AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Scheuermann disease (juvenile kyphosis) is a deformity in the thoracic or thoracolumbar spine in children. Patients have an increased kyphosis in the thoracic or thoracolumbar spine with associated backache and localized changes in the vertebral bodies.

Pathophysiology

Scheuermann 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 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. Reports of identical radiological changes in monozygotic twins and transmission over 3 generations suggested underlying heritability. In study by by McKenzie and Sillence, 12 probands were referred, and, upon radiological examination of their parents and siblings, 7 were shown to have familial Scheuermann disease with an autosomal dominant pattern of inheritance. Of the remaining 5 probands, 4 had chromosomal anomalies.

Patients with Scheuermann kyphosis generally are affected at 13-16 years, are taller than comparably aged peers, and have advanced skeletal versus chronologic age. Some affected children have disproportionate limb lengths. In a Finnish study, left-handedness was found to be a powerful determinant of hyperkyphosis in school children before puberty. An increased incidence of spondylolysis and spondylolisthesis also was reported in patients with Scheuermann kyphosis, and scoliosis in the region of kyphosis is reported in 20-30% of patients as well.

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

Frequency

United States

The prevalence rate of Scheuermann kyphosis is thought to be 0.4-8%.

International

No international data are available.

Mortality/Morbidity

Many authors believe that there are few adverse long-term sequelae of the condition, despite a paucity of available natural history data. Lowe suggests that if residual kyphosis remains less than 60° 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.

Sex

Boys are affected more frequently than girls.

Age

The condition affects children aged 13-16 years, and the diagnosis is rarely made in patients younger than 10 years.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

• Most patients with Scheuermann kyphosis 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.

Physical

• Patients with upper thoracic Scheuermann 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 to 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 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.

Causes

• Osteochondritis of the upper and lower cartilaginous vertebral plates has been incriminated.
• Trauma sometimes seems to be a causative factor.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Postural round back (the kyphosis is flexible)
Thoracic hyperkyphosis in patients with various types of skeletal dysplasia (eg, spondyloepiphyseal dysplasia congenita, Morquio disease)
Ankylosing spondylitis may present a similar picture.
Kyphosis in patients who had a laminectomy before skeletal maturity or who had radiation to the spine for a regional tumor (eg, Wilms tumor, neuroblastoma)
Kyphosis in patients with eosinophilic granuloma
Type II congenital kyphosis (failure of segmentation)
Hyperkyphosis secondary to vertebral epiphysitis in eunuchoidism, ovarian agenesis, and Wilson disease
The possibility of scoliosis must be assessed because of the high association with Scheuermann kyphosis.
Friedreich ataxia and neurofibromatosis may present with hyperkyphosis and scoliosis.
Thoracic hyperkyphosis may occur secondary to glucocorticoid-induced osteoporosis in Cushing disease.
In atypical cases, a generalized skeletal dysplasia and spinal tuberculosis must be excluded.

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Laboratory studies are not needed in diagnosing Scheuermann disease.

Imaging Studies

• Lateral radiograph of the spine demonstrates diagnostic changes.
• • Wedge-shaped vertebral bodies
  • Arcuate and rigid kyphosis
  • Narrow intervertebral disk spaces with calcifications
  • Prominent irregularities of the vertebral surfaces
  • The vertebral plates are poorly formed and develop multiple herniations of the nucleus pulposus known as Schmorl nodes. Note that the Schmorl nodes are not uncommon and may be seen in Wilson disease, sickle cell anemia, and spinal stenosis.
• Sorenson suggested the following radiologic criteria for the diagnosis of Scheuermann kyphosis:
• • Hyperkyphosis greater than 40°
  • Irregular upper and lower vertebral endplates with loss of disk space height
  • Wedging of 5° or more in 3 consecutive vertebrae

Histologic Findings

• 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 vertebra growth.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Rehabilitation Program

Physical Therapy

The treatment of Scheuermann disease is controversial. Some authors think that the natural history of thoracic Scheuermann kyphosis is benign, and, 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 kyphosis usually requires 12-24 months of treatment.

A newly designed brace for the treatment of Scheuermann disease was recently evaluated at Alfred I. duPont Hospital for Children. It was recommended to be worn until skeletal maturity (at least 16 mo) to induce improvement or halt progression of the disease. The results were comparable to previous reports in literature describing the effectiveness of the modified Milwaukee brace, and the duPont kyphosis brace had the advantage of concealability under normal attire.

Surgical Intervention

• Surgery rarely is indicated in patients with Scheuermann disease. Probably the 2 most common indications for surgery are spinal pain and unacceptable cosmetic appearance. These criteria are both subjective, so it is wise to be cautious in counseling these patients.
• 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.
• Cord decompression is indicated for the rare cases of patients who had 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 kyphosis is controversial. Patients treated with bracing or surgery have improved self-image, which they attributed to their treatment. Patients with kyphotic curves extending 70° at follow-up had an inferior functional result. Soo and colleagues suggested that by carefully selecting the appropriate treatment for patients with Scheuermann kyphosis 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. They felt that despite different treatment protocols, patients with Scheuermann kyphosis 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 positively influenced the outcome of treatment were compliance with conservative treatments (a body cast plus brace and exercise regimen) and an initial presence of the Risser sign of skeletal maturity.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Pain generally responds to nonsteroidal anti-inflammatory drugs (NSAIDs), as well as to temporary activity restriction.

Drug Category: Nonsteroidal anti-inflammatory drugs

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.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

• For rare cases 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.

Further Outpatient Care

• Patients with Scheuermann 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.

Complications

• Complications that may develop from Scheuermann 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.

Patient Education

• Patient education is very important throughout the treatment process. Patients who are treated with bracing need to be educated in the proper techniques of donning and doffing of their braces, as well as 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.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Failure to diagnose or treat properly

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Berkow R, Fletcher AJ. In: Merck Manual of Diagnosis & Therapy. 15th ed. Rahway, NJ:. Merck, Sharp & Dohme Research Laboratories;1987:2107-8.
• Dandy DJ. In: Essential Orthopedics and Trauma. Edinburgh, Scottland:. Churchill Livingstone;1989:316, 424.
• Faingold R, Saigal G, Azouz EM, et al. Imaging of low back pain in children and adolescents. Semin Ultrasound CT MR. Dec 2004;25(6):490-505. [Medline].
• Freehill AK, Lenke LG. Severe kyphosis secondary to glucocorticoid-induced osteoporosis in a young adult with Cushing''s disease. A case report and literature review. Spine. Jan 15 1999;24(2):189-93. [Medline].
• Greenfield GB. In: Radiology of Bone Diseases. 5th ed. Philadelphia, Pa:. JB Lippincott;1990:173-4.
• McKenzie L, Sillence D. Familial Scheuermann disease: a genetic and linkage study. J Med Genet. Jan 1992;29(1):41-5. [Medline].
• Millner PA, Dickson RA. Idiopathic scoliosis: biomechanics and biology. Eur Spine J. 1996;5(6):362-73. [Medline].
• Nissinen M, Heliovaara M, Seitsamo J, Poussa M. Left handedness and riskof thoracic hyperkyphosis in prepubertal schoolchildren. Int J Epidemiol. Dec 1995;24(6):1178-81. [Medline].
• Platero D, Luna JD, Pedraza V. Juvenile kyphosis: effects of different variables on conservative treatmentoutcome. Acta Orthop Belg. Sep 1997;63(3):194-201.
• Rauschmann MA, Habermann B, Engelhardt M, Schwetlick G. [Pott triad and Schmorl nodules. A historical overview of kyphosis with specialreference to tuberculous spondylitis and Scheuermann disease]. Orthopade. Dec 2001;30(12):903-14. [Medline].
• Riddle EC, Bowen JR, Shah SA, et al. The duPont kyphosis brace for the treatment of adolescent Scheuermann kyphosis. J South Orthop Assoc. 2003;12(3):135-40. [Medline].
• Soo CL, Noble PC, Esses SI. Scheuermann kyphosis: long-term follow-up. Spine J. Jan-Feb 2002;2(1):49-56. [Medline].
• Turek SL. In: Buckwalter JA, ed. Turek's Orthopaedics: Principles and Their Application. 5th ed. Philadelphia, Pa:. Lippincott-Raven;1994:467-70.
• Waldis MF, Kissling RO. Evaluation and treatment of Scheuermann''s disease. Schweiz Rundsch Med Prax. Oct 30 1990;79(44):1326-33. [Medline].
• Wilde PH, Upadhyay SS, Leong JC. Deterioration of operative correction in dystrophic spinal neurofibromatosis. Spine. Jun 1 1994;19(11):1264-70. [Medline].

Article Last Updated: Jan 26, 2007