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British Medical Books Health Issues Computerized Tomography

Scoliosis

Definition

  • an apparent lateral curvature of the spine
  • actually a triplanar deformity with lateral, anteroposterior and rotational components (Dickson et al., 1984)

Evaluation of the patient with scoliosis

History taking

  • deformity
    • when did it first appear
    • is it progressive?
    • Is there pain
    • family history of spine deformity or neurologic disease?
  • determine the status of growth
    • menses begun
    • pubic hair
  • ask about other observations or treatment
    • seen another doctor
    • treatment given

Physical examination
Examination of Other Areas

  • Skin
    • abN defects in the spine area (lipomas, hairy patches, hemangiomas or nevi)
    • cafe-au-lait spots (a sign of neurofibromatosis)
    • hyperelasticity - Ehlers-Danlos syndrome
  • Heart
    • murmurs – sonar
  • Uro-genital tract
    • horse-shoe kidney
    • sonar, IVP
  • Ears
    • congenital abN
    • preauricular skin tags - Goldenhar’s syndrome
  • Palate
    • high-arched palate - Marfan’s syndrome
    • cleft palate - congenital deformity
  • Hands
    • congenital anomalies, joint hyperelasticity (Ehlers-Danlos & Marfan’s sy)
    • muscle weakness (e.g. clawing of fingers in syringomyelia)
  • Hips
    • range of motion, especially for contractures
    • paralytic disorders - look for tightness of the extensors, flexors, adD, abD & ITB
  • Feet
    • diagnosis of neuromuscular problems
    • high arches suggest Friedreich’s ataxia or Charcot-Marie-Tooth sy
    • clubfeet, vertical tali or heel varus - spinal dysraphism
    • presence of both foot deformity & spine deformity in the same patient -> either a NM disorder or spinal dysraphism (e.g., diastematomyelia, intraspinal lipoma, filum terminale)
  • Pulmonary function testing
    • major reason for treating scoliosis preservation of lung capacity (especially thoracic scoliosis)
    • pulmonary function affected by the curve
    • serious error to use actual height for calculation
      • scoliosis causes loss of height & a falsely high value will be obtained
    • FVC & FEV1 & ABG

Neurologic examination

  • reflexes
    • deep tendon (biceps, triceps, patellar and Achilles-tendon reflexes)
    • superficial (abdominal)
    • pathological (Babinsky)
  • motor & sensory examination
  • straight-leg raising
  • Romberg test & finger-to-nose for any neuromuscular problem

Examination of the spine

  • curve
    • area of the curve (e.g., right thoracic, or double right thoracic & left lumbar)
    • magnitude of the curve
  • balance
    • deviation of a plumb line dropped from occiput or from 7th cervical vertebra to the gluteal cleft
      • measured in cm
    • shoulder elevation (also measured in cm)
    • prominence of one hip
    • are the iliac crests level?

On forward bending toward the observer

  • measured in cm or in degrees of deviation from the horizontal

Bending away from the examiner

  • deviation to one side
    • suggests cord or cauda equina irritation
    • bone scan & myelography indicated to rule out tumours
  • note whether there is pure scoliosis, pure kyphosis, pure lordosis or a combination of the above
    • rib hump due solely to rotation should never be called kyphoscoliosis
    • adolescent idiopathic scoliosis - lordosis of the thoracic spine, not kyphosis

Stage of development

  • Tanner system
    • breasts & pubic hair development
    • Tanner 2 & 3 - rapid phase of growth spurt

Radiologic evaluation

Positioning

  • standing views if can
  • sitting or supine if can’t stand

Curve pattern

  • side of convexity determines whether if left or right curve
  • site of a curve – where is the apical vertebra
    • cervical curve – apex between C2-C6
    • cervico-thoracic – apex C7-T1
    • thoracic curve – apex T2-T11
    • thoraco-lumbar – apex T11-L1
    • lumbar curve – apex L2-L4
    • lumbosacral – apex below L5
  • single or double pattern

Measurement of Curve

Cobb technique

  • first select end vertebrae of the curve
    • the most tilted from the horizontal
  • double curvature
    • both curves measured
  • transitional vertebra
    • upper end vertebra for the lower curve and the lower end vertebra for the upper curve
    • place only one line on this vertebra (upper and lower end-plates are parallel)
Scoliosis

Cobb method

Risser-Ferguson method

Rib-vertebral angle (RVA)

  • line perpendicular to the apical vertebral end plate & another line thru’ middle of the corresponding rib
  • angle formed by the inter-section -> RVA
  • RVA difference (RVAD)
    • difference between the values of the angles on the concave and convex sides of the curve
    • if initial RVAD > 20° -> progressive curve until proven otherwise
Scoliosis

Measurement of Kyphosis & Lordosis

  • on lateral view
  • as important as AP x-ray
  • Cobb technique applied

Evaluation of Rotation

  • small curve without rotation -> postural
  • length of the arthrodesis partly determined by the rotation

Nash & Moe method

  • evaluation of pedicles on the convex side of curve
  • grade I – grade IV

Pedriolle method

  • more complicated
  • in studies
Scoliosis

Side bending films

  • only preoperatively to determine which segment to fuse

Bone age determination

  • in conjunction with clinical assessment (Tanner staging)

Risser grading

  • ossification of the iliac crest apophyses on AP pelvis view
  • starts anteriorly from ASIS & proceeds posteriorly towards PSIS
  • divided into quarters & graded as Risser 1 to 4
  • Risser 5 complete fusion of apophysis
  • Grade 0 (no ossification) & 1 coincide with rapid growth

Vertebral ring apophysis

  • at upper & lower margins of vertebral body
  • fusion of the ring -> cessation of spinal growth

Pyle - Gruelich atlas

  • L hand & wrist AP to compare with atlases
  • to determine bone age

Special studies

Tomography

  • better definition of congenital anomalies
  • detection of osteoid osteomas - may produce scoliosis

Myelography

  • suspicion of spinal cord tumour, spinal dysraphism, or any neurologic problems 2° to curvature
  • always examine the entire spinal canal
  • water-soluble myelographic techniques

CT Scan

  • seldom useful in ordinary curvature problems
  • highly useful for bone tumours, infections, spinal stenosis, and some fractures
  • can be combined with water-soluble myelography(dysraphic problems, tumours, and cystic lesions)

MRI

  • evaluation of spinal canal & its contents
  • in all congenital scoliosis & infantile or juvenile idiopathic scoliosis
    • high incidence of dysraphism or intraspinal problems

Natural history

  • 2x mortality rate then normal population
  • << vital capacity if curve > 60°
  • if curve > 60° - progression after growth stops

Nonstructural scoliosis

Postural Scoliosis

  • children who do not voluntarily stand perfectly straight
  • usually thoracic kyphosis and lumbar lordosis, but also scoliosis of mild degree

Physical examination

  • no rib hump on forward bending
  • disappears in the prone position & when asked to stand in a very straight position

X-ray

    • long curve, extending from one end of the spine to the other
    • no rotation
    • supine x-ray usually perfectly straight & bending films show no areas of contracture
  • such curves do not progress nor become structural

Leg Length Discrepancy

  • results in a curvature of the spine in the standing position
  • functional or non-structural - no intrinsic stiffness of the curve in the spine
  • disappears in sitting, lying down or if leg length is corrected
  • does not become structural if presents for long period of time
    • spend little time standing on our two feet with even weight

Hysterical Scoliosis

  • emotionally disturbed teenagers develop a curvature of the spine

Physical examination

  • constant in the upright & sitting position
  • may or may not be present in the prone or supine position
  • always absent while sleeping & under anaesthetic

X-ray

  • long, sweeping, bizarre pattern curvature
  • not associated with rotation on the x-ray or true rib hump on forward bending
  • exclude spinal cord tumour or other neurologic pathology
  • not to treat by orthopaedic methods (e.g., exercises, braces, or casts) & never by surgery
    • psychiatric problem

Structural scoliosis
Idiopathic scoliosis

Definition

  • scoliosis of unknown cause
  • most common form of scoliosis
  • all possible causes must be excluded (congenital, neuromuscular etc.)
  • if painful -> further investigation (osteoid osteoma)
  • three types depending on the age of onset

Incidence/prevalence

  • 1.5 - 3%
  • infantile 1%
  • juvenile 10%
  • adolescent 89%

Aetiology

  • unknown
  • multifactorial
  • many theories

Infantile

  • genetic tendency – multifactorial
  • postural cause
    • plagiocephaly – flattened occiput after birth
  • associated factors
    • CDH
    • heart defect

Adolescent

  • familial pattern
    • autosomal or gender linked with incomplete penetrance
    • examine siblings
  • too rapid growth
  • paraspinal muscle weakness/abN

Presentation

  • asymmetry of the back
    • noticed by parents
    • school screenings
      • Adams forward bending test
      • waist & feet together, knees straight, palms opposed
      • any positive sent for X-rays
      • expensive, but worthwhile as early bracing alters the natural Hx of ~

Infantile idiopathic scoliosis

Definition

  • onset before 3 yrs of age
  • 1 % of all idiopathic ~
  • boys > girls
  • curves – left thoracic or thoracolumbar

Progression

  • 85 % non progressive
  • 15 % progressive
    • if RVAD > 20° -> progressive
    • benign & malignant type

Treatment

Observation

  • non progressive -> no treatment
  • follow up with X-rays every 6/12

Bracing

  • any curve > 35°
  • any progressive curve
  • serial cast first
  • Milwaukee brace full time
  • weaning according to correction
  • child observed until maturity

Surgery

  • if can’t control with brace
    • usually malignant progressive type
  • instrumentation without fusion
    • rod under fascia
    • protected in Milwaukee brace
    • spinal growth can occur
  • spinal fusion
    • at adolescent growth spurt
    • anterior & posterior fusion to avoid crankshaft effect

Juvenile idiopathic scoliosis

Definition

  • onset between 3 yrs & puberty
  • boys = girls
  • 10 % of all idiopathic ~
  • left thoracic curve in younger children
  • double or R thoracic in older child
  • intraspinal pathology common - MRI

Progression

  • 1/3 non progressive
  • 2/3 progressive
    • 50% managed with bracing
    • 50% needs surgery

Treatment

Observation

  • curve < 25°
  • follow up every 4-6/12 with repeat standing x-rays

Bracing

  • curve > 25°
  • progressive curve
  • Milwaukee brace
    • 24 hours a day
    • if curve maintained 20° or << -> weaning
      • out of brace 4 hrs -> x-ray
      • 3/12 later out for 6 hrs
      • continued until just night bracing
      • if still stable – out of brace
  • continue observing until maturity

Surgery

  • if progression in brace
  • depending on age - instrumentation without fusion or fusion

Adolescent idiopathic scoliosis

Definition

  • first detected at time of adolescent growth spurt
  • girls > boys
  • 89% of all idiopathic ~
  • right thoracic or double R thoracic L lumbar curve usually

Progression

  • > 5° (10°) increase from time of presentation
  • risk factors
    • larger the curve (>20-29°)
    • double curve
    • growth potential available (Risser 0 or 1)
    • girls
    • family hx. of scoliosis, rotational prominence, decompensation

Treatment

  • full Hx & clinical examination
  • complete neurologic assessment
    • any sign of intraspinal pathology -> MRI
  • exclude other possible causes
  • standing AP & lateral X-rays of whole spine
    • 36 inch cassette
    • balance seen better
    • measure curves (Cobb’s)
    • unusual curve pattern (L thoracic) -> MRI
  • evaluate growth potential
    • Tanner grading (menarche in girls, axillary hair in boys)
    • Tanner stage 2 indicates onset of adolescent growth spurt
    • Risser grading (iliac crest)

Observation

Growing child

  • age, pre-menarche, no axillary hair, Risser sign < 3
  • < 25° curve
    • follow up x-ray every 6-8/12 (< 20°)
    • x-ray 3-4/12 for larger curves
    • if progression > 5° from presentation -> non-operative rx
  • > 30° curve -> non-operative rx

Near end of growth

  • Risser sign > 3, post menarche, axillary hair
  • up to 45° observe
    • follow up every 4-6/12
    • until cessation of growth

Non-operative Rx

  • control curve & prevent progression
  • cosmesis should improve

Electric stimulation

  • muscles on convexity of curve
  • used in past
  • no effect on natural Hx.

Exercises, Manipulation

  • no benefit – no role

Orthotic Rx. (bracing)

  • in growing child
  • > 30-40° curve or curve < 29° , but progressive
  • alters natural hx. of scoliosis
    • failure – if patient progress > 5° in brace or needs surgery

Contraindication

  • completed growth
  • curve > 45° or < 25° without progression

Milwaukee brace

  • cervico-thoraco-lumbo-sacral orthosis
  • moulded pelvic section
  • 2 posterior & 1 anterior uprights connected to neck ring
  • pads attached to uprights according to curve pattern

TLSO

  • thoraco-lumbo-sacral orthosis
  • underarm or lower type
  • not used if apex above T8

Method

Full time wear (20 –22 hrs)

  • out for bathing or athletic activity
  • follow up every 4/12
    • standing x-ray in brace
    • measure for progression
    • adjust for growth

Weaning from brace

  • started at end of growth (no increase in height, Risser 4 or >, 12-18/12 post-menarche)
  • standing x-ray at 4 hrs out of brace -> if no loss -> 4 hrs out of brace daily
  • repeated every 4 months adding 4 hrs until night bracing
  • night bracing for 6-12 month, then out of brace for 1 week & x-ray

Operative Rx

Indications

  • child in a rapid growing phase with 40-45° curve
  • progression to a curve of 40-45° under bracing
  • mature adolescent with curve > 50-60°
  • other factors
    • cosmetic appearance
    • decompensation
    • thoracic lordosis

Pre-operative evaluation

  • full Hx. & clinical evaluation
  • complete neurologic examination
  • additional x-rays
    • lateral for saggital balance
    • bending views

Selection of fusion area

  • identify curve pattern
    • analyse clinical appearance
    • standing & supine bending views
  • single or double major pattern
    • all major curves need to be fused
    • compensatory curves not
  • King’s classification
    • classification of thoracic curves into 5 types (type I – V)
    • based on location, apex, magnitude & flexibility
    • attempt to determine fusion levels
    • King type II & III most common
    • fusion must extend from a non-rotated vertebra cranially to a non-rotated vertebra caudally
    • the lower end vertebra must be the stable vertebra (bisected by the central sacral line)
Scoliosis

Type I deformity

  • S-shaped curve in which both the thoracic and the lumbar curves cross the midline
  • lumbar curve usually larger and less flexible than the thoracic component
  • double major or double primary pattern ---> both curves need to be included in the fusion area
  • almost always T4 to L4

Type II curve

  • S-shaped combined thoracic & lumbar double curve pattern
  • thoracic curve greater in magnitude and less flexible than the compensatory lumbar curve
  • lumbar curve must cross the centre sacral line
  • only the thoracic curve needs to be fused

Type III

  • pure thoracic curve
  • compensatory lumbar curve never crosses the midline and much more flexible
  • fusion of only the thoracic curve
  • inferior level --> stable vertebra

Type IV curve

  • long thoracic curve with the apex in thoracic spine, but its lower end vertebra in lumbar spine
  • lower end vertebra typically L2, but rotation persists to L3 or even L4
  • decompensation of the thorax to the right occurs
  • important to distinguish type III pattern from type IV pattem because the latter needs a longer fusion and a different
  • inferior level of fusion --> stable vertebra
  • King et al did not describe or discuss primary thoracolumbar curves (apex at T12 or L2) or primary lumbar curves

“King V” pattern

    • double major thoracic pattern
    • identifying features
        1. a high left curve
          • apex usually at T3, the upper end vertebra T1 or T2 the lower end vertebra T5 or T6
        2. a second right thoracic curve
          • upper end vertebra T5 or T6; the apex at T8, T9, or T10 and the lower end vertebra at T11, T12, or L1, or even L2
        3. both curves “structural”
          • full curves (not fractional) and lack complete flexibility on side-bending
        4. a “positive T1 tilt”
          • a line drawn along the upper end plate of T1 higher on the left than on the right
        5. apical vertebra of the upper curve to the left of midline

Clinical evaluation

View of the patient from the front

  • the hair is tied up, and the gown is pulled down or off --> neckline, trapezius area, and the shoulder balance seen clearly
  • higher L shoulder
  • trapezius area prominence or fullness more readily seen from the front than the back

Viewed from the side

  • areas of abnormal lordosis and kyphosis
  • kyphosis in the T1 to T5 area of particular concern --> extending the fusion up to T2 or T1

Viewing from the back

  • overall balance
  • Is the head centered over the pelvis? Are the shoulders level? Are the trapezius prominence areas symmetric?
  • Is the thorax vertical or tilted? Is the thorax as a unit centered over the sacrum?
  • L scapula prominence, trapezius fullness

Forward bending examination (best done with the examiner seated and the patient bending toward the examiner)

  • rotational prominences and the amount of such prominence
    • look specifically at the high thoracic level (L), the low thoracic level (R), and the lumbar level (nil)
    • presence of high left thoracic prominence diagnostic of a structural left thoracic curve

Approach

  • posterior most common
    • subperiosteal exposure
    • facet excision
    • instrumentation for correction
    • autogenous iliac crest BG
  • anterior
    • for single L or Th-L curve
    • shorter fusion can be done
    • exposure from convexity
  • combined
    • for large stiff curves
    • to prevent crankshaft effect in a young child
    • one or two staged
  • thoracoplasty
    • if severe rib hump (> 5-6 cm)
    • excision of medial portion of rib

Instrumentation

  • corrects deformity
  • adds stability & early mobilisation
  • << pseudo-arthrosis

1st generation

    • Harrington hook-rod system

2nd generation

    • sublaminar wiring (Luque)

3rd generation

    • allows 3 dimensional correction
    • no need for bracing
    • CD – pedicle screws, hooks & rod
    • TSRH, Moss Miami etc.

Safety

  • monitoring possible – SSEP
  • Stagnara wake-up test
  • any change in neurology – reduce distraction or remove distraction rod
  • cord problems with correction
    • Harrington technique – 0.23%
    • CDI – 0.60%
    • sublaminar wiring – 0.86%

Preservation of spinal mobility

  • back pain after spinal fusion
  • minimum number of segments must be fused

Congenital scoliosis

Definition

  • curve d/t anomalous development of the vertebra
  • anomalies present at birth - curvature at birth or developing much earlier in life
  • receive less than optimum care - frequently progress to a serious degree
  • rigid & resistant to correction - must not be allowed to progress - early fusion necessary

Classification

Failure of Formation

  • partial (wedge)
  • complete (hemivertebra)

Failure of Segmentation

  • unilateral failure of segmentation (unilateral unsegmented bar)
  • bilateral failure of segmentation (bloc vertebrae)

Miscellaneous

Mixed

Progression

  • 25% - no progression
  • 50% progresses significantly -> treatment required
  • follow up at 6-month intervals until end of growth
    • high-quality X-rays
    • photographs
  • malicious anomaly - unilateral unsegmented bar -> early fusion
    • total lack of growth on concave side

Associated congenital anomaly

  • most frequently genitourinary tract -> IVP
  • congenital heart defects -> listen for murmurs
  • high frequency of spinal dysraphism
  • check x-rays for interpedicular widening or midline bony spicules
    • any evidence of hidden neurologic disorder
    • hair patches, dimples, hemangiomata
    • abN of the lower extremities
      • flat feet, cavus feet, vertical tali, clubfeet
      • atrophy of one calf, smaller foot & asymmetric reflex

X-ray evaluation

  • standing, sitting or supine AP & lateral
  • tomograms to see deformity better
  • MRI
    • always to exclude dysraphism

Orthotic Treatment

  • no role
    • primary deformity in the bones - rigid
    • more difficult to treat than idiopathic or paralytic curves

Indications

  • flexible curves in whom the curvature was primarily in non congenital vertebrae
    • three or four patterns

Goal

  • must control the curve in an acceptable alignment
  • main indication for bracing -> delay of surgery until a more optimal age
  • if curve progresses despite orthosis -> fusion must be done
  • Milwaukee brace

Surgical Treatment

  • most important
  • two questions:
    • what is the best procedure?
    • what is the best time for the procedure?

Posterior fusion

Aim

  • prevention of progression rather than correction

Usually without instrumentation

  • with instrumentation
    • >> risk of neurological damage
    • MRI evaluation for tethered cord
  • correction obtained with Risser cast or traction occasionally
  • abundant bone graft
    • thick fusion mass necessary to avoid bending of the fusion by the intact anterior growth plates

Anterior and posterior fusion

  • to prevent bending of the fusion mass - crankshaft phenomenon
  • if concave growth persists - progressive improvement of the curve

Convex hemi-epiphyseodesis

  • to arrest convex growth
  • must be < 5 yrs old – enough growth remaining
  • contraindicated if kyphosis at the deformity
  • combined anterior & posterior approach
  • postop. cast 4/12 then Milwaukee brace for 18/12

Hemivertebra excision

  • difficult & dangerous
  • in rigid angulated scoliosis
  • wedge osteotomy at the apex of a curve & fusion of the whole curve

Neuromuscular scoliosis
Preoperative Evaluation

Nutrition

  • Weight for-height percentile
  • Serum tests
    • total protein, albumin, Iymphocyte count

Respiratory system

  • History
    • aspiration, pneumonia, number of respiratory illnesses
    • anatomic anomalies including tracheomalacia & tracheostomy tube
  • Physical examination:
    • strength of cough, presence of rhonchi, stridor
  • Diagnostic tests:
    • CxR, ABG, pulmonary function tests (developmental age > 4)

Hematologic tests

  • PT/TT/PTT, CBC, Bleeding time (for patients on valproic acid)

Cardiovascular screening

  • Duchenne muscular dystrophy:
    • ECG, CXR, cardiac ECHO
  • Friedreich’s ataxia:
    • ECG, chest radiograph, cardiac ECHO if symptomatic or > 18 years old

Classification

  • endorsed by the Scoliosis Research Society

Idiopathic

Infantile

  • 0-3 years
  • Resolving
  • Progressive

Juvenile

  • 4 years-puberty onset

Adolescent

  • puberty onset to epiphyseal closure

Adult

  • epiphyses closed

Neuromuscular

Neuropathic

  • Upper motor neuron lesion
    • Cerebral palsy
    • Spinocerebellar degeneration
      • Friedreich’s
      • Charcot-Marie-Tooth
      • Roussy-Levy
    • Syringomyelia
    • Spinal cord tumour
    • Spinal cord trauma
    • Other
  • Lower motor neuron lesion
    • Poliomyelitis
    • Traumatic
    • Spinal muscular atrophy
    • Myelomeningocoele (paralytic)
    • Dysautonomia (Riley-Day)
    • Other

Myopathic

  • Arthrogryposis
  • Muscular dystrophy
    • Duchenne (pseudohypertrophic)
    • Limb-girdle
    • Facio-scapulohumeral
  • Congenital hypotonia
  • Myotonia dystrophica
  • Other

Congenital

Congenital scoliosis

Failure of formation

  • Wedge vertebra
  • Hemivertebra

Failure of segmentation

  • Unilateral bar
  • Bilateral (“fusion”)

Mixed

Associated with Neural Tissue Defect

  • Myelomeningocele
  • Meningocele
  • Spinal dysraphism
    • Diastematomyelia
    • Other

Neurofibromatosis

Mesenchymal

  • Marfan’s
  • Homocystinuria
  • Ehlers-Danlos
  • Other

Traumatic

  • Fracture or dislocation (non paralytic)
  • Post irradiation
  • Other

Soft tissue contractures

  • Postempyema
  • Burns
  • Other

Osteochondrodystrophies

  • Achondroplasia
  • Spondyloepiphyseal dysplasia
  • Diastrophic dwarfism
  • Mucopolysaccharidoses
  • Other

Tumor

  • Benign
  • Malignant

Rheumatoid disease

Metabolic

  • Rickets
  • Juvenile osteoporosis
  • Osteogenesis imperfecta

Related to lumbosacral area

  • Spondylolysis
  • Spondylolisthesis
  • Other

Thoracogenic

  • Post-thoracoplasty
  • Post-thoracotomy
  • Other

Hysterical

Functional

  • Postural
  • Secondary to short leg
  • Due to muscle spasm
  • Other