Congenital scoliosis: causes, symptoms, diagnosis, treatment

Scoliosis - lateral curvature of the spinal column, combined with its torsion.

ICD-10 code

• M41. Scoliosis.
• Q76.3 Congenital scoliosis caused by malformation of the bone.

Before a surgeon, there are usually three problems: identification of congenital anomalies, prospects of progression of deformation and treatment of scoliosis.

What causes congenital scoliosis?

The most common scoliosis on the basis of anomalies in the formation of vertebrae. Among such anomalies are wedge-shaped vertebrae and semi-vertebra.

Progression of the spinal deformity depends on such factors as type of anomaly, localization and number of abnormal vertebrae, presence (or absence) of fusion with adjacent vertebrae.

If the body of the sphenoid vertebra (semi-vertebra) is separated from the adjacent normally developed intervertebral disc, both vertebrae have growth plates and, consequently, grow at the same rate. The principal difference between them is that the wedge vertebra is initially deformed, moreover, due to the Hueler-Folkmann law, the degree of deformation gradually increases. The presence of growth plates leads to the progression of the deformation of the spine as a whole and becomes, thus, the most important prognostic factor. Such a vertebra was identified by IA. Movshovich as active. If the abnormal vertebra merges with one or both adjacent vertebrae, the progression of the deformation becomes benign. Such a wedge-shaped vertebra (half a vertebra) according to AI Movshovich is defined as inactive.

The second important factor in the progression of deformity is the number of abnormal vertebrae. If the wedge-shaped vertebrae (semi-vertebrae) are two or more and all are located on one side, this is a prognostically unfavorable sign. If the abnormal vertebrae are located on opposite sides of the spine and are separated by at least one normal vertebra, the prognosis of the progression of scoliosis can be quite favorable. Such vertebrae are called alternating.

Congenital scoliosis of the second group - deformities on the soil of the spinal segmentation anomaly. These disorders are met at any level, but most often in the thoracic spine. The block can be formed on any extent - both in the frontal and horizontal planes. The rate of progression of scoliosis on the basis of segmentation anomalies depends on the number of segments involved in the block zone and the safety of growth plates on the convex side of the deformation.

Congenital scoliosis in the most severe variant - type III deformations according to the Winter classification (mixed anomalies). This is a scoliosis, at the basis of development and progression of which lies one-sided blocking of the vertebrae in the presence of one or more wedge-shaped vertebrae on the opposite side (at the block level). The combination of two types of scoliosis anomalies mutually reinforces the effect of each of them, which already leads to catastrophic consequences at an early age.

Separate, although not numerous, group is congenital scoliosis on the basis of multiple developmental anomalies, which affect almost the entire vertebral column. At such patients sometimes there is no normally formed vertebra.

The attendant anomalies are very frequent. These include head and neck anomalies (cleft palate and upper lip, ear deformation, lower jaw deformation, absence of epiglottis, deficiency of VII and VIII pairs of cranial nerves), trunk (congenital heart defects, sternum deformity, absence of lung, tracheoesophageal fistula, esophageal stricture ), urinary system, extremities.

How is congenital scoliosis treated?

Conservative treatment of scoliosis

Congenital scoliosis is not treated with conservative methods.

[1], [2], [3]

Surgical treatment of scoliosis

Indications for surgical intervention in congenital scoliosis should be considered in terms of the severity of the already existing deformation and the prospects for its further progression.

It should be operated at the age at which indications for intervention are not in doubt, even if it is a very early age (2-5 years). Moreover, many surgeons are convinced that scoliosis treatment should begin at 3 years of age.

Orthopedic literature contains references to the most varied and numerous interventions that can change the natural course of congenital scoliotic deformation. The treatment of scoliosis depends, among other things, on the experience of the surgeon and equipping the clinic. There is no universal method, but in recent decades, most orthopedists tend to need anteroposterior stabilization of the spinal column (360 fusion).

Rear spidylodesis without tools

The posterior elephantoscope without instrumentation is the best method for deformations that progress clearly or are of such a nature that progression is inevitable, but at the same time so rigid that correction seems unrealistic. A classic example is a one-sided, non-segmented block.

The basic principles of surgical intervention are as follows.

• In the area of spinal fusion, the entire arc of curvature plus one segment should be included cranially and caudally,
• The posterior parts of the vertebrae must be exposed as widely as possible, that is, to the vertices of the transverse processes.
• The formation of the bone bed should be thorough and include resection of the facet joints and complete decortication of the posterior structures of the vertebrae.
• It is necessary to use a large number of transplants.

The formation of the block requires postoperative external immobilization. Using for this purpose corrective corsets such as Milwaukee or corsets with halothoursia (for cervical and thoracic deformities) allows some correction of scoliosis. In addition, the use of such devices contributes to the normalization of the balance of the trunk and the formation of the bone block under conditions close to normal in terms of biomechanics of the spinal column.

Lonstein et al. Emphasize that the results of posterior spondylodesis of white instrumentation are excellent, as long as the surgeon realizes that significant correction can not be considered the main goal. The main goal is stabilization, that is prevention of progression.

Many surgeons say that a small child can not be spondylosed, as this limits his growth. It is true that the formed vertebral body does not grow in length with the growth of the patient or grows slower than normal, but it must be remembered that with congenital scoliosis the blocked zone does not have growth potentials. This nature shortens the spine, not the surgeon, the child will have a longer torso after an early spondylodease, if this operation is not postponed.

Rear spondylodesis using tools

Supplementation of posterior spondylodesis with metal implants aims to achieve greater stabilization of the spine, which reduces the dependence on the quality of external immobilization, and also obtain a more significant deformation correction. The use of Harrington distracters for this purpose is associated with an increased risk of neurological complications. It is much more attractive to use CDI or its analogues. But any intervention with the use of metal implants requires a careful preoperative study of the contents of the spinal canal, as well as intraoperative monitoring of the spinal cord.

Anteroposterior fusion

Such intervention is optimal from the standpoint of stopping the progression of congenital scoliosis. The formation of the circular (360) bone block is accompanied by the destruction of growth plates on the convex side of the arch and balances both sides of the spine in terms of the potency of growth and, correspondingly, the increase in deformation. The implementation of ventral spinal fusion in patients with congenital scoliosis has peculiarities.

• The first feature is the need to identify abnormally developed and located intervertebral disks.
• The second feature is the abnormal location and branching of segmental vessels.

Ventral spondylodesis is expedient to perform immediately before dorsal, that is during one anesthesia.

[4], [5], [6]

Anteroposterior epiphysis

The principal difference from the previous intervention is that. That epiphiseospondilodez not only blocks the spine at a certain extent, but, stopping the growth of bone tissue on the convex side of the deformation, keeps it on the concave side.

Epiphiseospondilodez is shown to young children aged 1 to 5 years, if the deformation progression is documented, the arc length is small, the growth potentials on the concave side are preserved, and the actual deformation is purely scoliotic - without pronounced kyphosis or lordosis. The operation can be effective in patients older than 5 years.

Dubousset et at. Proposed a scheme for planning the operation of spinal focal disease, depending on the location of the anomaly and its nature. You can imagine each vertebra as a cube consisting of four parts (quadrants), each of which grows symmetrically around the spinal canal. If the growth processes go asymmetrically, which is the case in the case of congenital deformity of the spine, it is necessary to determine in advance which zones need blocking in order to restore the lost symmetry. Using a four-quadrant scheme allows you to decide exactly where (in the horizontal plane) the bone block should form.

The second component of the Dubousset scheme is the determination of the length of the spinal fusion along the length of the spinal column. If the epiphiseospondilodez is performed only at the level of the abnormal vertebra, this will only result in a stabilizing effect. If it is necessary to achieve correction of deformation during the continuing growth of the spine, it is necessary to include the above and below segments in the epiphysiospondylodease zone.

[7], [8], [9], [10], [11], [12]

Excision of the semi-vertebra

The first operation of this kind was described in 1928 by Royle, later many surgeons used it. In fact, excision - vertebrotomy on the convex side of curvature, if the operation did not lead to the formation of a block at the level of vertebrotomy, it can be considered unsuccessful. Excision of the semi-vertebra is associated with a real risk of neurological complications, since the lumen of the spinal canal must be opened in front and behind. Indication for the operation of the deformity of the spine on the soil of a single semi-vertebra. Experience shows that its implementation without the use of metal structures that provide compression on the convex side of the arc and thus the closure of the post-resection wedge-shaped defect often results in non-growth of bone surfaces and progression of deformity. The optimal age of the operation is up to 3 years, although at an older age it may turn out to be quite effective. In the case of lumbar scoliosis, the epiphiseospondylodesis is performed in front and behind on the convex side of the deformity with the capture of the level of the semi-vertebra and the two adjacent ones, cranial and caudal, in the thoracic and thoracolumbar spine, due to the risk of the above complications, two vertebral segments above and below the semi- . 

The first stage of the intervention is the removal of the semi-vertebral body. Access depends on the location of the anomaly. The body is removed completely to the base of the root of the arch. Together with the vertebral body, adjacent intervertebral discs and growth plates of adjacent vertebral bodies are removed. E.V. Ulrich recommends the use of a localized arch root to facilitate the identification of the posterior parts of the anomalous vertebra during the second stage of the intervention. To this end, a 6-8 cm long Kirschner's needle is inserted into the center of the base of the root of the arch and carried in the dorsal direction through soft tissues and the skin of the back. This gives the surgeon a clear and reliable reference point, which makes it possible to shorten the time to find the necessary half-hearth and not to expand without the need for access. In place of the resected semi-invertebrate, autografts are laid, the wound is sutured layer by layer.

The second stage is correction of deformity and posterior epiphysis. Access is median. Subperiosteal secrete posterior parts of the vertebrae on the convex side of the arch over three segments. The posterior structures of the abnormal vertebra are removed, after which a defect is formed, which is inverted by the apex towards the concavity of deformation. Two CDI hooks lead to the half-bow of the vertebrae located at the borders of this defect. The length of the rod should be less than the distance between the hooks before compression. The rod is inserted into the hooks, the nut is tightened on one of the hooks, with the help of the contractor, the hooks are brought together by the compressive force, and as a result the wedge-shaped postresection defect is eliminated and the deformation of the spine is corrected. Tighten the nut on the second hook. The operation is completed by stacking the autografts on the convex side of the deformation next to the instrumentation.

[13], [14], [15], [16],

Stage distraction without spondylodesis

This type of surgical treatment is designed for malignantly progressive forms of infantile and juvenile idiopathic scoliosis. Its use in congenital deformations is limited to rather rare forms, characterized by a multitude of anomalies throughout the thoracic and lumbar spine and a combination with the patient's young age and sufficient mobility of deformation.

Single-stage resection of the semi-vertebra and correction of deformation by segmental instrumentation (Shono operations)

Indication - scoliosis of adolescents on the basis of single semi-vertebrae of thoracic and thoracolumbar localization, which do not require prolongation of spondylodesis to the lower lumbar spine.

The patient's position is on his stomach. Bind the posterior parts of the vertebrae to the vertices of the transverse processes, identify the semi-vertebra. Its spinous process, arch and articular facets resect with preservation of the root of the arch and transverse process. These two structures are resected only under the condition of direct visualization of the spinal cord (in the thoracic region the corresponding half-vertebrae of the pebpo are subjected to excision for 3 cm). Resection of the body of the hemisphere begins at the base of the root of the arch and continues centrally to the anterior and ventral platelets. It is usually not necessary to remove them, since after application of the compressive force on the convex side of the deformation, they break and crumble like an empty egg shell. It is necessary to remove the tissue of the intervertebral discs and the end plates on both sides of the semi-vertebra. Resection of the root of the arch and semi-vertebral body is facilitated by the fact that the well-visible spinal cord is biased towards the concavity of deformation.

The next step is the implantation of screws and hooks in accordance with pre-operational planning. It is necessary to provide compression along the convex side of the deformation and distraction - along the concave side. Before correction, it is necessary to lay the autografts in the form of shavings between adjacent vertebrae in the defect formed after resection, otherwise there may be voids. The first implant rod on the convex side of the arc, pre-bending it in accordance with the normal sagittal contour of the spine. On this rod hooks or screws develop a compressive force to crush the end plates and close the triangular postresection defect. At the same time, scoliosis and local kyphosis are corrected. The second rod is implanted on the concave side of the arc. Distraction, however, should be dosed in order to avoid unnecessary tension of the spinal cord. The main role of the second rod is additional stabilization. Throughout the arc, curvatures are followed by posterior spinal fusion with autostimulation. Bed rest should be observed for 1-2 days. Immobilization by the corset is shown for 3 months,

Operations for violations of segmentation

Scoliosis in young children is treated with epiphiseospondylodease. The side and extent of the spinal fusion is determined in accordance with the Dubousset scheme. In older children and adolescents, surgical tactics, among other things, are dictated by the presence or absence of compensatory antiscavity. Optimal implementation of anteroposterior fusion using CDI, which allows to largely eliminate compensatory anticonviction and thereby normalize the balance of the trunk. In the most severe cases, and even in adult patients, it is possible to perform a wedge-shaped osteotomy of the block. During the operation, the spine is purposefully destabilized in order to obtain the necessary correction. The risk of complications increases in proportion to the resulting correction. The lost stability must be immediately restored to the operating table.