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Basics ⬇

Author: DavidMcClaskey, MD, ATC

Description

  • Juvenile osteochondrosis of the spine
  • Scheuermann kyphosis is rigid and most commonly affects the thoracic spine.
  • Vertebrae grow unevenly with regard to the sagittal plane (posterior angle is greater than the anterior), resulting in the signature “wedging” shape of the vertebrae, causing kyphosis.
  • Defined by anterior wedging ≥5 degrees in three or more adjacent vertebral bodies
  • Type I—thoracic spine only, apex of curve T7–T9
  • Type II—lower thoracic and lumbar spine involvement

Epidemiology

Prevalence

  • 1–8% in the United States (1)
  • Most common age of diagnosis is 12 to 17 yr; male to female ratio is at least 2:1.

Etiology and Pathophysiology

  • Results from mechanical stress on a weakened vertebral endplate
  • The vertebral endplate is weakened due to defective growth, likely from predisposing genetic defects. Hormonal abnormalities are postulated to contribute as well.
  • Defective endplate may also affect the health of the adjacent intervertebral disc, resulting in early disc disease.

Genetics

  • Suspected autosomal dominant inheritance
  • Seen more commonly in monozygotic twins
  • Heritability (percentage of all cases that can be attributed to genetics) is 74% (2,3).

Risk-Factors

  • Repetitive strain (particularly with flexion/extension) from strenuous sports or manual labor may increase or accelerate the severity of the kyphosis.
  • Contribution of elevated body mass index (BMI) to this condition is not well defined.

Commonly Associated Conditions

  • Scoliosis
  • Cervical or lumbar hyperlordosis (compensatory curvature due to the thoracic kyphosis)
  • Spondylolysis
  • The above conditions occur in up to 1/3 of patients with Scheuermann kyphosis.

Diagnosis ⬆ ⬇

History

  • Affected patient will present with cosmetic deformity, back pain, and hamstring tightness.
  • Thoracic kyphosis may produce pain at the thoracic spine and/or result in strain on the cervical or lumbar spine.
  • Pain is typically insidious with no traumatic event.
  • The pain worsens with activity and improves with rest.
  • Cosmetic deformity may be noticed by parents or during school-based screenings, preparticipation examinations, or routine wellness exams.
  • A family history of Scheuermann kyphosis may be present in most cases.

Physical Exam

  • Spinal range of motion should be carefully evaluated and documented in all planes of motion.
  • Physical exam shows a rigid, hyperkyphotic curve that is accentuated with forward bending (roundback).
  • The kyphosis does not resolve with postural maneuvers (extension, lying supine, prone, etc.). The kyphosis cannot be consciously corrected.
  • Loss of vertebral height, thus appearing shorter
  • May result in kyphoscoliosis, scoliosis, or cervical/lumbar hyperlordosis
  • Patients typically have tight hamstrings to compensate for excessive lumbar curvature.
  • Excessive curvature (>100 degrees) may cause restrictive lung disease, which can be confirmed with pulmonary function tests.
  • Rarely, may cause neurologic deficits, therefore, a complete neurologic exam is warranted
  • Look for findings that may suggest other causes of kyphosis, such as marfanoid body habitus, hypermobile joints (Marfan or Ehlers-Danlos syndrome), cafe-au-lait spots (neurofibromatosis), etc.

Differential Diagnosis

  • Postural kyphosis (improves with postural maneuvers)
  • Ankylosing spondylitis
  • Scoliosis
  • Neuromuscular disorders
  • Ehlers-Danlos syndrome
  • Marfan syndrome
  • Rickets
  • Congenital kyphosis (Klippel-Feil syndrome, vertebral anomalies (V), anal atresia (A), cardiac malformations (C), tracheo-oesophageal fistula (TE), renal dysplasia (R) and limb abnormalities (L) syndrome)

Diagnostic Tests & Interpretation

Initial Tests (lab, imaging)

  • X-rays:
    • Anteroposterior (AP)/lateral radiographs confirm the diagnosis, demonstrating kyphosis >40 degrees, or anterior wedging ≥5 degrees in three or more adjacent vertebral bodies (3)[B].
    • Cobb angle is used to measure angle of kyphotic deformity; this angle is the intersection of the lines drawn from the endplates of the terminal vertebrae, which are most tilted toward each other.
    • Sagittal balance can also quantify spinal deformity by measuring the horizontal distance between the center of C7 and the superior endplate of S1 on a lateral radiograph. An abnormal balance (plumb line passing more than 2 cm in front of, or behind, the posterosuperior S1 body) is predictive of dysfunction.
    • May show signs of an irregular vertebral endplate, including Schmorl nodes, loss of disc space height, spondylolysis, or spondylolisthesis (3)[B]
    • Schmorl nodes are the herniation of the nucleus pulposus through the vertebral end plate into the body of the adjacent vertebra.
  • Magnetic resonance imaging (MRI):
    • Useful for evaluating disc and spinal cord abnormalities in the presence of neurologic deficits
    • Can be used for preoperative planning

Treatment ⬆ ⬇

General Measures

  • Majority of cases treated with nonoperative management
  • Physical therapy:
    • Focuses on stretching and strengthening of the paraspinal and hamstring musculature as well as postural control
    • Manual medicine may have a role in treatment among providers who are familiar with treating this condition.
  • Lifestyle modification:
    • Avoidance of sports or exercises that places excessive pressure on the spine, such as weight lifting or rugby
    • OK to participate in athletics if kyphosis is nonpainful and being treated nonoperatively
  • Bracing:
    • Painful, mild to moderate kyphosis can be treated with bracing for 12 to 24 mo.
    • Most effective in the skeletally immature patient
    • Bracing may effectively slow progression but may not resolve the kyphosis (1)[B].
    • Types of braces: Milwaukee brace, kyphologic brace, thoracolumbosacral orthosis-style Boston brace
    • Efficacy of braces is limited by compliance; requires the patient to wear up to 18 to 20 hr/day
  • Emotional support:
    • Spinal deformity may negatively impact self-esteem and body image, particularly because this condition occurs during adolescence.
    • Treatment is time-consuming and may significantly impact daily activities.
    • Options for support groups and therapy should be explored and offered.

Medication

First Line

Nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen is typically used for pain control.

Issues for Referral

Referral for surgical correction should be considered for the following cases (4)[B]:

  • Persistent or worsening symptoms despite conservative management
  • Progressive kyphosis >70 degrees despite conservative management
  • Unacceptable cosmetic deformity
  • Pulmonary compromise (restrictive lung defect) secondary to kyphosis
  • Patient/parent preference

Surgery/Other Procedures

  • Significant variability among threshold to treat surgically
  • Curve magnitude typically exceeds 70 to 80 degrees among surgical candidates, but decision to operate is individualized and influenced by maturity, level of pain, response to conservative measures, and patient/parent preference (5)[B].
  • Surgery involves release of spine structures, correction of the kyphosis, and arthrodesis.
  • Arthrodesis may occur through combined anterior-posterior or posterior-only approach.
  • Even with surgery, the kyphosis may progress over time, both in the fusion as well as at the levels above or below the fusion (junctional kyphosis) (6)[B].
  • Risks of surgery include postoperative infection, neurologic injury, and reoperation. Risks are increased when more levels are fused.

Ongoing Care ⬆ ⬇

Follow-up Recommendations

  • A sports medicine or spine specialist should follow patients every 4 to 6 mo to monitor progression, particularly during skeletal maturation.
  • Serial imaging should be performed at 6 to 12 mo intervals until skeletally mature; can perform in shorter intervals if the kyphosis is progressive and the patient is skeletally immature

Patient Education

  • Kyphosis <60 degrees generally has few long-term complications.
  • Athletic activity is allowed for patients with nonpainful kyphosis in nonoperative treatment. Given the potential for progression, high-impact or sports that strain the spine should be avoided.

Prognosis

  • The majority of patients maintain high functional scores with conservative treatments.
  • A high incidence of back pain is reported among patients long term with both conservative and surgical treatments (6).
  • Kyphosis <60 degrees generally has few long-term complications.

Complications

  • Untreated Scheuermann kyphosis may result in a higher risk of back pain and lower quality of life in the later adult years.
  • Restrictive lung disease may occur with severe kyphosis (>100 degrees).
  • Surgical complications include junctional kyphosis, neurologic complications, hardware failure, infection, and lung or kidney injury (4).

Additional Reading ⬆ ⬇

  • Palazzo C, Sailhan F, Revel M. Scheuermann’s disease: an update. Joint Bone Spine. 2014;81(3):209–214.
  • Ristolainen L, Kettunen JA, Heliövaara M, et al. Untreated Scheuermann’s disease: a 37-year follow-up study. Eur Spine J. 2012;21(5):819–824.

References ⬆ ⬇

  1. 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.
  2. Damborg F, Engell V, Nielsen J, et al. Genetic epidemiology of Scheuermann’s disease. Acta Orthop. 2011;82(5):602–605.
  3. Gokce E, Beyhan M. Radiological imaging findings of Scheuermann disease. World J Radiol. 2016;8(11):895–901.
  4. Lonner BS, Newton P, Betz R, et al. Operative management of Scheuermann’s kyphosis in 78 patients: radiographic outcomes, complications, and technique. Spine (Phila Pa 1976). 2007;32(24):2644–2652.
  5. Polly DW Jr, Ledonio CG, Diamond B, et al. What are the indications for spinal fusion surgery in Scheuermann kyphosis? [published online ahead of print January 30, 2017]. J Pediatr Orthop. doi:10.1097/BPO.0000000000000931.
  6. Graat H, Schimmel J, Hoogendoorn R, et al. Poor radiological and good functional long-term outcome of surgically treated Scheuermann patients. Spine (Phila Pa 1976). 2016;41(14):E869–E878.

Clinical Pearls ⬆

  • Scheuermann kyphosis is distinguished as a rigid kyphosis that does not resolve with postural maneuvers and most commonly affects the thoracic spine.
  • The majority of cases are treated successfully with conservative management. Surgical considerations are influenced not only by the degree of kyphosis (typically >70 degrees) but also by individual patient considerations, including maturity, pain, response to conservative measures, and patient or parent preference.
  • The kyphosis may progress despite bracing and surgical management.
  • Mild-moderate kyphosis <60 degrees has fewer long-term complications than more severe kyphosis.