Scheuermann Disease

Scheuermann's 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's 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 the development of the condition, but the mode of inheritance has been debated.[8] Reports of identical radiologic changes in monozygotic twins and transmission over 3 generations suggested underlying heritability. In study by McKenzie and Sillence, 12 probands were referred, and upon radiologic examination of their parents and siblings, 7 were shown to have familial Scheuermann's disease with an autosomal dominant pattern of inheritance.[9] Of the remaining 5 probands, 4 had chromosomal anomalies.

Patients with Scheuermann's disease generally are affected at age 13-16 years, are taller[10] than comparably aged peers, and have advanced skeletal versus chronologic age. Some affected children have disproportionate limb lengths.

A Greek research report, however, found that although, in the study, children with Scheuermann's disease tended to be taller and weigh more than did other children, there seemed to be no correlation in children with the disease between these 2 factors and the magnitude and morphology of the main kyphotic curve.[10] The authors suggested that hormonal disturbances may be impacting the development of Scheuermann's disease and also causing, as a secondary result, height and weight increases.

A study by Hershkovich et al, however, suggested that height and body mass index (BMI) are associated with the risk and severity of spinal deformities in adolescents. The study, which involved the medical records of 829,791 males and females aged 17 years, including 103,249 who had been diagnosed with some degree of kyphosis or scoliosis, found a significantly higher rate of spinal deformities, and a greater likelihood of such deformities being severe, in underweight males and females. Greater height was also found to be associated with increased risk and greater severity of spinal deformities in males and females.[11]

In a Finnish study, left-handedness was found to be a powerful determinant of hyperkyphosis in school children before puberty.[12] An increased incidence of spondylolysis and spondylolisthesis also was reported in patients with Scheuermann's disease, and scoliosis in the region of kyphosis is reported in 20-30% of patients as well.[13]

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's disease. Scheuermann's disease may be associated with an epidural cyst with an ensuing neurologic deficit.

A retrospective study by Tyrakowski et al found no significant difference between skeletally mature and skeletally immature patients with Scheuermann’s disease with regard to radiographic sagittal spinopelvic parameters, including sagittal vertical axis, thoracic kyphosis, thoracolumbar kyphosis, lumbar lordosis, pelvic incidence, pelvic tilt, and sacral slope. However, both groups (33 patients each) had a significantly lower pelvic incidence and sacral slope than did children, adolescents, and adults without Scheuermann’s disease, causing the investigators to question the use of pelvic incidence in predicting the desired lumbar lordosis in Scheuermann’s disease cases.[14]

A study by Peleg et al suggested that a greater horizontal orientation of the sacrum may lead to a change in spinal biomechanics that in turn contributes significantly to the development of Scheuermann’s disease. The study involved the evaluation of the sacral anatomical orientation in 183 persons with Scheuermann’s disease and 185 controls.[15]

Frequency

United States

The prevalence rate of Scheuermann's disease is thought to be 0.4-8%.

International

A study by Armbrecht et al using found the prevalence of Scheuermann’s disease in Europe to be 8% in persons aged 50 years and above, with no difference between males and females. The sample, consisting of more than 10,000 individuals, came from 27 centers across Europe that participated in the European Vertebral Osteoporosis Study.[16]

Mortality/Morbidity

Many authors believe that there are few adverse long-term sequelae of Scheuermann's disease, despite a paucity of available natural history data. Lowe suggests that if residual kyphosis remains less than 60º 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.

Using the exercise tolerance test, a prospective, comparative study by Lorente et al found that adolescent Scheuermann’s disease patients with kyphosis of over 75° displayed significant respiratory inefficiency, with ventilation capacity and maximum oxygen uptake being lower than in healthy controls.[17]

A study by Liu et al suggested that a link exists between Scheuermann’s disease and thoracolumbar disk herniation (disk herniation between T10/11 and L1/2). The investigators found that the radiographic signs of Scheuermann’s disease, as well as the diagnosis of Scheuermann’s disease itself, were significantly more prevalent in the 63 patients in the study with symptomatic thoracolumbar disk herniation than they were in a group of 57 patients who underwent surgery for lower lumbar disk herniation. Moreover, in the patients with thoracolumbar disk herniation, the herniation was significantly more prevalent at segments displaying the radiographic signs of Scheuermann’s disease than at segments that did not.[18]

A study by Garrido et al of patients with untreated kyphosis in Scheuermann’s disease found, at mean 27-year follow-up, that a correlation existed between increases in the sagittal vertical axis (SVA) and declining health-related quality of life. Greater SVA was associated with worse function, pain, disability, and mental health scores, as measured using the Scoliosis Research Society-22 (SRS-22) questionnaire, the 36-item Short-Form Health Survey (SF-36), and the Oswestry Disability Index (ODI). Kyphosis in the patients, which had a mean value of 66° at skeletal maturity, had increased to 78° at follow-up.[19]

Sex

Scheuermann's disease affects boys more frequently than it does girls.

Age

Scheuermann's disease affects children aged 13-16 years, and the diagnosis is rarely made in patients younger than 10 years.

See the list below:

• Most patients with Scheuermann's disease 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.[20]

See the list below:

• Patients with upper thoracic Scheuermann's 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 with 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's 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.

See the list below:

• Osteochondritis of the upper and lower cartilaginous vertebral plates has been incriminated in the development of Scheuermann's disease.

• Trauma sometimes seems to be a causative factor.

See the list below:

• Laboratory studies are not needed in diagnosing Scheuermann's disease.

See the list below:

• Lateral radiography of the spine demonstrates diagnostic changes in Scheuermann's disease.[3, 4] See the image below.

  • Wedge-shaped vertebral bodies

  • Arcuate and rigid kyphosis

  • Narrow intervertebral disk spaces with calcifications

  • Prominent irregularities of the vertebral surfaces

  • Vertebral plates that are poorly formed and that develop multiple herniations of the nucleus pulposus known as Schmorl nodes (Note that Schmorl nodes are not uncommon and may be seen in Wilson disease, sickle cell anemia, and spinal stenosis.)

    Preoperative lateral of a patient with an 85º thoracic deformity secondary to Scheuermann's disease.

• Sorenson suggested the following radiologic criteria for the diagnosis of Scheuermann's disease:

  • Hyperkyphosis greater than 40°

  • Irregular upper and lower vertebral endplates with loss of disk space height

  • Wedging of 5° or more in three consecutive vertebrae

A study by Ristolainen et al found that, compared with controls, patients with Scheuermann’s disease demonstrated, on lumbar magnetic resonance imaging (MRI), a greater prevalence of Modic changes at levels L1/L2, L3/L4, and L5/S1, with such imaging also showing Schmorl nodes to be more prevalent than in controls (64% vs 8%, respectively). In addition, MRI revealed the dura sac to be larger in Scheuermann’s disease (mean 201 mm2 vs 152 mm2).[21]

See the list below:

• Histologic changes demonstrate that vertebral growth endplate cartilage is abnormal, with a decreased collagen/proteoglycan ratio on electron microscopic examination.

• Enchondral ossification is altered profoundly in affected segments, and there are increased proteoglycan levels.

• The matrix of the endplates is abnormal, thus interfering with normal vertebral growth.

Physical Therapy

The treatment of Scheuermann's disease is controversial.[5, 6] Some authors think that the natural history of thoracic Scheuermann's disease is benign and that therefore the condition needs no treatment. Whether orthotics or surgical treatments prevent any of the consequences that may occur is uncertain.

Some authors recommend treatment in the skeletally immature patient in the hope of preventing excessive deformity, which may cause pain and cosmetic concerns. Patients with mild, nonprogressive disease can be treated by reducing weight-bearing stress and avoiding strenuous activity. Exercise alone is not found to be beneficial.

When the kyphosis is more severe, recommendations include casting, a spinal brace, or rest and recumbency on a rigid bed. Orthotic management of Scheuermann's disease usually requires 12-24 months of treatment.

In a 2003 report on one type of brace for the treatment of Scheuermann's disease (evaluated at the Alfred I. duPont Hospital for Children), it was recommended that the orthosis be worn until skeletal maturity (at least 16 mo), in order to induce improvement or halt progression of the disease.[22] The results for this brace were comparable to previous reports discussing the effectiveness of the modified Milwaukee brace, and the duPont kyphosis brace had the advantage of being concealable under normal attire.

Surgery rarely is indicated in patients with Scheuermann's disease. Probably the two most common indications for surgery are spinal pain and unacceptable cosmetic appearance. These criteria are subjective, so it is wise to be cautious in counseling these patients.[7]

In patients with curves greater than 75° and with pain that is unresponsive to nonoperative measures, consider spinal fusion. Spinal fusion consists of an anterior release and fusion, as well as a posterior instrumentation and fusion performed under the same anesthesia on the same day. See the image below.

Postoperative lateral demonstrating a 2-rod leverage technique after an anterior release allowing reduction of the deformity to 47º.

A retrospective study by Riouallon et al indicated that with regard to functional and radiologic results, the effectiveness and stability of posterior-only spinal fixation in Scheuermann’s disease are the same as for combined anterior/posterior fusion. The investigators therefore recommended that the condition be treated with the anterior/posterior approach only when major deformation is present.[23]

However, a retrospective study by McDonnell et al indicated that better results can be derived from two-stage anterior/posterior fusion than from single-stage posterior fusion, in Scheuermann's disease. Significantly greater kyphosis correction was associated with the two-stage procedure. Moreover, there was no junctional failure with anterior/posterior fusion, compared with a rate of 12.9% for the posterior-only surgery. Nonetheless, the incidence of postoperative infection was greater for the two-stage operation than for the one-stage procedure.[24]

A multi-center, prospective cohort study by Polly et al indicated that patients with Scheuermann's disease who undergo spinal fusion tend to have a higher body mass index than those treated nonoperatively.[25]

Cord decompression is indicated for the rare patients who have neurologic deficits secondary to epidural cysts or increased kyphotic angulation.

The most frequently occurring complication is pseudoarthrosis, followed by instrumentation failure and secondary loss of correction.

The optimal treatment of Scheuermann's disease is controversial. Patients treated with bracing or surgery have experienced an improved self-image, which they have attributed to their treatment. Patients with kyphotic curves extending 70° at follow-up have had an inferior functional result. Soo and colleagues suggested that by carefully selecting the appropriate treatment for patients with Scheuermann's disease on the basis of the patient's age, the spinal deformity, and the severity of back pain, achieving a similar functional result at long-term follow-up is possible.[26] They felt that despite different treatment protocols, patients with Scheuermann's disease tend to achieve a similar functional result at long-term follow-up.

Flexible curves have been suggested as a positive predictor of a successful outcome of bracing.

Other variables that have positively influenced the outcome of treatment have been compliance with conservative treatments (a body cast plus brace and exercise regimen) and the initial presence of the Risser sign of skeletal maturity.

A retrospective study by Yrjälä et al comparing acute and chronic pain outcomes in adolescents (aged 10-21 years) who underwent posterior spinal fusion found that patients with spondylolisthesis reported more pain 2 years post surgery than did those with Scheuermann’s disease or adolescent idiopathic scoliosis (AIS). The Scoliosis Research Society 24-item questionnaire (SRS-24) demonstrated that at 2-year follow-up, the adolescents with spondylolisthesis had a mean pain domain score of 4.04, compared with 4.43 and 4.31 for patients with Scheuermann’s disease or AIS, respectively. While persistence of pain in the study was linked to the patients’ specific disease pathology, the severity of acute postoperative pain and the amount of in-hospital opioid use appeared to have a positive correlation with the number of fused vertebrae.[27]

A retrospective study by Nasto et al indicated that in patients with Scheuermann's disease who undergo posterior-only surgical correction, high preoperative pelvic incidence should be taken into account when planning correction of thoracic kyphosis in order to avoid too much reduction of lumbar lordosis and the development of proximal junctional kyphosis.[28]

Pain associated with Scheuermann's disease generally responds to nonsteroidal anti-inflammatory drugs (NSAIDs), as well as to temporary activity restriction.

Class Summary

Have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known, but they may inhibit cyclo-oxygenase activity and prostaglandin synthesis. Other mechanisms may exist as well, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell-membrane functions.

Ibuprofen (Ibuprin, Motrin)

DOC for patients with mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.

Naproxen (Anaprox, Aleve, Naprosyn, Naprelan)

For relief of mild to moderate pain; inhibits inflammatory reactions and pain by decreasing activity of cyclo-oxygenase, which results in a decrease of prostaglandin synthesis.

See the list below:

• Patients with Scheuermann's disease most commonly are treated successfully on an outpatient basis. Conservative measures may include rest, NSAIDs, bracing, and physical therapy. See Rehabilitation Program for more information. The physician should schedule regular follow-up visits over the course of treatment to monitor progress and modify the plan of care when necessary.

See the list below:

• For rare cases of Scheuermann's disease that require surgical intervention, the patient typically is hospitalized for a recovery time following the operation. Inpatient care focuses on pain control, mobility training, and patient education.

See the list below:

• Complications that may develop from Scheuermann's disease include chronic back pain, progressive deformity, and neurologic deficits. Following operative treatment, the most common complication is pseudoarthrosis, following by instrumentation failure and secondary loss of correction.

See the list below:

• Patient education is very important throughout the treatment process for Scheuermann's disease. Patients who are treated with bracing need to be educated in the proper techniques of donning and doffing of their braces, as well as in an appropriate wearing schedule. An exercise program needs to be presented to the patient, with a focus on spinal extension exercises and hamstring stretching. Education is necessary also to improve the patient's posture and overall body mechanics with daily activities.