Neuromuscular Scoliosis - Causes, Symptoms, and Treatment

Introduction

Spinal deformity is a frequent finding in neurological patients. Neuromuscular scoliosis (NMS) is a non-congenital spinal deformity that can occur in individuals with any sort of neuromuscular disorder. It is caused by a lack of muscle control induced by a neurological or degenerative muscular disorder, such as muscular dystrophy or cerebral palsy. Neuromuscular scoliosis accounts for a small percentage of the total number of scoliosis cases identified annually. However, the underlying abnormalities associated with this type of scoliosis frequently result in some of the more complicated and extensive curves encountered by pediatric orthopedic surgeons and present a distinct set of treatment difficulties.

What Is Scoliosis?

Scoliosis is a condition in which the spine curves sideways. In three dimensions, the curve is typically "S" or "C" shaped. The cause of the majority of cases is unknown; however, it is thought to be a combination of genetic and environmental factors. Scoliosis affects approximately three percent of the population. It usually appears between the ages of ten and twenty. Scoliosis is classified into different types. They are:

• Structural scoliosis.

• Non-structural scoliosis.

• Idiopathic scoliosis.

• Congenital scoliosis.

• Degenerative scoliosis.

• Neuromuscular scoliosis.

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• Lumbar scoliosis.

• Thoracolumbar scoliosis.

What Is Neuromuscular Scoliosis (NMS)?

Neuromuscular scoliosis is a form of scoliosis that occurs in children who have medical issues that limit their ability to control the muscles that stabilize the spine. NMS is commonly related to muscular dystrophy, spina bifida, and cerebral palsy. In neuromuscular scoliosis, the nerves and muscles cannot maintain proper spine and trunk balance and alignment. Neuromuscular curves are frequently connected with pelvic obliquity, in which one side of the child's pelvis is higher than the other. Kyphosis is frequently present at the same time.

Neuromuscular scoliosis (NMS) frequently worsens over time and progresses more quickly than adolescent idiopathic scoliosis (AIS), usually continuing after the person's growth and spine development have stopped. Conditions with systemic and neurologic involvement are more likely to display their progressive character. Neuromuscular scoliosis has a wide range of occurrences due to its diverse etiology. With increasing neuromuscular involvement, the likelihood and severity of the curves are likely to rise.

What Are the Causes of Neuromuscular Scoliosis?

Muscle weakness, paralysis, or neurological difficulties associated with an underlying disorder induce neuromuscular scoliosis. The level of nerve and muscle involvement associated with a child's neuromuscular disease influences the risk that they may develop NMS. Although NMS does not occur in all children with neuromuscular disorders, it is common in children who use wheelchairs. A child with some neuromuscular problems may be able to walk while they are younger but will need to use a wheelchair when they reach puberty. As they reach adolescence, these children will likely acquire NMS or have existing curve progress. The neuromuscular diseases associated with NMS are the following.

• Cerebral palsy.

• Multifocal disorders (spina bifida).

• Spinal cord injury.

• Spinal muscular atrophy.

• Muscle fiber disorders.

• Musculoskeletal tumors (benign or malignant).

• Polio.

What Are the Symptoms of Neuromuscular Scoliosis?

Neuromuscular scoliosis symptoms often occur early in a child's life. Symptoms associated with NMS are the following.

• Poor balance.

• Seating problems.

• Thoracic insufficiency syndrome.

• Poor coordination.

• Uneven buttocks or hips.

• Unevenly hanging arms.

• Uneven shoulder heights.

• Tilted shoulders.

• Leaning more to one side.

• Tilted pelvis.

• Uneven waistline.

• Prominent ribs (one side).

Children with NMS may also have various forms of spinal curvatures, such as lordosis (swayback) or kyphosis (hunchback). Larger upper back scoliosis curves or hyperlordotic curves (extreme swayback) may restrict the space in the chest for the lungs and create breathing issues.

How Is Neuromuscular Scoliosis Diagnosed?

The primary method for determining neuromuscular scoliosis and the degree of curvature is X-ray scans. It is usually taken from the front and side of the body. X-rays provide detailed views of dense structures like bone. The doctor will be able to examine the location of the spine curve and quantify the degree of curvature with an X-ray. An MRI (magnetic resonance imaging) scan provides a clear view of soft tissues, which enables the assessment of the spinal cord's condition. Other diagnostic tests like bone scans, muscle biopsies, bone density scans, nerve conduction studies, ultrasound, and electromyogram (EMG) are also used for the diagnosis of neuromuscular scoliosis.

What Is the Treatment for Neuromuscular Scoliosis?

Treatment for NMS will be determined by the age of the child, the underlying disease, and the nature and severity of the spinal curve.

• Non-Surgical Treatment - Non-surgical treatments like bracing, wheelchair modifications, environmental adaptation, and physical therapy can assist the child in adapting to ordinary situations and improving mobility. Bracing can prevent the child's spine curvature from worsening during growth phases but will not correct the curve.

• Surgical Treatment - The most common treatment for neuromuscular scoliosis is spinal fusion surgery, which involves stabilizing the spine. The treatment straightens the spine and solidifies the bone using metal rods, hooks, screws, and wires, known as instrumentation. The spine fuses six to twelve months following surgery, much like a fractured bone heals. During this time, the child must wear a brace. Normally, the instrumentation remains in the back without causing any issues. Patients with neuromuscular scoliosis are more likely to experience blood loss during surgery. Several steps are taken to address this, including using cell savers (machines that filter blood so it can be returned to the patient), designated donor blood, or autologous blood (blood donated by the patient before surgery), if necessary.

Conclusion

Neuromuscular scoliosis, or NMS, is a type of scoliosis that induces an uneven curvature of the spine. It is the second most frequent type of scoliosis related to nerve or muscle system abnormalities such as spinal cord damage, cerebral palsy, and spina bifida. These neuromuscular diseases cause muscles to become spastic, weak, or paralyzed. Thus without complete back muscular support, the spine develops an aberrant curve as it grows. Symptoms associated with neuromuscular scoliosis frequently occur in children at a young age. Treatment involves non-surgical management and surgical spinal cord fusion.