Scheuermann's Disease

Synonyms: Scheuermann disease, juvenile kyphosis, Scheuermann kyphosis¹

Scheuermann's disease is one of the osteochondroses. It is essentially osteochondrosis of the vertebrae.² It affects the thoracic or thoracolumbar spine in adolescents, causing increased kyphosis, poor posture and possible backache.¹ It is the most common cause of structural kyphosis in adolescents.^3,4

There is a separate article giving an overview of the Osteochondroses.

• Osteochondroses all involve a defect in ossification. In Scheuermann's disease there is a defect in the secondary ossification centres of the vertebral bodies. A few vertebral bodies may be involved or the whole thoracolumbar spine.¹
• Hereditary factors are thought to play a part in the pathophysiology but no definite mode of inheritance or genetic marker has been established so far.^1,5,6
• Other factors including a correlation between Scheuermann's disease and taller height, as well as an association with increased levels of growth hormone have also been suggested as possible causes.⁵ However, one study has disputed the increased weight and height in those with Scheuermann's disease as being part of the pathogenetic mechanism, suggesting that they were secondary results.⁷
• Mechanical factors and trauma have also been cited as possible causes.^1,5

• Usually presents in children 13-16 years old.
• Occurs more commonly in boys.¹

History

• Poor posture with increased kyphosis (usually noticed by parents).
• Pain is a more common presentation if the disease affects the lumbar spine. Pain is usually worse on activity.¹
• Neurological symptoms may rarely be the presenting feature.⁵
• Cardiorespiratory symptoms can also rarely occur.⁵

Examination

• It is normal to have a degree of kyphosis. Any degree of kyphosis of more than 40 degrees is considered abnormal.⁵
• Kyphotic deformity may be progressive.
• Upper thoracic kyphosis is best seen in the forward flexed position; lower thoracic kyphosis may be seen at the thoracolumbar junction.
• There is reduced flexibility of the spine.
• There may be tenderness above and below the apex of the kyphosis.¹
• Scoliosis may also be present and is associated with Scheuermann's disease.¹
• You should carry out a neurological examination but neurological signs are rare.

Includes:

• Postural kyphosis (known as postural round back deformity; kyphosis is flexible)
• Ankylosing spondylitis
• Glucocorticoid-induced osteoporosis in Cushing disease can lead to thoracic kyphosis
• Skeletal dysplasia
• Congenital kyphosis (failure of segmentation)
• Spinal tuberculosis (consider if atypical presentation)

• X-ray of spine in a standing position is the mainstay for diagnosis. AP and lateral views are taken. The whole thoraco-lumbar spine should be imaged. Changes can include:
  • Wedge-shaped vertebral bodies with anterior wedging of at least 5 degrees or more in at least 3 adjacent vertebral bodies (pathognomonic for Scheuermann's disease)
  • Hyperkyphosis (greater than 40 degrees)
  • Narrow intervertebral disc spaces
  • Irregular upper and lower vertebral endplates
  • Schmorl nodes - multiple herniations of the nucleus pulposus of the vertebral plates
• MRI and CT scanning may also be used.

• The management is controversial. Some think that no treatment is required as the disease usually follows a benign course with few adverse sequelae. Others believe that treatment in those who are skeletally immature will prevent future excessive deformity and pain.
• If treatment is carried out, for mild disease, avoidance of strenuous activity and weight-bearing may be all that is needed. A referral to a physiotherapist may be helpful. Observation and X-ray follow-up are carried out.⁵
• Non-steroidal anti-inflammatory drugs may be used for pain relief provided there are no contraindications.
• Casting, spinal braces (a Milwaukee style brace) and bed rest may be used in more severe disease. Braces may be advised for several years.¹
• Surgery is usually only used if there is neurological deficit, uncontrolled pain, unacceptable cosmetic appearance or documented progression.^1,3
• Cord decompression is carried out for neurological deficit and spinal fusion techniques are used for kyphosis correction and pain control.⁸

• Chronic back pain
• Progressive and permanent deformity
• Neurological deficit
• Cardiorespiratory problems

• The degree of clinical symptoms and signs generally depends on the degree of deformity. Those with mild deformity tend to have mild symptoms and signs.⁵
• Mild to moderate Scheuermann's disease rarely requires bracing or surgery.⁵
• A gradual loss of correction can occur after a brace is removed.⁵
• Neurological and cardiorespiratory complication risks are very low.^5,9
• Adolescents with severe deformity and symptoms have had significant deformity correction after surgery. However, a literature review in 2007 concluded that 'clinical outcomes data are not yet available, and the studies available do not have strong levels of evidence.'⁴