Neuromuscular scoliosis is a spine deformity often associated with cerebral palsy, spina bifida, muscular dystrophy, paralytic conditions and spine tumors.
Children who suffer from cerebral palsy (CP) have a significant chance of developing scoliosis during their early years and adolescence. The behavior of this scoliosis is closely associated with the severity of the CP disability and unlike idiopathic scoliosis, it continues to progress beyond skeletal maturity. Conservative measures may slow the progression of the curve, however, surgery remains the only definitive management option. Advances in surgical technique over the last 50 years have provided methods to effectively treat the deformity while also reducing complication rates. The increased risk of surgical complications with these complex patients make decisions about treatment challenging, however, with careful pre-operative optimization and post-operative care, surgery can offer a significant improvement in the quality of life. This review discusses the development of scoliosis in CP patient, evaluates conservative and surgical treatment options and assesses post-operative outcome.
Cerebral palsy (CP) is defined as a permanent, non-progressive abnormality of motor function that is a result of injury to the developing brain (1). It can occur pre, peri or post-natally from a variety of causes. The term encompasses a heterogeneous group of conditions characterized by abnormal muscle tone, movement, and posture. There is a strong link between CP and the development of scoliosis. It is estimated scoliosis occurs in between 21% and 64% of patients with CP (3–5). Spinal deformity is thought to occur before 10 years of age (5–7) and unlike in idiopathic scoliosis, has been shown to progress beyond skeletal maturity (8).
The development of scoliosis is strongly linked to the level of global disability caused by the CP. There are classification systems that describe the spectrum of disability that can occur under the umbrella term of CP. The most widely used of these is the Gross Motor Function Classification System (GMFCS), which splits children into 5 categories depending on their functional capacity.
Spina bifida is a condition that affects the spine and is usually apparent at birth. It is a type of neural tube defect (NTD).
Spina bifida can happen anywhere along the spine if the neural tube does not close all the way. When the neural tube doesn’t close all the way, the backbone that protects the spinal cord doesn’t form and close as it should. This often results in damage to the spinal cord and nerves.
Spina bifida might cause physical and intellectual disabilities that range from mild to severe. The severity depends on:
Muscular dystrophy refers to a group of disorders that involve a progressive loss of muscle mass and consequent loss of strength.
The main forms of muscular dystrophy may affect up to 1 in every 5,000 males.
The most common form is Duchenne muscular dystrophy. It typically affects young boys, but other variations can strike in adulthood.
Muscular dystrophy is caused by genetic mutations that interfere with the production of muscle proteins that are needed to build and maintain healthy muscles.
The causes are genetic. A family history of muscular dystrophy will increase the chance of it affecting an individual.
There is currently no cure, but certain physical and medical treatments can improve symptoms and slow the progression.
Paralysis is most often caused by damage in the nervous system, especially the spinal cord. Other major causes are stroke, trauma with nerve injury, poliomyelitis, cerebral palsy, peripheral neuropathy, Parkinson’s disease, ALS, botulism, spina bifida, multiple sclerosis, and Guillain–Barré syndrome.
A spinal tumor is an abnormal mass of tissue within or surrounding the spinal cordand/or spinal column. These cells grow and multiply uncontrollably, seemingly unchecked by the mechanisms that control normal cells. Spinal tumors can be benign (non-cancerous) or malignant (cancerous). Primary tumors originate in the spine or spinal cord, and metastatic or secondary tumors result from cancer spreading from another site to the spine.
Spinal tumors may be referred to by the region of the spine in which they occur. These basic areas are cervical, thoracic, lumbar and sacrum. Additionally, they also are classified by their location in the spine into three major groups: intradural-extramedullary, intramedullary and extradural.