What Is Spinal Dysraphism?

The coronal, sagittal or axial position of the spine deviates from the normal position and a morphological abnormality occurs, which is called spinal deformity.

Basic Information

English name

spinal deformity

Visiting department

orthopedics

Common causes

Idiopathic, congenital, neuromuscular, interstitial, traumatic, etc.

Common symptoms

"Razorback" deformity; kyphosis

Causes of spinal deformity

According to the causes of spinal deformity, it can be divided into idiopathic, congenital, neuromuscular, interstitial, and traumatic. For scoliosis, idiopathic is a common cause; for kyphosis, neurofibromatosis type I and Scheumann disease are common coronary malformations, and the location of the malformations is divided into the upper thoracic and middle thoracic segments. Thoracolumbar / waist.

Idiopathic

That is, idiopathic scoliosis is not very clear in terms of etiology, but it has a certain relationship with genes and inheritance. In addition, there are also reasons for the imbalance of the distribution of the paraspinal muscle itself. Morphology refers to the fact that the vertebral body has no structural abnormality, the vertebral body is normally separated, has a symmetrical pedicle, and the normally developed lamina and articular processes.

2. congenital

(1) Congenital scoliosis For congenital scoliosis or kyphosis, it usually refers to the spinal deformity caused by the abnormal structure of the vertebral body itself. The type of pathological structure is usually divided into incomplete vertebral bone formation or incomplete separation. Vertebral body insufficiency can occur in the coronal or sagittal position, causing scoliosis or kyphosis.

(2) Congenital spinal deformity is due to the abnormal development of the spinal cord and medulla during embryonic development (6 weeks before embryonic development). It is usually accompanied by developmental deformities of other organs, which can be abbreviated with VACTERL, namely V-vertebral developmental abnormality, A-anal atresia, C-cardiovascular malformation, TE-tracheoesophageal fistula, R-kidney dysplasia, L- Limb dysplasia. It may also be accompanied by lesions such as scapular scapula (SprengelDeformity) and Klippel-Feil syndrome.

(3) Congenital kyphosis deformity is caused by congenital fusion (separation disorder) of the vertebral body, and the pathogenesis of congenital scoliosis is similar, but the morphology only affects the sagittal curvature. Similarly, due to the formation of horn-shaped kyphosis, spinal cord compression may easily occur, or bilateral lower limb paralysis due to spinal cord blood flow disorders.

3. Disease factors

(1) Neuromuscular scoliosis is mainly caused by the muscular system of the whole body, resulting in the weakness of the chest and back muscles and the paraspinal muscles that cannot support the spine.

(2) Neurofibromatosis is also an important cause of scoliosis. Neurofibromatosis itself is caused by a genetic defect that causes neural crest cells to develop abnormally and cause multiple system damage.

(3) Marfan syndrome is also the cause of scoliosis. The incidence of male and female is similar. It is a chromosomal dominant genetic disease (caused by 15q21.1 mutation of chromosome 15), but about 25% of patients are caused by chromosomal mutation.

(4) There are two main types of scoliosis in adults . One is that idiopathic scoliosis develops into adolescence and the corresponding symptoms appear. It is called adult idiopathic scoliosis; the other is in adulthood. Due to disc degeneration during this period, degenerative adult scoliosis is said to be the most common type. It also includes adult manifestations of congenital scoliosis, paralytic scoliosis, and deformity after trauma.

(5) The main cause of Scheumann's disease is the occlusion of the nutritional vessels of the endplate caused by minor trauma, which makes the endplate lose its blood supply.

(6) Scoliosis caused by spinal tuberculosis is one of the main causes of local kyphosis. Because the tuberculosis lesion invades the vertebral body and the intervertebral space, the disc tissue disappears, and the vertebral bodies merge with each other, forming a local " "Angular" kyphosis. The vertebral bodies are fused with each other, but the pedicle and posterior structures (including articular processes, lamina, spinous processes, etc.) still exist, which is very likely to cause spinal cord compression and paraplegia. Tuberculosis bacteria form localized packages and cause disease.

(7) Ankylosing spondylitis is a chronic progressive disease that invades the spine and affects the sacroiliac and surrounding joints. Marie-Strumpell disease or VonBechterew disease is an autosomal dominant hereditary disease, which mainly causes arthralgia of the articular processes and joints. The primary site is the attachment of ligaments and joint capsules. Synovial membranes of the joints are characterized by granulomas. Inflammation causes ossification of the ligaments, which in turn leads to joint stiffness, and the entire spine joints form a "bamboo-like" change, accompanied by obvious fracture looseness.

Clinical manifestations of spinal deformity

From the appearance, lateral curvature can produce back hump deformities, "razor back" deformities, and sometimes even "funnel chest" or "chicken breast" deformities. At the same time, this back deformity can be accompanied by bilateral shoulder imbalance or pelvis. Imbalance and unequal length of both lower limbs; Kyphosis, especially tuberculous kyphosis of the thoracic spine, can cause obvious local malformations, decrease in height, decrease in thoracic and abdominal volume, and even cause neurological, respiratory, and digestive functions At the same time, for patients with spinal bone structure dysplasia, it can be accompanied by meningocele, invisible spina bifida and other neurodevelopmental manifestations. In addition, congenital scoliosis may be accompanied by abnormalities in the cardiovascular system, tracheo-esophageal fistula, polycystic kidney disease and other organs.

Spinal deformities can be divided into cervical, thoracic, and lumbar deformities according to location. According to morphology can be divided into lordosis, scoliosis and kyphosis.

Idiopathic scoliosis

Idiopathic scoliosis is the most common "razorback", and some patients may also find bilateral shoulder and pelvic imbalances. Nervous damage is rarely seen.

2. Congenital Scoliosis

In clinical manifestations, lumbosacral sinus tracts, increased hairs, local bulges, subcutaneous lipomas, etc. It is usually accompanied by developmental deformities of other organs, which can be abbreviated with VACTERL, namely V-vertebral developmental malformation, A-anal atresia, C-cardiovascular malformation, TE-tracheoesophageal fistula, R-kidney dysplasia, L- Limb dysplasia. It may also be accompanied by lesions such as scapular scapula (SprengelDeformity) and Klippel-Feil syndrome.

3. Neuromuscular scoliosis

One of the most common causes is cerebral palsy. Cerebral palsy is mostly due to hypoplasia of the nervous system during the neonatal period, leading to continuous muscle atrophy and developmental imbalance. At the same time, these children are distinguished from other types of scoliosis and are often accompanied by systemic diseases such as joint dislocations, epilepsy, Chilean disorders, and even bedsores. Most of the onset time began in infancy or adolescence, the spine bone itself developed well, and the shape of the vertebral body was not changed. However, because the lower limbs were not able to walk, most children could only do it in a wheelchair with obvious lower limb muscles Atrophy, the hip joint is prone to adduction contraction on one side, and abduction dislocation on the other side. At the same time, this muscle atrophy can further affect intercostal muscles (such as Duchenne syndrome) that support respiratory function, causing early death.

Spine deformity The spine shape usually shows a pronounced "C" curvature, which continuously changes from the cervical spine to the thoracic spine to the lumbar spine without obvious compensatory scoliosis. Large degree of lateral curvature, often affecting the segment, poor spine flexibility, often accompanied by significant pelvic tilt.

4. Neurofibromatosis

In NFI patients, milk-coffee spots and peripheral neurofibromatosis can usually be seen on the skin surface, which are mostly located in the non-exposed part of the trunk. In addition, Lisch nodules can be seen in the eye, which are upper eyelid fibroids or plexiform neurofibroma. The orbit can touch the mass or the convex eye beats, and the slit lamp can see the orange yellow round small nodules of the iris, which is a hamartoma, which is unique to NFI. Performance can increase with age.

5. Marfan syndrome

Males and females have a similar incidence, also known as spider palm malformations. The age of onset of spinal deformity is early. 50% of the patients first appeared at the age of 6. Although the appearance is similar to idiopathic scoliosis, the Cobb angle is large, the progress is fast, the curvature is stiff, it is not easy to correct, and it is easy to appear intervertebral body. The lateral displacement of the bone graft is prone to non-union.

6. Adult scoliosis

Pain is the main manifestation, accompanied by the symptoms of lumbar spinal stenosis. Inquiry of medical history should include evaluation of daily function, and the impact of spinal deformity on work and life. Examination includes examination of spinal deformity, musculoskeletal system, and nervous system, including the length of both lower limbs to avoid rebalancing the body after orthopedics.

7. Scheumann disease

Also known as diabetic disease, adolescents have round backs. According to the site of the disease, it can be divided into thoracic spine and lumbar spine Scheumann disease. According to the definition, the angle between the trailing edge and the leading edge of three consecutive vertebral bodies is greater than 5 °, and the "worm-like" change of the vertebral body adjacent to the endplate occurs at the same time. Loss of blood supply.

8. Scoliosis due to spinal tuberculosis

Most patients with spinal tuberculosis caused severe deformities, most of them are adults, and most of them are caused by tuberculosis or invisible infection in adolescence, and they are actively treated.

9. Congenital Scoliosis

The pathogenesis of congenital kyphosis is similar to that of congenital scoliosis, but its morphology only affects the sagittal curvature. Similarly, due to the formation of horn-shaped kyphosis, spinal cord compression may easily occur, or bilateral lower limb paralysis due to spinal cord blood flow disorders.

10. Ankylosing spondylitis

Most of the clinical manifestations gradually developed from sacroiliac joints to spinal articular processes and costal joints. It is easy to cause kyphosis of the spine and cause local pain in patients. The main reason for the patient's visit was due to the stiff kyphosis of the spine, and he couldn't look up. At the same time, as the thoracic kyphosis increases, the patient's respiratory dysfunction and digestive function also cause a certain decrease. In addition, the disease can affect the cardiovascular system, eyes, ears and other organs.

Spine deformity diagnosis

The diagnostic criteria for scoliosis or kyphosis in imaging, including the measurement of the Cobb angle, that is, the angle between the most inclined vertebral bodies that form the ends of the scoliosis or kyphosis (head and tail) The most basic description of any spinal deformity.

For the evaluation of the occurrence of vertebral body rotation, Nash-Moe typing is usually used, which is to judge by the degree of symmetry of the bilateral pedicle. Through bilateral symmetry description of the pedicle of the vertebral pedicle, the vertebral body rotation information can be obtained on the ordinary X-ray.

And the evaluation of the patient's growth potential Risser sign. Risser's sign evaluates the growth potential of a patient through the closure of the epiphysis on both patella surfaces.

Spinal deformity treatment

Idiopathic idiopathic scoliosis

Divided into two methods of surgical treatment and conservative treatment:

There is some controversy about the standard of surgery. Most scholars believe that the Cobb angle of the lateral curve is above 45 °, or that the shoulder or pelvis is out of balance. Surgical treatment can be considered. The surgical methods can be divided into lateral anterior surgery and posterior surgery for fixed orthopedic implant Bone fusion.

Conservative treatment can be divided into observation and brace treatment. For observable patients, it is generally considered to have a certain growth ability (that is, the Risser sign is less than 3), and the local Cobb angle is less than 25 °, or the Risser sign is 4 or 5, and the spine has stopped Growth, but the local Cobb angle does not meet the surgical standard; brace treatment refers to patients with a certain growth ability of the spine (Risser sign is less than 3), and the local Cobb angle is between 25 ° and 45 °. For the choice of brace, you can choose the corresponding type according to the position of the scoliosis apex. Generally, the scoliosis apex is above the T7 level, you can choose Milwaukee brace, and below the T7 level, you can choose Boston brace.

2. Congenital Scoliosis

The treatment of congenital scoliosis is divided into observation and surgical treatment. The brace treatment is not effective for this type of patients, because the brace correction force cannot correct the congenital deformities existing in the vertebral body. If the patient does not have obvious appearance abnormalities, such as asymmetry of shoulder joints and bilateral hips, deformity of the back, and he does not feel discomfort, he can consider to continue observation, the time is about 4-6 months; otherwise, he can consider surgical treatment, surgery according to different ages, The position of the malformation and the balance of the whole body can choose different surgical methods. Such as hemivertebral epiphysis fusion, hemivertebral resection, scoliosis and in situ fusion. At present, with the improvement of surgical instruments and concepts, for the treatment of congenital hemivertebra, the main considerations are resection of the hemivertebra, internal fixation and surgery. Congenital scoliosis can cause deformity to progress at any age.

3. Neuromuscular scoliosis

General conditions, orthopedic conditions, and spinal deformities were evaluated simultaneously. For such patients, a supportive seat is necessary. Brace treatment is basically ineffective after the onset of pubertal growth. Surgical fixation usually requires fusion from the upper thoracic spine (T1 or T2) to the pelvis.

4. Neurofibromatosis

For the treatment principle of neurofibromatosis type I, patients should be divided into two groups without developmental malnutrition and developmental malnutrition. The former's spine curvature is similar to the appearance of idiopathic scoliosis, and the processing principles, such as fusion, are similar. However, in terms of actual angle and bone quality, the two are significantly different. The curvature of neurofibromatosis progresses significantly. At the same time, more pseudo joints are formed after surgery, and defects in vertebral body development are visible. For the group with no developmental malnutrition less than 35 °, experimental brace treatment is feasible; simple posterior approach surgery is available from 35 ° to 45 °; combined anterior and posterior approach is available above 60 ° to increase the fusion rate. The developmental dystrophy group usually had vertebral "shell-like" changes, severe rotation of the vertebrae, increased pedicle distance, and "pencil" signs on the convex ribs, which were related to intraspinal tumors and dural widening. Progression in curvature often develops before the age of seven.

The brace treatment failed completely. Due to the large curvature and vertebral dysplasia, surgical treatment usually cannot provide sufficient power to control the progress of deformity, and it is easy to form local pseudo joints and postoperative curvature progress. There is no significant difference in the incidence of pseudoknee joints caused by malnutrition and non-nutrition, but the fusion rate can be improved by 360 ° fusion, especially in patients with kyphosis, to avoid the progress of deformity. Compared with other types of scoliosis, neurofibromatosis is more prone to early correction and fusion, but has little effect on trunk growth.

5. Marfan syndrome

The brace treatment is not effective for horse formula syndrome or horse formula disease.

Surgical treatment is mainly performed by posterior fixation orthopedic fusion, combined with anterior surgical intervertebral bone graft fusion if necessary. Preoperative evaluation is very important, especially Marfan syndrome is easy to be complicated by pathological changes of important organs such as cardiopulmonary aorta, so it is indispensable for the overall evaluation before operation.

6. Adult scoliosis

Non-surgical treatment is mainly functional exercise, using N-saids anti-inflammatory and analgesic drugs to alleviate symptoms, and using braces to give certain support, non-surgical treatment cannot relieve the progress of deformity.

Indications for surgical treatment: progression of malformations; poor spinal balance; malformations severely affect cardiopulmonary compensation; neurological impairment.

7. Scheumann disease

Treatment of Scheumann's disease can be considered with brace treatment, provided that the local Cobb angle forming the kyphosis is less than 50 °. Since most of these diseases occur during the young period, you can consider wearing braces to delay the progress of the deformity. If the local Cobb angle is greater than or equal to 50 °, and the patient suffers from back pain at the same time, it indicates that the amount of local muscle tension is increased due to the progress of local kyphosis, and surgical treatment should be considered to ease its progress. The surgical approach considers posterior fixation and bone graft fusion.

8. Congenital Kyphosis

For this kind of deformity, there is no possibility of self-relief. It can only rely on surgery to remove the fused vertebral body, so that the spinal cord is compressed on the ventral side. At the same time, strong support is obtained.

9. Ankylosing Scoliosis

The main surgical method is posterior surgery, the main purpose is to stabilize the spine and restore the sagittal imbalance, so that the patient can look up with both eyes and resume normal life. The surgical procedure will be performed through the pedicle osteotomy (PSO) or through the articular process osteotomy (SPO).