Spinal deformity

Introduction to spinal deformity The coronal, sagittal or axial position of the spine deviates from the normal position, and the morphological abnormality occurs, which is called spinal deformity. The human spine develops faster during the embryonic period and all structures are completed within a few weeks. After the spine is formed, it has its shape and stability. The entire development process is divided into four phases. The first phase, called the spinal cord, is formed on the fifteenth day of the embryo, and the remaining part of the embryo survives, called the nucleus pulposus; the second phase is called the membranous phase, starting from the 21st day to the end of three months; the third phase is the cartilage Period, from 5 to 6 weeks to before birth; the fourth period is the skeletal period, from two months to the completion of a part of birth. When the embryo develops to the 14th to 21st day, the lamellar cell layer separates the amniotic cavity and the yolk sac. From then on, it is divided into ectoderm, mesoderm and endoderm. The ectoderm forms a neural plate, which is submerged into a sulcus, which in turn develops into a neural tube. The spinal cord originates from the mesenchymal cells and is located in the deep layer of the neural tube. A pair of body segments are formed around the notch along the midline. The mesoderm gradually evolves into a "bone joint" along the neural tube, forming a "bone joint", developing along the neural tube and the notochord, and finally forming a vertebral body. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: brain swelling and meningocele, tracheoesophageal fistula, polycystic kidney disease

Cause of spinal deformity

Genetic factors (15%)

Idiopathic scoliosis is not very clear from the etiology, but it has a certain relationship with genes and heredity. In addition, there are also reasons for the imbalance of the paravertebral muscles themselves. Morphology means that the vertebral body itself has no structural abnormalities, the vertebral body is normally separated, has a symmetrical pedicle, and develops normal lamina and articular processes.

Congenital factors (15%)

(1) Congenital scoliosis, usually refers to spinal deformity caused by abnormal structure of the vertebral body itself. The type of pathological structure is usually divided into vertebral bone formation or insufficiency.

(2) Congenital kyphosis deformity is caused by congenital fusion of the vertebral body (separation disorder), and the pathogenesis of congenital scoliosis is similar, but the morphology only affects the sagittal curvature. . Also due to the formation of kyphosis, it is easy to cause compression of the spinal cord, or the symptoms of lower limb paralysis due to blood supply disorders of the spinal cord itself.

Disease factor (50%)

(1) Neuromuscular scoliosis: Neuromuscular scoliosis, mainly due to systemic muscle system lesions, resulting in muscle weakness and damage caused by the muscles of the chest and back, which can not be well supported by the spine.

(2) Neurofibromatosis: Neurofibromatosis is also an important cause of lateral (posterior) curvature of the spine. Neurofibromatosis itself is caused by genetic defects that cause abnormalities in neural crest cell development leading to multiple systemic damage. According to clinical manifestations and gene mapping, it is divided into neurofibromatosis type I (NFI) and type II (NFII).

(3) Marfan syndrome: Marfan syndrome is also the cause of scoliosis. The proportion of men and women is similar. It is a chromosomal dominant disease (caused by 15q21.1 mutation on chromosome 15), but about 25% of patients are Caused by chromosomal variation.

(4) adult scoliosis: There are two main pathological types of adult scoliosis. One is that the idiopathic scoliosis in adolescence progresses to adulthood and the corresponding symptoms appear, called adult idiopathic scoliosis; The second is due to the degeneration of the intervertebral disc in adulthood, called denovo Scoliosis. Scheumann's disease: Scheumann's disease is again a disease of the disease, and the teenager has a round back. According to the location of the disease, it can be divided into thoracic and lumbar Scheumann's disease. According to the definition, the angle between the trailing edge of the three vertebral bodies and the leading edge is greater than 5°, and the insect-like changes of the vertebral body adjacent to the endplate and the changes of Schmorl nodules can be diagnosed. The main reason is that micro-trauma leads to occlusion of the endplate nutrient vessels, which causes the endplate to lose blood supply.

(5) Scoliosis caused by spinal tuberculosis: Spinal tuberculosis is one of the main causes of local kyphosis. Because tuberculosis invades the vertebral body and intervertebral space, the intervertebral disc tissue disappears and the vertebral bodies merge with each other to form a local part. "Angular" kyphosis. The vertebral bodies merge with each other, but the pedicle and posterior structures (including the facet joints, lamina, spinous processes, etc.) still exist, which is very likely to cause compression of the spinal cord and cause paraplegia. Most of the patients with severe malformations caused by spinal tuberculosis are adults. Most of the onset is due to tuberculosis or invisible infection during adolescence. Tuberculosis forms a localized package and causes disease.

(6) Ankylosing spondylitis: is a chronic progressive disease that invades the spine and involves the ankle joint and surrounding joints. (Marie-Strumpell disease, or Von Bechterew disease), is an autosomal dominant genetic disease, which mainly causes vascular hyperplasia of the facet joints. The primary site is the attachment site of the ligament and joint capsule, and the synovial formation of the joint is granuloma. The characteristic synovitis causes ossification of the ligament, which in turn forms the rigidity of the joint, and the entire spine joint forms a "bamboo-like" change with obvious fracture loosening.

Spinal deformity prevention

Stabilizes the spine and restores the sagittal imbalance, allowing the patient's eyes to look flat and return to normal life. Adjust daily life and workload, and regularly carry out activities and exercise to avoid fatigue.

Spinal malformation complications Complications, brain swelling, meningocele, tracheoesophageal fistula, polycystic kidney disease

Decreased height, reduction of chest and abdominal volume, and even damage to nerve function, respiratory function, digestive function, etc.; also for patients with dysplasia of the spine bone structure itself, may be accompanied by meningocele, stealth spina bifida and other neurodevelopment Abnormal performance. In addition, congenital scoliosis may also be associated with abnormal cardiovascular system, tracheal-esophageal fistula, polycystic kidney disease and other multiple organ abnormalities.

Symptoms of spinal deformity Common symptoms Spinal horny deformity Bamboo spine Spinal curvature Bone spine Post spine Physiological curvature disappears Scoliosis

From the shape, the side bend can produce back bulge deformity, resulting in "razor back" deformity, and some even produce "funnel chest" or "chicken chest" deformity, combined with this back deformity, can be accompanied by bilateral shoulder joint imbalance or pelvis Unbalanced, and unequal length of both lower extremities; kyphosis, especially tuberculous kyphosis, can cause significant local deformity, reduced height, reduced chest and abdominal volume, and even neurological, respiratory, and digestive functions. The damage, etc.; at the same time for the spine bone structure itself dysplastic patients, may be accompanied by meningocele, invisible spina bifida and other neurodevelopmental abnormalities. In addition, congenital scoliosis may also be associated with abnormal cardiovascular system, tracheal-esophageal fistula, polycystic kidney disease and other multiple organ abnormalities.

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

Idiopathic scoliosis

Idiopathic scoliosis is the most common "razor back", and some patients may also find bilateral shoulder imbalance and pelvic imbalance. The performance of nerve damage is usually rare.

Congenital scoliosis

Congenital spinal deformity is usually accompanied by developmental malformations of other organs. It can be abbreviated by VACTERL, which is V-vertebral developmental malformation, A-anal atresia, C-cardiovascular malformation, TE-tracheal esophageal fistula, R-kid development. Poor, L-limb dysplasia. It may also be accompanied by lesions such as Sporngel Deformity and Klippel-Feil syndrome.

Neuromuscular scoliosis

The most common cause is cerebral palsy. Most of the cerebral palsy is caused by hypoxia caused by hypoxia in the neonatal period, resulting in persistent muscle atrophy and developmental imbalance. At the same time, such children are distinguished from other types of scoliosis, often with systemic diseases such as joint dislocation, epilepsy, Chilean disorders, and even acne. Most of the onset time begins in infancy or adolescence. The spine bones are well developed and the shape of the vertebral body is not mutated. However, since the lower limbs cannot walk, most of the children can only be in a wheelchair with obvious lower limb muscles. Atrophy, the hip joint is prone to one side of the adduction contracture, and the other side of the abduction dislocation. At the same time, this muscle atrophy can further affect the intercostal muscles that support respiratory function (such as Duchenne syndrome), causing early death.

Neurofibromatosis

In the case of NFI patients, milk-coffee spots (Café au lait) and peripheral nerve multiple neurofibroma can be seen on the skin surface, mostly located in the non-exposed areas of the trunk. In addition, Lisch nodules can be seen in the eye. They are fibroids of the upper jaw or plexiform neurofibroma. The eyelids can touch the mass or the pulsation of the convex eye. The slit lamp can be seen in the iris miliary orange round small nodules, which are hamartomas and are unique to NFI. Performance can increase with age. The diagnostic criteria are more than 6 prepubertal, diameter greater than 5mm (diameter greater than 15mm after puberty) with high diagnostic value; systemic and axillary freckles are also one of the characteristics; two or more neurofibromas or plexiform neurofibromas were found; And relatives have NFI patients; 2 or more Lisch nodules; bone damage. The location of the gene lesion is mostly located at 17q11.2.

Most patients with NFII are central neurofibromatosis or bilateral acoustic neuroma, and the first-degree relatives of the diagnostic criteria have NFII with one side acoustic neuroma, or with neurofibromatosis, meningioma, glioma, and Schwann cell tumor. Two. The location of the gene lesion is at 22q.

Adult scoliosis

Clinical manifestations of pain are the main manifestations, accompanied by symptoms of lumbar spinal stenosis. Inquiries about medical history should include an assessment of daily function, the impact of spinal deformity on work and life. The examination included examination of the spinal deformity, musculoskeletal system and nervous system, including the length of the lower limbs, to avoid re-integration of the body after orthopedics.

Imaging examinations include the full lateral position of the spine and the dynamic position (left and right Bending images) to clarify the mobility of the disc. The imaging differences between adult idiopathic scoliosis and adult degenerative scoliosis are: 1. The former usually has Two curvatures, the latter usually only one; 2, the former is well compensated for the trunk and pelvis, the latter is often decompensated; 3, the former has no obvious degeneration of the intervertebral space, the intervertebral space is equal and there is no obvious sagittal or coronal Sliding; the latter intervertebral space degeneration is severe, bilateral unequal height, visible obvious slip, and changes in endplate hyperplasia; CT clear bone changes, especially in patients with severe osteoporosis; MRI To clarify the situation of disc degeneration and nerve compression, some scholars even use discography to determine the level of distal fusion and disc.

Ankylosing spondylitis

Most of the clinical manifestations are from the ankle joint to the spinal joint and rib joint. It is easy to cause kyphosis of the spine, causing local pain in the patient. The reason for the patient's visit is mainly due to the stiff kyphosis of the spine, but can not look up at the front. At the same time, due to the increase of thoracic kyphosis, the patient's respiratory dysfunction, digestive function also caused a certain decline. In addition, the disease can affect the cardiovascular system, the eyes, the ears and other organs.

Examination of spinal deformity

Imaging criteria for the diagnosis of scoliosis or kyphosis, including the measurement of the Cobb angle, that is, the angle between the most oblique vertebral bodies that form the scoliosis or the sulcus (the foremost and the most end) is The most basic description of any spinal deformity.

The evaluation of the occurrence of vertebral rotation is usually judged by the Nash-Moe classification, that is, by the symmetry of the bilateral pedicles. The symmetry description of the bilateral sides of the vertebral pedicle is used to obtain information on the rotation of the vertebral body on the normal X-ray.

As well as the Risser sign for the evaluation of the patient's growth potential. The Risser's sign evaluates the patient's growth potential by closing the epiphysis of the bilateral humerus.

Diagnosis and diagnosis of spinal deformity

Imaging criteria for the diagnosis of scoliosis or kyphosis, including the measurement of the Cobb angle, that is, the angle between the most oblique vertebral bodies that form the scoliosis or the sulcus (the foremost and the most end) is The most basic description of any spinal deformity.

The evaluation of the occurrence of vertebral rotation is usually judged by the Nash-Moe classification, that is, by the symmetry of the bilateral pedicles. The symmetry description of the bilateral sides of the vertebral pedicle is used to obtain a letter of vertebral rotation on a common X-ray.

As well as the Risser sign for the evaluation of the patient's growth potential. The Risser's sign evaluates the patient's growth potential by closing the epiphysis of the bilateral humerus.

Marfan syndrome

Diagnostic criteria

1. The specific content of the standard

(1) skeletal system

Main criteria: The following performances are at least 4 - chicken breast; funnel chest needs surgical correction; the ratio of upper volume / lower volume is reduced, or the ratio of upper limb span length / height is greater than 1.05; wrist sign, indication is positive; scoliosis is greater than 20 degrees, or spinal advancement (curvature); elbow abduction decreased (<170 degrees); middle metatarsal joint dislocation formed a flat foot; any degree, acetabular lordosis (ankle joint invagination) (X On-chip ok).

Secondary criteria: moderate funnel chest: abnormally enhanced joint activity; high zygomatic arch, overcrowded teeth; facial characterization: long head - normal head index of 75.9 or less, patella hypoplasia, eyeball invagination, constriction, The cleft palate is oblique.

Conditions to be met for skeletal system involvement: There are at least two primary criteria or one primary criterion plus two secondary criteria.

(2) Eye system

Main criteria: lens dislocation.

Secondary criteria: abnormal flat cornea (measured by the corneal surface meter); increased axial length of the eye (ultrasound measurement); dilated pupil of the iris or ciliary muscle. Eye system involvement is subject to standards: primary criteria or at least two secondary criteria.

(3) Cardiovascular system

Main criteria: ascending aortic dilatation with or without aortic regurgitation, and at least Valsava's sinus dilatation; ascending aortic dissection.

Secondary criteria: mitral valve prolapse with or without mitral regurgitation; primary pulmonary artery dilatation (under valvular or peripheral pulmonary stenosis and other obvious causes, age less than 40 years); mitral annulus calcification (age Less than 40 years old; descending aorta or abdominal aorta dilatation or dissection (under 50 years old).

Conditions to be met for cardiovascular involvement: There is one primary criterion or one secondary criterion.

(4) Lung system

Main criteria: None.

Secondary criteria: spontaneous pneumothorax; pulmonary apical bullae (confirmed by chest radiograph).

If one is present, the lung system is considered to be involved.

(5) Skin and body capsule

Main criteria: None.

Secondary criteria: dermatogly atrophy (traction marks), unrelated to significant overweight, pregnancy or repeated compression; recurrent hernia or incisional hernia.

A skin or body capsule is considered to be involved in the presence of a secondary standard.

(6) Hard brain (ridge) membrane

Main criteria: dural bulging found in CT or MRI.

Secondary criteria: None.

(7) Family or genetic history

Main criteria: one of the parents, children, or siblings meets the diagnostic criteria; there are known mutations in the FBNI gene that cause Marfan syndrome; there are known FBNI gene haplotypes that are identical to those in their family. .

Secondary criteria: None.

Since family or genetic history is significant in diagnosis, there must be one in the main criteria.

2. Diagnostic premise: Marfan syndrome and Marfan body shape are easily confused at the time of diagnosis

Diagnosis of Marfan syndrome:

For a specific case: if there is no family or hereditary history, at least two major systems of major systems and another organ are involved; if a mutation in a known Marfan syndrome is detected, there is a major standard and The diagnosis of a two-system system can be diagnosed; patients with a family history can have a primary standard of one system and a second system can be diagnosed.

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