Paediatrics: Spine: scoliosis

Spine: scoliosis

Idiopathic scoliosis

•   Lateral curvature (>10°) +/– rotation deformity of the spine without an identifiable cause. Description of curvature based on direction of apical convexity. There are 3 types.

•   Infantile (<3yrs old): left > right side; males > females; associated plagiocephaly (skull flattening), hip dysplasia, and other congenital defects. May be s to underlying spinal abnormality.

•   Juvenile (3–10yr olds): may be s to underlying spinal abnormality; high risk of curve progression (70% require treatment with 50% needing brace and 50% surgery).

·  Adolescent: most common in 11–16-yr-olds. Females > male.

Incidence

•   Curves >10°, 2% incidence.

·  Curves >30°, 0.2% incidence.

•   Right thoracic curves > double major (right thoracic and left lumbar) > left lumbar > right lumbar.

Risk factors

Positive family history; daughters of affected mothers more likely to be affected, Marfans, neurofibromatosis.

Disease progression

Risk factors for curve progression: age <12yrs; skeletal immaturity; female; curve magnitude >20°; spine at greatest risk of curve progression during puberty. Natural history after skeletal maturity is curves < 30° unlikely to progress, if >50° 2/3 will progress. Severe curves (Cobb angle >100°) associated with pulmonary dysfunction, early mortality, pain, poor self-image.

Symptoms

Onset of symptoms, rate of progression, Is it painful? (inflammatory or neoplastic.) Ask about respiratory and neurological symptoms.

Signs

•   Inspect child standing.

•   Describe scoliosis as the side to which the spine is convex (shoulder on convex side is elevated).

•   Inspect: pelvic height—limb length difference; waistline asymmetry; trunk shift; spinal deformity; rib rotational deformity (rib hump).

•   Bend—touch toes. Is it fixed?

•   Adam’s forward test: asymmetry of the posterior chest wall on forward bending. If scoliosis disappears: postural (80% of scoliosis).

•   Full neurological examination including abdominal reflexes.

•   Lower limb examination (other causes of postural scoliosis: unilateral muscle spasm; unequal leg length).

True idiopathic scoliosis

Painless, convex to the right in the thoracic spine, not associated with any neurological changes.

Investigations

• Standing PA and lateral X-rays full spine.

• MRI if pain, neurological changes, rapidly progressive curve, excessive kyphosis, left thoracic/thoracolumbar curves, considering surgery.

Treatment

Based on maturity of patient, magnitude of deformity, and curve progres-sion. The aim is to prevent further progression.

Non-operative

• Close observation (6-monthly X-rays for curves <25°).

• Bracing is controversial. May slow/halt curve progression—the more it’s worn, the more effective it is. Consider for children with curves <40°.

• Manipulation and casting for young children with more severe curves

Operative

• Anterior/posterior spinal fusion with instrumentation for severe deformities (>45°).

• Posterior instrumentation without fusion

• Vertebral expanding prosthetic titanium rib

Congenital scoliosis

Most common congenital spinal disorder.

Aetiology 

Abnormal vertebral development in the first trimester.

Associations 

Can be isolated deformity or associated with other con-genital abnormalities: Spinal (40%)> genitourinary (20%) > heart disease (10–15%), also associated with syndromes, e.g. VACTERL.

Disease progression

Risk of progression dependent on morphology and growth potential of vertebrae. Greatest risk during periods of rapid growth (<2yrs and >10yrs old).

Treatment 

Early diagnosis; often need surgery.

Scoliosis secondary to neuromuscular disorders

Symptoms and signs

Progresses more rapidly and may continue after maturity; longer curves involving more vertebrae and less likely to have compensatory curves.

Associations

• Skeletal: pelvic obliquity, bony deformities, cervical involvement.

• Pulmonary: more frequent, ‘fall’ lung function and pneumonia.

• Neurological: brainstem, proprioception, Klippel–Feil syndrome.

• Upper motor neuron disease: cerebral palsy, spina bifida, spinocerebellar degeneration, syringomyelia, spinal cord tumour/trauma, tethered cord, diastematomyelia.

• Lower motor neuron disease: poliomyelitis, spinal muscular atrophy, (myopathic: DMD).

Syndromes: neurofibromatosis, Marfan’s.