Pathophysiology: Scheuermann disease refers to osteochondrosis of the secondary ossification centers of the vertebral bodies. The lower dorsal and upper lumbar vertebrae are involved initially. The process may be limited to several bodies or may involve the entire dorsal and lumbar spine.
Scheuermann disease probably is heterogeneous (ie, not a single entity but a group of conditions sharing similar features). The etiology and pathogenesis are a matter of debate. Many theories have been advanced, including mechanical, metabolic, and endocrinologic causes. A definite hereditary component is involved in development of the condition, but no mode of inheritance has been identified.
Patients with Scheuermann kyphosis generally are affected at 13-16 years, are taller than comparably aged peers, and have advanced skeletal versus chronologic age. Some affected children have disproportionate limb lengths. In a Finnish study, left-handedness was found to be a powerful determinant of hyperkyphosis in school children before puberty. An increased incidence of spondylolysis and spondylolisthesis also was reported in patients with Scheuermann kyphosis, and scoliosis in the region of kyphosis is reported in 20-30% of patients as well.
According to some authors, the presence of an adjacent area of lordoscoliosis below the region of hyperkyphosis testifies to the common nature of the pathogenesis of idiopathic scoliosis and Scheuermann disease. Scheuermann kyphosis may be associated with an epidural cyst with an ensuing neurologic deficit.
Frequency:
- In the US: The prevalence rate of Scheuermann kyphosis is thought to be 0.4-8%.
- Internationally: No international data are available.
Mortality/Morbidity: Many authors believe that there are few adverse long-term sequelae of the condition, despite a paucity of available natural history data. Lowe suggests that if residual kyphosis remains less than 60° at skeletal maturity, the patient has an excellent prognosis for minimal problems in adult life.
Pain may be present but generally ceases when growth is complete. Minimal spinal malalignment may persist when the disorder becomes quiescent. Early development of marginal osteophytes may occur. Acute myelopathy secondary to cord compression at the apex of the thoracic kyphosis has been reported.
Sex: Boys are affected more frequently than girls.
Age: The condition affects children aged 13-16 years, and the diagnosis is rarely made in patients younger than 10 years.
History:
- Most patients with Scheuermann kyphosis present with a history of deformity. The parent brings the child to the physician because of poor posture or referral from a school screening program.
- The incidence of pain is low, although 20% of patients may complain of discomfort in the region of the kyphosis. In patients with lumbar localization of kyphosis, the pain appears more pronounced—as many as 80% may report lower back pain. The pain is usually intermittent and is characterized as dull and aching; it is related generally to activity and is relieved by rest.
Physical:
- Patients with upper thoracic Scheuermann disease present with a kyphotic deformity best demonstrated in the forward flexed position.
- Decreased flexibility of the spine is noted, indicating the structural nature of the kyphotic deformity, in contrast to patients with flexible postural kyphosis.
- Patients may have tenderness to palpation above and below the apex of the kyphosis.
- A high association exists between scoliosis and Scheuermann disease. Patients also may have a hyperlordosis in the lumbar spine.
- Lower thoracic kyphosis is localized at the thoracolumbar junction; in general, any kyphotic deformity present at this level must be considered abnormal.
- Hamstring tightness may be present in these patients.
- A careful neurologic examination is recommended, although neurologic deficits are extremely rare.
Causes:
- Osteochondritis of the upper and lower cartilaginous vertebral plates has been incriminated.
- Trauma sometimes seems to be a causative factor.
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