Scoliosis in children

One of the most common orthopedic diseases is scoliosis in children, or lateral curvature of the spine, combined with its torsion (ICD-10 code M41). The frequency of scoliotic deformations of the spine, according to various authors, ranges from 3 to 7%, with 90% being idiopathic scoliosis. Scoliosis in children occurs in all races and nationalities, and is more common in women - up to 90%.

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Epidemiology of scoliosis in children

The prevalence of any pathological condition can only be determined by screening large populations. This is the number of individuals in the population with scoliotic deformity at any given time. Two types of surveys have been conducted: chest radiography-based tuberculosis screening and school screening. The number of subjects screened ranged from 10,000 to 2,500,000, with the incidence of spinal deformities (the vast majority of which were idiopathic scoliosis in children) ranging from 1.0 to 1.7%. All studies considered only deformities exceeding 10° according to Cobb.

Is the prevalence of scoliosis the same throughout the world? Are there racial, national, or geographic differences? In Japan, a study of 2,000 schoolchildren in Chiba found structural deformities in 1.37% of cases, and Takemitzu, in a study of 6,949 schoolchildren in Hokkaido, found scoliosis in children in 1.9% of cases. Skogland and Miller, who conducted a study in northern Norway, found scoliosis in children in Lapps in 0.5% of cases, and in the rest of the population in 1.3%.

Dommisse in South Africa found scoliosis in children in 1.7% of cases in a survey of 50,000 white children in Pretoria (90% of scoliosis is idiopathic). Segil in Johannesburg examined Caucasian and Negroid (Bantu) schoolchildren and found deformities of 10° or more in 2.5% of Caucasians and only 0.03% in Negroids. Span et al. examined 10,000 schoolchildren aged 10-16 in Jerusalem. Deformities were detected twice as often in Jewish schoolchildren as in Arabs.

According to the etiological factor, scoliosis is classified as follows.

• Idiopathic scoliosis in children, i.e. scoliosis of unknown cause.
• Congenital scoliosis in children is caused by congenital malformations of the vertebrae.
• Scoliosis in children with systemic congenital diseases of the musculoskeletal system (Marfan syndrome, Ehlers-Danlos syndrome, etc.).
• Paralytic scoliosis in children, as a result of poliomyelitis or after a spinal cord injury.
• Neurogenic scoliosis in children, the cause of which is degenerative-dystrophic diseases of the spine or other neurological diseases.
• Cicatricial scoliosis in children is the result of severe burns or extensive surgical interventions on the chest organs.

Classification of scoliosis types by localization of the main arc (apex) of curvature.

• Upper thoracic (apex of curvature: III-IV thoracic vertebra).
• Thoracic (apex of curvature: VIII-IX thoracic vertebra).
• Thoracolumbar (apex of curvature: XI-XII thoracic vertebra or I lumbar).
• Lumbar (apex of curvature of the II-III lumbar vertebra).
• Combined (or S-shaped) with the same amount of curvature and torsion in both arcs located in different parts of the spine, the difference in size is no more than 10°.

Depending on the direction of the convexity of the curvature arc, a distinction is made between left-sided, right-sided and combined scoliosis in children (combines two main differently directed curvature arcs).

To determine the severity of scoliosis, the classification of V.D. Chaklin (1963) is used, based on measuring the angle of the main arc of curvature on an X-ray of the spine, taken in a direct projection with the patient standing: I degree - 1-10°, II degree - 11-30°, III degree - 31-60°, IV degree - more than 60°.

An example of a diagnosis formulation: “idiopathic right-sided thoracic scoliosis in children, grade III.”

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Pathogenesis of scoliosis

The main pathogenetic links in the development of idiopathic scoliosis are pathological rotation of the vertebrae in the horizontal plane, displacement towards the nucleus pulposus, frontal tilt of the spine and torsion of the vertebrae.

Etiological classification of scoliosis

I. Idiopathic scoliosis in children

• Infantile scoliosis in children (from birth to 3 years).
  • Self-resolving.
  • Progressive.
• Juvenile scoliosis in children (3 to 10 years old).
• Adolescent scoliosis in children (over 10 years old).

II. Neuromuscular scoliosis

A. Neuropathic scoliosis in children.

• 1. Scoliosis in children due to damage to the upper motor neuron:
  • cerebral palsy:
  • spinocerebellar degeneration;
    • Friedreich's disease;
    • Charcot-Marie-Tooth disease;
    • Roussy-Levy disease;
  • syringomyelia;
  • spinal cord tumor;
  • spinal cord injury;
  • other reasons.
• Based on lower motor neuron damage:
  • polio;
  • other viral myelitis;
  • injury;
  • spinal muscular atrophy:
    • Werdnig-Hoffmann disease;
    • Kugelberg-Welander disease;
    • myelomeningocele (paralytic).
• Dysautonomy (Riley Day syndrome).

B. Myopathic scoliosis in children

• Arthrogryposis
• Muscular dystrophy,
• Congenital hypotension,
• Dystrophic myotonia.

III. Congenital scoliosis in children

A. Violation of formation.

• Wedge-shaped vertebra.
• Half-vertebra.

B. Violation of segmentation.

• Unilateral scoliosis in children.
• Bilateral scoliosis in children.

B. Mixed anomalies.

IV. Neurofibromatosis.

V. Mesenchymal pathology.

• Marfan syndrome.
• Ehlers-Danlos syndrome.

VI. Rheumatoid diseases.

• Juvenile rheumatoid arthritis.

VII. Traumatic deformities.

• After the fracture.
• After surgery:
  1. Postlaminectomy.
  2. Postthoracoplastic.

VIII. Scoliosis in children due to contractures of extravertebral localization.

• After empyema.
• After burns.

IX. Osteochondrodystrophic scoliosis in children.

• Dystrophic dwarfism.
• Mucopolysaccharidosis (eg, Morquio disease).
• Spondyloepiphyseal dysplasia.
• Multiple epiphyseal dysplasia.
• Achondroplasia.

X. Scoliosis in children due to osteomyelitis.

XI. Metabolic disorders.

• Rickets.
• Osteogenesis imperfecta.
• Homocystinuria.

XII. Scoliosis in children due to pathology of the lumbosacral joint,

• Spondylolysis and spondylolisthesis.
• Congenital anomalies of the lumbosacral joint.

XIII. Scoliosis in children due to tumors.

• A. Vertebral column.
  • Osteoid osteoma.
  • Histiocytosis X.
  • Other.
• B. Spinal cord.

The term "idiopathic" applied to scoliosis as a nosological entity means that its origin at this stage of development of medical science remains unknown. Since Hippocrates first gave a clinical description, and Galen proposed a number of terms to define spinal deformities (scoliosis in children, kyphosis, lordosis, strophosis), thousands of years have passed, but there is no single point of view on the root cause of idiopathic scoliosis. Many proposals and hypotheses have been put forward, some of them are hopelessly outdated (scoliosis in school-age children, rachitic scoliosis in children) and deserve no more than a mention.

Studies of vertebral and paravertebral structures revealed numerous morphological and chemical changes in the tissues. However, in no case was there any reason to categorically assert that the noted deviations from the norm were the cause of the development of scoliotic deformation, and not the consequence of its drinking.

The hormonal status of patients with scoliosis has been studied repeatedly - deviations in the functioning of the pituitary-adrenal system and the adrenal cortex itself have been found, and changes in the content of sex hormones have been identified.

M.G. Dudin studied the content of direct-acting osteotropic hormones (calcitonin, parathyroid hormone, somatotropin, and cortisol) in patients with scoliosis. Non-progressive course of idiopathic scoliosis was noted with high concentrations of cortisol and parathyroid hormone. Scoliosis progresses in children with the opposite ratio: high content of calcitonin and somatotropin. According to M.G. Dudin, two systems influence the growth of the spine - the nervous and endocrine systems. Increased hormone synthesis stimulates the growth of the spine, which creates unfavorable conditions for the spinal cord. Due to the peculiarities of the anatomical relationships between the bone structures of the spine and the spinal cord, a situation arises in which the anterior sections of the spinal column are longer than the posterior ones. Compensation for this condition, which arose as a result of the imbalance in the action of the nervous and endocrine systems, occurs due to the twisting of the elongated anterior sections of the spine around the relatively shortened posterior ones. Clinically and radiographically, this is manifested by torsion of the vertebrae.

Dysfunction of the nervous and endocrine systems becomes the trigger for the development of idiopathic scoliosis and in accordance with the theory of Sevastik. It is based on the dysfunction of the sympathetic nervous system. As a result, hyperemia of the left half of the chest develops, as a result of which the ribs on this side begin to grow rapidly. It is the asymmetric growth of the ribs that causes gross deformation of the chest and spine. Sevastik emphasizes that scoliosis itself in children is secondary in relation to the deformation of the rib cage of the chest.

In recent years, there have been increasing reports of the familial nature of idiopathic scoliosis. Various studies have proposed models of inheritance of idiopathic scoliosis: multifactorial, sex-linked inheritance, autosomal dominant inheritance of the disease with incomplete penetrance of genotypes. Several attempts have been made to identify the gene responsible for the development of idiopathic scoliosis. Structural genes of extracellular matrix components: elastin, collagen and fibrillin were considered as candidates. However, none of the genes demonstrated sex-linked inheritance responsible for the development of the disease. Thus, the mechanisms of inheritance of idiopathic scoliosis have not yet been established.

It is known that there are gender differences in the manifestation of this disease. It is diagnosed in girls much more often than in boys.

The sex ratio among patients with idiopathic scoliosis varies from 2:1 to 18:1. Moreover, polymorphism increases with increasing severity of the disease. This makes the analysis of the inheritance of idiopathic scoliosis particularly difficult.

The studies showed that in all groups of relatives the purity of idiopathic scoliosis is significantly higher than the average for the population. This confirms the known data on the family aggregation of the pathology under study. In addition, it turned out that the frequency of scoliosis among the sisters of probands is higher than among the brothers. This also agrees well with the known data. At the same time, no significant differences were found in the frequency of idiopathic scoliosis in the fathers and mothers of probands.

Segregation analysis showed that the inheritance of severe (grades II-IV) forms of scoliosis can be described within the framework of an autosomal dominant majorgene diallelic model with incomplete penetrance of genotypes depending on gender and age. At the same time, the penetrance of genotypes carrying the mutant allele is approximately twice as high in girls as in boys. This is in good agreement with the known data on the frequency of idiopathic scoliosis in children. If the existence of the majorgene can be proven with a high degree of certainty, it can be expected that further studies will localize it and find a way to influence the majorgene in order to prevent the development of the pathological process.

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How is scoliosis recognized in children?

In pediatric practice, timely diagnosis of idiopathic scoliosis is of primary importance. This is a severe orthopedic disease, expressed in multiplanar deformation of the spine and chest. The name "idiopathic" implies a cause of the disease unknown to modern science. However, various signs of dysplastic development of the skeleton and their combination play an important role in the predisposition to this type of scoliosis: hypoplasia of the vertebrae, disruption of their segmentation, bone dysplasia of the lumbosacral spine, underdevelopment or asymmetry of the development of the 12th pair of ribs, anomalies in the development of teeth and bite, asymmetry and deviations in the development of the skull, flat feet, etc. A careful neurological examination reveals deviations in tendon-muscle reflexes, which may indicate myelodysplastic processes. The detection of these signs allows us to talk about the dysplastic nature of idiopathic scoliosis.

A complete interpretation of the diagnosis of scoliosis in children requires determining the etiology, localization and direction of the arc of curvature of the spine, as well as the severity of the scoliosis.

X-ray examination

Spinal radiography is performed in direct and lateral projections. In a standing position. Radiographs are used to determine the localization of the curvature arc, measure its size, the degree of pathological rotation (according to the projection of the bases of the vertebral arches), the shape and structure of the vertebral bodies and intervertebral discs, identify dysplastic signs of bone tissue development, assess the degree of osteoporosis, bone age (according to the degree of ossification of the apophyses of the vertebral bodies) to determine the prognosis of the further course of the disease. X-ray functional examination is performed to determine the stability or mobility of the deformation. This is also important to know to determine the prognosis of the disease and indications for further treatment.

Differential diagnostics

Initial idiopathic scoliosis in children should be differentiated primarily from postural disorders in the frontal plane. The cardinal distinguishing feature is the presence of pathological rotation and torsion of the vertebrae in scoliosis, and clinically - the appearance of a costal hump and muscle ridge. In addition, idiopathic scoliosis in children should be differentiated from spinal deformities caused by other diseases: congenital scoliosis in children, neurogenic scoliosis, cicatricial scoliosis after chest surgery and burns, scoliosis against the background of systemic hereditary diseases.

Congenital scoliosis develops as a result of malformations of the vertebrae, which are determined radiologically.

The most common anomaly is lateral wedge-shaped vertebrae and hemivertebrae. They can be found in any section of the spine, but more often in the transitional ones, and can be single or multiple. Sometimes this anomaly is combined with other malformations of the vertebrae and spinal cord. If the lateral hemivertebrae are on one side, the curvature quickly becomes pronounced, rapidly progressing, and there are indications for surgical treatment, since such a deformation can lead to neurological movement disorders due to compression of the spinal cord.

If the hemivertebrae are located on opposite sides or the hemivertebra fuses with the normally developed vertebra above and below (i.e. a bone block is formed), the course of scoliosis in such cases is more favorable.

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How is scoliosis treated in children?

The goal of conservative treatment of scoliosis is to prevent further progression of the spinal deformity. Treatment principles: axial unloading of the spine and creation of a strong muscular corset by using physical therapy methods. Therapeutic gymnastics - a special set of exercises corresponding to the individual diagnosis, is performed initially on an outpatient basis, under the supervision of a physical therapy specialist, and then at home for 30-40 minutes. Massage of the back and abdominal muscles is prescribed (2-3 courses per year for 15 sessions). Regular swimming in the "breaststroke" style is recommended. Physiotherapy treatment includes electrophoresis of drugs to improve the trophism of the spinal cord, bone and perivertebral soft tissues in the area of the main arc of curvature, electrical myostimulation on the convex side of the deformity. To unload the spine, when necessary, ready-made orthopedic corsets can be used. For the treatment of progressive scoliosis, corrective corsets of the Cheneau type are currently used in world practice.

Comprehensive conservative treatment is carried out in a local clinic, in specialized kindergartens or boarding schools, in sanatoriums for children with disorders and diseases of the musculoskeletal system.

Surgical treatment of scoliosis

Its goal is the maximum possible correction of the deformed spine and its lifelong stabilization under conditions of normal balance of the body. For surgical treatment, various technologies are used depending on the localization and severity of the curvature (including the use of corrective metal structures and osteoplastic fixation of the spine).

The optimal age for surgical treatment is 15-16 years, when growth potential is reduced and the risk of loss of correction is minimal.

How to prevent scoliosis in children?

Given the unclear cause of scoliosis, there is no specific prevention. However, if a child is found to have a posture disorder, courses of therapeutic exercise, general strengthening massage and regular swimming are recommended. In preschool and school classes, teachers should monitor the child in terms of maintaining correct posture.

What is the prognosis for scoliosis in children?

The prognosis of the course of scoliosis depends on a combination of anamnestic, clinical and radiological signs. The earlier pronounced scoliosis first appears in children, the more progressive its course becomes. Thoracic scoliosis is less favorable than lumbar scoliosis. Scoliosis in children progresses most during puberty, and after the end of spinal growth (complete ossification of the vertebral apophyses), the curvature stabilizes.

Idiopathic scoliosis in children is inherited in rare cases (6-8%).

With adequate conservative treatment, carried out until the child's growth is complete, scoliosis in children has a more favorable prognosis.