Legg-Calvé-Perthes syndrome
Encyclopedia
Legg–Calvé–Perthes syndrome is a degenerative disease
of the hip joint
, where growth/loss of bone mass leads to some degree of collapse of the hip joint and to deformity of the ball of the femur and the surface of the hip socket. The disease is characterized by idiopathic
avascular osteonecrosis of the capital femoral epiphysis
of the femoral head leading to an interruption of the blood supply
of the head of the femur
close to the hip joint. The disease is typically found in young children, and it can lead to osteoarthritis in adults. The effects of the disease can sometimes continue into adulthood. It is also known more simply as Perthes disease, ischemic necrosis of the hip, coxa plana, osteochondritis and avascular necrosis of the femoral head, Legg–Perthes Disease or Legg–Calve-Perthes Disease (LCPD).
It is named for Arthur Legg, Jacques Calvé and Georg Perthes
and was first described by Karel Maydl
.
Although no-one has identified the cause of Perthes disease it is known that there is a reduction in blood flow to the joint. It is thought that the artery of the ligamentum teres femoris closes too early, not allowing time for the medial circumflex femoral artery to take over. For example, a child may be six years old chronologically but may have grown only four years old in terms of bone maturity. The child may then engage in activities appropriate for six years old child but lacking the bone strength of an older child, these activities may lead to flattening or fracture of the hip joint. Genetics do not appear to be a determining factor, but it has been suggested that a deficiency of blood factors with anticoagulant property used to disperse blood clots may lead to blockages in the vessels supplying the joint, but these have not been proven. It has also been suggested that there is a deficiency of proteins C and S which also act as blood anticoagulants; if that were the case it is possible that their deficiency could cause clot formation in ligamentum teres femoris artery and hinder blood supply to the femoral head. However there is no evidence of this, over the years many theories have been published but none have stood up to professional research.
at the hip joint and a painful or antalgic gait
. There may be atrophy of thigh muscles from disuse and an inequality of leg length. In some cases, some activity can cause severe irritation or inflammation of the damaged area including standing, walking, running, kneeling, or stooping repeatedly for an extended period of time.
The first signs are complaints of soreness from the child, which are often dismissed as growing pains
, and limping or other guarding of the joint, particularly when tired. The pain is usually in the hip, but can also be felt in the knee ('referred pain
'). In some cases, pain is felt in the unaffected hip and leg, due to the child favoring the injured side and placing the majority of their weight on the "good" leg. It is predominantly a disease of boys (4:1 ratio). Perthes is generally diagnosed between 5 and 12 years of age, although it has been diagnosed as early 18 months. Typically the disease is only seen in one hip, but bilateral Perthes is seen in about 10% of children diagnosed. (The differential diagnosis for Bi-lateral Perthes disease is Multiple Epiphyseal Dysplasia.}
Onset of pain may be up to 4 hours after inactivity. Knee pain is felt in the back of the knee rather than in the front, not unlike a localized charley horse
. This lasts for an hour or so and returns nightly on inactivity.
s of the hip confirm the diagnosis. X-rays usually demonstrate a flattened, and later fragmented, femoral head. A bone scan
or MRI may be useful in making the diagnosis in those cases where x-rays are inconclusive. Neither bone scan nor MRI offer any additional useful information beyond that of x-rays in an established case. If MRI or bone scans are necessary, a positive diagnosis relies upon patchy areas of vascularity to the capital femoral epiphysis (the developing femoral head).
Treatment has traditionally centered on removing pressure from the joint until the disease has run its course. Options include traction
(to separate the femur from the pelvis and reduce wear) braces (often for several months, with an average of 18 months) to restore range of motion, physiotherapy, and surgical intervention when necessary because of permanent joint damage. To maintain activities of daily living, custom orthotics
may be used. These devices internally rotate the femoral head and abduct the leg(s) at 45 degrees. Orthoses can start as proximal as the lumbar spine (LSO), and extend the length of the limbs to the floor. Most functional bracing is achieved using a waist belt and thigh cuffs derived from the Scottish-Rite Orthosis. These devices are typically prescribed by a physician
and implemented by a certified orthotist. For older children, the distraction method has been found to be a successful treatment, using an external fixator which relieves the hip from carrying the body's weight. This allows room for the top of the femur to regrow. Many children need no intervention at all and are simply asked to refrain from contact sports or games which impact the hip. The Perthes Association has a "library" of equipment which can be borrowed to assist with keeping life as normal as possible, newsletters, a helpline, and events for the families to help children and parents to feel less isolated.
Modern treatment focuses on removing pressure from the joint to increase blood flow, in concert with physiotherapy. Pressure is minimized on the hip through use of crutch
es or a cane, and the avoidance of running-based sports. Swimming is highly recommended, as it allows exercise of the hip muscles with full range of motion while reducing the stress to a minimum. Cycling is another good option as it also keeps stress to a minimum. Physiotherapy generally involves a series of daily exercises, with weekly meetings with a physiotherapist to monitor progress. These exercises focus on improving and maintaining a full range of motion of the femur within the hip socket. Performing these exercises during the healing process is essential to ensure that the femur and hip socket have a perfectly smooth interface. This will minimize the long term effects of the disease. Use of zoledronic acid has also been investigated.
Perthes disease is self limiting, but if the head of femur is left deformed there can be a long-term problem. Treatment is aimed at minimizing damage while the disease runs its course, not at 'curing' the disease. It is recommended not to use steroids or alcohol as these reduce oxygen in the blood which is needed in the joint. As sufferers age, problems in the knee and back can arise secondary to abnormal posture and stride adopted to protect the affected joint. The condition is also linked to arthritis of the hip, though this appears not to be an inevitable consequence. Hip replacements are relatively common as the already damaged hip suffers routine wear; this varies by individual, but generally is required any time after age 50.
Caucasians are affected more frequently than other races. Males are affected 3-4 times more often than females.. Children of sufferers of the disease themselves have a very slightly increased risk; 1 in 100 male children of adults with Legg–Calvé–Perthes syndrome also exhibit the syndrome. It is most commonly seen in persons aged 3–12 years, with a median of 6 years of age. In the US, 1 in 1200 children younger than 15 years will have this condition, while the incidence in the UK is 1 in 2000.
and Coxa Magna.
s, Pug
s, Jack Russell Terrier
s, and Dachshund
s can be affected. Hip pain is usually seen by the age of 6 to 8 months. The disease is bilateral in 12 to 16 percent of cases. X-ray
s are necessary to make the diagnosis and show increased opacity and focal lysis in the head of the femur, and later in the disease, collapse and fracture of the neck of the femur. The recommended treatment is surgical removal of the head of the femur
, but conservative treatment (rest, exercise restriction, and pain medication) may be effective in a limited number of cases (less than 25 percent, according to some studies). The prognosis is excellent with surgery.
Degenerative disease
A degenerative disease, also called neurodegenerative disease, is a disease in which the function or structure of the affected tissues or organs will progressively deteriorate over time, whether due to normal bodily wear or lifestyle choices such as exercise or eating habits...
of the hip joint
Joint
A joint is the location at which two or more bones make contact. They are constructed to allow movement and provide mechanical support, and are classified structurally and functionally.-Classification:...
, where growth/loss of bone mass leads to some degree of collapse of the hip joint and to deformity of the ball of the femur and the surface of the hip socket. The disease is characterized by idiopathic
Idiopathic
Idiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...
avascular osteonecrosis of the capital femoral epiphysis
Epiphysis
The epiphysis is the rounded end of a long bone, at its joint with adjacent bone. Between the epiphysis and diaphysis lies the metaphysis, including the epiphyseal plate...
of the femoral head leading to an interruption of the blood supply
Circulatory system
The circulatory system is an organ system that passes nutrients , gases, hormones, blood cells, etc...
of the head of the femur
Femur
The femur , or thigh bone, is the most proximal bone of the leg in tetrapod vertebrates capable of walking or jumping, such as most land mammals, birds, many reptiles such as lizards, and amphibians such as frogs. In vertebrates with four legs such as dogs and horses, the femur is found only in...
close to the hip joint. The disease is typically found in young children, and it can lead to osteoarthritis in adults. The effects of the disease can sometimes continue into adulthood. It is also known more simply as Perthes disease, ischemic necrosis of the hip, coxa plana, osteochondritis and avascular necrosis of the femoral head, Legg–Perthes Disease or Legg–Calve-Perthes Disease (LCPD).
It is named for Arthur Legg, Jacques Calvé and Georg Perthes
Georg Perthes
Georg Clemens Perthes was a German surgeon and X-ray diagnostic pioneer who was born in Moers, Germany.-Biography:...
and was first described by Karel Maydl
Karel Maydl
Karel Maydl was a Czech surgeon who was a native of Rokytnice nad Jizerou, Bohemia.In 1876 he received his medical doctorate at Prague, and following graduation was a surgical assistant to Carl Wilhelm Heine . Afterwards he worked with Eduard Albert at the Universities of Innsbruck and Vienna...
.
Cause
Legg believed trauma to be the cause, Calve ricketts, and Perthes infection. Presently, a number of factors have been implicated including heredity, trauma, endocrine, inflammatory, nutritional, and altered circulatory hemodynamics.Although no-one has identified the cause of Perthes disease it is known that there is a reduction in blood flow to the joint. It is thought that the artery of the ligamentum teres femoris closes too early, not allowing time for the medial circumflex femoral artery to take over. For example, a child may be six years old chronologically but may have grown only four years old in terms of bone maturity. The child may then engage in activities appropriate for six years old child but lacking the bone strength of an older child, these activities may lead to flattening or fracture of the hip joint. Genetics do not appear to be a determining factor, but it has been suggested that a deficiency of blood factors with anticoagulant property used to disperse blood clots may lead to blockages in the vessels supplying the joint, but these have not been proven. It has also been suggested that there is a deficiency of proteins C and S which also act as blood anticoagulants; if that were the case it is possible that their deficiency could cause clot formation in ligamentum teres femoris artery and hinder blood supply to the femoral head. However there is no evidence of this, over the years many theories have been published but none have stood up to professional research.
Signs and symptoms
Common symptoms include hip, knee, or groin pain, exacerbated by hip/leg movement. The pain feels like a tooth ache, possibly severe. There is a reduced range of motionRange of motion
Range of motion , is the distance that a movable object may normally travel while properly attached to another object. It is also called range of travel, particularly when talking about mechanical devices and in mechanical engineering fields...
at the hip joint and a painful or antalgic gait
Antalgic gait
Antalgic gait is a form of gait abnormality where the stance phase of gait is abnormally shortened relative to the swing phase. It can be a good indication of pain with weight-bearing.-Conditions associated with an antalgic gait:* Coxalgia...
. There may be atrophy of thigh muscles from disuse and an inequality of leg length. In some cases, some activity can cause severe irritation or inflammation of the damaged area including standing, walking, running, kneeling, or stooping repeatedly for an extended period of time.
The first signs are complaints of soreness from the child, which are often dismissed as growing pains
Growing pains
Growing pains are pain symptoms relatively common in children. Typically, they are located in the muscles, rather than the joints, of the leg and less commonly the arm. They are usually felt on both sides, and appear late in the day or at night, waking the child, with pain varying from mild to very...
, and limping or other guarding of the joint, particularly when tired. The pain is usually in the hip, but can also be felt in the knee ('referred pain
Referred pain
Referred pain is pain perceived at a location other than the site of the painful stimulus. An example is the case of ischemia brought on by a myocardial infarction , where pain is often felt in the neck, shoulders, and back rather than in the chest, the site of the injury...
'). In some cases, pain is felt in the unaffected hip and leg, due to the child favoring the injured side and placing the majority of their weight on the "good" leg. It is predominantly a disease of boys (4:1 ratio). Perthes is generally diagnosed between 5 and 12 years of age, although it has been diagnosed as early 18 months. Typically the disease is only seen in one hip, but bilateral Perthes is seen in about 10% of children diagnosed. (The differential diagnosis for Bi-lateral Perthes disease is Multiple Epiphyseal Dysplasia.}
Onset of pain may be up to 4 hours after inactivity. Knee pain is felt in the back of the knee rather than in the front, not unlike a localized charley horse
Charley horse
A charley horse is a popular North American colloquial term for painful spasms or cramps in the leg muscles, typically lasting anywhere from a few seconds to a few hours. Also known as a "Tommy Horse"...
. This lasts for an hour or so and returns nightly on inactivity.
Diagnosis
X-RayX-ray
X-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
s of the hip confirm the diagnosis. X-rays usually demonstrate a flattened, and later fragmented, femoral head. A bone scan
Bone scan
A bone scan or bone scintigraphy is a nuclear scanning test to find certain abnormalities in bone which are triggering the bone's attempts to heal. It is primarily used to help diagnose a number of conditions relating to bones, including: cancer of the bone or cancers that have spread to the bone,...
or MRI may be useful in making the diagnosis in those cases where x-rays are inconclusive. Neither bone scan nor MRI offer any additional useful information beyond that of x-rays in an established case. If MRI or bone scans are necessary, a positive diagnosis relies upon patchy areas of vascularity to the capital femoral epiphysis (the developing femoral head).
Treatment
The goal of treatment is to avoid severe degenerative arthritis. Orthopedic assessment is crucial. Younger children have a better prognosis than older children.Treatment has traditionally centered on removing pressure from the joint until the disease has run its course. Options include traction
Traction (orthopedics)
In orthopedic medicine, traction refers to the set of mechanisms for straightening broken bones or relieving pressure on the spine and skeletal system.There are two types of traction: skin traction and skeletal traction....
(to separate the femur from the pelvis and reduce wear) braces (often for several months, with an average of 18 months) to restore range of motion, physiotherapy, and surgical intervention when necessary because of permanent joint damage. To maintain activities of daily living, custom orthotics
Orthotics
Orthotics is a specialty within the medical field concerned with the design, manufacture and application of orthoses. An orthosis is an orthopedic device that supports or corrects the function of a limb or the torso...
may be used. These devices internally rotate the femoral head and abduct the leg(s) at 45 degrees. Orthoses can start as proximal as the lumbar spine (LSO), and extend the length of the limbs to the floor. Most functional bracing is achieved using a waist belt and thigh cuffs derived from the Scottish-Rite Orthosis. These devices are typically prescribed by a physician
Physician
A physician is a health care provider who practices the profession of medicine, which is concerned with promoting, maintaining or restoring human health through the study, diagnosis, and treatment of disease, injury and other physical and mental impairments...
and implemented by a certified orthotist. For older children, the distraction method has been found to be a successful treatment, using an external fixator which relieves the hip from carrying the body's weight. This allows room for the top of the femur to regrow. Many children need no intervention at all and are simply asked to refrain from contact sports or games which impact the hip. The Perthes Association has a "library" of equipment which can be borrowed to assist with keeping life as normal as possible, newsletters, a helpline, and events for the families to help children and parents to feel less isolated.
Modern treatment focuses on removing pressure from the joint to increase blood flow, in concert with physiotherapy. Pressure is minimized on the hip through use of crutch
Crutch
Crutches are mobility aids used to counter a mobility impairment or an injury that limits walking ability.- Types :There are several different types of crutches:...
es or a cane, and the avoidance of running-based sports. Swimming is highly recommended, as it allows exercise of the hip muscles with full range of motion while reducing the stress to a minimum. Cycling is another good option as it also keeps stress to a minimum. Physiotherapy generally involves a series of daily exercises, with weekly meetings with a physiotherapist to monitor progress. These exercises focus on improving and maintaining a full range of motion of the femur within the hip socket. Performing these exercises during the healing process is essential to ensure that the femur and hip socket have a perfectly smooth interface. This will minimize the long term effects of the disease. Use of zoledronic acid has also been investigated.
Perthes disease is self limiting, but if the head of femur is left deformed there can be a long-term problem. Treatment is aimed at minimizing damage while the disease runs its course, not at 'curing' the disease. It is recommended not to use steroids or alcohol as these reduce oxygen in the blood which is needed in the joint. As sufferers age, problems in the knee and back can arise secondary to abnormal posture and stride adopted to protect the affected joint. The condition is also linked to arthritis of the hip, though this appears not to be an inevitable consequence. Hip replacements are relatively common as the already damaged hip suffers routine wear; this varies by individual, but generally is required any time after age 50.
Incidence
Perthes is rare, occurring in approximately 5.5 of 100,000 children per year (and therefore a lifetime risk of developing the disease is just under 1 per 2000).Caucasians are affected more frequently than other races. Males are affected 3-4 times more often than females.. Children of sufferers of the disease themselves have a very slightly increased risk; 1 in 100 male children of adults with Legg–Calvé–Perthes syndrome also exhibit the syndrome. It is most commonly seen in persons aged 3–12 years, with a median of 6 years of age. In the US, 1 in 1200 children younger than 15 years will have this condition, while the incidence in the UK is 1 in 2000.
Prognosis
Children younger than 6 have the best prognosis since they have time for the dead bone to revascularize and remodel, with a good chance that the femoral head will recover and remain spherical after resolution of the disease. Children who have been diagnosed with Perthes' Disease after the age of 10 are at a very high risk of developing osteoarthritisOsteoarthritis
Osteoarthritis also known as degenerative arthritis or degenerative joint disease, is a group of mechanical abnormalities involving degradation of joints, including articular cartilage and subchondral bone. Symptoms may include joint pain, tenderness, stiffness, locking, and sometimes an effusion...
and Coxa Magna.
Legg–Calvé–Perthes disease in dogs
This is also known as aseptic necrosis of the femoral head. Toy and small breeds, particularly Toy Poodles, Yorkshire TerrierYorkshire Terrier
The Yorkshire Terrier is a small dog breed of terrier type, developed in the 19th century in the county of Yorkshire, England to catch rats in clothing mills. The defining features of the breed are its size, to , and its silky blue and tan coat...
s, Pug
Pug
The pug is a "toy" breed of dog with a wrinkly, short-muzzled face, and curled tail. The breed has a fine, glossy coat that comes in a variety of colors, and a compact square body with well-developed muscle. They have been described as multum in parvo , referring to the pug's personality and...
s, Jack Russell Terrier
Jack Russell Terrier
The Jack Russell terrier is a small terrier that has its origins in fox hunting. It is principally white-bodied smooth, rough or broken-coated which is commonly confused with the Parson Russell terrier and the Russell terrier with the term "Jack Russell" commonly misapplied to other small white...
s, and Dachshund
Dachshund
The dachshund is a short-legged, long-bodied dog breed belonging to the hound family. The standard size dachshund was bred to scent, chase, and flush out badgers and other burrow-dwelling animals, while the miniature dachshund was developed to hunt smaller prey such as rabbits...
s can be affected. Hip pain is usually seen by the age of 6 to 8 months. The disease is bilateral in 12 to 16 percent of cases. X-ray
X-ray
X-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
s are necessary to make the diagnosis and show increased opacity and focal lysis in the head of the femur, and later in the disease, collapse and fracture of the neck of the femur. The recommended treatment is surgical removal of the head of the femur
Femoral head ostectomy
Femoral Head Ostectomy is a surgical procedure that removes the femoral head and neck from the femur. FHO surgery is performed to alleviate pain. It is a salvage procedure, reserved for condition where pain can not be alleviated in any other way. It is common in veterinary surgery...
, but conservative treatment (rest, exercise restriction, and pain medication) may be effective in a limited number of cases (less than 25 percent, according to some studies). The prognosis is excellent with surgery.
External links
- Perthes Association (UK)
- - information about therapies, treatments and a comprehensive medical glossary in plain english
- Legg-Calvé-Perthes Disease The National Osteonecrosis Foundation
- Legg-Calvé-Perthes Radiology