legg calve perthes disease-umy
DESCRIPTION
ETIOLOGY,PATHO,STAGING, MANAGEMENT ,..ALL IN ONETRANSCRIPT
Legg Calve Perthes Disease Presentor-Umesh Yadav
FIRST DESCRIBED BY LEGG (USA), AND WALDENSTORM IN 1909, AND BY PERTHES(GERMANY) AND CALVE(FRANCE) IN 1910
Disorder of the hip in young children Usually ages 4-8yrs As early as 2yrs, as late as teens Boys:Girls= 4-5:1 Bilateral 10-12% No evidence of inheritance
Epidemiology
Unknown Past theories: infection, inflammation,
trauma, congenital Most current theories involve vascular
compromise◦ Sanches 1973: “second infarction theory”
sometimes called as “coronary artery disease of hip”
Etiology
Etiology: blood supply
Infants 1. Metaphyseal arteries .2. Lat epiphyseal arteries3. Lig teres – insignificant
4 mts – 4 years1. Lat epiphyseal2. Metaphyseal art. decrease in number (due to appearance of growth plate).
Blood supply to femoral head
4 yrs to 7 years1. Epiphyseal plate forms a barrier to
metaphyseal vessels. Pre-adolescent
1. After 7 yrs arteries of lig teres become more prominent and anastomose with the lateral epiphyseal vessels.
Blood supply to femoral head
-- Susceptible child : delayed bone age-- Trauma-- Hereditary factors : controversial(HLA-A
antigens in lymphocytes)-- Coagulopathy : protein c& s-- Hyperactivity-- Passive smoking-- SynovitisFACTORS UNLIKELY TO BE ETIOLOGY--- Endocrinopathy-- Urban envt.
Etiology
Histologic changes described by 1913 Secondary ossification center= covered by
cartilage of 3 zones:◦ Superficial◦ Epiphyseal◦ Thin cartilage zone
Capillaries penetrate thin zone from below
Pathogenesis
Pathogenesis: cartilage zones
Epiphyseal cartilage in LCP disease:◦ Superficial zone is normal but thickened◦ Middle zone has 1)areas of extreme
hypercellularity in clusters and 2)areas of loose fibrocartilaginous matrix
Superficial and middle layers nourished by synovial fluid
Deep layer relies on blood supply
Pathogenesis
Physeal plate: cleft formation, amorphis debris(Bone dust), blood extravasation
Metaphyseal region: normal bone separated by cartilaginous matrix
Epiphyseal changes can be seen also in greater trochanter, acetabulum
Pathogenesis
Often insidious onset of a limp,excaberated by activity.
C/O pain in groin, thigh, knee
Few relate trauma hx Can have an acute onset
Presentation
Decreased ROM, especially abduction and internal rotation: initially due to muscle spasm
Abductor limp Trendelenburg test often
positive Muscular atrophy of
thigh/buttock/calf Limb length discrepency
Physical Exam
Coxa magna Premature physeal growth arrest Central-short neck,trochanteric
overgrowth Lateral-externally tilted head trochantric overgrowth acetublar deformity Irregular headOsteochondoirtis dessicans
RESIDUAL--
Imaging
AP pelvis Frog leg lateral Key= view films
sequentially over course of dz
Arthrography MRI role
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WALDENSTROM Modified Elizabethtown Classification Catterall classification Salter-Thompson Classification Lateral Pillar Classification
CLASSIFICAION
Four Waldenstrom stages:◦ 1) Initial stage◦ 2) Fragmentation stage◦ 3) Reossification stage◦ 4) Healed stage
Radiographic Stages
Stage of Avascular Necrosis Ischemia
A part ( anterior) or whole of capital femoral epiphysis is necrosed. On X-ray –
◦ The ossific nucleus looks smaller◦ Classically of Perthes’, looks dense◦ The articular cartilage remains viable & becomes thicker than normal – increased joint space.
PATHOGENESIS
PATHOGENESIS
Stage of REVASCULARIZATION / FRAGMENTATION Ingrowths of highly vascular & cellular connective tissue.
Necrotic trabecular debris is resorbed & replaced by vascular
fibrous tissue the alternating areas of sclerosis and
fibrosis appear on X- ray as fragmentation of epiphysis.
New immature bone laid on intact
necrosed trabeculae by creeping
substitution further increases
the density of ossific nucleus on
X-ray.
The femoral head may extrude from acetabulum at this stage.
Stage of REVASCULARIZATION / FRAGMENTATION (contd.)
PATHOGENESISStage of Ossification / Healing New bone starts forming and epiphyseal
density increases in the lucent portions of the femoral head.
Remodeling / Residual stage This is the stage of remodeling and there is no
additional change in the density of the femoral head.
Depending on the severity of the disease the residual shape of the head may be spherical
or distorted.
PATHOGENESIS
Modified Elizabeth Town classification
Stages
I Sclerotic A: no loss of height B: loss of height II Fragmentation A: early B:late III Healing A: peripheral B:>1/3epiphysis IV Healed
220 days
240 days
255 days
Stage Ia - the initial stage of the disease, characterized by sclerosis of the
epiphysis without any loss of epiphyseal height .
Stage Ib - epiphysis is sclerotic and there is loss of height of the epiphysis. In this stage the epiphysis is still in a single piece and no fragmentation is visible in either anteroposterior or lateral views .
Stage Iia- epiphysis has just begun to fragment; one or two vertical fissures in the epiphysis are seen in either view .
In stage IIb - fragmentation of the epiphysis is advanced, but there is no evidence of new bone formation lateral to the fragmented epiphysis .
In stage IIIa - evidence of new bone formation at the periphery of the necrotic fr agment; the new bone is not of normal texture and covers less than one-third the circumference of the epiphysis .
In stage IIIb - new bone is of normal texture and covers more than one-third the circumference of the epiphysis .
In stage IV the healing is complete and there is no radiologically identifiable avascular bone
extent of epiphyseal involvement and percentage of collapse as seen in x-ray (both AP and Lateral view)
most commonly used◦ 4 groups based on amount of femoral head
involvement◦ Also presence of sequestrum, metaphyseal rxn,
subchondral fx
Catterall classification
Group I-25% antrocentral head
Group II-50% antrolateral
Group III-75% head
Group IV
Lateral Pillar Classification 3 groups:
◦ A) no lateral pillar involvment◦ B) >50% lat height intact◦ C) <50% lat height intact
Simplification of Catterall Based on status of lateral margin of capital
femoral epiphysis Group A (Catterall I & II equivalent) Group B (Catterall III & IV equivalent)
Salter-Thompson Classification
Catteral ‘head at risk sign’
ClinicalRadiographic
Progressive loss of movement more of ABduction
Pain
(1) lateral subluxation of the femoral head from the acetabulum,
(2) speckled calcification lateral to the capital epiphysis,
(3) diffuse metaphyseal reaction (metaphyseal cysts),
(4) a horizontal physis (5) Gage sign
Physeal disruption
Femoral head extrusion
Gage’s sign
Rarefaction in the lateral part of the epiphysis and subjacent metaphysis.
Unilateral
Tuberculosis hip Synovitis Slipped femoral capital
epiphysis Lymphoma Eosinophilic granuloma
Bilateral
Hypothyroidism
Multiple epiphyseal dysplasia
Spondyloepiphyseal dysplasia
Sickle cell disease
DIFFERENTIAL DIAGNOSIS
60% of kids do well without tx AGE is key prognostic factor:
◦ <6yo= good outcome regardless of tx◦ 6-8yo= not always good results with just
containment◦ >9yo= containment option is questionable,
poorer prognosis, significant residual defect ◦ --Flat femoral head incongruent with
acetabulum= worst prognosis
Prognosis
CONTAINMENT of the femoral head
Minimize enlargement of the femoral head
Prevent or correct GT overgrowth
Prevent secondary degenerative arthritis of the hip
Aims of Treatment
Weight Relief
Containment by bracing or casting
Surgical Containment
Greater trochanteric arrest
Treatment options
disease progresses and resolves stage wise, which cannot be bypassed or hurried.
Improve ROM 1st
Bracing: Removable abduction orthosis Pietrie cst
-Wean from brace when improved x-ray healing signs Check serial radiographs
◦ Q3-4 mos with ROM testing Orthotic treatment is discontinued when the
disease enters the reparative phase and healing is established
Non-operative Tx
Treatment (Orthosis)
Atlanta Scotish Rite Brace
petrie abduction cast
The radiographic evidence of healing are1. Appearance of irregular ossification in the
femoral head.
2 . Increased density of femoral head should disappear.
3 . Medial segment of femoral head should increase in size and height.
4 . Metaphyseal rarefaction involving the lateral cortex of the metaphysis should ossify.
5 . There should be intact lateral column.
If non-op tx cannot maintain containment Surgically ideal pt:
◦ 6-9yo◦ Catterral II-III◦ Good ROM◦ In collapsing phase
Operative Tx
CE angle of Weiberg Indicator of acetabular depth It is the angle formed by a
perpendicular line through the midpoint of the femoral head and a line from the femoral head center to the upper outer acetabular margin.
Normal = 20 to 40 degrees Angle >25 = good, 20-25=
fair, < 20 = poor
Surgical Containment
Femoral VDRO Pelvic osteotomies
Varus 20 Shelf Derotation 20-30 Redirectional
Displacement
VARUS OSTEOTOMIES
varus osteotomy :-◦ INDICATIONS- patients with a spherical femoral head,
little or no acetabular dysplasia (center-edge angle of at least 15 to 20 degrees),lateral overloading, and a valgus neck-shaft angle of more than 135 degrees.
◦ DISADVANTAGES-varus angulation that may not correct with growth (especially in an older child),
◦ further shortening of an already shortened extremity,
◦ the possibility of a gluteus lurch produced by decreasing the length of the lever arm of the gluteal musculature,
◦ the possibility of nonunion of the osteotomy, ◦ requirement of a second operation to remove
the internal fixation
Contd.
FEMORAL OSTEOTOMY
ADVANTAGE-Anterolateral coverage of the femoral head, lengthening of the extremity (possibly shortened by the avascular process), and avoidance of a second operation for plate removal.
DISADVANTAGES-1)inability sometimes to obtain proper containment of the femoral head, especially in older children;
2)an increase in acetabular and hip joint pressure that may cause further avascular changes in the femoral head;
3)an increase in leg length on the operated side compared with the normal side that may cause a relative adduction of the hip and uncover the femoral head.
Eg.-Salter’s ostoeotomy
INNOMINATE OSTEOTOMY
SALTER OSTEOTOMY
Aims of treatment Relieve pain Correct Trendelenburg gait Minimize the risk of development of degenerative arthritis
Salvage surgery for sequelae of Perthes’ds
Valgus osteotomy
Joint distraction
Surgical dislocation and osteochondroplasty
Cheilectomy(Osteochondroplasty)
Arthrodesis
Salvage surgery Options
Greater trochanter advancement
Lengthening of the femoral neck
Improving acetabular coverage of the femoral head by periacetabular osteotomy
Salvage surgery Options
THE SHELF PROCEDURE (STAHELI)
INNOMINATE OSTEOTOMY WITH MEDIAL DISPLACEMENT OF ACETABULUM (CHIARI)
Valgus extension osteotomy indication -hinge abduction of hip Cheilectomy indication – malformed femoral head with lateral protuberance Coxa plana Chiari osteotomy indication – malformed femoral head with lateral subluxation Trochanteric advancement indication – premature capital femoral physeal arrest Greater trochanteric epiphysiodesis indication – premature capital femoral physeal arrest Shelf augmentation procedure indication – coxa magna coxa magna & lack of acetabular coverage
TREATMENT Reconstructive procedures
Patients presenting at 8+yrs Have a worse prognosis Severe femoral head deformity more likely Deformity at maturity predicts outcome Particularly if Catterall III or IV Or Herring C
(B/C) Girls have a poorer prognosis
Late Onset Perthes
In some patients collapse was more pronounced in the middle pillar rather than the lateral.
Neither the Catterall grouping nor the Herring grading correlated with the final outcome
Osteoporosis premature fusion of: the triradiate cartilage,
trochanteric growth plate and the capital femoral growth plate.
Radiological features
The outcome of the disease in adolescents is poor.
Many of the patients with the destructive pattern required salvage surgery to relieve pain. It is likely that patients with the other patterns of the disease will develop degenerative changes in due course.
UMY
Thank you for attention !