Epiphysial aseptic necrosis, Osteochondrosis

 


Definition
  Aseptic necrosis of bony epiphyses, probably due to ischemia;
  • Frieberg's disease (Metatarsal head)
  • Johansson Larsen disease (Patella)  
  • Kienboch's disease (Lunate)
  • Kohler's disease (Tarsal Navicular)
  • Osgood Schlatter's disease (Tibial tubercle)
  • Panner's capitellum of the humerus
  • Perthes' disease (Upper Femoral Epiphysis)
  • Scheurmann's disease (Vertebral bodies)
  • Sever's disease (Calcaneum)
  • Sinding-Larsen-Johannson disease (Patella)
  • Summary
     

    Osteochondrosis,  Epiphysial aseptic necrosis sometimes called osteochondritis, is a condition of defective bone formation in the epiphysis or growing part of the skeleton. No one knows the exact cause, although it is probably due to circulation disturbance to that part of the bone.

    Osteochondrosis can occur in many areas of the body, but the more common examples are as follows.

    • (Albert H Freiberg, 1868–1940, American surgeon), osteochondrosis of the metatarsal head, usually the second. Trauma or vascular insufficiency have been proposed as mechanisms but the exact aetiology is unknown. The condition most commonly affects athletic adolescent girls aged 10–15 years and can be unilateral or bilateral. The condition typically presents with focal pain and tenderness, the symptoms often present initially after significant activity and increase with time. Conventional radiographs may be normal initially but later show osteopenia and a subchondral fracture or irregularity of the articular surface which can progress to sclerosis, fragmentation and flattening of the metatarsal head (Fig.1) and later reconstitution. Bone scintigraphy or MR imaging may allow an earlier diagnosis than conventional radiographs.

       

    • Kienbock’s disease occurs in the lunate of the hand. It usually occurs in patients age 20 to 30 years of age, more commonly in males.
      (Robert Kienbock, 18711953, Austrian radiologist), osteochondritis of the lunate probably secondary to repetitive trauma. It is more common in adults but is occasionally seen in children, typically athletic adolescents and particularly gymnasts. The condition is often associated with shortening of the ulna, negative ulnar variance. The presenting complaint is usually pain, soft tissue swelling and stiffness.

      Conventional radiographs will show irregular contour, fragmentation, sclerosis and collapse of the lunate. MR imaging shows initial decreased signal on T1- and increased signal on T2-weighted or STIR sequences reflecting oedema. Later there may be decreased signal or signal void on T1- and T2-weighted sequences reflecting bone death or sclerosis, this signal void may be limited to the mid body of the lunate. There may be an associated synovitis seen best on intravenous contrast-enhanced fat suppressed T1-weighted sequences.


       

    • Kohler’s disease occurs in the navicular bone of the foot, and occurs in children between 3 and 9 years of age. The child presents with a painful foot, and diagnosed on X-rays which show a collapse of the navicular bone. Treatment consists of using an arch support, which relieves the pain. Total restoration usually occurs over a period of 12 to 18 months.
      (Alban Kohler, 18741947, German radiologist), osteochondritis of the navicular bone of the foot. The disease typically presents with a limp, is more common in boys than girls and seen especially between the ages 35 years. Examination shows focal pain and tenderness. Radiographs show irregular contour, fragmentation and sclerosis of the navicular with compression in the anteroposterior direction. Scintigraphy may show reduced or increased focal activity. The symptoms usually resolve within three to four months with no long term complications. The radiographic appearances may, however, take one to two years to resolve. The condition must be distinguished from irregular ossification of the navicular occurring as a phase of normal development, in this respect the existence of earlier radiographs is helpful.

       
       

    • Legg-Calve-Perthes' disease occurs in the hip between the ages of 6 to 9 years of age, more commonly in boys. Although the less severe forms recover with no problems, the more severe forms in older boys can lead on to serious disability.
       

      (Arthur Thornton Legg, 18741939, American surgeon; Jacques Calv, 18751954, French orthopaedic surgeon; Georg Clemens Perthes, 18691927, German surgeon), idiopathic avascular necrosis of the capital femoral epiphysis. The condition generally affects Caucasian children aged 312 years of age, with the commonest age being 48 years. Boys are affected approximately 4 times as commonly as girls. Bilateral involvement occurs in approximately 15% of cases but is usually asymmetrical.

      The child characteristically presents with a limp. This may be painless or the child may describe hip, groin, thigh or knee pain often aggravated by activity. Clinical examination reveals an antalgic limp, limited and painful hip rotation, particularly internal rotation. Pathological stages are of initial bone infarction or necrosis, followed by revascularisation, active resorption of dead bone and increasing immature new bone deposition with gradual formation of mature bone.

      The diagnosis is usually confirmed on radiography and initial radiographic evaluation should include an AP and frog-lateral view of the pelvis. The earliest abnormality is usually a non-specific hip joint effusion, most easily detected by ultrasound. This may be suggested by widening of the medial aspect of the hip joint, however, conventional radiographs will often appear normal at this stage. Specific radiographic changes then develop with subchondral fissure-fracture in the antero-lateral aspect of the capital femoral epiphysis, this aspect is best demonstrated on the frog-lateral radiograph  As the condition progress there is fragmentation and sclerosis of the capital femoral epiphysis with reduction in height and possible ill defined focal lucencies within the proximal metaphysis particularly the antero-lateral aspect adjacent to the physis . The capital femoral epiphysis gradually becomes more flattened with lateral extrusion of the cartilage producing broadening of the femoral neck. In mild disease the femoral head may in time be restored to a normal size and shape. More severe Perthes results in a permanently flattened distorted femoral head  with a short wide femoral neck..

      MR imaging is sensitive in the early diagnosis of Perthes and for assessment of progression. The normal yellow marrow of the capital femoral epiphysis shows high signal intensity on both T1- and T2-weighted images relative to the lower signal intensity of red marrow within the metaphysis. During the early infarction stage of Perthes there may be patchy or diffuse reduction in signal intensity within the capital femoral epiphysis on both T1- and T2-weighted images. There may also be heterogeneous reduction in signal in the proximal metaphyseal region. A joint effusion would be seen as high signal within the joint space on T2-weighted images. Later during the fragmentation repair stage there may be mixed high and low signal areas. As revascularization and reossification proceed the epiphysis is restored to more uniform high signal intensity. Bone scintigraphy is also sensitive for the early diagnosis of Perthes and demonstrates focal perfusion defects.

      Ultrasound can better demonstrate the initial hip joint effusion, however, this finding is non specific and more commonly due to transient synovitis of the hip. Persistence of a hip joint effusion for longer than two weeks should raise the suspicion of Perthes.

      Later in the disease ultrasound may demonstrate thickening of the articular cartilage and progression with irregularity, flattening and fragmentation of the capital femoral epiphysis. In the healing phase ultrasound may demonstrate new bone formation and recalcification earlier than on conventional radiographs.

      With advanced Perthes the aim of imaging is to assess the severity of femoral head deformity and the degree of subluxation or uncovering. These features are generally adequately assessed with AP radiographs of pelvis. Three-dimensional CT may be helpful in preoperative planning and arthrography is helpful to assess range of movement and reducibility.

       
       

    • Osgood-Schlatter disease occurs in the tibial tubercle apophysis at the insertion of the patellar tendon. It occurs between the ages of 10 to 15, more commonly in boys. It is probably a form of overuse syndrome, due to repeated avulsion stress on the tibial tubercle. X-rays are usually normal, and the diagnosis is usually made clinically by localized tenderness over the tibial tubercle. Treatment consists of rest for a few days and use of non-steroidal anti-inflammatory medication. An Osgood-Shlatter brace to protect the patellar tendon and tibial tubercle may be used for sports. There is usually no need to restrict activities, as long as the child can tolerate the pain. No long term disability is expected, although there have been reports of cases where the tibial tubercle stays prominent and even tender long after maturation.
       

      Robert B. Osgood, 18731956, American orthopaedic surgeon; Carl Schlatter, 18651930, Swiss surgeon), osteochondritis or a traction apophysitis of the anterior tibial tuberosity resulting from repetitive microtrauma. This overuse phenomenon is common in athletic pubescent children and more frequent in boys than girls. The condition has a usual age range of 1015 years, is usually unilateral and is characterized by focal pain and tenderness over the tibial tuberosity. Clinical examination reveals focal swelling over the tibial tuberosity with point tenderness. The diagnosis is usually clinical and the role of radiographs is to exclude other more sinister pathology such as tumour or infection.

      Radiographs may show soft tissue swelling overlying the tibial tuberosity, fragmentation of the tibial tuberosity ossification centre and thickening of the distal patellar tendon, possibly with intratendinous calcification. Ultrasound is more valuable than radiography for demonstrating the features of Osgood Schlatter disease and may be useful in the assessment of clinically atypical cases. The initial sonographic features are fragmentation of the tibial tuberosity apophysis and swelling of the overlying cartilage. With more advanced disease there may be a focal thickening and heterogenicity of the distal patellar tendon, sometimes with intratendinous echogenic foci representing calcification or ossification close to its insertion site. In severe cases there may be a deep pretibial bursitis seen as a hypoechoic fluid collection deep to the inferior aspect of the patellar tendon. This combination of findings infers a compression mechanism with the distal third of the patellar tendon levering onto the anterior tibial tuberosity.

      Treatment is limitation of activity so as to control the pain, knee pads, nonsteroidal anti-inflammatory agents and quadriceps and hamstring flexibility exercises. Symptoms may continue intermittently for 1824 months but usually resolve when the tibial tuberosity becomes fully ossified and the apophysis fuses


       

    • Panner’s disease occurs in the capitellum of the distal humerus at the elbow, and usually between the ages of 5 and 10, more commonly in boys. The patient presents with pain and inability to extend the elbow fully after a trivial injury. X-rays are diagnostic, and treatment consists of using a sling and restriction from strenuous activity involving the elbow. The bone reconstitutes after a year or two, and the patient usually resumes full function.
      (Hans Jessen Panner, 18711930, Danish radiologist), osteochondritis of the capitellum of the humerus in a child. The condition usually reflects a chronic stress injury and is seen particularly in young baseball pitchers, hence it is also termed little league elbow. In this instance it may be associated with chronic avulsion injury of the medial epicondyle. The condition usually presents with pain and stiffness at the elbow. Fragmentation of the capitellum is seen in the acute phase (Fig.1).

      Imaging features are similar to those of osteochondritis dissecans in the knee. The defect has low signal on T1- and high signal on T2-weighted images in the acute phase, with a breach in the articular cartilage seen on a high signal extending to the articular surface. The lesion may heal but leave a loose body in the joint, best imaged by CT. If the precipitating trauma is removed healing will usually occur.

       
       

    • Sever’s disease occurs in the heels, at the insertion of the Achilles tendons, and may be bilateral. It occurs between the ages of 10 to 12 in boys, and younger in girls. The child complains of pain in the heel with running, and is tender over the heel at the insertion of the Achilles tendon. X-rays are not diagnostic, and treatment consists of using heel lifts to relieve the stress on the heel. Spontaneous resolution is the usual course.
      (James W. Sever, 18781964, American orthopaedic surgeon),
      inflammation of the calcaneal apophysis. This is the secondary ossification centre that serves as the site of insertion for the Achilles tendon and the origin of the plantar aponeurosis (fascia). Excessive use of these structures in young adolescent dancers or athletes may result in calcaneal apophysitis. The calcaneal apophysis is well seen on a lateral radiograph, however, conventional radiographs are unhelpful for imaging calcaneal apophysitis as the normal calcaneal apophysis can have a very variable appearance with irregularity, fragmentation and sclerosis. The apophysis may also appear multipartite due to its ossification from several centres which later coalesce.
      In Sever's disease the role of conventional radiographs is to exclude other possible causes of heel pain such as calcaneal stress fracture, infection or neoplasm. Sever's disease is generally a clinical diagnosis but if imaging is required MR imaging will demonstrate oedema in the fatty marrow of the apophysis and in severe cases the adjacent body of calcaneus and heel pad (Fig.1). These changes are best seen on STIR or fat suppressed T2-weighted sagittal or transverse sequences



     

    • Sinding-Larsen-Johannson disease occurs at the inferior pole of the patella, at the proximal insertion of the patellar tendon. It occurs in the same age group as in Osgood-Sclatter disease, between 10 to 15 years of age. Etiology is probably similar, and so is the outcome. No long-term disability is expected, and treatment is symptomatic only.

      (C M F Sinding-Larsen, Norwegian surgeon, born 1874; S. Johansen, born 1880, Swedish surgeon), proximal patellar tendonitis secondary to repetitive microtrauma and possible traction aphophysitis of the distal pole of patella. The likely mechanism involves chronic impingement. The condition is most commonly seen in boys aged 911 years of age. Clinical examination reveals point tenderness at the inferior pole of the patella and symptoms typically resolve with conservative treatment.

      Conventional radiographs may show fragmentation of the inferior pole of patella. Ultrasound may likewise show fragmentation of the inferior pole of patella but also demonstrates the proximal patellar tendonitis with thickening of the tendon and heterogeneous hypoechogenicity within. Sinding-Larsen Johansen syndrome should be distinguished from a sleeve fracture which represents an acute avulsion injury of the knee extensor mechanism and may be demonstrated on radiographs or ultrasound as a fracture of the inferior pole of the patella.

       


      Scheuermann’s Disease of the Thoracic and Lumbar Spine
      Scheuermann's disease, or Scheuermann's Kyphosis, is a condition in which the normal roundback in the upper spine (called a kyphosis) is increased. Most people with Scheuermann's disease will have an increased roundback (e.g. a hunch back or hump back) but no pain.

      The name of this condition comes from Scheuermann, the person who in 1921 described changes in the vertebral endplates and disc space that can occur during development and lead to kyphosis, or roundback deformity of the thoracic spine (upper back).

      There is some confusion in terminology, however, as Scheuermann also described changes that occur in the disc spaces of the lumbar spine that can lead to back pain. This is really another condition, called juvenile disc disorder, but is often confused with Scheuermann’s kyphosis and is sometimes called Lumbar Scheuermann’s disease. To avoid confusion, the preferred terminology is now:

      Scheuermann’s disease (or Scheuermann’s kyphosis) to describe the condition of adolescent kyphosis

      Juvenile disc disorder (or juvenile discogenic disorder) to describe the condition of adolescent degenerative disc disease.


Disorders characterized by primary or secondary osteonecrosis

Summary with links to http://www.medcyclopaedia.com, Amersham Health

Disorder Site Probable mechanism
 
     
 
Legg Calv Perthes disease Femoral head Osteonecrosis, perhaps due to trauma
 
Freibergs infraction Metatarsal head Osteonecrosis due to trauma
 
Kienbocks disease Carpal lunate Osteonecrosis due to trauma
 
Kohlers disease Tarsal navicular Osteonecrosis or altered sequence of ossification
 
Panners disease Capitulum of humerus Osteonecrosis due to trauma
 
Thiemanns disease Phalanges of hand Osteonecrosis, perhaps due to trauma
 

 

Disorders related to trauma or abnormal stress without evidence of osteonecrosis

Disorder Site Probable mechanism
Osgood Schlatter disease Tibial tuberosity Trauma
 
Blounts disease Proximal tibial tuberosity Trauma
 
Scheuermanns disease Discovertebral junction Trauma
 
Sinding Larsen Johansson disease Patella Trauma
 

 

Diseases due to variations in ossification

Disorder Site Probable mechanism
Severs phenomenon Calcaneus Normal variation in ossification
 
van Necks phenomenon Ischiopubic synchondrosis Normal variation in ossification
 


  
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