Neuromuscular scoliosis
ANATOMY OF THE SPINE
The human spine is formed by individual vertebrae and connective tissue discs in between. The vertebrae form the spinal canal. There are seven cervical, twelve thoracic, and five lumbar vertebrae. The intervertebral discs are the link between the individual vertebral bodies.
Normal anatomy of the spine. Longitudinal section and cross sections through the cervical, thoracic and lumbar spine.
The little joints that link the vertebrae together are known as facet joints. They help to stabilize the spine and, together with the intervertebral discs, allow a certain degree of mobility of the spinal cord. The spinal canal should be wide enough to allow nerve roots to float freely in cerebrospinal fluid.
The front border of the spinal canal is built by the vertebral bodies and intervertebral discs, the side by the intervertebral joints (facets) and back by the ligamentum flavum (yellow band) and vertebral arches. Discs consist of an outer fibrous ring (annulus fibrosus), which surrounds an inner gel-like center (nucleus pulposus).
The spinal cord and nerve roots lie within the spinal canal. The spinal cord extends downwards approx. to the 1st lumbar vertebra. Below, only nerve roots are present in the spinal canal. At the level of the intervertebral disc the nerve roots pass through the neural root foramina to exit the spinal canal. The spinal cord and nerve roots conduct electric-like signals from the skin and joints to the brain, and process of movement is initiated from the brain to the muscles.
DESCRIPTION
Diseases of the brain, spinal cord, and muscular system including cerebral palsy, muscular dystrophy, spinal muscular atrophy, spina bifida and others, may cause a spinal deformity known as neuromuscular scoliosis. Neuromusccular scoliosis progresses more rapidly even after maturity than the idiopatic one. It is commonly associated with pelvic obliquity and has a high rate of perioperative pulmonary complications.
CAUSE OF SYMPTOMS
Gradually progressing compression of nerve roots
Gradually progressing deformation of the spine
Gradually progressing inflammation and degeneration in the intervertebral joints
SYMPTOMS AND SIGNS
Total/partial paralysis and numbness from the site of the spinal cord damage downwoads
Visible spine deformity
Chest deformity (protrusion on one side and concavity on the other)
Waist asymmetry and pelvic tilt
Shoulder height differences
Inclined head position
Pain and stiffness along the spine
THE DIAGNOSIS IS BASED ON
Medical history Clinical exam and at least one of the following tests:
Magnetic resonance imaging (MRI) Computer tomography additionally you may have to do:
Spirometry
Radiographs Genetic tests Study of creatine kinase (CK/KK) levels
Muscle biopsy
Electromyography TREATMENT
Treatment may consist of surgical and non-surgical methods. Non-surgical treatment rarely can significantly improve the condition of the patient, but aims to prevent secondary vertebral complications and to delay the need for surgical stabilization. It includes:
Non-surgical treatment may include
Immobilization with brace
Physiotherapy Electrotherapy Ultrasound therapy Ergotherapy WHEN SHOULD AN OPERATION BE PERFORMED?
The scoliosis angle increases
loss of ability to sit
WHAT IS THE GOAL OF SURGERY?
Prevent the progression of the scoliosis curve
Improve balance while sitting
Reduce pain
HOW IS SURGERY PERFORMED?
Non-fusion procedures (e.g. growing rods) in small children, allow for continued spinal growth.
Anterior/posterior spinal fusion in younger patients, prevents crankshaft phenomenon.
WHICH OTHER DISEASES SHOULD BE EXCLUDED (DIFFERENTIAL DIAGNOSIS)?
Cerebral Palsy Myelomeningocele Spinal muscular atrophy Muscular dystrophy (Duchenne and Becker) Syringomyelia Polyomyelitis Spinocerebellar ataxia (SCA) Arthrogryposis multiplex congenita (АМС) The condition is treated by medical spetialists in the field of:
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