LEGG CALVÉ PERTHES DISEASE

A disorder sometimes easily mistaken for hip dysplasia is Legg Calvé Perthes disease, perhaps more
frequently referred to by the dog fancier as Legg Perthes. This is an avascular (pertaining to inadequate
blood supply), aseptic (not infected), developmental osteonecrosis (dying of bone tissue) of the femoral
head and neck, found almost entirely in toy or other small breeds. It can be described as a localized
tissue anemia. On radiographs, it often looks as if the bone is rotting away, and lameness with variable
pain is the major or only symptom. It has a history in human medicine, too. In fact, that's where it was
first discovered in 1910 by three researchers working independently. Legg (U.S.), Calvé (France) and
Perthes (Germany) saw a flattening of the femoral head (coxa plana) in affected youngsters and all first
thought that trauma was at the heart of its etiology. They determined that this ischemic osteonecrosis
of the femur in children was differentiated from osteomyelitis. Phemister in 1930 found that it occurred
also in adults. Schnelle, also in the 1930s but working with dogs, first saw the canine disorder in
Wirehaired Fox Terriers, and Moltzen Nielsen in Germany about the same time saw it mostly in the
Wires but also in a few other breeds. Since then, 3 to 10 month-old puppies of many other small, toy,
and miniature breeds have been found to be affected.

Phemister concluded that it was an avascular phenomenon rather than an infection and he coined
the term cavitation for its unique ability to hollow out bone. This is the same term used to
describe a very different and occasional phenomenon when distracting joints during the PennHIP
evaluation of laxity. Today LCPD (Legg Calvé Perthes disease) is confirmed to be a disease of
ischemia and micro-infarctions of the marrow microcirculation and is known by several different
names, including: ischemic osteonecrosis, avascular necrosis, corticosteroid induced
osteonecrosis, dysbaric osteonecrosis (when seen in deep sea divers and tunnel diggers with
caisson's disease), and radiation osteonecrosis. Several local and systemic causative or etiologic
factors (environmental and genetic) have been implicated. The following list was compiled in a
study on a similar jawbone disorder in humans, known as neuralgia-induced cavitational
osteonecrosis (NICO).

In 1920 G. V. Black described a progressive death of bone, cell by cell in human jawbones,
differing from osteomyelitis and appearing to soften the bone, often hollowing out the cancerous
portions of large areas of bony tissue or producing a soft intramedullary mass enclosing small
particles of necrotic bone. He called this new disease chronic osteitis, noted its extensive bone
destruction, and suggested that the lesion be opened freely, and every particle of softened bone
removed until good sound bone forms all the walls of the cavity. If all necrotic bone is removed,
generally, the case makes a good recovery.” Black's Disease, ischemic osteonecrosis of the
jaws, was apparently forgotten by the dental profession until the 1970s when a series of reports
began to emerge describing an unusual osteomyelitis of the jaws in patients with chronic facial
pain; different names were used, including most recently, NICO (neuralgia-inducing cavitational
osteonecrosis). Dr. J. E. Bouquot, of the Maxillofacial Center in Morgantown, WV says that its
features are seen in ischemic osteonecrosis in other bones, where it is predominantly an aseptic
and painful phenomenon. See the paragraphs under CAUSE.

SIGNS

Radiographic (X ray) signs of Legg Perthes are usually gross and discouraging, as many cases
are not referred to the vet or the specialist for diagnosis until the dog has been limping for a long
time or the disease has progressed to the point that it becomes a more real problem to the owner.
These small dogs put so little weight on their tiny hip joints that they almost can compensate for
discomfort by walking on their forelimbs instead of their four limbs”. Many are couch
potatoes” or spend much time being carried, but even then, picking up an affected dog in a
certain manner can put more pressure on the joint than normal locomotion, so pain at that time is
often the stimulus to do something about it. Owners and vets have reported incredible pain”
and constant, progressive discomfort, inability to stay long in any one position, and bone lysis
(loss through a process akin to dissolving or consuming) at other areas in the limb distal (further
away, the opposite of proximal) to the hip.

Radiographic features in NICO include:

1. Poorly demarcated radiolucency or moth eaten” appearance as seen on the radiograph,

2. Irregular vertical trabeculae (a type of bone cell),

3. A cotton wool or ground glass” radiopacity, sometimes referred to as ghost

marrow”,

4. Flecks and streaks radiating outward and giving rise to the term eagle's nest”,

5. Soap bubble radiolucency, and more.

These are similar to those in LCPD. The earliest radiographic signs of LCPD include an
increased radiodensity (opacity as seen on the radiograph) in the lateral part of the epiphysis of
the femoral head. Lateral means the part away from the mid line or medial; the outside”.
Resorption of necrotic (dying, rotting or decomposing) trabecular bone cells is next accompanied
by a lysis (dissolving or being consumed) of bone, and these are replacement attempts by the
body (similar to the attempt to replace bone that takes place during HD remodeling). Eventually
we find fracture or collapse like a frame house riddled by termites. As HD may or may not be
concurrent, the congruity of the ball and socket coxofemoral joint might still be maintained until
collapse.

Although NICO and facial neuralgias seem to be associated with more intense pain in the jaws
than is experienced in cases of ischemia of many other bones, LCPD in toy breeds is also
frequently very painful. The jaws are the only bones with large sensory nerves but there are
enough nerves near the femoral head to produce the pain response to the toxins, mediators, and
other biochemical products of the disease process. NICO is the second most common form of
osteonecrotic disease, affecting more than 68,000 U.S. adults, seen from l6 years of age to as old
as 94. As in Legg-Perthes, the disease may smolder” for years before symptoms are
stimulated. In the human jaw, this may be brought on by crown preparation or other dental work,
and in dogs, the sudden appearance may result from a small trauma, such as bumping or
jumping. In this way, it resembles osteoporosis that can be suddenly manifested by a fall or even
a hard sneeze.

CAUSE

From Etiologies for ischemic osteonecrosis”, as summarized by Dr. Bouquot:Local Factors:

o Trauma (mild or severe) or surgery

o Radiation therapy for cancer

Strong association but unproven:

o Intraosseous inflammation/infection

o Rheumatoid arthritis

o Prosthetic obstruction of blood flow to marrow

o Intraosseous malignancy (especially lymphoma and metastatic carcinoma)

Systemic (more widespread throughout the body, some more genetic) Factors:

o Corticosteroid therapy (long and short term); Cushing's syndrome

o Variable atmospheric pressures in occupation (caisson's disease, as in divers and miners)

o Alcoholism/pancreatitis

o Systemic lupus erythematosus (with or without corticosteroids)

o Familial hypofibrinolysis disease (plasminogen activator inhibitor deficiency)

o Sickle cell disease

 o Thrombophilia (Protein C & Protein S deficiencies)

o Gauchers disease

Strong association but unproven:

o Pregnancy/high dose estrogen therapy

o Antiphospholipid antibody syndrome

o Disseminated intravascular coagulation (DIC)

o Cigarette smoking

o Chemotherapy for cancer

Remember that some of the above, as is the case with numerous other diseases, may be entirely
environmental, entirely genetic, or a combination; often, a genetic tendency or weakness is
brought to light by an environmental incident. The most probable cause of LCPD is a genetic
weakness that allows abnormal or inadequate blood supply to the ossifying epiphyses. These are
the ends or caps of long bones that are changing from cartilage in the embryo to bone in the
adult. Depending upon breed and particular bone portion, all normal ossification is usually
complete by 12 months of age. Compression/pinching of the blood vessels in that area leads to
the necrosis (death) of cartilage and bone tissue. One idea was that some of these little dogs have
excess and premature levels of androgen and estrogen (hormones) that influence this process.

More than 80% of NICO patients who failed to improve after their initial surgery had one or
more coagulation disorders, including high levels of plasminogen activator inhibitor (the major
inhibitor of fibrinolysis), low levels of Protein C or Protein S or resistance to activated Protein C
(associated with a tendency to form blood clots), high levels of lipoprotein A, and other
abnormalities. These coagulation abnormalities can all be considered as having genetic origins,
as the inability of the body to produce normal biochemical or immunological systems or actions
is very often derived from defective genes (wrong chemicals or molecules).

TREATMENT

Antibiotics are over-prescribed and administered in most disorders, whether by an M.D. or a

veterinarian, but they may temporarily help to diminish the associated pain of NICO in cases
with incidental or secondary infections. However, they are very unlikely to effect cure. The
abnormal bony tissues in the human maxillary condition (NICO) usually must be surgically
removed via decortication and curettage in the case of the jaw, and similarly, complete excision
of the femoral head in LCPD. When the abnormal jawbone tissue is removed, the defect
frequently heals and pain subsides or disappears. Since such diseases are often merely indicative
of underlying systemic disorders, NICO has a strong tendency to recur and/or to develop in
additional jawbone sites, often requiring multiple repetitions of the same surgical procedures. We
have not seen anything similar in LCPD, probably because the disorders are sufficiently different
in genetic and developmental aspects. Also, because in LCPD, we remove the entire portion of
affected bone. Thirty percent of NICO's human patients have recurrence of pain after jaw
surgery, but there is nothing like that phenomenon in the canine hip disorder. Dogs that have
femoral head excision make a good” recovery in nearly all cases. Not breed-worthy or super-
athletic, but able to live a fairly normal life without pain. Various treatments have been
suggested for human LCPD, but the usual one is excision (surgical removal) of the femoral head
and neck, again with a similarity to the operation performed on dogs. Because humans are
bipedal and carry all our (greater) weight through the hips, LCPD is of greater seriousness in
humans than in small canine breeds. Fortunately, there are many reports of improvement in
surgical operations and follow-up therapy in human hips, so we don't have to face the same type
of surgery that is best for our dogs.

Conservative treatment (as opposed to ‘heroic measures such as surgery) has been suggested
for those unilaterally limping dogs (lame on only one side and supported well by the other limb)
with good congruity and no collapse or deterioration. The dog's worse limb is put into an Ehmer
sling for a time, perhaps as much as a couple of months, then the dog is kept in a crate to
minimize activity for another few weeks perhaps, during which time the dog is periodically
radiographed. If this approach is successful, the resorbed bone is replaced in a normal manner
and radiopacity returns, indicating normal bone cells and regained strength. In such an incidence,
aseptic necrosis is halted and then reversed by keeping the dog's weight off the limb. Lameness
has been reported to cease in perhaps a quarter of dogs treated conservatively, but much of this
estimate depends on owners' reports rather than always being followed up by veterinary
examination. In my opinion, surgery in dogs is almost demanded by the history of the disease in
canines, and the results I have seen.

A syndicated column called To Your Good Health in the Clarksburg (WV) Telegram of June 30,
1994 included a brief discussion by Paul Donohue, M.D., responding to a reader's request for
advice. Her 8 year old child had recently been diagnosed with Legg Calvé Perthes disease and
she saw no improvement after 3 months in a brace. By the way, human infants with HD are put
into slings or casts, which keep the legs spread apart until the joint begins to strengthen. (Did you
know that people get HD, too?) Anyway, Dr. Donohue told her that the Legg Calvé Perthes
disorder involved a cutting off of the blood supply to the epiphysis (top part of the femur) and
that it might take more than a year for the brace to rest the hip enough so that restoration of blood
supply can help restore bone there. If unsuccessful after that long a wait, surgery may be needed.
So you see, your dogs aren't the only ones at risk for this problem.

Some cases of Legg-Perthes go unreported or misdiagnosed. To some veterinarians, the

radiograph looks like hip dysplasia, and it is not sent in to OFA, GDC, or PennHIP for diagnosis
and recording of data. If you come across a confirmed case of Legg Perthes in your non-toy
breed, please get me a copy of the radiograph and medical report, even if only on loan till I can
make a copy for my orthopedics files.

Pathophysiology
Understanding the pathophysiology of Perthes' disease has come a long way since its first
description. The disease goes through 4 stages in 2 to 4 years following first presentation:

 Stage 1, ischaemia: a variable sector of the femoral head is involved depending on the
severity. Radiographs are normal. The femoral epiphysis stops growing during this period
which lasts 6 to 12 months. The articular cartilage continues to grow and is thickened.
This is evident as "Waldenstrom sign" (increased joint space and apparent mild
pseudosubluxation) on x-ray.

 Stage 2, resorption, fragmentation, re-vascularisation and repair: a subchondral fracture

may be seen in the early stages in the infracted area (crescent, Salter's or Caffrey's sign).
Dead trabecular fragments are resorbed and replaced with fibrous tissue which may
become calcified. Loss of structural support results due to resorption of the underlying
cancellous bone in the proximal femoral epiphysis. This leads to the deformation of this
epiphysis when subjected to the normal weight-bearing forces through the hip joint. Bone
is re-vascularised with new lamellae laid on dead trabeculae resulting in the necrotic
bony nucleus appearing fragmented. Resorption is usually complete after 12 to 18
months. Cysts appear in the proximal femoral metaphysis; increased severity could lead
to osteolysis of the superolateral portion of the femoral head (Gage sign on x-ray).

 Stage 3, re-ossification and resolution: re-ossification typically starts at the epiphyseal

margin (paraphyseal ossification). Occasionally, re-ossification through the physis results
in a bony bridge leading to growth arrest in the femoral neck. Resolution is usually
complete within 6 to 24 months resulting in healing, or a residual deformity in more
severe cases.

 Stage 4, re-modelling: femoral head collapse during re-modelling can lead to flattening
and distortion of the head. Five classes of structural hip joint changes, based on the
sphericity of the femoral head and associated acetabular congruence, have been
described. These classes are closely linked to the final prognosis of degenerative changes
in the hip joint and demonstrate that sphericity is not as important a prognostic variable
as the hip joint congruence. An aspherical femoral head with a good congruence of the
joint will at worst lead to a moderate hip osteoarthritis. However, an aspherical
incongruous joint will lead to severe osteoarthritis at a younger age.
 Stulberg classification and prognosis of future hip arthritis

legg-Calve-Perthes Disease (LCPD)

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What is Legg-Calve-Perthes Disease?

Legg-Calve-Perthes is a disease of the hip. With this disease, the head of the femur (thighbone)
that sits in the hip joint breaks down. This happens because the hip joint does not get enough
blood. The disease is temporary and will resolve over time.

Who gets Legg-Calve-Perthes Disease?

A child with this disease is often very active compared to other children. The onset of the disease
usually occurs when the child is between 3 and 12 years old. It is most common in children ages
5 to 7 years old. Boys are 3 to 5 times more likely than girls to develop the disease. 

What are the symptoms?

The child often has a limp and may lean side to side when walking. The limp may get worse late
in the day or after activities, but gets better with rest.

Some children complain of pain in the groin, front of the thigh, or knee that gets worse with
physical activity. The pain often is worse late in the day and some children may have pain at
night.
Most children have muscle spasms that limit the movement in their hip.

How is it Diagnosed?

X-rays are used to confirm diagnosis. A bone scan or MRI may also be used.

What are the Stages of Legg-Calve Perthes Disease?

1. Initial Stage (lasts about 6 months)

o The child’s symptoms may get better and then get worse

2. Fragmentation Stage (lasts about 8 months)

o This is the stage where the head of the femur breaks down
o The child’s pain and limp become more obvious
o There is more loss of motion in the hip
o The degree of symptoms may vary from child to child

3. Healing Period (lasts about 4 years)

o New bone grows in the head of the femur
 The pain and limp usually start to improve
 Some limitation of hip motion continues
  

o The child will gradually return to normal activities

4. Residual Stage (till growth is done)

o The shape of the head of the femur may continue to change until growth stops

What is the Treatment for Legg-Calve-Perthes Disease?

Your doctor may tell your child to limit impact activities, like jumping and running. Your child
may need to use a walker or crutches. This is important if the child has a lot of pain with
walking.

Your doctor may also suggest physical therapy to:

 Stretch muscles that are tight

 Strengthen muscles around the hip
 Work on walking to reduce limping