Scheuermann’s disease

Author(s): Edward Hurvitz, MD

Originally published:10/27/2015

Last updated:10/27/2015

1. DISEASE/DISORDER

Definition:

Scheuermann disease, (juvenile kyphosis), is a curving deformity of the thoracic or thoracolumbar spine in children that causes an increase bowing or rounding of the back in the sagittal plane. Typically defined by anterior vertebral wedging of at least 5° in 3 or more adjacent thoracic vertebral bodies. Often, it produces pathologic thoracic kyphosis (>40°; normal is 20°-40°).

Etiology:

Scheuermann disease was described as an autosomal-dominant mode of inheritance. However, there is a predominance in men, which suggests a complex, multifactorial pattern of inheritance.1 Chromosomal anomalies may also be responsible for the development of the disease.2

There have been many other etiologic theories, including mechanical factors, increased growth caused by excess growth hormones, osteoporosis caused by calcium metabolism abnormalities, inflammatory disease, hypovitaminosis, and others, but there has been no definitive association with Scheuermann disease and any of these etiologies.3

Epidemiology including risk factors and primary prevention:

The prevalence rate of Scheuermann disease in the United States is between 0.4% and 8%.

There is a 2:1 male to female ratio.4,5

Patients with Scheuermann disease generally are affected between ages 13 and 16 years, and the diagnosis is rarely made in patients younger than 10 years.

Patho-anatomy/physiology:

Three adjacent wedged vertebral bodies of at least 5° each are pathognomonic for Scheuermann disease. Anatomic findings may also include a thickened anterior longitudinal ligament with narrowed intervertebral disks. The vertebral bodies are wedged, and traumatic disk herniations through the endplates (Schmorl nodes) are often found.

Histologic changes demonstrate a low ratio of collagen to proteoglycans in the endplate matrix. It has been hypothesized that the relative decrease in collagen leads to an alteration in the ossification of the endplate.

The bone mineral density was found to be lower then controls indicating that there is a highly significant association that exists between osteoporosis and Scheuermann disease.6

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time):

Signs and symptoms:

  1. Pain: there is a 50% incidence of severe thoracic back pain in patients with thoracic deformities and a higher incidence of back pain in patients with thoracolumbar deformities. The pain is typically located just distal to the apex of the deformity and radiates laterally in a paraspinal pattern.
  2. Progressive deformity: patients and family members may notice a deformity worsening over time, starting intially as poor posture. With kyphosis, the thoracic curve is usually between 45° and 75°. Clothes may fit differently, and shoulders appear more rounded.

Specific secondary or associated conditions and complications:

Kyphosis of around 95° can present with neurologic compromise or myelopathy.7 However, there are cases showing neurologic compromise with an kyphosis of only 53° to 56°.8

Kyphotic curves greater than 100° can produce restrictive lung disease, with the apex of the curve located in the upper thoracic region.9 There is a decrease in lung volume and impedence on the movement of ribs, consequently affecting the mechanics of the respiratory muscles.

Cosmetic concerns should be specifically and aggressively discussed with the individual patient because these concerns ultimately may be the driving force behind the patient’s decision process.

2. ESSENTIALS OF ASSESSMENT

History

  1. History of deformity, including when it was first noted and some sense of the progression over time (eg, Did the parents note poor posture when younger? Did the school say something about the patient’s posture?).
  2. Family history due to suggested genetic etiology.
  3. Pain, which may be the initial symptom, including relation to activity (often worse with activity, relieved by rest).10
  4. Changes in neurologic function (numbness, weakness, bowel or bladder issues) suggesting myelopathy.

Physical examination:

  1. Spine range of motion examination, including assessment of kyphosis, lordosis and scoliosis, and overall flexibility. Kyphosis can be measured with gravity-dependent inclinometers.11 Adam’s forward bending test helps to differentiate from postural irregularities.
    • In Scheuermann disease, there is decreased flexibility, increased kyphosis, and kyphosis is present on Adam’s test. Scoliosis (seen in about one third of patients3) and hyperlordosis may be present.
  2. Spine palpation.
    • May be tender above and below the apex of the kyphosis.
  3. Full range of motion examination.
    • Hamstring tightness may be present in these patients. There may be hip and shoulder joint contractures.
  4. Neurologic examination; although, neurologic deficits are extremely rare.

Clinical functional assessment: mobility, self care cognition/behavior/affective state

FIM may be helpful in assessing day to day activities of daily living and mobility. For example, there are certain patients that may use a wheelchair as a result of cardiopulmonary or mechanical limitations, and serial FIM assessments can document their functional course.

Laboratory studies

No genetic markers or laboratory studies exist to confirm the diagnosis of Scheuermann disease.

Imaging

Anteroposterior (AP) and lateral radiographs of the entire thoracolumbar spine should be incorporated. Annual follow-up imaging should continue until the child stops growing or there is no further increase in curvature. If there is no evidence of frontal plane curve on baseline films or follow-up physical exams, decreased frequency of AP films can be considered to reduce radiation exposure.

Evidence of increased pain, suspicion of rapid progression, and/or neurologic compromise (focal neurologic deficits) would require immediate imaging.

The angle between the endplates of each respective vertebral body should be measured using the Cobb technique. This technique measures the tilting angle of the end vertebral bodies that are most tilted in the kyphotic deformity, both proximally and distally on AP radiographs.

In addition to wedge-shaped vertebral bodies, radiographs may show disk space narrowing and Schmorl nodes that develop from herniations of the nucleus pulposus because of poorly formed irregular vertebral endplates. However, Schmorl nodes are not pathognomonic for Scheuermann disease.

Magnetic resonance imaging (MRI), computed tomography (CT) scan, and CT myelography can be helpful adjunctive studies in planning the care of a patient with Scheuermann disease.

If surgery is necessary, MRI may help the surgeon define the anatomy. In particular, there may be coexistent thoracic spinal stenosis, syrinx, or other intrathecal abnormality that could affect surgical care.

Lumbar disks can also be assessed with MRI, which can help the surgical decision making in terms of which levels to incorporate in the fusion.

Supplemental assessment tools

Pulmonary function tests may be used to assess lung function of these patients; they are used prior to any surgical procedure (eg, spinal fusion).

Early prediction of outcomes

One third of the patients with curves of 74° or more failed bracing and progressed to surgery.12

Social role and social support system

Family and social support should be assessed because they are very important in maintaining a good mental and physical state.

Patients have similar levels of education, absenteeism from work, physical activity, medication use, and social interactions as age-matched controls. Those with Scheuermann disease typically have less demanding jobs.

3. REHABILITATION MANAGEMENT AND TREATMENTS

Available or current treatment guidelines

No established clinical guidelines exist.

At different disease stages

Scheuermann disease does not have any specific treatment plan.13 Treatment should be tailored to each individual based on symptoms, age, and nature of the curve. Most can be managed without bracing and surgery. Observation and simple radiograph follow-ups may be the most reasonable treatment plan,14 along with education and encouragement to resume normative activities.

Anti-inflammatory medications can provide short-term pain relief.

Physical therapy may not alter the natural history of a progressive kyphosis. However, a thoracic extention program can be useful in reducing symptoms.

Adolescents with progressive kyphosis whose curves reach at least 45° are ideal candidates for bracing. Typically, a Milwaukee brace can be used.12 While the brace is on, an approximate 50% correction should be expected. However, once the brace is removed, a gradual loss of correction is expected over time. Over a 5-year time span after discontinuation of bracing, many patients maintained at least 3° of improvement as demonstrated on radiographs. For curves with an apex below T9, a thoracolumbosacral orthosis (eg, 4-valve orthosis, Gschwend erection corset) may be used.15

Surgery is rarely indicated in patients with Scheuermann disease. The 2 most common indications for surgery are spinal pain and unacceptable cosmetic appearance.15

Spinal fusion surgery is usually done on curves greater than 75° and consist of an anterior release and fusion and a posterior instrumentation and fusion.

Coordination of care

Multiple specialists, including physiatry, psychology, physical therapy, and occupational therapy, should be involved with patient care. These specialists can coordinate a comprehensive biopsychosocial approach to improve and preserve function and quality of life.

Patients are treated successfully on an outpatient basis. They may benefit from a coordinated and conservative program that may include rest, nonsteroidal anti-inflammatory drugs, bracing, and physical therapy. For those that may require surgical intervention, the patient typically is hospitalized for a recovery time after the operation. Patient care focuses on pain control, mobility training, and patient education, which requires the multidisciplinary team.

Patient & family education

Patient and family education is very important throughout the treatment process. Patients who are treated with bracing need to be educated on an appropriate wearing schedule and in the proper techniques of donning and doffing their braces. Spinal extension exercises and hamstring stretching should also be incorporated. Education is necessary to improve the patient’s posture and overall body mechanics.

Measurement of Treatment Outcomes including those that are impairment-based, activity participation-based and environmentally-based

The FIM (for adults) and WeeFIM (for kids) may be useful. The Barthel Index, which only tests for functional motor items and no functional cognition items, is useful in this condition where there are no cognitive deficits.

Translation into Practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Watchful waiting is generally the best treatment course.

4. CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE

Video-assisted thoracoscopic surgery (VATS) is a promising new technique in the treatment of kyphosis as an alternative to open thoracotomy for anterior release procedures. This technique is more expensive than open thoracotomy, but with fewer complications.15 With this technique, there is decreased pain and perioperative morbidity, shorter hospital stays, and fewer intensive care days.

Combined VATS release and posterior spinal fusion is a viable option for the treatment of the more severe and rigid curves.16

A unique development that has been successfully applied to patients with osteoporotic kyphosis is the Spinal Proprioceptive Extension Exercise Dynamic program.17 This program may have some beneficial applications in the treatment of progressive kyphosis. The dynamic effect of a weighted kypho-orthosis is to facilitate recruitment of back extensors. This orthosis increases a patient’s percepton of spinal joint position to encourage extention rather then flexion. Patients who wore the orthosis for 20-minute sessions twice daily and participated in specific proprioceptive exercises for 4 weeks had significally reduced kyphosis and back pain, improved back strength, and increased level of physical activity.17

5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE

Controversies and gaps in the evidence-based knowledge

Current controversies in the treatment of patients with Scheuermann disease revolve around bracing care and the surgical decision-making process. Proponents of bracing state that bracing postpones indefinitely the need for surgical intervention. Others believe that bracing does not affect the ultimate prognosis of the curve and that, if these patients are monitored long enough, the curve will continue to settle and resume its prebracing course of progression.

REFERENCES

1. Damborg F, Engell V, Nielsen J, Kyvik KO, Andersen MØ, Thomsen K. Genetic epidemiology of Scheuermann’s disease. Acta Orthop. 2011;82:602-605.

2. McKenzie L, Sillence D. Familial Scheuermann disease: a genetic and linkage study. J Med Genet. 1992;29:41-45.

3. Lowe TG. Current concepts review: Scheuermann disease. J Bone Joint Surg Am. 1990;72:940-945.

4. Damborg F, Engell V, Andersen M, Kyvik KO, Thomsen K. Prevelence, concordance, and heritability of Scheuermann kyphosis based on a study of twins. J Bone Joint Surg Am. 2006;88:2133-2136.

5. Murray PM, Weinstein SL, Spratt KF. The natural history and long-term follow-up of Scheuermann kyphosis. J Bone Joint Surg Am. 1993;75:236-248.​

6. Lopez R, Burke S, Levine D, Schneider, R. Osteoporosis in Scheuermann’s disease. Spine J. 1988;13:1099-1103.

7. Lonstein JE, Winter RB, Moe JH, et al. Neurologic deficits secondary to spinal deformity. A review of the literature and report of 43 cases. Spine (Phila Pa 1976). 1980;5:331-355.​

8. Ryan MD, Taylor TK. Acute spinal cord compression in Scheuermann’s disease. J Bone Joint Surg Br. 1982;64:409-412.

9. Murray PM, Weinstein SL, Spratt KF. The natural history and long-term follow-up of Scheuermann kyphosis. J Bone Joint Surg Am. 1993;75:236-248.

10. Haveman LM, van Es HW, ten Berge-Kuipers M. [Complaints of back pain in childhood: find curable causes] [Dutch]. Ned Tijdschr Geneeskd. 2008;152:353-358.

11. Lewis JS, Valentine RE. Clinical measurement of the thoracic kyphosis. A study of the intra-rater reliability in subjects with and without shoulder pain. BMC Musculoskelet Disord. 2010;11:39.

12. Sachs B, Bradford D, Winter R, et al. Scheuermann kyphosis. Follow-up of Milwaukee-brace treatment. J Bone Joint Surg Am. 1987;69:50-57.​

13. Tsirikos AI, Jain AK. Scheuermann’s kyphosis; current controversies. J Bone Joint Surg Br. 2011;93:857-864.

14. de Mauroy J, Weiss H, Aulisa A, et al. 7th SOSORT consensus paper: conservative treatment of idiopathic & Scheuermann’s kyphosis. Scoliosis. 2010;5:9.

15. Papagelopoulos PG, Mavrogenis AF, Savvidou OD, Mitsiokapa EA, Themistocleous GS, Soucacos PN. Current concepts in Scheuermann’s kyphosis. Orthopedics. 2008;31:52-58; quiz 59-60.

16. Soto JH, Parikh SN, Al-Sayyad MJ, et al. Experience with combined video-assisted thoracoscopic surgery (VATS) anterior spinal release and posterior spinal fusion in Scheuermann’s kyphosis. Spine. 2006;30:2176-2181.​

17. Sinaki MS. Exercise for patients with osteoporosis: management of vertebral compression fractures and trunk strengthening for fall prevention. PM R. 2012;4:882-888.

Author Disclosure

Edward Hurvitz, MD
Nothing to Disclose

Related Articles