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 !