a series of images, oftenX-rays, of ajointafterinjectionof a contrast medium

Congruithaving the same size and shape**In the early stage, when no radiological abnormality is yet visible in the child with an avascular lesion, a defect is seen in the femoral head image on radionuclide scanning.Characteristic features include a photopenic void in proximal femoral epiphyses as compared with the contralateral side.

*Perthes' disease. (A) The lateral aspect of the right femoral head does not show up on radionuclide scanning. (B) On X-ray, the involved area looks smaller than on the scan. The right ossific nucleus is flattened, irregular and smaller than that on the normal left side. It shows collapse. The growth plate and metaphysis are irregular.*The ossific nucleus flattens and the normal bright signal related to marrow fat diminishes following' loss of the normal circulation. The signal seen from this region varies with the stage of disease and healing, and may range from low early on in the disease to a mixture of high and low when revascularisation occurs or if cysts are present. The bone deformity is visualized. Metaphyseal irregularity is seen and an abnormal relationship of the entire head to the acetabulum.MRI also shows thickening of the non-ossified cartilage of the femoral head, especially laterally, and of the acetabulum, especially the labrum. The degree of acetabular covering of the developing femoral head, as well as articular congruity, is seen both at MRI and arthrography, though arthrography allows the relationship between the head and socket to be assessed dynamically as a precursor to surgery

irregular foci of low signal intensity or linear segments replace the normal high signal intensity of bone marrow in the femoral epiphysis on T1- and T2-weighted images.

*Legg-Calve-Perthes Disease.AP and frog-lateral views of both hips show a right capital femoral epiphysis which is smaller in size (black arrows) than the left (white arrow), an early sign of this disease.*Frontal radiographs of the pelvis demonstrate sclerosis and fragmentation of the left proximal femoral epiphysis (arrows) in this young boy, compatible with advanced stage of Legg-Calve-Perthes disease.*The left superior femoral epiphysis is markedly flattened, sclerotic and appear to be undergoing fragmentation. Alignment is unremarkable and the pelvis and acetabulum appear normal.The right hip appears normal.

*BilateralPerthe's diseasein a 17 year old male.*Legg-Calv-Perthes disease. Technetium-99m diphosphonate bone scan shows a photon-deficient defect in the right femoral head.**