Scheuermann's Kyphosis

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Definition/Description

Scheuermann kyphosis, also known as Scheuermann disease, juvenile kyphosis or juvenile discogenic disease, is a condition of hyperkyphosis that involves the vertebral bodies and discs of the spine identified by anterior wedging of greater than or equal to 5 degrees in 3 or more adjacent vertebral bodies. The thoracic spine is most commonly involved, although involvement can include the thoracolumbar/lumbar region as well. Image R severe case - pre-operative image of a 22 year old male with Scheuermann's Disease

• Most commonly, diagnosis is made in adolescents aged 12 to 17 years who present after their parents notice a postural deformity or “hunchbacked” appearance.
• Pain in the affected hyperkyphotic region may also be the cause of initial evaluation
• Scheuermann's disease is the most common cause of kyphotic deformity in adolescents.

There are two major forms of Scheuermann's kyphosis.

• The thoracic form is most common and has an apex between T7-T9.
• Secondly, the thoracolumbar form can occur with an apex between T10-T12 and is more likely to continue into adulthood.

 

Clinically Relevant Anatomy

The spine of an adult is naturally shaped in an S-curve. The cervical and lumbar regions are concave (lordosis), and the thoracic and sacral regions are convex (kyphosis). According to the Scoliosis Research Society, the thoracic spine has a kyphosis between 20 to 40 degrees. A spinal deformity is considered when the curve is greater (or lesser) as mentioned degrees.

Scheuermann’s disease is a structural deformity of the vertebral bodies and spine. The kyphosis of the thoracic region will be around 45 to 75 degrees. Also there will be vertebral wedging greater than five degrees of 3 or more adjacent vertebrae.  The wedge shaped bodies characterize the rigid hyperkyphosis we see in Scheuermann’s disease. The hyperkyphosis can be compensated by a lumbar and cervical hyperlordosis.

Etiology

Definitive and universally accepted etiology of Scheuermann kyphosis remains undetermined.

• An hereditary component is understood to contribute to this condition's development, although the mode of transmission is still unclear.
• One growing theory, supported by histologic findings, suggests discordant vertebral endplate mineralization and ossification during growth which causes disproportional vertebral body growth and resultant classic wedge-shaped vertebral bodies that lead to kyphosis
• Other theories proposed include mechanical, metabolic, and endocrinologic causes, but the real cause is still unclear.
  

Epidemiology

• Prevalence: 1% to 8% in the United States
• Sex: Male to female ratio is at least 2:1
• Age: Most commonly diagnosed in adolescents 12-17 years
• Rarely diagnosed in children less than 10 years.
• Classification: Type I (Classic) - Thoracic spine involvement only, with the apex of curve T7-T9Type II - Thoracic and lumbar involvement, with the apex of curve T10-T12

Characteristics/Clinical Presentation

According to Sorenson , Scheuermann’s kyphosis is characterized by following criteria.

• Three or more adjacent vertebrae must be wedged 5° or more
• Must be no evidence of congenital, infectious or traumatic disorders of the spine.

The adolescent will present with

• Cosmetic/postural deformity
• Possible Subacute thoracic pain, usually no identifiable inciting event. The pain is worse with activity and improved with rest.
• Deformity is typically appreciated in the early-mid teenage years by child, parents, or on a school screening exam.
• Physical exam shows rigid hyperkyphotic curve, accentuated with forward bending.
• Hyperkyphosis does not resolve with an extension or lying prone/supine, further supporting the “rigid” nature of this deformity.
• Other associated findings on an exam might include cervical or lumbar hyperlordosis, scoliosis, and tight hamstrings. Although neurologic deficits are uncommon, a thorough neurologic exam must be completed
• Muscle stiffness and fatigue, especially at the end of the day
• Decreased flexibility of the torso
• In severe cases, heart and lung function can be impaired or severe neurological symptoms can occur. These symptoms are extremely rare.

Patients with Scheuermann's disease may also complain of inability to participate in physical exercise, work and activities of daily living secondary to pain or the presence of their deformity negatively affecting their cosmetic appearance.
Possible to see cutaneous skin pigmentation at the area of greatest curvature due to skin friction on chair backs.
The natural history of Scheuermann’s kyphosis is unclear, with conflicting reports as to the severity of pain and physical disability.

Differential Diagnosis

• Postural kyphosis (flexible postural deformity)
• Hyperkyphosis attributable to another known disease state
• Postsurgical kyphosis
• Ankylosing spondylitis
• Scoliosis

Diagnostic Procedures

History and physical along with AP/lateral radiographs comprise the essential components for evaluating Scheuermann kyphosis. Lateral radiographs are required for diagnosis, and diagnostic criteria including the following:

• Rigid hyperkyphosis, greater than 40 degrees
• Anterior wedging, greater than or equal to 5 degrees in three or more adjacent vertebral bodies

Other Associated Findings Noted on AP/Lateral Radiographs

• Irregular vertebral endplates
• Schmorl nodes
• Loss of disc space height
• Scoliosis
• Spondylolysis/spondylolisthesis
• Disc herniation

Typically not a necessity, MRI can be helpful to further evaluate anatomic changes or for pre-operative planning. CT imaging is usually not needed. There are also no specific laboratory tests or histologic findings necessary for the diagnosis of Scheuermann kyphosis.

Outcome Measures

Following self-reported outcome measures can be used after an operative treatment  or for untreated Scheuermann’s disease as well :

1.  Scoliosis Research Society Instrument (SRSI): This questionnaire can be used for follow- up of an individual to see if the patient reports improvements in level of activity, pain, personal relationships etc.
2. Back pain and disability scores: Visual Analogue Scale (VAS), Quebec Back Pain Disability Scale (QBPDS), Roland-Morris Disability Questionnaire, Oswestry Disability Index (ODI) and SF-36: measures Quality of Life (QoL) across eight domains, which contain a physical component score (PCS) and a mental component score (MCS)

Examination

• The most significant feature of patients with Scheuermann’s disease is the thoracic kyphosis.
• Often the kyphosis is accompanied by a lumbar and/or cervical hyperlordosis.
• The cervical lordosis can be increased with a protrusion of the head. The shoulders mostly are positioned anteriorly.
• These abnormalities can be accompanied by a mild to moderate scoliosis.
• Patients with Scheuermann's disease are well muscled compared to patients with postural kyphosis.
  The examination consist of:

1. Postural assessment: examination of the posture from anterior, posterior and lateral view
2. Neurological screening: rarely the spinal cord can be stretched over the posterior aspect of the vertebral bodies at the apex of the curvature which may cause neurological signs of impending paraplegia with clonus and hyperreflexia.
3. Adam’s forward-bend test: Scheuermann’s kyphosis can be accompanied by a scoliosis.
4. Muscle length testing: the disease can be accompanied by tightness of the M. Pectoralis, M. Hamstrings, M. suboccipitalis and the hip flexors with possible contractures of the anterior shoulder and hip
5. Range of motion: Flexibility of the extremities and spine can identify impairments and track changes over time.
6. Muscle strength testing: strength of the abdominals, core, trunk extensors and gluteal muscles must be assessed
   

Medical Management

Nonoperative Management

1. Stretching, lifestyle modification, NSAIDs, plus/minus physical therapy

Indication

• Kyphosis less than 60 degrees and asymptomatic

Course

• Majority of patients fall into this category
• Patients typically do well without significant longterm sequelae

2. Extension bracing plus above

Indication

• Kyphosis 60 to 80 degrees plus/minus symptomatic

Course

• Bracing typically required for 12 to 24 months
• Milwaukee brace (see image R), Kyphologic brace or Thoracolumbosacral orthosis-style Boston brace
• Most effective in skeletally immature patients
• Typically, does not improve curve but rather impedes progression

Operative Management

Spinal fusion, typically combination of anterior release + fusion as well as posterior instrumentation + fusion

Indications

• Kyphosis greater than 75 degrees causing unacceptable deformity
• Kyphosis greater than 75 degrees with associated pain
• Neurologic deficit/spinal cord compression
• Severe refractory pain

Course

• Majority of patients experience symptomatic improvement as well as improved curve deformity towards normal
• Operative/postoperative complications must be considered
  

Physical Therapy Management

Conservative treatment of Scheuermann’s kyphosis remains controversial. This can be divided by bracing techniques and physiotherapy exercises. Both have conflicting reports to their effectiveness.  The treatment differs between skeletally immature and skeletally mature patients. (level of evidence: 4)

Bracing

Bracing is widely regarded as being efficacious in the treatment of Scheuermann's kyphosis in the skeletally immature patient with a kyphotic curve of more than 45°. Many studies have proven that for patients who are already skeletally mature, bracing is not an effective treatment. Bracing has been used primarily for the treatment of deformity; the results of brace treatment for relieving pain have not yet been published. Bracing is also used to decrease stress on the anterior wall of the vertebral body.  (level of evidence: 1B)
The overall results of brace treatment seem reproducible and promise a permanent correction of vertebral deformity.  (level of evidence: 2B)

According to Lowe, brace treatment is almost always successful in patients with kyphosis between 55 degrees and 80 degrees if the diagnosis is made before skeletal maturity. A greater kyphosis is almost never treated successfully without surgery in symptomatic patients.

• Bracing therapy has a few shortcomings:
  as the bracing time increases, the probability to develop low back pain increases
• in adolescents the compliance is usually low

Ideally, bracing should begin at the onset of puberty, be worn for approximately 2 years, and removed at the end of skeletal maturity. Braces should be worn 22-24 hours daily for the first 12- 18 months, and the part time (at night) until the patient reaches skeletal maturity. (level of evidence: 5)
Braces that could be used for the treatment of Scheuermann’s kyphosis are:

• Milwaukee brace
• Gschwend brace
• Kyphologic brace
• SpinoMed brace

Other bracing strategies have been attempted such as the use of a soft brace but this however has not shown to be successful. With a soft brace no correction can be achieved in rigid curvatures.

Physiotherapy

According to Bezalel et al (2014), the literature rarely mentions physical therapy as an effective treatment for Scheuermann’s disease. Although the little evidence, it is often used as the first choice of treatment.  (level of evidence: 1A)

Treatment of the Scheuermann's disease depends on the severity and the progression of the disease, the presence or absence of pain and the age of the patient.
Physical therapy for postural improvement is often recommended, focusing on hamstring and pectoralis stretching and trunk extensor strengthening as well as improving function. These exercises can be effective when the thoracic spine has not developed a relevant stiffness and when the sagittal curve is not too high: Cobb angles from 44° to 55°.  (level of evidence: 1B)

Scheuermann's disease in adults is regarded to be a different entity from that of the teenager because the major manifestation is pain and not aesthetic quality. The functional rehabilitation on an outpatient basis is the favoured treatment and referral for surgery or dorso-lumbar braces is rare.
According to Pizzutillo, effective interventions for adolescents with postural kyphosis include exercises to relieve lower extremity contractures and strengthen abdominal musculature, coupled with practiced normal posture standing and sitting. Several studies reported that adolescents with Scheuermann’s Kyphosis who have undergone physical therapy showed improvement in Cobb angle and respiration.  Adolescents with a kyphosis less than 60° are mostly treated with only flexibility-exercises.  (level of evidence: 4)

Skeletally immature patients with Scheuermann's kyphosis benefit from a similar exercise program but also require the use of a spinal orthosis. Bracing of the spine in patients with Scheuermann's kyphosis results in permanent correction of vertebral deformity.  (level of evidence: 1B)

The kyphotic posture can be treated with the Schroth method. This program is developed in the 1920s in Germany by Katharina Schroth. The trunk is divided into three regions (cervical, thoracic and lumbar segments). The program consist of special exercises to correct the positions of these three blocks in the sagittal plane. The exercises are combined with self-elongation of the vertebral column, a correction of breathing techniques and a re-education of the neuromuscular system to improve postural perception. A mirror can be used to synchronize the corrective moments and to improve the postural perception. During the exercises, the patient uses corrective active trunk muscle forces and learns to maintain an erect posture. Later the corrected posture must be maintained throughout activities of daily living. Cooperation and motivation are important factors of success of the Schroth method. All exercises must be tailored to the individual patient.  (level of evidence: 4)

Bezalel et al concluded that the Schroth therapy could decrease the thoracic curve angle of patients with Scheuermann’s Kyphosis. The efficacy and effectiveness of this method should be investigated in further research. (level of evidence: 4)

Examples of Schroth exercises are:

Berdishevsky investigated the effect of an intensive outpatient rehabilitation and bracing program in an adult patient with Scheuermann’s disease. The physical therapy consist of exercises based on the Schroth program and on the Principles of Correction of the Barcelona Scoliosis Physical Therapy School (BSPTS).  (level of evidence: 4) There are five Principles of Correction:

• Trunk elongation and expansion
• Symmetrical sagittal straightening: identical exercises must be performed for both sides of the trunk (right and left):
        Bilateral thoracic expansion in a posterior to anterior (PA) direction to reduce the thoracic hyperkyphosis
        Bilateral lumbar expansion in an anterior to posterior (AP) direction to reduce the hyperlordotic low back
• Shoulder traction: the traction enhances the expansion of the thorax and corrects the spine
• Correction of breathing: it allows the subject to feel an increased expansion in his/her initially collapsed regions. The goal is to expand the thorax in a back-to-front and a lateral direction.
• Muscle activation by increasing tension: to achieve the best possible correction, muscle balance, stabilization and to increase the proprioceptive input. It helps to integrate the ‘corrected body schema’ in the brain.

Berdishevsky concluded that intensive physical therapy with these methods and combined with bracing (SpinoMed brace) was successful to treat an adult patient with Scheuermann’s kyphosis. The patient compliance with the exercises and the brace is important for a positive result.  (level of evidence: 4)

For Scheuermann’s patients extension sports are advised: such as gymnastics, aerobics, swimming, basketball, cycling and hyperextension exercises. Some sports should be discouraged: like sports associated with jumping, marked stress and functional overuse of the back.  (level of evidence: 4)

Postoperative physical therapy is necessary and must contain breathing exercises, mobilizations and strengthening exercises.  (level of evidence: 1A)

More clinical trials are necessary to evaluate the effectiveness of conservative interventions, especially different exercises and manual therapies. These should also be combined/compared with braces. (level of evidence: 1A)

Clinical Bottom Line

Scheuermann's kyphosis can present as a progressive kyphotic deformity with possible pain in adolescents aged 12-17 years. Treatment of the Scheuermann's disease depends on the severity and the progression of the disease, the presence or absence of pain and the age of the patient. Early diagnosis and treatment with bracing or a spinal orthosis can help to stop the kyphotic process and results in permanent correction of vertebral deformity. Physical therapy (Schroth therapy, Principles of Correction of the Barcelona Scoliosis Physical Therapy School, ...) is recommended for postural improvement. Physical therapy can be combined with bracing. More evidence for the efficiency of these therapies is needed.

Key Research

1. Bezalel, Tomer, and Leonid Kalichman. "Improvement of clinical and radiographical presentation of Scheuermann disease after Schroth therapy treatment." Journal of bodywork and movement therapies 19.2 (2015): 232-237
2. Berdishevsky, Hagit. "Outcome of intensive outpatient rehabilitation and bracing in an adult patient with Scheuermann’s disease evaluated by radiologic imaging—a case report." Scoliosis and spinal disorders 11.2 (2016): 40.
   

Resources

Deutchman: Schroth Method Exercises for Scoliosis:

Strott: The Scheuermann’s Disease Fund:

Spine Universe. Scheuermann's Kyphosis (Scheuermann's disease): abnormal curvature of the spine. Available on: www.spineuniverse.com/conditions/kyphosis/scheuermanns-kyphosis-scheuermanns-disease

Tribus C. Medscape Reference. Scheuermann Kyphosis. Available on: emedicine.medscape.com/article/1266349-overview#showall

http://cirrie.buffalo.edu/encyclopedia/en/article/125/

http://www.spine-health.com/conditions/spine-anatomy/lumbar-spine-anatomy-and-pain

http://emedicine.medscape.com/article/1899031-overview#a2

http://www.innerbody.com/anatomy/skeletal/thoracic-vertebrae-lateral