PERTHE’S DISEASE

LCP is a self limiting disease characterized by

avascular necrosis of femoral head with collapse and
subsequent regeneration.
It is also known as coxa plana, pseudocoxalgia, coxa
juvenile etc. Its cause and treatment are still topics of
controversy.
Cause is unknown. The cause of AVN has been related
to hereditary, metabolic and mechanical dysfunctions.

Trauma, infection, transient synovitis also have been

implicated.
LCP occurs between 2-12 years most frequently from
4-8 years. Males are affected four times ore than
females.
Primary feature is AVN of the proximal femoral
epiphyseal region as a result of temporary loss of blood
supply. Collapse then occurs followed by resorption of
the dead bone and replacement with newly formed
immature cells.
LCP is most often grouped in 4 stages based on
radiologic findings.
Avascular stage
Fragmentation
Re ossification
Healing stage
Smaller ossific nucleus which is radiodense than neck
of femur.
A subchondral radiolucent zone called crescent sign
can also be present.
Generally no symptoms during this period.
Lasts from several months to a year.
Necrotic bone is resorbed and replaced with new
immature bone cells.
This new bone is laid on dead bone resulting in head
within a head appearance.
The bone is easily molded at this stage either into
normal or abnormal shape depending on the forces
exerted on it. Hip becomes painful with effusion and
decreased motion.
This stage lasts for 1-3 years, which is a vulnerable
phase.
 During this reparative stage remodeling continues
until femoral head is reconstituted. Bone is still
biologically plastic .
 Lasts for 1-3 years.
Residual deformity stage.
Resorbing cells are replaced by normal cells.
Femoral neck and head may be deformed as a
result of the disease process.
Joint incongruity and decreased ROM
Hip is at risk of degenerative joint disease.
Incidence is higher in first born children.
Those with low birth weights.
Children with LCP has short stature.
It occurs more in those with a lower socioeconomic
status.
It occurs bilaterally in 20% of cases.
 Group A: lateral pillar full height maintained.
 Group B:over 50 % of height is maintained.
 Group C: less than 50 % of the height is maintained.
 Results are worse than other two groups
Lateral pillar system was proposed by Herring in 1992.
In this classification femoral head is divided into 3
parts.
Lateral, Central and Medial
Involvement of the lateral pillar is more predictive of
the outcome than the total head of femur.
AP radiograph is taken to determine the classification.
Group A: less than one half of the epiphysis is
involved. Individuals do well without treatment.

Group B: greater than one half of the upper epiphysis

is involved. Poor prognosis. Results are improved with
containment.
Two basic systems of classification for LCP are used.
The Salter Thompson System Lateral Pillar System.
Salter Thomson System was developed in 1971 and is
based on subchondral fracture found in LCP. It divides
LCP into 2 groups, group A and B.
Perthe’s disease (Legg-Calvé-Perthes disease) is a
relatively common disease of the hip in
childhood, affecting 10 of every 100 000 children.
The disease was described nearly simultaneously
in the year 1910 by G. C. Perthes in Germany, A.
T. Legg in the United States, and J. Calvé in
France.
Insidious onset
Prolonged course of symptoms
Pain in hip, groin or knee can be present.
Pain aggravated with activity and relieved with rest.
A limp without pain is seen in few patients.
Antalgic gait of several months duration
Limited hip movements, particularly abduction and
internal rotation.
A flexion or adduction contracture of the hip can occur.
Atrophy of thigh, calf and gluteal muscles.
 The affected children come to medical attention
because of limping. Pain is typically located in the
groin area and is often present only during physical
activity; in 25% of patients, the pain radiates into the
thigh and knee.
 There may also be knee pain without groin or thigh

pain, and, in such cases, the diagnosis is often

delayed.
 It follows, therefore, that any child presenting with

knee pain should have the hip carefully examined as

well.
 AP and frog lateral x-rays are taken.
 Degree of femoral head involvement is seen in x ray.
 Crescent sign is best seen in frog lateral view.
 Femoral head appears fragmented.
 Bone scan
 MRI
 Arthrography
 CT
 Important to rule out infectious etiology (septic
arthritis, toxic synovitis)
 Others:

◦ Chondrolysis -Neoplasm
◦ JRA -Sickle Cell
◦ Osteomyelitis -Traumatic AVN
◦ Lymphoma -Medication
Perthes disease results when the blood supply to the
capital femoral epiphysis is blocked. There are many
theories about what causes this problem with the blood
supply, yet none have been proven. There appears to be
some relationship to nutrition. Children who are
malnourished are more likely to develop this condition.

Children who have abnormal blood clotting also have a

higher risk of developing Perthes disease. These
children have blood that clots easier and quicker than
normal. This may lead to blood clotting that blocks the
small arteries going to the femoral head.
 Late 19th century: “hip infections” that
resolved without surgery
 First described in 1910

 Clinicalrisk factors for a poor outcome

include a later age of onset, overweight,
severe limitation of the range of motion, and
female sex.
 Decreased ROM, especially abduction and
internal rotation
 Trendelenburg test often positive
 Adductor contracture
 Muscular atrophy of thigh/buttock/calf
 Limb length discrepancy
 60% of kids do well without treatment.
 AGE is key prognostic factor:
 <6yo= good outcome regardless of treatment.

◦ 6-8yo= not always good results with just

containment
◦ >9yo= containment option is questionable,
poorer prognosis, significant residual defect
The main prognostic factors are the patient's age
at the onset of the disease, the degree of
limitation of range of motion, the extent of
involvement of the femoral epiphysis, and any
additional radiographic "head-at-risk" signs.
Depending on the severity of the disease, the treatment
options range from observation and frequent follow-up
to reconstructive hip surgery. The goal of all treatments
is to prevent premature coxarthrosis. This goal is best
met by adherence to the principle of containment, i.e.,
the maintenance or restoration of joint congruence while
biological plasticity is still present.

Flatfemoral head incongruent with acetabulum= worst

prognosis
 The goal of all forms of treatment is to
prevent deformity of the femoral head
and incongruence of the affected hip. The
extent of incongruence in adolescence
determines the severity of the deformity,
and thus also the probability of early
secondary coxarthrosis.
 In order to achieve these goals, treatment is
based on the principle of containment, i.e., the
maintenance or restoration of the central
position of the femoral head. The type of
treatment that is to be provided is determined
on the basis of the radiological severity of the
disease, the presence or absence of "head-at-
risk signs," the extent of limitation of mobility
of the hip joint, and the age of the patient.
 A regularprogram of physiotherapy is
needed to optimize mobility.

 Improve ROM 1st

 Bracing:

◦ Removable abduction orthosis

◦ Petrie casts
 Wean from brace when improved X-ray healing
signs
If contracture of the adductor musculature is also
present, treatment with botulinum toxin, combined
with intensive physiotherapy, may increase the range of
motion in abduction and thereby improve containment.
During the entire course of treatment, the foremost
treatment goal is the attention of free mobility of the
hip in all directions, and especially free rotation and
abduction, with maintenance of the central position of
the femoral head in the acetabulum.
Good range of motion of the hip, with at least
30° of abduction, is a prerequisite for the
success of operative containment therapy.
Any greater restriction of hip mobility is
considered to contraindicate surgery.

Ideally, the child should be in the early phase of

the disease (the fragmentation or early repair
phase) at the time of surgery, so that the femoral
head will still possess the remodeling potential
that is present in this phase.
 If the range of motion of the hip is restricted,
an adequate range of abduction must be
achieved before surgery.
 It may be necessary for the patient to be

hospitalized for intensive physiotherapy and

supportive therapy with anti-inflammatory
drugs; sometimes, adductor tenotomy will be
necessary.
The disturbed growth of the epiphysis of the
femoral head can lead to coxa vara in the late
stage of the disease, with relative shortening of
the femoral neck and a high-standing trochanter.
In such cases, the indication for surgical
lengthening of the femoral neck or a valgus
osteotomy should be evaluated, so that leg
shortening and muscular insufficiency, if present,
can be treated, and the optimal anatomy and
function of the proximal femur can be restored.
 Check serial radiographs
◦ 3-4 months with ROM testing
 Continue bracing until:
◦ Lateral column ossifies
◦ Sclerotic areas in epiphysis gone
 Ifnon-op treatment cannot maintain
containment
 Surgically ideal patient:

◦ 6-9 years
◦ Catterral II-III
◦ Good ROM
◦ In collapsing phase
 Surgical options:
◦ Excise lat extruding head portion
Acetabular (innominate) osteotomy to
cover head
◦ Varus femoral osteotomy
◦ Arthrodesis
 Physeal arrest patterns
 Irregular head formation
 Osteochondritis dessicans (a joint
condition in which a piece of cartilage,
along with a thin layer of the bone
beneath it, comes loose from the end of a
bone.)
Follow-up visits are used to monitor the symptoms, hip mobility,
and to make sure that the condition is not deteriorating. The
surgeon will take X-rays during the recheck visits to follow the
healing of the femoral head.

Patients with Perthes disease are always at higher risk of

developing osteoarthritis of the hip. The end result is that most
patients with Perthes disease will require an artificial hip at some
point in the future.
Most patients do not develop problems for 40 years or more. How
soon patients have problems with their hip is directly related to
how much deformity is present once the condition heals.
In general, the more round the femoral hip is at that time, the
longer the hip will stay free of pain.
Exercise therapy
Quadriceps sets
 
•Sit in long sitting with your legs straight out in front of you.
•Put a rolled towel underneath your knee.
•Then squeeze your knee down into the roll towards the ground.
•Hold it for 3 seconds, and repeat 10 times.
 

Four-Way Straight Leg Raises

Flexion (front of hip)
Abduction (outside of hip)
Adduction (inside of hip)
Extension (backside of hip)
10 reps in all four directions
 
•The patient lies on the back, with buttocks at the edge

of the table.
•The left limb is kept in knee to chest position.

•The therapist applies force on the lower the thigh to

stretch in extension, while the opposite thigh is bent.

•Stretching has to be repeated 3 times and maintained

for 20 seconds.
 
•The patient lies on the back, with buttocks at the edge

of the table.
•The left limb is kept in knee to chest position.

•The therapist applies force on the lower the thigh to

stretch in extension, while the right knee is maintained

in flexion.
•Stretching has to be repeated 3 times and maintained

for 20 seconds.