Orthopedics - Oleksa A.P. 2006

Scoliosis
Congenital, dysplastic, and neurodysplastic scoliosis

According to E.A. Abalmasova (1965), L.K. Zakrevsky (1966), and V.D. Chaklin (1973), congenital Scoliosis accounts for 2-12% of all cases of this spinal deformity (T.V. Spilyotina, T.A. Ermak, S.D. Shevchenko, 2001).

Congenital scoliosis arises from a spinal development anomaly caused by impaired Embryogenesis, particularly During the first 3-6 weeks and less frequently in the subsequent 3-8 weeks of intrauterine fetal development (Ya.L. Tsivyan, 1978; Robert B., Winter M.D., 1983).

Regardless of sex, these anomalies can occur in any part of the spine in the form of one or more hemivertebrae with additional Ribs and their potential synostosis, unclosed vertebral arches, articular process anomalies, intervertebral blocks, etc.

Consequently, various Congenital spinal deformities develop, including scoliosis (Fig. 279).

The apex of the curvature in congenital scoliosis is formed by an anomalous additional triangular hemivertebra or an underdeveloped articular process or half-arch of a vertebra.

Anomalies of the cervical vertebrae result in a congenital bony form of Torticollis, whereas additional hemivertebrae in the thoracic region are accompanied by extra ribs, and those in the lower thoracic and lumbar regions lead to lateral curvature.

Congenital thoracic scolioses have a more complex clinical course than other Congenital Malformations, although congenital Kyphosis caused by a posterolateral hemivertebra is also prone to rapid progression.

Depending on the severity of clinical manifestations, congenital spinal deformity is either detected by parents immediately after the child's birth or somewhat later. Physicians encounter no diagnostic difficulties, as the existing deformity or local spinal curvature is fixed and non-correctable upon examination of the child.

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Fig. 279. S-shaped scoliosis in an 8-year-old girl.

The Nature of the spinal deformity and the presence of additional abnormal vertebrae and ribs are determined radiologically at four months of age with gonad shielding. Sonography allows for earlier detection.

As the child grows, congenital scoliosis will progress if cartilaginous growth plates are present within the wedge-shaped vertebra, or if unilaterally synostosed ribs lag behind in growth, thereby deforming the rib cage and spine. Spinal curvature typically remains localized; however, during the child's growth, adjacent vertebrae and intervertebral discs may occasionally become involved, leading to The formation of sweeping curves of both the primary and compensatory curvatures.

Cases occasionally occur where hemivertebrae are located opposite one another at the same or adjacent levels. If they contain growth plates, the hemivertebrae grow evenly, and scoliosis may go unnoticed or be barely perceptible and non-progressive. When two or more wedge-shaped hemivertebrae are present on one side of the lumbar spine, an S-shaped scoliosis develops due to primary and compensatory spinal curvatures, which progresses with the growth of the vertebrae and hemivertebrae, yet without their rotation (torsion).

Congenital Developmental anomalies of the spine are frequently associated with Sprengel's deformity, Klippel-Feil Syndrome, etc.

The presence of a longitudinal congenital spinal cleft (rachischisis) in the lower lumbar region leads to The Development of significant scoliotic deformity.

G.I. Turner, R.V. Vreden, and V.D. Chaklin previously suggested that lumbosacral vertebra Dysplasia weakens the Base of the spine, which may serve as a basis for the development of scoliosis. These dysplastic scolioses may present with segmental sensory disturbances, acrocyanosis, and Urinary Incontinence in children, indicating a combined anomaly of Spinal Cord and vertebral development. E.A. Abalmasova observed Asymmetry of the knee Reflexes, diminished or absent abdominal or knee reflexes, and even absent Achilles reflexes, accompanied by vasomotor symptoms. Other manifestations include hypertrichosis in the sacral or lower lumbar region, high-arched feet (pes cavus), Hammer Toes, etc.

Based on experimental and clinical physiological studies, V.A. Lyashenko (1958) suggested that a lesion affecting individual motor Neurons in the anterior horns of the spinal cord on one side results in Muscle imbalance and the primary curvature of scoliosis.

However, this assertion remains controversial, much like other theories regarding the Etiology of scoliosis (V.P. Skrygin, 1949 — gliomatosis; S.A. Reinberg, 1968 — pathological process in the neuromuscular apparatus of the spine).

Congenital scolioses are characterized by progressive spinal curvature accompanied by subsequent chest deformation in the form of a rib hump on the convex side. This leads to hemodynamic disorders, Atelectasis, compensatory emphysema, and pneumosclerosis. Furthermore, these functional disorders develop in parallel with the severity of the deformity.

Deformation of the lumbar spine causes internal organ dislocation; consequently, this symptom complex is referred to as scoliotic disease. Timely Treatment is essential to prevent such functional impairments. Children are provided with plaster beds that are replaced as they grow, along with corrective pads, strap-pelotes, collars, three-point-support corrective braces, gymnastic exercises, and muscle massage. Although these measures cannot eliminate vertebral anomalies, they effectively prevent the progression of the deformity in a growing child. Therefore, conservative and general strengthening therapy is highly beneficial.

Since conservative treatment is merely palliative and used in young children to halt the progression of scoliosis, surgical intervention is advisable once the child's body has physically strengthened.

Preoperative patient preparation and reliable Anesthetic Management are of paramount importance. The surgical Procedure involves the resection or removal of the additional wedge-shaped vertebra, followed by correction of the spinal axis using traction or staged plaster jackets. Patients must wear a brace until skeletal maturity is reached, removing it only for physical therapy and massage, and utilizing a plaster bed during Sleep.

In older children and adolescents with established S-shaped scoliosis, Surgical treatment does not differ from the aforementioned approach.