Perthes disease

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Classification according to ICD-10
M91.1 Juvenile osteochondrosis of the femoral head (Perthes-Legg-Calvé disease)
ICD-10 online (WHO version 2019)

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

Perthes' disease was discovered in 1910 by the German surgeon Georg Clemens Perthes . At the same time, there were further initial descriptions by Jacques Calvé in France and Arthur T. Legg in America, which is why the term “Legg-Calvé-Perthes' disease” is also used in the English-speaking world.

Synonyms

  • Legg-Calvé-Perthes (Waldenström) Syndrome (LCP)
  • Osteochondropathia deformans coxae juvenilis
  • Coxa plana idiopathica
  • juvenile femoral head necrosis
  • idiopathic necrosis of the femoral head in children
  • Maydl disease

Occurrence

The prevalence is around 1: 1200. The disease occurs predominantly in boys with white skin (these are affected approx. 4 times more often than girls), mostly between the ages of 5 and 9 (rarely until the end of puberty, but also as early as 2 years). Both sides are affected at the same time in around 15% of children. In addition to osteochondrosis dissecans , Perthes disease is the most common aseptic bone necrosis . The disease is relatively common with an annual incidence of five cases per 100,000 inhabitants (exact data for the Federal Republic are not available).

causes

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

  • Circulatory disorders: Discussed are possibly present vascular malformations that affect the blood supply to the femoral head even under normal vascular bed.
  • hormonal dysregulation
  • Increase in pressure in the bone or joint space
  • genetic factors: a multifactorial inheritance is suspected here. Direct relatives in particular (e.g. siblings) are at a significantly higher risk

Perthes disease is a disease of the femoral head in childhood that is probably caused by circulatory disorders. In the early stages it leads to joint irritation with joint effusions, so that there is a similarity to rheumatic diseases. In the further course of the process, the femoral head ball sintered together, often associated with a lateral migration from the joint ball bearing in the pelvis, also known as the socket. Later there is a permanent deformation of the head and pan with a corresponding movement disorder. The leg remains shortened. The early wear of the hip joint is predetermined. In children aged 2 to 12 years of age, hip pain and limping, knee pain and laziness to walk should be considered. The bony, but not the cartilaginous changes in the hip joint can be seen with X-rays.

Typical course in the X-ray image

M. Perthes on the left hip joint (right side of the picture). Clearly the inhomogeneity of the head epiphysis
  • Initial stage: apparent widening of the joint space in the hip joint.
  • Infraction stage : subchondral infraction lamella "contour doubling"
  • Condensation stage: In this stage, compression of the bone substance can be seen.
  • Fragmentationsstadium: A scholliger collapse of the femoral head epiphysis is seen with flattening and possibly cross-broadening and projection of the femoral head from the pan.
  • Repair stage: the femoral head gradually re-ossifies.
  • Healing stage: The remodeling processes are complete, there may be a deformation of the femoral head if there was incorrect loading during the repair phase.

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

The first signs are restricted mobility and / or pain in the hip / knee area. Joint effusion is diagnosed in the sonogram. In addition, incipient changes in the epiphysis can be seen at the front in the fragmentation stage. Changes can be seen in the X-ray from the infraction stage. In the case of clinical suspicion, magnetic resonance imaging (MRI) can detect the disease in the initial stage.

In the course of the M. Perthes the femoral head enlargement occurs, which can lead to an incongruence of the femoral head ball to the joint ball bearing in the pelvis. Both parts of the joint can be badly deformed. One speaks of a loss of containment. Deformities in the sense of loss of containment (beginning or pronounced) can be treated surgically. These operations include a transection of the pelvis ( Salter pelvic osteotomy ) or the thighbone (intertrochanteric varicating femur osteotomy) to restore the congruence of the joint. The inserted implants must be removed again.

If the infestation is severe or the children are at increased risk, imaging must be repeated at least every four months until the regeneration stage is reached. Thereafter, no further deformation is to be expected, apart from an elevated trochanteric position. This should be slowed down by sclerosing the growth plate so that there is no limping gait. It is also not yet standardized whether an X-ray or MRI is used. Further, a is arthrography for the evaluation of the containment possible. Recently, there have also been studies of containment diagnostics in ultrasound in comparison to MRI and X-rays, which can give a good statement about the joint situation.

diagnosis

There is a restriction in the splay and rotation mobility in the hip joint, so that there is a positive four-digit sign . Internal rotation and abduction are restricted, and later also flexion and extension. Pain is not necessarily present, but can develop in the hip as well as in the knee area as rest, stress or start-up pain. Early symptoms are e.g. B. a u. U. discrete limping and / or laziness. The changes mentioned above can often be seen in the X-ray image. In addition to the X-ray pelvic overview, a second level (axial, Lauenstein image ) is always required. A magnetic resonance tomography (MRT) examination is usually carried out as a further diagnosis . Since the initial phase usually only lasts a few weeks, but the symptoms often only appear in the condensation phase, a diagnosis is only made then. Then x-rays are sufficient in two planes.

Radiological risk signs

The signs of risk indicate a prognostically rather unfavorable course:

Differential diagnosis

therapy

The course over time cannot be influenced. The duration of the illness depends on the severity and can last from a few months to the end of the illness after several years. The aim of therapy is to relieve the weakened femoral head and prevent deformations from occurring during the repair. Many ways have been tried over the years to achieve these goals, but none of them have absolutely prevailed. The treatment strategies often differ regionally.

Conservative : The attempt is made to relieve the hip joint or to bring it into a position that has a beneficial effect on healing (so-called containment).

  • Thomasschiene (only causes relief, according to a study even leads to increased pressure in the femoral head. However, this study only refers to measurements in one case, in an adult.)
  • Mainz orthosis (provides relief and containment)
  • Texas Scottish Rite orthosis (only effects containment, is no longer used by the developers themselves)
  • Pelvic leg cast in abduction or relief position (causes relief and containment)
  • Petrie Cast (containment only, the femoral head is fully loaded)
  • Atlanta splint (containment only, the femoral head is fully loaded)
  • Snyder Sling (full relief, walking is only possible on forearm supports)
  • Braad plaster of paris (full relief, walking only possible on forearm supports )
  • Forearm crutches (crutches - do not guarantee relief if used incorrectly)
  • wheelchair

Pelvic plaster casts and relieving orthoses are used less today than in the past. There are studies according to which orthoses such as the Thomas or Mainz splint do not lead to complete relief, but rather to increased pressure in the area of ​​the hip joint due to the changed joint position under certain circumstances.

It is therapeutically important to reduce the load on the hips; jumping or hopping should be avoided (sports prohibited). “Stepping down” and the use of crutches are also effective in relieving pressure on the femoral head. Regular imaging checks are necessary to determine the need for surgery .

It is deceptive that children often do not take the disease seriously enough, namely when they are pain-free. Often they do not use their crutches or use them incorrectly. This can lead to irreversible deformation of the femoral head and later to coxarthrosis .

Surgical therapy also aims to prevent deformities and restore joint congruence. One speaks of "containment therapy" because the femoral head is again completely covered by the acetabulum and the hip joint is to be recentered (contained hip):

These are relatively difficult interventions in which the bones must be cut. In some cases, even after such an operation, relief from crutches, wheelchairs or orthoses is required over a longer period of time.

Opinions about the meaning and success of the various treatment methods differ widely, there are hardly any good comparative studies, and retrospective studies are not very meaningful with diverging results. There is also great inconsistency in treatment methods in individual countries.

The necessary psychosocial care for children and parents is often lacking. Accordingly, self-help groups are becoming more important, in which everyday problems in particular can be discussed and experiences can be exchanged, for example on dealing with aids in everyday life and reacting to being "stared at".

Development of the Perthes treatment

Conservative therapy

decade Typical treatment / procedure
1920s no treatment
1930s Patients were prescribed to lie down permanently
1940s unencumbered running
1950s no uniform treatment
1960s Containment by orthosis

Surgical treatment

forecast

The prognosis depends mainly on the healing result. After growth is complete, it is divided into five groups in the Stulberg classification, depending on how spherical the femoral head is and to what extent it fits congruently to the acetabulum. Among other things, the severity of the femoral head necrosis and the late age of onset have a significant influence on this.

In a multicenter long-term study on 56 non-surgically treated children with 58 affected hip joints, it was found after a mean age of 20.4 years that pain, osteoarthritis and increasing restrictions were very common. 76% reported at least occasional hip pain, with 39% reporting pain daily or several times a week. A hip prosthesis had already been implanted in 3 cases , 44% showed moderate to severe and 30% slight signs of osteoarthritis on the X-ray. 31 of 56 had anterior femoro-acetabular impingement , a further 18 patients had lateral and 14 patients had posterior impingement, which was clearly associated with pain. A coxa magna, i.e. an oversized femoral head, was found in 70%. Since this study was only carried out on patients after conservative treatment, it is not possible to make any statements about the long-term results after surgical therapy, nor can any statements be made about which therapy shows better results. However, the healing result according to Stulberg is a good predictor for later hip problems and premature osteoarthritis. Both signs of osteoarthritis (as Tönnis grade 2 or 3) as well as occasional pain and coxa magna were more common the worse the healing result according to the Stulberg classification:

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

literature

Basics - initial descriptions

  • J. Calvé: Sur une forme particulière de pseudocoxalgie greffée sur déformation caractéristiques de l'extrémité supérieure du fémur. In: Revue de chirurgie. 30/1910, pp. 54-84.
  • A. Legg: An obscure affection of the hip joint. In: Boston Medical and Surgical Journal. 162/1910, pp. 202-204.
  • G. Perthes: About arthritis deformans juvenilis. In: German journal for surgery . 107/1910, pp. 111-117.

Important classifications

  • A. Catterall: The natural history of Perthes' disease. In: Journal of Bone and Joint Surgery. British Volume. 53/1971, pp. 37-53.
  • EJ Eyring, DR Bjornson, CA Petersen: Early diagnostic and prognostic signs in Legg-Calvé-Perthes disease. In: The American Journal of Roentgenology. 92/1965, pp. 382-387.
  • JA Herring, B. Jeffrey, JJ Williams, JS Early, RH Browne: The lateral pillar classification of Legg-Calvé-Perthes disease. In: Journal of Pediatric Orthopedics. 12/1992, pp. 143-150.
  • RB Salter, G. Thompson: Legg-Calvé-Perthes disease. The prognostic value of the subchondral fracture and a two group classification of the femoral involvement. In: Journal of Bone and Joint Surgery. American Volume. 66/1984, pp. 479-489.
  • SD Stulberg, DR Cooperman, R. Wallenstein: The natural history of Legg-Calvé-Perthes disease. In: Journal of Bone and Joint Surgery. American Volume. 63/1981, pp. 1095-1108.

Diagnostic literature

  • W. Konermann, G. Gruber, J. Gaa: Standardized sonographic examination of the hip joint. In: Ultrasound Med. 2000 Jun; 21 (3), pp. 137-141.
  • J. Kramer, S. Hofmann, A. Scheurecker, C. Tschauner: Perthes disease. In: Radiologist. 2002 Jun; 42 (6), pp. 432-439.
  • MH Stuecker, J. Buthmann, AL Meiss: Evaluation of hip containment in legg-calve-perthes disease: a comparison of ultrasound and magnetic resonance imaging. In: Ultrasound Med. 2005 Oct; 26 (5), pp. 406-410.

German-language standard textbook

  • K.-P. Schulitz, H.-O. Dustmann: Perthes disease. Etiopathogenesis, differential diagnosis, therapy and prognosis. 2nd Edition. Springer-Verlag, Berlin / Heidelberg / New York 1998.

genetics

  • Y. Miyamoto, T. Matsuda, H. Kitoh et al .: A recurrent mutation in type II collagen gene causes Legg-Calvé-Perthes disease in a Japanese family . In: Hum. Genet. tape 121 , no. 5 , June 2007, p. 625-629 , doi : 10.1007 / s00439-007-0354-y , PMID 17394019 .

Individual evidence

  1. ↑ Guideline on Movement Disorders. (No longer available online.) Archived from the original on March 4, 2016 ; accessed on April 16, 2018 . Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. @1@ 2Template: Webachiv / IABot / www.awmf.org
  2. ↑ a b A. Noelle Larson, Daniel J. Sucato, John Anthony Herring, Stephen E. Adolfsen, Derek M. Kelly, Jeffrey E. Martus, John F. Lovejoy, Richard Browne, Adriana DeLaRocha: A Prospective multicenter study of Legg- Calvé-Perthes disease - functional and radiographic outcomes of nonoperative treatment at a mean follow-up of twenty years. In: Journal of Bone and Joint Surgery (Am). April 4, 2012, Volume 94-Am, pp. 584-592.
  3. ^ Original table by George E. Fulford, Edinburgh 1984.

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