Congenital vertebral malformation

　　One, incomplete vertebral segmentation According to Winter's observation, it is divided into the following four types

　　1, the lateral unsegmented vertebral body is partially segmented on one side, eventually leading to severe scoliosis.

　　2, the anteriorly unsegmented vertebral body is unsegmented in front, leading to kyphosis deformity of the spine.

　　3, the bilateral undistributed posteriorly causes kyphosis deformity.

　　4, the longitudinal axis of symmetrically bilateral undistributed vertebral bodies does not grow, and does not occur angular or rotational deformities.

　　Two, incomplete vertebral formation can be partial or complete.

　　When a unilateral vertebral body is incomplete, the vertebral body appears wedge-shaped or oblique square, and it is shown as a small incomplete vertebral body on the X-ray film. The hemivertebral body is caused by incomplete formation on one side, and the hemivertebral body and adjacent vertebral bodies may not be distributed, partially segmented or segmented. Partially segmented hemivertebral bodies can lead to asymmetrical vertebral growth, and when two hemivertebral bodies appear on one side, severe scoliosis may occur. When a hemivertebral body appears at the posterior side of the vertebral body, it can lead to posterior kyphosis deformity. A hemivertebral body that is partially segmented and fused with an adjacent vertebral body has relatively mild scoliotic deformity. An unssegmented hemivertebral body fused with two adjacent vertebral bodies generally does not cause progressive scoliosis. According to the Nasca report, 60 cases of hemivertebral patients were divided into 6 types:

　　1, Simple redundant hemivertebra: It can fuse with one or two adjacent vertebrae, and may have pedicles and redundant ribs when it occurs in the thoracic spine.

　　2, Simple trapezoidal hemivertebra.

　　3, Multiple hemivertebrae.

　　4, Multiple hemivertebrae with fusion of one side of the vertebra.

　　5, Flat rare hemivertebra: There are an equal number of hemivertebrae on both sides, which generally does not cause scoliosis of the spine.

　　6, Posterior hemivertebra: It is prone to cause kyphosis.

　　1, Vertebral deformity can involve one or several vertebrae. If one vertebra is involved, it can cause lateral deformity. If multiple vertebrae are involved, it can cause trunk atrophy and form an 'S' shaped vertebral curvature.

　　2, This disease often occurs with other deformities, such as congenital heart disease, Sprengel deformity, cleft palate, etc.

　　Among vertebral deformities, hemivertebrae account for the majority, and most of the congenital scoliosis is caused by hemivertebrae. The development of spinal deformity varies due to different vertebral lesions, and the degree of deformity development is also different. Nasca reported that the progression speed of scoliosis is 1° to 33° per year on average, with an average of 4°. Generally, the following points should be noted:

　　2, Range of vertebral involvement: The deformity develops more slowly in the cervical-thoracic and lumbar segments than in the thoracic segment. Due to the tilt of the head and neck and the lowering of the shoulders, the external deformity is not very serious. In the lumbar segment, the deformation does not cause external deformity due to the non-compensation of the pelvic tilt. Generally, it does not cause external deformity. In congenital scoliosis, the position of the hemivertebra is more posterior, the deformity of the lateral posterior convexity is more severe, and the prognosis is worse.

　　1, Specificity of deformity: Unsegmented on one side, the development of scoliosis is fast, and the deformity develops quickly in the thoracic spine. Additionally, hemivertebra patients are in the peak period of growth and development during the adolescence of 11 to 19 years old, and the deformity also develops quickly, requiring close observation.

　　Congenital vertebral malformation is a congenital disease, and there is currently no effective preventive measure. Early diagnosis and early treatment are the key to the prevention and treatment of this disease. Maintaining good living habits and ensuring a nutritious diet can, to some extent, prevent the disease.

　　Patients with this condition can undergo X-ray examination and MRI examination to confirm the diagnosis:

　　1, X-ray examination:X-ray films can clearly identify the type of vertebral malformation and should include both anteroposterior and lateral views of the entire spine to estimate the possible correction angle during surgery.

　　2, MRI examination:Those who have the condition should undergo MRI examination, which can exclude syringomyelia or tethered cord syndrome.

　　For those with neurological symptoms, it is necessary to perform myelography to further confirm the diagnosis.

　　Patients with congenital vertebral malformation generally have no special dietary requirements. It is recommended that patients maintain a normal diet, with light food being preferable, and to eat less of high-salt, high-sugar, and high-fat foods, as well as spicy and刺激性 food.

　　One, Non-surgical Treatment

　　The Milwaukee brace can prevent the development of long-segment flexible scoliosis, but it has a poor effect on short, rigid scoliosis. X-ray films taken in traction and natural states can measure the flexibility of the spine. The angles of the original curve and the compensatory curve should be recorded during the period of wearing the brace to monitor their development. If the degree of curvature becomes significantly worse during the period of brace fixation, surgical treatment should be considered. It is best not to use brace fixation for those with a curvature exceeding 50°.

　　II. Surgical Treatment

　　The surgical method is determined according to the type and severity of spinal deformity, the progression speed of spinal curvature, the location of the deformity, and the age of the patient:

　　1. In-situ posterior fusion of the spine is suitable for patients with mild to moderate spinal deformity, good appearance, and slow development of deformity, especially suitable for unilateral unsegmented patients to undergo this type of surgery. The operation should be performed before the age of 5, which is beneficial for controlling the development of deformity. Autologous iliac bone can be used as a bone source, and the fusion range includes the upper and lower two normal vertebrae.

　　2. Unilateral vertebral epiphysis fixation surgery fuses the anterior and posterior sides of the vertebral convexity to prevent excessive growth, allowing the concave side of the spine to continue to grow, achieving the goal of correction. However, this type of surgery is not suitable for those with excessive kyphosis.

　　3. Correction and fusion of scoliosis is suitable for patients with severe spinal deformity. Preoperative traction of the spine is required to prevent the sudden elongation of the spine during the operation. The methods of internal fixation and correction that can be selected include Harrington rod and Luque rod.

　　4. Hemivertebra resection is suitable for spinal deformity and maladjustment caused by the sacral connection. Early surgery is advocated to prevent secondary bony changes. After the hemivertebra is resected, it is fixed with a compression rod. If there is a conus medullaris or incomplete nerve closure, a hemilateral fusion surgery between the convex side of the lumbar and sacral 1 vertebra should be performed first.

　　5. Spondylectomy is suitable for patients with age or adolescence, with unilateral unsegmented vertebral bodies, stiff lesion sites, and severe angular kyphosis. If there is rib fusion in the lateral part of the spine, it should be removed. The osteotomy and fusion surgery should be performed through the anterior approach of the spine. This operation is relatively difficult and must be completed by an experienced spinal deformity orthopedic surgeon. The risk of spinal cord injury caused by the operation is relatively high, and the patient and the parents should be informed clearly before the operation.