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Paediatrics: Spine: 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.

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.

 

 

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