Neuromuscular
Scoliosis

What is neuromuscular scoliosis?

Scoliosis as a consequence of an underlying condition.

Neuromuscular scoliosis is a lateral curvature of the spine that develops as a consequence of a neurological or muscular underlying condition. Here the deformity is not the disease itself but its effect on the trunk and back muscles — the absent or disturbed muscular balance allows the growing spine to deviate sideways.

The most common underlying conditions include:

• Cerebral palsy — the most frequent cause of neuromuscular scoliosis, particularly in more severely affected, non-ambulatory children.
• Spinal muscular atrophy (SMA) — a genetic loss of motor neurons with an early, often rapidly progressing curve.
• Muscular dystrophies, e.g. Duchenne muscular dystrophy — scoliosis frequently develops once the ability to walk is lost.
• Myelomeningocele (spina bifida) and other disorders of the spinal cord.

How neuromuscular differs from idiopathic scoliosis

Neuromuscular scoliosis behaves differently from the far more common idiopathic scoliosis in several ways:

• Earlier onset — often already in early childhood (early-onset).
• Rapid progression — the curve can continue to progress even after growth is complete.
• Long, C-shaped curves — instead of the typical S-shape of idiopathic scoliosis.
• Pelvic involvement — often combined with pelvic obliquity; in a pronounced form as the so-called windswept deformity, in which trunk and pelvis are tilted against each other.

Diagnostic considerations

The examination is tailored to what the patient is able to do. In non-ambulatory children and adolescents the assessment in the seated position takes priority: sitting balance, head and trunk control, and the impact of the deformity on care, nutrition and breathing. Imaging (standing or seated full-spine radiograph) is adapted to the individual situation.

Central to this is interdisciplinary coordination: paediatric neurology, physiotherapy, orthotics and — in muscular conditions — pulmonology and cardiology work together to define the course and treatment goals jointly.

Conservative options and their limits

In neuromuscular scoliosis, conservative measures are aimed primarily at preserving the ability to sit and comfort — but as a rule they cannot halt progression permanently:

• Seating systems and adapted wheelchair configurations to improve sitting balance.
• Brace therapy — in neuromuscular curves usually symptom-relieving and supportive rather than curative; it may delay the timing of surgery but rarely corrects the curve permanently.

Surgical considerations

When a long curve increasingly compromises sitting balance, care or organ function, surgical correction is considered. Characteristic features of neuromuscular scoliosis are:

• Extended fusion with pelvic fixation (sacropelvic instrumentation) — because the curves often reach into the pelvis, the fusion frequently has to be carried down to the pelvis to achieve a stable, level sitting axis.
• Elevated perioperative risk — the underlying condition, reduced lung function, nutritional status and greater blood loss increase the demands on planning and aftercare.
• Interdisciplinary preoperative preparation — optimisation of nutrition, breathing and, in certain muscular dystrophies, cardiological assessment are an integral part of the planning.

Precisely for these demanding procedures, specialised infrastructure pays off: Klinikum Traunstein offers particular expertise in anaesthesia as well as a large paediatric unit with an affiliated social-paediatric centre (SPZ) — an environment that supports the safe care of children and adolescents with neuromuscular underlying conditions before, during and after surgery.

Growth-guiding systems in early-onset forms

If neuromuscular scoliosis begins very early, while the spine and chest are still growing strongly, growth-guiding procedures are an option. They stabilise the curve while still allowing further growth, instead of an early rigid fusion. More on the pages about MAGEC rods and NEMOST (in German).