paediatric spinal deformities 2

8/3/2019 Paediatric Spinal Deformities 2

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Congenital

o Failure of Formation - Partial Unilateral (wedge vertebra), Complete Unilateral

(Hemivertebra)

o Failure of Segmentation - Unilateral (Unilat. unsegmented bar), Bilateral (Block

vertebra)

o Mixed

Others

o Neurofibromatosis

o Neural defects - Myelomeningocoele, spinal dysraphismo Connective Tissue -Marfans, homocystenuria, Ehlers-Danlos

o Traumatic Fracture/dislocation, irradiation

o Tumours

o Bone Dysplasias - Achondroplasia, spondyloepiphyseal dysplasia, diastrophic

dwarfism, mucopolysaccharidoses

o Rheumatoid

o Metabolic - Rickets, juvenile osteoporosis, Osteogenesis imperfecta

o Soft tissue - burns, postempyema

Functional - postural, leg length, muscle spasm, Hysterical

Assessment:Clinical

Range of motion and spine flexibility should be assessed.

With the patient standing a plumb line dropped from the spinous process of C7 should fall

in the gluteal cleft. The distance from this line to the gluteal cleft is a measure of

compensation.

Adam's Test/Position - patient bends forward. A 'rib hump' will appear with a structural

scoliosis on the side of the curve convexity.

With the patient sitting & facing away from examiner, pelvic obliquity should be assessed

(for structural vs. functional curve)

Neurological examination is essential, with attention to sexual development and

associated congenital anomalies.


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With congenital spinal deformity attention should be paid to the cardiac, neurological and

genitourinary systems.

Radiological:

Standing PA film of whole spine on one film

Lateral bend films - Supine with maximum voluntary lateral bend;

Determines flexibility.

Differentiates structural from compensatory curves.

Indicated in preoperative evaluation for Double curve, Low curve to see if L4 corrects.

Lateral films Standing - To measure kyphosis and lordosis

Cobb angle - involves drawing perpendicular lines through the end plates of the most tiltedvertebrae of the curve and measuring the angle of the intercept. These vertebrae are known asthe end-vertebrae, while the vertebra at the centre of the curve is the apical vertebra. Linedrawn along upper end plate of upper end vertebra and lower end plate of lower endvertebra. Perpendiculars drawn from these lines. Angle of intersection measured. For doublecurve, one vertebra is upper end vertebra for lower end curve and lower end vertebra for uppercurve (transitional curve). Only one line drawn on this vertebra

End vertebra = most tilted

Apical vertebra = at centre of curve

Stable vertebra = bisected by mid-sacral line

Neutral Vertebra = see both pedicles equally


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Rib-vertebral Angle Difference (RVAD) of Mehta - is the difference between the angle formed

by a vertical line through the centre of the apical vertebral body on an AP film and the rib on the

convex side and the same angle on the concave side. RVAD of more than 200 or overlap of the

head of the rib over the vertebra are associated with a high likelihood of progression.
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Risser's staging - Indicates skeletal maturity and physiological ageBased on ossification of the iliac crest apophysis & graded 0-5 from anterior to posterior.

Mohr's Method - measures the amount of rotation at the apexof the curve

A painful functional scoliosis may require a bone scan to exclude tumouror infection

IDIOPATHIC SCOLIOSIS

Aetiology

Endocrine system - Patients with idiopathic scoliosis often tallerPostural equilibrium - Abnormalities in the vestibular system in the brain stem in scoliotics havebeen demonstrated

Neurotransmitter :No specific neurotransmitter defect identified

Genetics - Increased incidence in affected relatives found, Mother and father - 80%, Mother andsister - 20%, Mother - 10%, Sister - 3%. Indicative of multifactorial mode of inheritance
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Pathogenesis

Lordosis may be the biomechanical initiator of deformity. Thoracic lordosis lies in front of normal

axis of rotation. This causes rotation of lordotic section in flexion. Changes of vertebral shape are

effects secondary to rotation of lordosis

Right-sided prevalence explained by: Normal asymmetry of spine to right identified. Probably due

to descending aorta on left.

Increased incidence in girls explained by Normal flattening of thoracic kyphosis at age 12, which

corresponds to female growth spurt

Pathology

Intervertebral disc- Decrease in glycosoaminoglycan content in nucleus pulposis with

increase in collagen content found

Vertebral body - structures on concave side are hypoplastic. Structures on convex side

hypertrophied

Paravertebral musculature- Differences in muscle fibres on either side of curve

Ligaments and tendons -Collagen metabolism found to be normal. Posterior longitudinal

ligament thickened

Classification - Classified according to time of onset (old SRS)

Infantile- Curve occurs between birth and age 3

Juvenile - Curve occurs between age 3 and onset of puberty (about age 10)

Adolescent - Curve occurs between onset of puberty (about age 10) and cessation

of skeletal growth (about age20)

Alternative classification proposed =

Early onset Curve occurs before age 5 yrs

Late onset Curve occurs after age 5 yrs


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Various curve patterns (in decreasing incidence):

Right thoracic

Double major - right thoracic and left lumbar

Thoracolumbar Double major - right thoracic and left thoracic

Left lumbar

Natural history

Progression is signified by an increase in the curvature (Difficult to accurately quantify). Cobb's

angle lacks exact precision (variations of +/- 3o between examiners).

Progression is defined as:

Two sequential x-rays showing more than 5oof change. Not all curves progress. The larger the

curve at presentation the more likely it is to progress.

Most likely to progress:

1. Female sex

2. Young age at Diagnosis

3. Skeletally immature - Risser < 2 - Risk at or before Risser 2 - 50%, Risk after Risser 2

-


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70% of curves progress after skeletal maturity & progress an average of 20o . Curves less than

30o tend not to progress, Curves of 50-75o usually progress, Esp. Thoracic curves by a rate of

1o per year

Untreated scoliosis can lead to:

Back pain

Cardiopulmonary dysfunction: Respiratory function is reduced by: Nil with curve < 60o ;

1/3 with curve 60-100o ; 1/2 with curve > 100o

Mortality - Only increased if curve >100o

Cosmesis

Examination

Assessment of back - Area of curve; Deviation of plumb line from C7 (cm) [Compensation];

Shoulder elevation (cm), scapular prominence; Flank prominence, asymmetrical loin creases;

Adam's forward bending test - Presence and height of rib hump (spirit level, cm) [Hump

corresponds to convexity of curve] Deviation to one side during bending; Angulation when viewed

from side; Neurological examination; Assessment of physical maturity; Signs of other conditions

(NB - cavovarus, skin)

20% of right curves have pathology, 80% of left curves have pathology

Indication for MRI:

Abnormal neurological exam

Presence of lower limb abnormalities; pes cavus or cavovarus

If Apical kyphosis is seen

Structural abnormalities on plain films

Left thoracic/thoraco lumbar curve

Painful scoliosis

Rapid curve progression

Associated syndromes

Juvenile onset scoliosis

Excessive kyphosis


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Classification -King-Moe

Type I - lumbar dominant (10%) - S-shaped curve, Both thoracic and lumbar curves cross

midline, Lumbar curve larger or more rigid

Type II - thoracic dominant (33%) - S-shaped curve, Both thoracic and lumbar curves

cross midline, Thoracic curve larger or more rigid

Type III - thoracic (33%) - Thoracic curve, Lumbar curve does not cross midline

Type IV - long thoracic (10%) - Long thoracic curve, L5 over sacrum, L4 tilted into curve

Type V - double thoracic (10%) - Double thoracic curve, T1 tilted into upper curve, Upper

curve structural

Treatment

Depends on

Magnitude of curve

Growth potential - Rapid growth precedes menarche (girls) and axillary hair (boys) and

Risser 2. Decreased growth occurs after this
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Curve treatment:

rapid growth decreased growth

45o surgical surgical or observation

Observation

Review every 3-6 months while significant growth potential. Frequency depends on

magnitude of curve

Non-surgical treatment

Indications: Still growing, Risser 0, 1 or 2, Around onset of menarche (girls) or axillary hair (boys),

Add bone age if unclear, Curve 25o to 45o or30o + documented progression

Milwaukee brace - If apex of curve is above T8. Standard orthosis foradolescent thoracic

idiopathic scoliosis. Provides passive correction by pressure on convex side and active

correction by muscle contraction pulling body away from pads. Patient not weaned into brace.

Seen after 2-3 weeks for adjustment, Then seen every 3-6 months. Brace adjusted. X-ray taken

to assess response, in brace, to assess progression. Brace worn 23 hours a day. Allowed out to

play sport. If curve progresses beyond 45o, surgery indicated. Weaning commenced once skeletal

maturity reached

Underarm orthoses - Thoracolumbar spinal orthosis (TLSO) [Boston]- If apex of curve belowT8

Surgical treatment

Indications

Curve > 40-45o with documented progression

Low lumbar curve

Adolescent or young adult

Idiopathic thoracic curve

Goals


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Reduction of rib hump

Correction of rotation

Achievement of rigid fixation to obtain solid fusion

Selection of fusion area

Must fuse structural curve and not compensatory curve (non-structural) - decide on

bending films

Must not fuse less than the measured curve and usually more

Avoid fusion to L5 (L4 or sacrum)

Avoid fusion above T1

Center lower end of fusion on vertical line from center of S1

Fuse down to Neutral vertebra (pedicles symmetrical on PA film) & Stable vertebra (one

bisected by vertical line through sacrum in level pelvis)

Fuse level above and level below measured curve

Posterior

Surgical principles - Complete facet joint excision on both sides (convex and concave).

Replacement of facet joint area by autogenous bone. Complete decortication of all

exposed laminae and transverse processes. Addition of extra autogenous graft from iliac

crest.

Instrumentation -

Harrington instrumentation was standard for scoliosis surgery. No longer used.

Luque instrumentation - L-shaped rods and sublaminar wires. First segmental fixation

system. Can preserve and improve sagittal curves. Significant drawbacks with wires, esp.

Neurological complications

Winsconsin instrumentation. No longer used

Cotrel-Dubousset instrumentation - Most recent development. Scoliosis corrected by

combination of initial distraction and subsequent rotation. Advantages = Rigid fixation so

no postoperative support necessary, Greater correction achieved (50%-75%), Rotational

deformity corrected so rib hump addressed, Allows preservation and recreation of sagittal

contour, Versatile because pedicle fixation possible. Disadvantages = Technically more


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difficult, Increased risk of neurological complications, Implants large and may be

palpable, Very expensive.

Anterior

Indications - Lumbar or thoracolumbar curves (because fewer motion segments required

for anterior & avoids Crankshaft Phenomena), To achieve mobility, Rigid curve, To

achieve growth arrest, Skeletally immature patient, To supplement anterior fusion,

Neuromuscular curve

Advantages - Less levels instrumented. Better rotational correction

Disadvantages - Requires anterior approach, Does not produce lumbar lordosis, Can be

overcome with allograft shape

Instrumentation - Dwyer system was first system used. Largely replaced by Zielke system

Early complications

Neurological injury during surgery. Wake-up test often used

Blood loss - Risk of transmission of disease with transfusion

Wound infection

Pneumothorax

Dural tear - During ligamentum flavum removal or hook or wire insertion

Abnormal sagittal alignment

Incorrect fusion levels

Inappropriate ADH secretion - Postoperative SIADH; If not diagnosed, iatrogenic fluid

overload and even death may occur

Late complications

Pseudarthrosis - Solid fusion should occur by 6 months Rod or wire breakage - Due to pseudarthrosis or fatigue failure. If pain persists or

correction lost, fixation must be removed

Back pain - Appears to be due to Fusion below L4 , Loss of lumbar lordosis
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CONGENITAL SCOLIOSIS

Congenital scoliosis is a developmental curvature of the spine caused by vertebral anomalies that

develop in the embryonic period. Although present

at birth, clinical deformity may only become evident with growth

Classification:

Failure of Formation - Partial Unilateral (wedge vertebra), Complete Unilateral

(Hemivertebra)

Failure of Segmentation - Unilateral (Unilat. unsegmented bar), Bilateral (Block vertebra)

Mixed

The unilateral bar with contralateral hemivertebrae must be recognized because this combination

leads to the most severe and rapidly progressive deformity of all types of

congenital scoliosis

Bilateral failure of segmentation results in block vertebra, which generally does not lead to

deformity with growth

The hemivertebra is a failure of formation in which one of the complements of the sclerotomal

tissue, which combine centrally to form the vertebral body, fails to develop normally

Types (most to least common):

Fully segmented nonincarcerated vertebra - there is a disk space above and below the

hemivertebra (fully segmented) and the hemivertebra is shifted laterally

Semisegmented vertebra

Nonsegmented, or incarcerated, hemivertebra, which is the least common, is in

alignment with the spine.

Management:

Before undertaking surgical correction of congenital scoliosis, magnetic resonance imaging

(MRI) or myelography should be performed to see if there are any associated intraspinal

anomalies. Should an anomaly, such as diastematomyelia, be discovered, it should be resected

before correction of the scoliotic curve


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Management options:

Anterior and posterior spinal fusion alone may be done to control progression of the

spinal deformity.

Hemiepiphysiodesis, in which one third to one half of the vertebral end plates areremoved along the convexity of the curve anteriorly and fusion performed

Hemivertebra excision. This is the procedure of choice for a lumbar hemivertebra,

particularly at the L5 level, due to its significant potential for truncal imbalance

Crankshaft Phenomenon:

in skeletally immature pt, isolated posterior arthrodesis with instrumentation of a

lordotic curve may act as a posterior tethering bar, producing lordosis & bending of

the fusion mass as the unfused anterior vertebral bodies continue to grow; - in the study

by MHH Noordeen et al 1999, it was found that there was significant growth plateactivity in patients in Risser stage 4, which makes it unlikely that the crankshaft

phenomenon is caused soley by end plate activity; - risk factors: - open triradiate

cartilages: - with a closed triradiate cartilage, there should be less than a 5% chance ofdeveloping crankshaft syndrome; - physiologic youth: - girls younger than 11 years; -

boys younger than 13 years; - Risser grade 0 or 1; - potential risk of crankshaft

phenomena appears highest in children with normal growth potential of anterior vertebral

body growth plates, such as may be in children with juvenile scoliosis; - patients withcongential scoliosis may have a reduced risk for the crankshaft phenomena due to

abnormal anterior growth plates; - Radiographs: - crankshaft phenomenon is evident

with more than 10 deg of progression of the Cobb angle or the rib-vertebral angle

(assuming that other causes of curve progression such as pseudoarthrosis is not present); -rib-vertebral angle difference is most sensitive; - Prevention: - to prevent occurance of

this deformity, anterior and posterior arthrodesis should be performed in physiologicallyyoung patients with progressive congenital scoliosis > 50 deg;

Klippel-Feil Syndrome

= Multiple fused cervical segments due to failure of segmentation of cervical somites at 3-8wgestation.

Associated with: congenital scoliosis, renal aplasia, Sprengel's deformity, congenital heartdisease, brain stem abnormalities.

Triad= low posterior hairline + short web neck + limited cervical ROM. (seen in


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BACK PAIN IN A CHILD

Differential Diagnosis:

1. Herniated Disc in the Child

2. Osteomyelitis of the Spine

3. Tuberculous Spondylitis4. DISCITIS5. Spinal Cord Tumors

6. Primary Bone Tumors - eosinophilic granuloma ; ewing's sarcoma ; metastaticneuroblastoma

7. Slipped Vertebral Apophysis:

8. Spondylothisthesis

9. Kyphosis - Scheuermann's disease is the most common cause of pain in the thoracic andthoracolumbar regions of the back;

10. Klippel-Feil syndrome: - pain is usually due to hypermobility or instability of adjacentvertebral segment or to degenerative osteoarthrosis;

11. Diastematomyelia: - frequently associated with a cutaneous malformation overlyingdefect, is more likely to present with neurological abnormalities involving lowerextremities, such as unilateral cavus foot or calf atrophy, rather than with back pain;

SPONDYLOLYSIS & SPONDYLOLISTHESIS

SPONDYLOLYSIS

caused by a defect in the pars interarticularis

usually a fatigue fracture from repetitive hyperextension stresses (gymnasts)

most common cause of LBP in adolescents

Radiology

o plain x-rays demonstrate 80% of lesions

o oblique views - additional 15% picked up - 'Scottie dog' sign (Lachapelle)

CT - may miss fracture

Bone Scan - incr. uptake indicates an acute lesion which will probably heal

Non-union is common

SPONDYLOLISTHESIS

"spondy" refers to the vertebrae and "listhesis" means "to slip"

usually L4/5 and L5/S1

Normally laminae & facets constitute a locking mechanism preventing forward slippage

Classification: (Newman, Wiltse, McNab)
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Congenital/Dysplastic (20%)superior articular facets are congenitally defective

associated with spina bifida occulta

Isthmic (50%)

caused by spondylosis (L5/S1)

a. Lyticfatigue fracture of the pars interarticularisb. Elongated but intact pars interarticularis

c. Acute fracture of the pars interarticularis

Degenerative (25%) degenerate facet joints (L4/L5)

Post-traumaticfrom an acute fracture in some other portion of the vertebra that allows a slip t

An isolated pars fracture is not seen with this lesion.

Pathological tumours, Paget's

Post-operative

Severity of Slip:

1. Percentage of slip of the AP diameter of the vertebra below:

Grade I - < 25%

Grade II - 25-50%

Grade III - 50-75%

Grade IV - 75-100%

Grade V - >100% (spondyloptosis)

2. Slip Angle

normal = > 0 degrees

Clinical
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may be discovered only incidentally on x-rays

injury may aggravate any symptoms

usually pain begins insidiously during the second or third decade with walking andstanding

Flattening of the back

Spinous process step-off

Claudication may signal the development of lateral stenosis

Radiology

Plain x-rays should be taken standingo grade slip

o slip angle

Oblique x-rays

Bone scans - (see above)

MRIo evaluates disc

o but can see a pseudodisc herniation due to rotatory element of slip

Treatment

Non-operative:

reduce sports

adolescents - x-rays every 6 months until maturity

Risk factors for Slip progression:

1. young age at presentation2. females

3. slip angle > -10 degrees4. high grade slip5. dome shaped sacrum6. inclined sacrum (>30deg. beyond vertical)

Operative:

Indications:o slip > 50% or progressing in adolescents

o back and/or leg pain unresponsive to non-operative treatment

o functionally significant neurological deficit

Grade I & II - in situ fusion (& decompression);o Repair of the pars defect & fixation using a lag screw or wires has been

described for low grade slips (


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paediatric spinal deformities 2

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