Guest Author - Wollie Woehler

Neuromuscular diseases are often the cause of Scoliosis, a common deformity in patients with some of these diseases. Scoliosis is a musculoskeletal condition, which primarily affects children and adolescents where there is an abnormal lateral curvature of the spine. This abnormal curvature either bends the spinal column to the left or to the right. Looking at most people’s spines from the back, it has the appearance of a straight line. Because of the abnormal curvature, the spine of someone with scoliosis more closely resembles the letter S or C. The name comes from the Greek word ‘scoliosis’ which means ‘crookedness.’ Scoliosis is relatively common, affecting approximately 3% of the population. The condition has been known for centuries as it is described in very early medical histories.

In neuromuscular spinal deformities, progression occurs much more frequently than in idiopathic scoliosis - progression often continues into adulthood. The long-term effects of the spinal deformity in patients with neuromuscular conditions includes loss of the ability to sit, pulmonary function may be markedly affected causing the patient to need assistive oxygen devices at night to breathe easily while sleeping.

According to the Scoliosis Research Society, Scoliosis associated with neuromuscular diseases can be divided into naturopathic and myopathic types.

The naturopathic conditions have been subdivided into those with upper and lower motor neuron lesions. The group with upper motor neuron lesions includes diseases such as cerebral palsy, syringomyelia, and spinal cord trauma.

The group with lower motor neuron lesions includes poliomyelitis and spinal muscular atrophy. The myopathic conditions include arthrogryposis, muscular dystrophy, and other forms of dystrophy.

Not all patients with neuromuscular diseases need surgery to correct or stabilize the spine. Visits to a physiotherapist or proper back bracing should be considered before surgery is done. Where surgery becomes necessary, a thorough assessment of a patient with neuromuscular scoliosis’s nutritional status and pulmonary function is extremely important. Detecting and correcting malnutrition preoperatively, helps prevent postoperative wound infection and healing problems.

An orthopedic examination, which includes assessment of all extremities and joints for contractures. Spinal deformity, decomposition, and shoulder balance should be done and documented. Ambulatory status is also evaluated, and patients are classified as walkers, sitters, or non-sitters.

The two main indications for surgery are curve progression and deterioration in sitting ability. A detailed preoperative history and assessment should include:
- An evaluation of respiratory competency,
- Cardiac status,
- Nutrition, possible feeding difficulties,
- Seizure disorders,
- Urologic status, and
- Metabolic bone disease.
This is important to ensure that the patient can tolerate reconstructive spinal surgery.

Malignant hyperthermia occurs with some frequency in certain neuromuscular disorders and is triggered by inhalational anesthetics and succinyl choline. This should be a consideration in all patients with neuromuscular conditions who are undergoing general anesthesia. (Next week we will discuss malignant hyperthermia in more detail as it is life threatening for some patients when undergoing surgerie.)

A highly rated book on neuromuscular scoliosis is:
The Scoliosis Sourcebook

Another great book is
Raising a Child with a Neuromuscular Disorder: A Guide for Parents, Grandparents, Friends, and Professionals