Definition of the disease. Causes of the disease
Scoliosis is a complex spinal deformity in both the frontal and sagittal planes, accompanied by vertebral twisting.
In the early stages of the disease, only lateral curvature occurs, but as the degree of deformation increases, curvature in the sagittal plane joins, and vertebral twisting around the vertical axis (in the horizontal plane) increases.
All authors note that among girls, the frequency of scoliotic disease development is significantly higher and relative to boys, it ranges from 1:3 to 1:6. The prevalence of scoliosis among girls is explained by several reasons: muscle weakness due to less activity and mobility of girls compared to boys, hormonal background characteristics, less mature physical development.
Causes of scoliosis development:
- Congenital – spinal developmental anomalies such as complete and additional congenital wedge-shaped vertebrae, asymmetric synostosis (fusion) of vertebral bodies and transverse processes, congenital rib synostosis, etc.;
- Neuro-muscular – insufficient and weak musculo-ligamentous apparatus of the spine, congenital hypotonia (reduced tone) of muscles against the background of syringomyelia (a chronic disease of the central nervous system), myopathies (chronic progressive neuro-muscular diseases), cerebral palsy (CP), multiple sclerosis, etc.;
- Syndrome-related – develop against the background of connective tissue dysplasia in Marfan syndrome, Ehlers-Danlos syndrome;
- Idiopathic – causes of development are not established;
- Secondary – develop after fractures, surgeries, scar contractures (joint movement restrictions), surgical interventions on the thoracic cage.
According to the works of I.A. Movshovich (1964, 1965, 1969), the development and progression of scoliotic disease occur with the realization of three factors:
- The presence of dysplastic changes in the spinal cord, vertebrae, discs, which disrupt normal spinal growth;
- Metabolic and hormonal disorders that create a general pathological background of the body and contribute to the manifestation of the first factor;
- Static-dynamic disorders in the form of increasing asymmetric load on the spine during the period of skeletal growth, leading to wedge-shaped vertebral growth.
Symptoms of scoliosis
Signs of scoliosis can be detected during body examination in three positions: standing from the front, side, and back; when leaning forward; and lying down.
During frontal examination, attention is drawn to the presence of asymmetry in facial features, neck and shoulder contours, waist triangles, and asymmetrical positioning of nipples. Deformation of the chest and leaning of the torso to one side are also noted.
During lateral examination, a curved posture and sagittal profile of the spinal column are determined, along with the presence of kyphotic (backward curvature) or lordotic (forward curvature) components of spinal deformity.
During back examination, lateral deviation of the spinous processes line of the spine, presence of rib hump, and lumbar roll are identified. The rib hump (gibbus) forms due to chest deformation caused by spinal curvature and rotational displacement around the longitudinal axis in the thoracic region. The posterior (dorsal) rib hump is always located on the convex side of the curvature and can be flat or sharp.
The lumbar muscle roll on the convex side of the curvature arises from the elevation of the long back muscles as the transverse processes of the vertebrae twist and rotate. These muscles form a protrusion under the skin, which is a well-defined and clearly contoured rounded roll located paravertebrally (along the spine). The appearance of this symptom indicates spinal curvature along the vertical longitudinal axis and is a manifestation of vertebral torsion (rotation). According to Movshovich, this concept should distinguish between two elements: vertebral deformation due to asymmetric growth of its individual parts and rotation-sliding of one vertebra relative to the adjacent one, combined with rotation of the entire scoliotic curve towards the convexity of the deformity. The presence of vertebral torsion occurs after the development of curvature in the frontal and sagittal planes and indicates the progression of scoliotic disease. Gibbus and muscle roll are best determined in the Adams position – when the body is bent forward.
Also evaluated are the mobility of the spinal column, paravertebral tenderness upon palpation, length of the lower limbs, and pelvic tilt. The latter symptom is determined in a standing position by the level of the iliac crests, often associated with the length of the lower limbs – if one is shorter, there is a difference in the level of pelvic tilt.
The length of the lower limbs, that is, the distance from the anterior superior iliac spine (bony prominence) of the iliac bone to the outer ankle, should be equal. Limb length is measured lying on the back, legs together. The length of the legs is assessed by comparing the ankles under the condition that the tip of the nose, navel, and the junction of the feet are on the same straight line.
Pathogenesis of scoliosis
Currently, the elucidation of the etiology and pathogenesis of scoliosis revolves around the structural elements of the spinal column, spinal musculature, collagen structures, endocrine system, vestibular apparatus, and genetic predisposition.
Several theories of the etiology of idiopathic (of unknown origin) scoliosis are relevant, namely the genetic, endocrine, and neuromuscular theories.
However, despite numerous studies and proposed theories, there are still no definitive mechanisms of inheritance for idiopathic scoliosis and clarity regarding the influence of hormonal profile on disease development.
In the thoracic region, as the thoracic curvature and torsion develop, there is displacement of the thoracic vertebral bodies towards the convex side of the curvature. As a result, the anterior surface of the thoracic vertebrae faces towards the convexity of the curve, the root of the arch on the convex side elongates, the vertebral foramen widens on the convex side and appears narrower on the concave side. The vertebrae become wedge-shaped deformed. Wedge-shaped deformation also occurs with intervertebral discs, which are significantly narrowed on the concave side and undergo deep dystrophic changes.
In scoliosis, the spine changes position and shape: the transverse processes on the convex side deviate backward and become more massive. The articular processes on the concave side assume a more horizontal position, their articular facets (zygapophyseal joints, connecting the vertebrae in the posterior segment) widen. New articulating surfaces are formed on adjacent arches. The spinous processes of the thoracic vertebrae also deviate towards the convex side, the half-arch of the convex side is shortened compared to the half-arch of the concave side.
Significant changes occur in the ligamentous apparatus: as the curvature develops, the anterior longitudinal ligament shifts towards the convex side, where it becomes fibrous and thins. On the concave side, part of the anterior longitudinal ligament thickens, becoming stronger and more tense, which contributes to additional fixation of the deformation.
The spinal canal in scoliosis becomes uneven and narrows on the concave side of the curvature, while expanding on the convex side. The dural sac (protective membrane of the spinal cord) and its contents are pressed into the anterior-lateral wall of the concave part of the spinal canal, being separated by a significant layer of epidural adipose tissue from the convex surface of the curvature wall of the spinal canal. The dural sac may be fixed to the concave bony wall of the spinal canal by fibrous adhesions and mimic thickening of the dura mater along the concave surface of the curvature.
Significant changes occur in the ribs and thoracic cage as a whole on the convex side of the curvature in the frontal, sagittal, and horizontal planes. The ribs are positioned more vertically and may overlap each other, intercostal spaces widen, the rib in the area of its angle deforms, forming a hump. On the concave side of the curvature, the ribs come closer together, fibrous adhesions may occur between them, and intercostal muscles undergo extreme degeneration.
Classification and Stages of Scoliosis
In the etiological classification, the following groups of scoliosis are distinguished:
- Congenital scoliosis group – deformity develops due to gross abnormalities in the development of the spine, such as wedge-shaped vertebrae, hemivertebrae, rib fusion, transverse processes, etc.
- Neuromuscular scoliosis group – develops due to myopathies, cerebral palsy, syringomyelia, multiple sclerosis, spinal cord injury, etc.
- Connective tissue dysplasia-related scoliosis group – Marfan syndrome, Ehlers-Danlos syndrome.
- Post-traumatic scoliosis group – develops after fractures, surgeries, in the context of scar contractures (joint motion limitations) after burns, purulent complications, and surgical interventions on the organs of the chest.
- Scoliosis group due to contractures of non-spinal localization.
- Scoliosis group due to rare pathologies – scoliosis due to osteomyelitis, metabolic diseases (homocystinuria, imperfect osteogenesis), tumors.
- Non-structural scoliosis group includes postural (habitual), dynamic (resemble postural scoliosis but can spontaneously disappear and recur), antalgic (reflexive spinal curvature in the frontal plane with hypertonicity of lumbar and thoracic muscles), inflammatory, etc.
Idiopathic scoliosis and dysplastic types of scoliosis, based on congenital features of the lumbar-sacral spine, stand out as separate groups, for example:
- spina bifida posterior – non-closure of the vertebral arch;
- lumbarization – the first sacral vertebra is partially or completely separated from the sacrum, forming an additional lumbar vertebra;
- sacralization – the fifth lumbar vertebra fully or partially fuses with the sacrum, etc.
In 80% of cases, doctors encounter this form of the disease in everyday practice.
Types of scoliosis based on the location of the primary curvature:
- Cervicothoracic (or upper thoracic);
- Thoracic;
- Thoracolumbar;
- Lumbar;
- Combined, or S-shaped, when two primary curves of curvature occur.
Radiological classification according to V.D. Chaklin:
- Grade 1. Scoliosis angle 1°-10°;
- Grade 2. Scoliosis angle 11°-25°;
- Grade 3. Scoliosis angle 26°-50°;
- Grade 4. Scoliosis angle > 50°.
Scoliosis classification by the shape of the curvature:
- C-shaped scoliosis (with one curve of curvature);
- S-shaped scoliosis (with two curves of curvature);
- Z-shaped scoliosis (with three or more curves of curvature).
Forms of scoliosis based on changes in the static function of the spine:
- Compensated (balanced) – when compensatory counter-curvature develops in the lumbar spine with thoracic scoliosis;
- Uncompensated (unbalanced) – when scoliotic curvature is present only in the thoracic or lumbar regions of the spine.
Complications of scoliosis arise from the formation and progression of this complex spinal pathology, leading to deformity of the thoracic cage, disruption of the proper positioning of organs in the thoracic and abdominal cavities, and functional impairments in many body systems. Additionally, it is accompanied by pronounced cosmetic defects.
The progressive curvature of the spine most commonly causes:
- Changes in the anatomy and function of the thoracic cage. This results in a reduction in lung function and significant ventilation insufficiency, leading to chronic hypoxia.
- Development of right ventricular insufficiency due to hypertension in the pulmonary circulation and the formation of the “scoliotic heart” symptom complex (shortness of breath, chest pain, difficulty breathing, fainting, poor tolerance of any physical exertion, increased heart rate, chest pain, numbness in the limbs, night sweats, edema of the lower extremities, cyanosis or blueness of the lips, cold hands and feet).
- Dropping of the liver and kidneys into the pelvic cavity, disrupting the functions of these organs and the motor-evacuation function of the intestine due to serious disruptions in the topography of the internal organs. In severe forms of scoliosis, the shape and position of the kidneys change, and the ureters lose their physiological curves and follow the curve of the curved spine. All of this weakens the functional capacity of the kidneys, leading to a decrease in glomerular filtration and an increase in the level of endogenous creatinine. Normal urodynamics may be disrupted, leading to the development of inflammatory diseases of the urinary system.
- Dystrophic changes in the intervertebral discs and synovial joints of the spine. These develop relatively early and manifest as pain syndrome characteristic of osteochondrosis with radicular syndrome or a range of other autonomic symptoms. All of this significantly reduces the residual employability of adults and worsens their quality of life.
- Severe spinal disorders up to spastic and even flaccid lower limb paralysis (reduction in muscle strength) and paralysis. This occurs in some cases with disorders of blood, lymph, and cerebrospinal fluid circulation, when stagnation occurs. A symptom complex of functional spinal insufficiency develops in the form of a disturbance in the pain sensitivity of a radicular nature, anisoreflexia (irregularities) of tendon and periosteal reflexes, and neurological pain symptoms up to progressive ischemic myelopathy.
As a result of all these factors, asthenization (exhaustion) of the body occurs, and a person may become disabled due to pronounced functional and organic disorders.
Scoliosis Diagnosis
Spondylography is the mandatory final diagnostic method for specifying the degree and localization of spinal curvature. It objectifies the visual clinical picture of the pathology, displaying the state of the vertebral growth potential and structural morphological changes in the curved spine. X-ray imaging is usually performed standing in two projections: anteroposterior with the capture of the iliac crests and lateral. In some cases, additional examination of the spinal column in a supine position and functional spondylograms with lateral body inclination are required.
Initially, spondylograms are visually analyzed, followed by a series of simple geometric constructions to determine the magnitude of the scoliotic curve angle. The Cobb method is the most common, where the extreme vertebrae of the formed curve are identified, and two straight lines are drawn along the cranial (upper) endplate of the upper vertebra and the caudal (lower) endplate of the lower vertebra. From these lines, perpendiculars are drawn and dropped, and the intersection determines the angle equal to the magnitude of the scoliotic deformation.
Additionally, it is crucial to determine the presence of vertebral rotation around the vertical axis of the spine and assess the degree of their rotation. It is also necessary to identify the presence of active vertebral bone growth plasticity, which is determined by Sadov’s radiological tests from S-0 to S-IV. Local age is assessed based on the development of apophyseal zones (growth zones) of vertebral bodies and often lags behind the chronological age in children with severe and progressive forms of scoliosis. Furthermore, the Risser test is evaluated based on the state of the iliac crest apophyses and the process of their fusion from R-0 to R-V.
The following characteristics are determined based on the results of the examinations:
- Type of scoliotic deformation;
- Anatomical type of scoliosis;
- Deformation parameters characterizing the degree of spinal curvature in the frontal plane;
- State of vertebral bone maturity as one of the most important background factors for predicting the natural course of the disease.
Improper frontal plane posture on an X-ray taken standing will show spinal column curvature without signs of pathological vertebral rotation, whereas on a supine X-ray, the curvature will be absent.
Treatment of Scoliosis
Both conservative and surgical methods are used for treating scoliosis. Conservative treatment aims to correct and stabilize the curved spine, create optimal conditions for the child’s growth and development, prevent secondary pathology of internal organs, and prevent early degenerative changes in the spinal column. Conservative measures include specialized therapeutic exercises (physical therapy), brace therapy, and adjunctive methods.
Medication Treatment
If a scoliosis patient suffers from severe chronic pain, symptomatic therapy is prescribed, including:
Nonsteroidal anti-inflammatory drugs (NSAIDs): Used to alleviate severe pain caused by exacerbation of the inflammatory process.
Corticosteroids: Hormonal drugs with pronounced anti-inflammatory effects, typically prescribed if scoliosis is bothering the patient against the background of an exacerbation of rheumatoid condition.
Chondroprotectors: Auxiliary drugs used in the comprehensive treatment of musculoskeletal system pathologies, although their effectiveness is debated and not fully proven.
Calcium preparations: Calcium is an essential building material for bones. Deficiency can lead to bone demineralization, making them more brittle.
Muscle relaxants: These drugs relax tense muscles and help relieve strain on the overloaded spine.
Heating ointments and gels: Improve blood circulation.
Surgical Treatment
In cases of ongoing progression of scoliosis and lack of effect from adequate conservative treatment, surgical correction of the scoliotic curve is indicated. The approach to surgical treatment of children with idiopathic scoliosis should be strictly individualized. The choice of surgical intervention method depends on the patient’s age, degree of bone plasticity, severity, and mobility of spinal deformity. In recent years, there has been a trend towards using metal constructions with transpedicular supporting elements. This type of spinal system allows for greater correction of curvature in the treatment of scoliosis in adult patients, stable fixation in the postoperative period, reduction of the length of the metal fixation zone, and promotes true derotation of vertebral bodies at the apex of the curvature. Postoperative treatment includes respiratory gymnastics, massage of the lower and upper limbs, physical therapy, and physiotherapy. Surgical treatment is carried out in specialized vertebrological centers or large multi-profile hospitals.
Bracing
Treating children with severe and progressive scoliosis during continued growth is a complex medical task. It aims to restrain the progression of the curvature, stabilize the deformity, and reduce the initial scoliotic curve. Currently, brace therapy is the main and widely recognized method in the treatment of patients with idiopathic scoliosis of 2-4 degrees. One of the modern promising directions in this method is the use of a special orthosis – an asymmetric actively corrective Chêneau brace. The brace applies pressure to the apex of the curved area. The brace design includes spaces (expansion zones) on the convex side of the curvature for rib movement during breathing and displacement of tissues and organs, facilitating derotation and correction of the scoliotic curve. Bracing is indicated and performed with a combination of specific clinical-radiological and organizational criteria:
Scoliotic deformity with a main curve angle of 20° and above, with the deformity apex not higher than Th6-Th8. At this stage of the disease, the possibilities of complete correction of the existing deformity are lost, and physiological parameters of postural body balance are disturbed, increasing the risk of scoliosis progression.
Presence of active vertebral bone plasticity.
Disciplined compliance with the patient’s treatment regimen, wearing the brace for 18-23 hours a day, with mandatory control X-ray examination and examination by the attending orthopedist once every 4 months.
Chêneau brace treatment continues until 18-20 years old, then bone maturity of the spine is assessed based on X-rays and the results of brace cessation tests. In case of relative stability of the spinal deformity, gradual discontinuation of the brace is carried out, with preservation of the night wearing regimen until 20-22 years old.
Taping
Taping refers to the application of elastic adhesive tape or tape to affected areas of the body. According to modern research data, this technique does not significantly affect the condition of patients with scoliosis.
Physical Therapy
Physical therapy is the leading treatment method, indicated for deformities up to 20°-25° by Cobb angle. Patients are taught an individual set of physical therapy exercises in a specialized clinic under the guidance of a physiotherapy specialist or physiotherapist and engage in daily home exercises. At this stage, the involvement of all family members in the treatment process is crucial. Children with initial degrees of scoliosis are not restricted in motor activity. Active outdoor games, swimming, skiing, and walking can stabilize the deformity.
Massage and Physiotherapy
Scoliosis can be treated with physiotherapeutic procedures, manual massage techniques, trigger point massage, and other manual techniques. Effective methods include electrophoresis with vasodilators, paraffin-ozokerite applications, mud therapy, hydro massage, magnet therapy, laser therapy, ultrasound, amplipulse therapy, electrostimulation of paravertebral muscles. These procedures help normalize neurotrophic processes in the vertebrae and surrounding tissues, improve local blood circulation in the spine and spinal cord at the apex of the curvature.
Features of Treatment in Children and Adolescents
In childhood and adolescence, when scoliosis often debuts and actively progresses, treatment can pose challenges. This is due to psychological issues such as the child’s lack of self-awareness, absence of pain sensations, low motivation for treatment, and preserving a certain lifestyle. The responsibility for creating a therapeutic and supportive environment lies with the parents, the child’s immediate environment, medical professionals, and school staff. The correct motivation of the child and their active participation in the treatment process are crucial for successful scoliosis treatment.
Regardless of the etiology of scoliosis, initial stages of the disease are treated conservatively, and active treatment continues until skeletal growth is complete. In males, skeletal growth typically ends around 22-24 years of age, while in females, it ends 1-2 years earlier.