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Scheuermann's Disease

Scheuermanns Disease

Scheuermann’s disease, or juvenile kyphosis, is a rigid spinal kyphosis caused by osteochondrosis and wedging of the thoracic or thoracolumbar vertebrae. It is one of the most common causes of structural kyphosis in adolescents and leads to poor posture and backache.


Presentation

History of deformity and poor posture are the two most common clinical features. Parents often notice increasing kyphosis and poor posture in children when they bring them in for diagnosis. Onset of manifestations appear during puberty and the features are dependent on the location of changes. Those who have thoracic kyphoses may not have any symptoms other than slight deformity. Some of the patients may complain of discomfort in the region of kyphosis, but may not have much pain. Those who have thoracolumbar kyphoses may have straight flat back. The symptoms are seen during puberty. With lumbar localization of kyphosis, the pain may be more pronounced. About 80% of the patients with thoracolumbar kyphoses complain of intermittent, lower back pain. In most of the cases the pain may be relieved with rest [10].

The deformity with upper thoracic kyphoses can be best noted in a forward flexed position. Flexibility of the spine is considerably decreased and this differentiates it from flexible postural kyphosis. The upper and lower portion of the kyphosis may be tender on palpation. Thoracolumbar kyphosis may result in hamstring tightness.

Upper Back Pain
  • Your child will normally complain of severe and sudden upper back pain and stiffness during periods of rapid growth spurts this will be aggravated by sporting activities.[clinic-hq.co.uk]
  • Learn more: Thoracic Spine Anatomy and Upper Back Pain Kyphosis is any kind of excessive curvature of the thoracic spine.[spine-health.com]
  • Other patients with this condition may experience acute upper back pain, achiness and / or stiffness particularly during a period of rapid growth.[physioadvisor.com.au]
  • Other patients may experience upper back pain, generally mild to moderate, especially during rapid growth. Symptoms are usually exacerbated with increased physical activity, such as repetitive or forceful lifting and/or bending.[bendandmend.com.au]
  • Why Kinesiology Tape Helps Reduce Swelling and Bruising Quicker Call PhysioWorks Book Online Related Conditions General Information Upper Back Pain Posture Syndromes Posture Braces Joint Injuries Facet Joint Pain Muscle-related Injuries Back Muscle Pain[physioworks.com.au]
Spine Pain
  • pain and neck pain, i did have physio at the time but i now suffer really badly, after 4 children and several moree pregnancies i can hardly bend over some days and suffer from migraines etc, i did have acupuncture once which helps with the pain, what[mumsnet.com]
Distractibility
  • Anterior Kostuik-Harrington distraction systems. Orthopedics. 1988 Oct. 11(10):1379-91. [Medline]. Horn SR, Poorman GW, Tishelman JC, Bortz CA, Segreto FA, Moon JY, et al.[emedicine.com]
Forgetful
  • Moreover, it is necessary to exclude all curves below 10 degrees shown on the a.p. standing X-ray, not forgetting the fact that a seemingly mild scoliosis might be due in part to misleading projections and positions.[ncbi.nlm.nih.gov]
  • I took up pilates after advice form the physio and it took about a year, plus daily exercises but I now can pretty much forget I have it.[mumsnet.com]
Spastic Paraplegia
  • CLINICAL PRESENTATION: The authors report on a 15-year-old boy with progressive incomplete spastic paraplegia presenting segmental dysplastic thoracic spinous processes and Scheuermann's disease.[ncbi.nlm.nih.gov]
Hyperesthesia
  • Excellent maintenance of correction of the deformity in these patients has occurred with no serious complication to date in 24 patients followed from 19 months to 4 years although "transient hyperesthesia" occurred in 16% of patients.[ncbi.nlm.nih.gov]
Meningism
  • OBJECTIVES: Reported is the case of a patient with Scheuermann's disease who experienced spastic paraparesis caused by multilevel disc herniations and intraspinal meningeal cyst occurring together.[ncbi.nlm.nih.gov]

Workup

A certain degree of kyphosis is normal in all people. Kyphosis more than 50˚ is considered to be abnormal [11]. Radiographical studies help in the identification of diagnostic changes, characteristic of the disease [12]. The most prominent among the findings are:

  • Wedging of vertebral bodies
  • Rigid kyphosis
  • Narrow intervertebral spaces 
  • Calcification of intervertebral space
  • Irregularities on vertebral surface
  • Poorly formed vertebral plates 
  • Schmorl nodes, or herniations of nucleosus pulposus

According to Sorenson, the criteria for confirmatory diagnosis of Scheuermann’s disease include three components:

  • Kyphosis greater than 40˚
  • Irregularity in the endplates with loss of disk space
  • Wedging of 5˚ or more in three consecutive vertebrae

Histologic studies may show reduced ratio of collagen to proteoglycan. Electron microscopic studies may also show abnormalities in the endplate cartilage. The endplate matrix will be abnormal and this interferes with the normal growth of the vertebrae. Proteoglycan levels are increased considerably affecting the ratio to collagen.

Schmorl's Nodes
  • A unitary concept of childhood lumbar disc disease, Schmorl's nodes and Scheuermann's disease is suggested.[ncbi.nlm.nih.gov]
  • All had various disk problems including Scheuermann's disease, Schmorl's nodes and limbus vertebrae. All patients shared loss of disk height, altered disk hydration and variable herniation of nuclear material.[ncbi.nlm.nih.gov]
  • Incidences of associated SD and four radiographic signs of SD that were Schmorl's node, irregular vertebral end plate, posterior bony avulsion of the vertebra and wedge-shaped vertebra, average thoracolumbar kyphotic angle and incidences of disc herniation[ncbi.nlm.nih.gov]
  • MRI Dorso Lumbar region spine sagittal T2w images show: Multiple and contiguous involvement of vertebral bodies, the anterior wedging, antero posterior elongation, associated Schmorl's nodes, end plate irregularity and disc space narrowing.[neuroradiologycases.com]
  • Some of the disc then pushes into the vertebra and these are called Schmorl's nodes and are typically seen on an X-Ray. These Schmorl's nodes are present for life but are do not appear to cause any problems in the future.[physioworks.com.au]

Treatment

Physiotherapy, brace treatment and surgery are the three major suggested options in the treatment of Scheuermann’s disease. Physiotherapy is recommended in patients who have fixed kyphosis during pubertal growth spurt. It helps to reduce pain, particularly if kyphosis affects only a short segment. But, physiotherapy may not be useful in controlling the progression of the disease. A suitable sporting activity may be recommended, if the patient does not enjoy physiotherapy. Sporting activities help to manage postural abnormalities. Active, corrective physiotherapy is effective in case of fixed kyphosis. Rowing, cycling, drop handlebars and weight-lifting are generally not recommended for this condition.

Brace treatment is suggested for patients with kyphosis more than 50˚. Brace treatment may help in correcting the kyphosis in patients who have sufficient growth potential. With adequate growth spurts, wedging of vertebrae may be straightened by compensatory growth. Compliance with wearing brace is very important in a good outcome. Some of the braces that are useful in treatment are straightening braces with three-point action, and extending braces with neck ring. A schedule of exercise that helps in thoracic extensor strengthening and endurance may be used with bracing. Spinal extensor strengthening programs and aerobic exercise are helpful in reducing pain associated with this disease.

Surgery is rarely suggested for patients with Scheuermann’s disease. Unacceptable cosmetic appearance and spinal pain are the two indications for which surgery may be recommended. Spinal fusion is one option for patients with kyphosis greater than 75˚, particularly when the patient is not responding to non-surgical measures of management. Cord decompression is suggested for patients who have neurologic deficits and high degree of kyphosis.

Non-steroidal anti-inflammatory drugs are used to relieve pain. Restricting activity and relaxing also helps to reduce pain.

Prognosis

Patients with thoracic kyphoses lesser than 50˚ may not have any problem in adulthood. Pain is also less in them. The only burden in these patients is the psychological stress regarding their appearance. If the kyphoses is more than 50˚, pain may be severe when compared to a normal individual. Patients with more than 70˚ kyphoses may have disease progression in adulthood also [9]. If thoracolumbar vertebrae are affected, pain is more intense in adults. This is because of the shift in the center of gravity which is compensated by increased postural work by paravertebral muscles. Early diagnosis and treatment can help in controlling the symptoms successfully. 

Etiology

Etiology of Scheuermann’s disease is thought to be multifactorial. Osteochondritis of the upper and lower vertebral plates is considered to be one of the etiologic factors in the development of this disease. Mechanical factors and trauma are also implicated in the occurrence of juvenile kyphosis [1]. Hereditary factors are thought to be involved in the causation of the disease, but the mode of inheritance or presence of genetic markers are not yet clearly established [4]. Tall structure and higher BMI are also thought to be associated with the development of this condition [5]. Juvenile osteoporosis was also found to play a role in progressive kyphosis. Scheuermann’s disease is associated with endocrine abnormalities, inflammatory diseases, and certain neuromuscular disorders. The etiology of this association may indicate influence of genetic factors.

Epidemiology

Incidence of Scheuermann’s disease varies considerably in different reports. It ranges from 1% to 15% and the difference arises from the variation in the specified threshold value of kyphosis. Prevalence of this disease in United States is about 0.4 to 8%. Contradictory reports are also available regarding the prevalence of the disease in two genders. It is usually seen in children between the age group of 13 to 16 years [6]. Diagnosis is rarely made in children younger than 10 years. In a study, about 30% of tall girls, above 5 ft 11 inches, were found to have juvenile kyphosis [7].

Sex distribution
Age distribution

Pathophysiology

Scheuermann assumed aseptic bone necrosis as the major causative factor of this disease. But some later studies showed that weakening of the cartilaginous ring apophyses in the endplates of vertebra result in Scheuermann’s disease. Ring apophyses represent growth regions of the vertebrae. A number of theories have been put forward to explain the pathogenesis of this condition. This includes mechanical, metabolic, and endocrinologic theories.

One of the theories propose that the strength of apophyses weakens during pubertal growth spurt. According to this theory, increased pressure during growth spurt causes the disk tissue to protrude through apophysis into the body of the vertebra. This protrusion reduces the volume of nucleus pulposus, and thus, the disk appears narrow. Growth spurt creates an anterior pressure that impairs the growth of cartilaginous ring apophysis overall, leading atrophy. It also results in wedge vertebral formation. As kyphosis progresses, anterior pressure increases, thus leading to a vicious circle.

The genetic component thought to be involved in the development of the disease is not clearly defined. Transmission of the disease across three generations and the presence of identical changes in monozygotic twins are evidences for the presence of a hereditary component in this disease. An autosomal dominant pattern of inheritance was shown in some of the familial Scheuermann’s disease [8]. Hormonal disturbances were implicated in the development of the disease with secondary effects like height and weight gain [6].

Prevention

Prevention of Scheuermann’s disease may not be possible as it can be caused by several factors. But future dysfunctions and problems can be prevented by taking adequate care. Having good flexibility and strong core muscles help in maintaining a good posture and controlling the symptoms. It will also help in limiting all future issues associated with the disease.

Summary

Scheuermann’s disease, or juvenile kyphosis, is a rigid spinal kyphosis caused by osteochondrosis and wedging of the thoracic or thoracolumbar vertebrae [1]. It is one of the most common causes of structural kyphosis in adolescents and leads to poor posture and backache [2]. Originally described by Scheuermann in 1921, two variants of the same disease were described later by Alexander – the classical form and the traumatic form [3]. In the classical form changes occur in T7 to T10 levels, while the traumatic form is characterized by changes in the thoracolumbar junction. Severe deformity of the vertebrae is associated with pain. Early diagnosis helps in successful treatment of the condition, mostly using nonsurgical interventions like exercises and bracing.

Patient Information

Scheuermann’s disease or juvenile kyphosis is a developmental disorder of the spine characterized by increased round back or hunch back. People who have such a hunch may or may not have pain. A normal person has a bend (kyphosis) at the upper back and this forms a part of the S-shape of a normal spine. A bend or angle more than 45˚ is considered abnormal. Patients with Scheuermann’s disease have an excessive kyphosis which changes the appearance.

The actual cause of the disease is not yet completely defined. Many factors are implicated in the development of this spine condition. Mechanical factors, hormonal disturbances, poor posture, and genetic factors, are all thought to be involved in the development of Scheuermann’s disease. Abnormal growth of the vertebral body results in increased kyphosis. Anterior part of the growth plate stops growing while the posterior part resumes growing, resulting in hunch. If the hunch back is severe, the patient may experience pain which may be intermittent and dull. It is normally noted in children between 13-16 years of age.

Those who have mild form of this disease may not have any other symptoms other than the change in appearance. The symptoms often appear during puberty and the severity may depend on the site of kyphosis. Patients who have kyphosis at the lower back may have pain and a strikingly flat back. The curve may increase in size when the spine grows. Pain, when present, increases with activity. Degree of pain may vary from one person to another. Radiography is the most common diagnostic technique used to confirm Scheuermann’s disease.

There is no standard treatment for this disease. Physiotherapy, bracing and surgery are the three commonly used methods for treating the condition. Physiotherapy is suggested if the kyphosis is present in the upper back and is less than 50˚. Those who do not prefer this method may also opt for some sporting activity other than rowing, cycling and weight-lifting. Bracing is considered for patients who have kyphosis of more than 50˚ and is still in the growing phase. It helps to stabilize the bend and also to correct it if the patient is still in growth spurt. The only precondition for success of this technique is the compliance from the patient to wear the brace continuously. Pain killers and rest are recommended to reduce pain associated with the disease. Surgery is recommended for curves greater than 70˚, and is cosmetically unacceptable. The spine is pulled straight with the help of rods. Pain eventually reduces and the most of the patients may be able to get back to normal routine soon.

References

Article

  1. Ristolainen L, Kettunen JA, Heliövaara M, Kujala UM, Heinonen A, Schlenzka D. Untreated Scheuermann's disease: a 37-year follow-up study. Eur Spine J. 2012;21(5):819-824. 
  2. Lowe TG, Line BG. Evidence based medicine: analysis of Scheuermann kyphosis. Spine. 2007 1;32(19 Suppl):S115-9.
  3. Alexander CJ. Scheuermann’s disease: a traumatic spondylodystrophy? Skeletal Radio. 1977;1:209-221. 
  4. Damborg F, Engell V, Andersen M, et al. Prevalence, concordance, and heritability of Scheuermann kyphosis based on a study of twins. J Bone Joint Surg Am. 2006;88(10):2133-2136.
  5. Tsirikos AI, Jain AK. Scheuermann's kyphosis; current controversies. J Bone Joint Surg Br. 2011;93(7):857-864. doi: 10.1302/0301-620X.93B7.26129.
  6. Fotiadis E, Kenanidis E, Samoladas E, Christodoulou A, Akritopoulos P, Akritopoulou K. Scheuermann's disease: focus on weight and height role. Eur Spine J. 2008;17(5):673-8.
  7. Skogland LB, Steen H, Trygstad O. Spinal deformities in tall girls. Acta orthop Scand. 1985;56:155-157. 
  8. McKenzie L, Sillence D. Familial Scheuermann disease: a genetic and linkage study. J Med Genet. 1992;29(1):41-45.
  9. Bradford DS. Vertebral osteochondrosis (Scheuermann’s kyphosis). Clin Orthop. 1981;158:83-90. 
  10. Haveman LM, van Es HW, ten Berge-Kuipers M. [Complaints of back pain in childhood: find curable causes]. Ned Tijdschr Geneeskd. 2008;152(7):353-8.
  11. Sugrue PA, O'Shaughnessy BA, Blanke KM, et al. Rapidly progressive Scheuermann's disease in an adolescent after pectus bar placement treated with posterior vertebral-column resection: case report and review of the literature. Spine (Phila Pa 1976). 2013;38(4):E259-62.
  12. Summers BN, Singh JP, Manns RA. The radiological reporting of lumbar Scheuermann's disease: an unnecessary source of confusion amongst clinicians and patients. Br J Radiol. 2008;81(965):383-5.

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Last updated: 2018-06-22 02:40