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DISEASE/DISORDER

Definition:

Scheuermann disease, (juvenile kyphosis), is a growth disturbance  with curving deformity of the thoracic or thoracolumbar spine in adolescents that causes an increase bowing or rounding of the back in the sagittal plane. Typically it is 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 is characterized by defective growth of the vertebral cartilage endplate, which probably results from a predisposing genetic background that affects the quality of matric components and chondrocytes.  An autosomal-dominant mode of inheritance is suspected. 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 10%.4, 5

The sex ratio is considered to be close to 1.1

Patients 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 than 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):

Three forms: 1

The classocal form: fixed dorsal thoraci kyphosis.

The thoracolumbar form: apex of kyphosis at T11-T12.

The lumbar form: loss of lumbar lordosis.

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 and lumbar deformities.
  2. Progressive deformity: starting initially as a poor posture, deformity may get worse over time. The thoracic kyphosis curve is usually between 45° and 75°.
  3. 15% to 20 % patients are associated with scoliosis. 1

Specific secondary or associated conditions and complications:

Kyphosis of around 95° can present with neurologic compromise or myelopathy which is often triggered by trauma. 1,7 However, there are cases showing neurologic compromise with an kyphosis of only 53° to 56°.8

Kyphotic curves greater than 100° with the apex of the curve located in the upper thoracic region can produce restrictive lung disease.9 There is a decrease in lung volume and impedance 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.

ESSENTIALS OF ASSESSMENT

History

1. History of deformity, the progression over time, body image dissatisfaction, restrictive lung disease, hamstring tightness, and in rare cases, neural impairment (numbness, weakness, bowel or bladder issues), as well as family history are obtained. 9

2. Pain generally is subacute without a clear episode of precipitating trauma. The pain is worse after activity and at the end of the day and improves with rest. It tends to improve with skeletal maturity. However, long-term follow-up suggests an increased prevalence of back pain in adulthood. 9

 Physical examination:

  1. Spine range of motion examination, including assessment of rigid kyphosis, lordosis and scoliosis, and overall flexibility. Kyphosis can be measured with gravity-dependent inclinometers.10 Adam’s forward bending test helps to differentiate a relatively sharp angulation from postural irregularities.
    • In Scheuermann disease, the rigid kyphosis does not flatten with forward bending, extension, or lying supine (in contrast to the curvature of poor posture). 11 Scoliosis and compensatorylumbar hyperlordosis may be present.
  2. Spine palpation.
    • May be tender above and below the apex of the kyphosis.
  3. Joint range of motion examination.
    • Hamstring tightness may be present. 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 assessments 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. Standing lateral spine radiographs are necessary for the diagnosis (anterior wedging of ≥5° in at least three adjacent vertebral bodies). 12.

Besides vertebral wedging, typical radiographic findings are: irregularities of the vertebral endplates, disc material herniation through the endplates (Schmorl’s nodes), narrowing of the disc spaces, and lengthening of the vertebral bodies. 13 Concomitant spondylolysis may occur in patients with lumbar pain.

Schmorl’s nodes and Modic changes (endplate MRI signal change) on lumbar MRI, back pain and physical function restrictions seem to be more prevalent among patients with Scheuermann’s disease. 14 Evidence of increased pain, suspicion of rapid progression, and/or neurologic compromise (focal neurologic deficits) would require immediate imaging.

Magnetic resonance imaging (MRI), computed tomography (CT) scan, and CT myelography can be helpful adjunctive studies if surgery is planned in, to evaluate coexistent thoracic spinal stenosis, syrinx, or other intrathecal abnormality.

Supplemental assessment tools

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

Early prediction of outcomes

One third of the patients with curves of 74° or more failed bracing and progressed to surgery.15

Social role and social support system

Familial and social support should be assessed as they are important for patients to maintain both physical and mental health.

Scheuermann patients typically have less physically demanding jobs. However, there is no correlation with decreased level of education, absenteeism from work, medication use, or social interactions.

REHABILITATION MANAGEMENT AND TREATMENTS

Available or current treatment guidelines

No established clinical guidelines exist.

At different disease stages

The treatment for Scheuermann disease remains controversial.  16, 17 Treatment should be tailored to each individual based on symptoms, age, and nature of the curve.

For kyphotic angles less than 70°, treatment usually consists of physical therapy including techniques of the Schroth method, 18 anti-inflammatory medications and heating pads for analgesia, avoidance of precipitants, observation and simple radiograph follow-ups. Physical therapy may not alter the natural history of a progressive kyphosis but can be useful in reducing symptoms.

Bracing is considered efficacious in skeletally immature adolescents. It is indicated for painfulScheuermann’s disease or mild kyphosis (between 45.and 65.) or with ineffective physiotherapy 1. Typically, a Milwaukee brace can be used.15 The brace is expected to be worn for about 16-24 hours a day until the individual reaches skeletal maturity. While the brace is on, about 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 maintain at least 3° of improvement.. 

In a retrospective, observational study released in 2017, conservative treatment with Milwaukee brace and physiotherapy was effective for halting kyphosis progression in 97.5% of Scheuermann’s kyphosis, which could be advised for cases up to 90° of kyphosis before skeletal maturity. 19

For curves with an apex below T9, a thoracolumbosacral orthosis (eg, 4-valve orthosis, Gschwend erection corset) may be used.20

Surgery is indicated in patients with greater than 70° thoracic curves, 16 greater than 25° to 30° thoracolumbar curves, curve progression despite bracing, unacceptable cosmetic deformity and uncontrolled pain.

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 may benefit from a coordinated and conservative program that may include rest, nonsteroidal anti-inflammatory drugs, bracing, and physical therapy on an outpatient basis. 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 essential throughout the treatment process. Patients who are treated with bracing need to be educated on an appropriate wearing schedule and brace donning and doffing techniques. Spinal extension exercises and hamstring stretching should also be incorporated. Education is necessary for postural and body mechanics  improvement.

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. Other questionnaires such as Scoliosis Research Society Instrument (SRSI), Oswestry Low Back Pain Disability and, the Barthel Index, 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.

CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE

Video-assisted thoracoscopic surgery (VATS) is a promising  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.21 Combined VATS release and posterior spinal fusion is a viable option for the treatment of the more severe and rigid curves.22

A systematic review of treatment approaches for Scheuermann kyphosis concluded that surgery affords superior correction and maintenance of correction relative to bracing. Posterior-only fusion may provide greater correction and similar loss of correction compared to anterior-posterior approaches along with a smaller complication profile. This posterior-only approach has concomitantly gained popularity over the combined anterior-posterior approach in recent years. 22  

A unique development that has been successfully applied to patients with osteoporotic kyphosis is the Spinal Proprioceptive Extension Exercise Dynamic program.24 The dynamic effect of a weighted kypho-orthosis is to facilitate recruitment of back extensors. This orthosis increases a patient’s perception of spinal joint position to encourage extension rather then flexion, which helps atients with  significally reduced kyphosis and back pain, improved back strength, and increased level of physical activity.24

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.

Further study is also needed to address natural history of the disease and identify the prognostic factors of back pain during adulthood. 1

REFERENCES

  1. Palazzo C, Sailhan F, Revel M. Scheuermann’s disease: an update. Joint Bone Spine. 2014 May;81(3):209-14.
  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. Zardar ZM, Ames RJ, Lenke L. Scheuermann’s Kyphosis: Diagnosis, Management, and Selecting Fusion Levels. J Am Acad Orthop Surg. 2019 May 15;27(10):e462-e472.
  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. 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.
  11. Ginsburg GM, Bassett GS. Back Pain in Children and Adolescents: Evaluation and Differential Diagnosis. J Am Acad Orthop Surg 1997; 5:67.
  12. Lowe TG. Scheuermann’s disease. Orthop Clin North Am 1999; 30:475.
  13. Bradford DS. Juvenile kyphosis. Clin Orthop Relat Res. 1977;128:45–55.
  14. Leena Ristolainen, Jyrki A. Kettunen, Heidi Danielson, Markku Heliövaara. Magnetic resonance imaging findings of the lumbar spine, back symptoms and physical function among male adult patients with Scheuermann’s disease. Journal of Orthopedics 21 (2020) 69-74.
  15. 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.​
  16. Loughenbury, P. R., & Tsirikos, A. I. (2017). Scheuermanns kyphosis: diagnosis, presentation and treatment. Orthopaedics and Trauma, 31(6), 388–394. doi: 10.1016/j.mporth.2017.09.010
  17. Hart, E. S., Merlin, G., Harisiades, J., & Grottkau, B. E. (2010). Scheuermannʼs Thoracic Kyphosis in the Adolescent Patient. Orthopaedic Nursing, 29(6), 365–371. doi: 10.1097/nor.0b013e3181f83761
  18. Bezalel, T., Carmeli, E., Levi, D., & Kalichman, L. (2019). The Effect of Schroth Therapy on Thoracic Kyphotic Curve and Quality of Life in Scheuermann’s Patients: A Randomized Controlled Trial. Asian Spine Journal, 13(3), 490–499. doi: 10.31616/asj.2018.0097,
  19. Etemadifar MR, Jamalaldini MH, Layeghi R. Successful brace treatment of Scheuermann’s kyphosis with different angles. J Craniovertebr Junction Spine. 2017;8(2):136–143. doi:10.4103/jcvjs.JCVJS_38_16
  20. Karimi, M. T. (2016). Effect of brace on kyphosis curve management: A review of literature. Health Rehabil. 1(1): 1-4.
  21. Papagelopoulos PG, Mavrogenis AF, Savvidou OD, Mitsiokapa EA, Themistocleous GS, Soucacos PN. Current concepts in Scheuermann’s kyphosis. Orthopedics. 2008;31:52-58; quiz 59-60.22. 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.​
  22. Huq S, Ehresman J, Cottrill E, Ahmed AK, Pennington Z, Westbroek EM, Sciubba DM. Treatment approaches for Scheuermann kyphosis: a systematic review of historic and current management. J Neurosurg Spine. 2019 Nov 1;32(2):235-247.24. Sinaki MS. Exercise for patients with osteoporosis: management of vertebral compression fractures and trunk strengthening for fall prevention. PM R. 2012;4:882-888.

Original Version of the Topic:

Edward Hurvitz, MD. Scheuermann’s disease. 10/27/2020

Author Disclosure

Yuxi Chen, MD
Ipsen, Research Grant paid to institution: PI for Pediatric lower limb spasticity study

Andrew Bloomfield, MD
Nothing to Disclose

Amara Nasir, MD
Nothing to Disclose