congenital vertebral deformity
Introduction
Introduction to congenital vertebral deformity Congenital vertebral deformity is caused by incomplete or incomplete segmentation during vertebral body development. It may form part or all of incompleteness. When part of the unilateral vertebral body is incomplete, the vertebral body appears wedge-shaped or rhomboidal. The X-ray film shows the cutting work as a small hypoplasia experience life, and the semi-vertebral body is completely incompletely formed by one side. As a result, the semi-vertebral body and the adjacent vertebral body may not be distributed, half-segmented or segmented, and the segmented half-vertebral body causes asymmetry of the vertebral body growth. basic knowledge The proportion of illness: 0.002% Susceptible people: children Mode of infection: non-infectious Complications: congenital heart disease
Cause
Causes of congenital vertebral deformity
Congenital vertebral malformations are thought to be related to autosomal dominant and recessive inheritance, and Winter reported a family history of 1%.
According to different vertebral lesions, it can be divided into vertebral body segmentation and vertebral insufficiency.
Vertebral segmentation
According to Winter's observation, it is divided into the following four types:
(1) Lateral unsegmented vertebral body segmentation occurs on one side, eventually leading to severe scoliosis.
(2) The front unsegmented vertebral body is not segmented in front, resulting in kyphosis deformity.
(3) The rear side is not distributed and causes kyphosis.
(4) Symmetry The longitudinal axis of the undistributed vertebral body does not grow, and no angulation or rotational deformity occurs.
2. Vertebral dysplasia
It may form part or all of incompleteness. When part of the unilateral vertebral body is incomplete, the vertebral body appears wedge-shaped or rhomboidal. The X-ray film shows the cutting work as a small hypoplasia experience life, and the semi-vertebral body is completely incompletely formed by one side. As a result, the semi-vertebral body and the adjacent vertebral body may not be distributed, half-segmented or segmented, and the segmented half-vertebral body causes asymmetry of vertebral body growth, especially when two semi-vertebral bodies appear on one side, severe scoliosis may occur. When a semi-vertebral body appears behind the vertebral body, it causes kyphosis to form an angular deformity. The semi-segmental hemivertebra is fused with an adjacent vertebral body. The scoliosis is relatively light, and the non-sectioned vertebral body and the adjacent two vertebral bodies Fusion, generally does not cause progressive scoliosis, according to the Nasca report together 60 patients with hemivertebra, divided into 6 types: 1 simple excess vertebral body: can be merged with one or two adjacent vertebral bodies, occurs in Thoracic vertebrae may have pedicle and excess ribs, 2 simple wedge-shaped semi-vertebral, more than 3 semi-vertebral bodies, more than 4 semi-vertebral bodies with one side vertebral body fusion, 5 flat dilute vertebral bodies: both sides have number Equal semi-vertebral body, generally does not cause scoliosis, after 6 Hemivertebrae: can lead to kyphosis.
Prevention
Congenital vertebral deformity prevention
The disease is a congenital disease, no effective preventive measures, early diagnosis and early treatment is the key to the prevention and treatment of this disease.
Complication
Congenital vertebral malformation complications Complications congenital heart disease
Vertebral malformations can involve one or several. Involving a vertebral body can cause a lateral deformity. If multiple vertebral bodies are involved, the trunk can be atrophied and the "S" shaped spine is bent.
The disease is often combined with other malformations, such as congenital heart disease, Sprengel malformation, cleft palate and so on.
Symptom
Congenital vertebral deformity symptoms Common symptoms Spinal horn deformity Scoliosis thoracic deformity Thoracic and lumbar segment and lumbar lordosis disappearing vertebral division
In the vertebral deformity, the majority of the vertebral body is the majority, and most of the congenital scoliosis is caused by the hemivertebra. The development of the spinal deformity is different due to the different vertebral lesions. The degree of malformation is also different. Nasca reports the side of the spine. The rate of convex progression is 1° to 33° per year, with an average of 4°. In general, the following points should be noted:
1. The particularity of malformation: unilateral unsegmented, scoliosis develops rapidly, and the lesions develop rapidly in the thoracic spine. In addition, the hemivertebra is in the growth and development peak between 11 and 19 years old. Malformation is also developing rapidly and needs to be closely observed.
2, the extent of spinal involvement: the development of deformity of the neck and thoracic segment and the lumbar segment is slower than that of the thoracic segment. Due to the inclination of the head and neck and the reduction of the shoulder, the appearance of the deformity is not very serious. Reimbursement, generally does not cause appearance deformity, congenital scoliosis, the position of the semi-vertebral body is more posterior, the more severe the lateral kyphosis, the heavier the prognosis.
Examine
Examination of congenital vertebral deformity
Patients with this disease can undergo X-ray examination and MRI examination to confirm the diagnosis:
(1) X-ray examination: X-ray films can identify the type of vertebral deformity, and should include the full-shoulder lateral radiograph to preliminarily estimate the angle that may be corrected during surgery.
(2) MRI examination: those who have conditions need to do MRI examination, can rule out spinal cord longitudinal or tethered syndrome.
For those with neurological symptoms, myelography must also be performed for further diagnosis.
Diagnosis
Diagnosis and diagnosis of congenital vertebral deformity
diagnosis
Record the patient's height in detail, including standing height and sitting height, rotation of the chest and back, degree of scoliosis, and results of double lower extremity social examination, whether combined with other malformations such as congenital heart disease, Sprengel malformation, cleft palate, etc. X-ray film can be clearly defined The type of vertebral malformation should include the full lateral radiograph of the spine in order to preliminarily estimate the angle that may be corrected during surgery. If there are neurological symptoms, the myelography must be performed. If necessary, MRI should be performed, except for the longitudinal fissure or tether system. Sign.
Differential diagnosis
It is mainly distinguished from the vertebral malformation caused by the acquired day. The main points of identification are as follows:
(1) History of trauma: There is a clear history of trauma, and the direction of external force is mostly from the top to the front of the flexion type external force, the swelling of the soft tissue around the vertebral body can be used as an indication of the role of indirect external force.
(2) Clinical manifestations: local pain at the wound, tenderness, limited lumbar activity, when the vertebral fracture is severely compressed, it may be accompanied by malformation and spinal cord injury.
(3) Imaging changes: In addition to the wedge-shaped changes in the affected vertebral body, the anterior horn of the vertebral body is also seen. The marginal cortex is wrinkled, interrupted, and has a step-like bulge. At the same time, the vertebral body is high due to trabecular bone compression. Density image.
