Legg–Calvé–Perthes syndrome

The Perthes disease is an orthopedic children's disease. The cause is a circulatory disorder (ischemia) and death (necrosis) of bone tissue of the femoral head. The children develop limping, knee pain and hip rotation restrictions.

The Perthes disease was discovered in 1910 by the German surgeon Georg Clemens Perthes. At the same time made ​​more first descriptions by Jacques Calvé in France and Arthur T. Legg in America, which is why the term " Legg - Calvé -Perthes ' ​​disease" is commonly used in the English -speaking world.

Synonyms

• Legg - Calvé - Perthes ( Waldenstrom's ) syndrome ( LCP)
• Osteochondropathia deformans coxae juvenilis
• Coxa plana idiopathic
• Juvenile femoral head necrosis
• Idiopathic infantile femoral head necrosis
• Maydl disease

Occurrence

The prevalence is approximately 1:1200. The disease occurs predominantly in boys with white skin color ( they are about 4 times more frequently affected than girls) usually between 5 and 9 years (rarely even to the end of puberty, but even with 2 years ) on. In approximately 15% of children, both sides are affected simultaneously. In addition to the osteochondritis dissecans of Perthes' disease is the most common aseptic bone necrosis. The disease is with an annual incidence of five cases per 100,000 inhabitants (exact data for the Federal Republic of missing) relatively common.

Causes

The causes of Perthes' disease are still largely unknown and so some possible causes are discussed:

• Circulatory disorders: Discussed are any existing vascular malformations that impair the blood supply to the femoral head also in normal vascular bed.
• Hormonal dysregulation
• Pressure increase in the bone or joint space
• Genetic factors: Here is a multifactorial inheritance is suspected. Particularly direct relatives (eg siblings) carry a significantly increased risk

In Perthes disease is a likely caused by circulatory disorders disease of the femoral head in childhood. In the early stages it leads to joint irritation with joint effusion, so that there is a similarity with rheumatic diseases. In the course of regular adhesive occurs sintering together of Hüftkopfkugel, often associated with a lateral Emigration from the joint ball bearing in the pelvis, also acetabulum. Later, there is a permanent deformation of the head and socket with a corresponding movement disorder. The leg is shortened. The early wear of the hip joint is predetermined. In children aged 2 to 12 years, must be considered in radiation of pain in the knee and Gehfaulheit to this disease in the occurrence of hip pain and limping. With X-rays the bone, but not the cartilaginous changes of the hip joint can be recognized.

Typical behavior of the radiograph

• Initial stage: Apparent joint space widening in the hip joint.
• Infraktionsstadium: Subchondral Infraktionslamelle " Konturdoppelung "
• Condensation stage: In this stage, a compression of the bone can be seen.
• The fragmentation: A scholliger collapse of the femoral epiphysis is seen with flattening and possibly cross- broadening and protrusion of the femoral head from the pan.
• Reparationsstadium: It comes to a gradual re - ossification of the femoral head.
• Ausheilungsstadium: The conversion processes are completed, under certain circumstances is a deformity of the femoral head before, when it has come in the reparative improper load.

Duration: Usually two to four years, but also shorter and longer courses are possible of up to six or eight years.

First signs are restricted movement and / or pain in Hüft-/Kniebereich. In the sonogram a joint effusion is diagnosed. In addition, incipient changes in the epiphysis are front visible in the fragmentation. Changes are visible in the X-ray image from Infraktionsstadium. In case of clinical suspicion magnetic resonance imaging ( MRI) can detect the disease already in the initial stage.

In the course when it comes Perthes' disease of the hip joint to a Hüftkopfvergrößerung, which can lead to joint ball bearing in the basin to a mismatch of Hüftkopfkugel. Both parts of the joint are difficult to deform. One speaks of a loss of containment. Deformities in the sense of containment loss (beginning or developed) can be treated surgically. These operations include a division of the tank ( pelvic Salter ) and the femur ( femoral osteotomy Intertrochanteric varus ) to produce the congruence of the hinge again. The introduced implants must be removed.

The imaging must be repeated at least every four months until the regeneration stage is reached in seldom attack or high-risk children. Thereafter, no further deformation is expected, except for a Trochanterhochstand. This should be slowed by the obliteration of the growth plate in growth, so that no limping gait arises. Whether an X-ray or MRI are used, is also not uniform. Furthermore, a arthrography for the assessment of the containment is possible. Recently, there are also studies of the containment diagnostic ultrasound in comparison to MRI and X-ray, which can give a good indication of the joint situation.

Diagnosis

It comes to the limitation in the Abspreiz and rotational mobility in the hip joint, so that there is a positive quad characters. The internal rotation and abduction are limited, later, the flexion and extension. Pain is not necessarily present, but can develop in both the hip and the knee than peace, stress or pain when starting in the course. Early symptoms such as a discrete may limping and / or run laziness. Radiographically, the changes mentioned above can often be seen. To this end, in addition to the X-ray pelvis overview always a second plane ( axial, Lauenstein Recording) As required further diagnosis is usually a magnetic resonance imaging ( MRI) procedure performed. Since the initial phase will last only a few weeks, but the discomfort often Setting up until the condensation phase, it also comes only for diagnosis. Then rich radiographs in two levels.

Radiographic risk signs

The risk signs indicate a prognostically more unfavorable course:

• Lateralization of the femoral head
• Involvement of the metaphysis
• Calcification lateral to the epiphysis stove
• Horizontal position of the epiphysis
• Lateral illumination in the epiphysis ( Gage 's sign)

Differential Diagnosis

• Coxitis fugax
• Bacterial coxitis
• Young Hüftkopflösung ( slipped capital femoral epiphysis )
• Congenital Gelenkdysplasie
• Hypothyroidism
• Tumors
• Multiple epiphyseal dysplasia
• Spondyloepiphyseal dysplasia
• Meyer's dysplasia

Therapy

The time course can not be influenced. The duration of the disease depends on the severity and may last from a few months to complete the disease after several years. In therapy, the objective is to relieve the weakened femoral head and to prevent the occurrence of deformation during the repair. Over the years many ways have been tried to achieve these goals, but it was not one of them absolutely enforced. Treatment strategies often differ regionally.

Conservative: This is an attempt to relieve the hip or bring it into a position that is favorable to a healing effect (so-called containment ).

• Thomas splint ( causes only relief, according to a study leads even to an increased pressure in the femoral head. This study, however, only refers to measurements in a case in an adult. )
• Mainz orthosis ( causes discharge and containment )
• Texas Scottish -Rite orthosis ( only causes containment is no longer employed by the developers themselves )
• Beck leg plaster cast in abduction or discharge position ( causes discharge and containment )
• Petrie Cast (only containment of the femoral head is fully loaded )
• Atlanta- rail ( only containment of the femoral head is fully loaded )
• Snyder Sling ( full relief, walking only on crutches possible)
• Braadgips ( full relief, walking only on crutches possible)
• Forearm crutches ( crutches - guarantee no reduction in false application)
• Wheelchair

Beck plaster and relieving orthoses are used less frequently today than in the past. There are studies, according to which not lead orthotics such as the Thomas splint or Mainz rail to a complete relief, but by the altered joint position under certain circumstances, to increased pressure in the region of the hip joint.

Therapeutically important is the reduction of Hüftbelastung, it should be avoided to jump or bounce (sports ban). "Steps reduce " and the use of crutches are just as effective for reducing the pressure on the femoral head. Regular imaging controls are needed to recognize the need for surgery.

Is deceptive, that the children take the disease is often not sufficiently serious, namely if they are painless. Often they will not use their crutches or incorrect. This can lead to irreversible deformation of the femoral head and later to coxarthrosis.

Surgical therapy has also the goal of avoiding deformities occurring and to restore the joint congruency again. This is called " containment therapy " because the femoral head again completely covered by the acetabulum and thus the hip joint should be recentered ( contained hip ):

• Salter pelvic osteotomy, usually in conjunction with a
• Intertrochanteric corrective osteotomy for example, as DVO ( derotation varus osteotomy ) or as DVEO ( derotation Varisations - Extensionsosteotomie )
• Triple osteotomy combined with intertrochanteric corrective osteotomy

It is relatively difficult procedures in which the bone to be cut. In part, further requires a relief to crutches, in a wheelchair or orthotic even after such an operation for a long time.

The opinions on the meaning and the success of different treatment methods are very far apart, good comparative studies hardly exist, and retrospective studies are of little relevance with divergent results. It is also a great lack of uniformity in methods of treatment in individual countries.

A necessary psychosocial care of children and parents often lack. Accordingly, important self-help groups in which particular emphasis on discussing everyday problems and experiences can be exchanged, such as the handling of tools in everyday life and the response to that one is " staring " is.

Development of Perthes treatment

Conservative therapy

Surgical treatment

Forecast

The prognosis depends mainly on Ausheilungsergebnis. This is divided after completion of growth in the classification according to Stulberg into five groups, depending on how the spherical femoral head and how it fits congruent with the acetabulum. Then, among others, the severity of the femoral head and a late age of onset have a significant impact.

In a multicenter long-term study of 56 non- surgically treated children with 58 affected hips showed average 20.4 years after that pain, arthritis and increasing restrictions were very common. 76 % reported at least occasional hip pain, with 39% who reported daily or several times weekly pain. Already in 3 cases, a hip prosthesis was implanted, 44 % showed moderate to severe and 30 % mild signs of arthrosis on radiographs. In 31 out of 56 was a front femoro - acetabuläres impingement, and another 18 patients had a lateral and 14 patients a posterior impingement on, that was significantly associated with pain. A coxa magna, ie an oversized trained femoral head, was found in 70%. Since this study was performed only on patients after conservative treatment, no information on the long -term outcome after surgical treatment are possible, yet which therapy shows better results. However, the Ausheilungsergebnis by Stulberg is a good predictor of later hip problems and premature osteoarthritis. Both signs of arthrosis (as Tönnis grade 2 or 3 ) and occasional pain and coxa magna were more frequent, the worse was the Ausheilungsergebnis according to the Stulberg classification:

• Stulberg I and II: 61% pain indication, 52 % coxa magna, 22 % osteoarthritis
• Stulberg III: 89 % pain indication, 76 % coxa magna, 61 % osteoarthritis
• Stulberg IV or V: 85 % pain indication, 92 % coxa magna, 62 % osteoarthritis