Spine

Congenital Kyphosis

Definition

 

Kyphosis due to congenitally anomalous vertebrae

- characterized by severe angular deformity

- prominent gibbus at the apex of the curve

 

Classification

 

Type I - Failure of formation

 

Failure formation anteriorly + preservation of posterior elements

- hemivertebrae / wedge vertebrae

- most common

- produces the worst deformity

- NHx is one of relentless progression

- 7per year

- most likely to result in paraplegia

 

Congenital Kyphosis XrayCongenital Kyphosis CT

 

Type II - Failure of segmentation

 

Anterior unsegmented bar

- better prognosis

- produces more rounded kyphosis

- deformity progresses more slowly

- paraplegia is uncommon

 

Type III

 

Mixed pattern

 

Clinical Features

 

Severe deformity maybe obvious at birth

- less obvious deformities may not appear until later

- may be accentuated during adolescent growth spurt

 

Progression occurs to end of growth & often after growth complete

 

Mechanism

 

Due to

- growth differential

- erosion of vertebral body from mechanical pressure

 

Issues

 

Deformity can become very severe (Type I)

- breakdown of the overlying soft tissues

- compression of abdominal viscera

- impairment of pulmonary function

 

Paraplegia may occur (Type I)

 

Management

 

Non operative not effective

 

Work up

 

MRI / Neurosurgery

- exclude intraspinal pathology

 

Echocardiogram

- cardiac abnormality

 

Abdominal US

- renal abnormality

 

Operative

 

Type I

 

Ideal treatment is early detection & early posterior fusion

- best results if fusion by age 3

- can be done as early as 6/12

 

A.  < 5 years & curve < 50o

- posterior spinal fusion

 

B.  > 5 years and > 500

- anterior and posterior fusion

 

C.  Neurology

- anterior decompression first

 

Congenital Kyphosis CTCongenital Kyphosis Correction Lateral

 

Type II 

 

If early, can treat with anterior osteotomy of unsegmented bar

- i.e. epiphyseolysis

 

If late, requires posterior fusion

 

 

 

 

 

Neck

Atlanto-axial Rotational Instability

Pathology

 

Paediatric populations predisposed

- due to lateral mass anatomy and ligamentous laxity

- both alar ligaments and facet capsules must be torn to occur

 

Aetiology

 

Due to forced rotation and lateral tilt

Can be spontaneous

 

Presentation

 

Most patients have torticollis and limited rotation

 

Cock robin position 

- lateral flexion and rotation to other side

 

Fielding Classification

 

1. Rotatory fixation without anterior displacement

 

2. Rotatory fixation with anterior displacement 3 - 5 mm

 

3. Rotatory fixation with anterior displacement > 5mm

- indicates disruption of both facet joints and transverse ligament

 

4. Rotatory fixation with posterior displacement

 

Associations

 

Downs

RA

Klippel Feil

Morquio

SED

Achondroplasia

 

DDx

 

Torticollis

Atlantoaxial rotatory fixation

Ondontoid fracture

Os Ondontoid

 

Grisel's syndrome

 

Pyogenic atlanto-axial subluxation

- after upper respiratory tract infection

- inflammation induced ligamentous laxity

- can be post tonsillectomy

 

Present with painful torticollis and limited rotation

 

Xray

 

Widened atlanto - lateral mass interval

 

Dynamic CT

 

Head turned to left then to right

- demonstrate fixed subluxation

 

Management

 

Atlanto-axial instability

 

1.  Early presentation

- i.e. first day or two

- trial soft collar / analgesics

- see 1 week later

 

2.  Reduce any anterior displacement with halter traction

- add sequential weight

- check X-ray to ensure no C0/1 displacement

- valium and Phenergan

- if successful apply HTB

- flexion extension views out of HTB for residual instability

 

3.  Failure halter traction / Trial skeletal traction

 

4.  Failure skeletal traction / Open reduction and fusion

 

Residual instability

 

C1/2 Gallie fusion

 

Chronic

 

> 3/12

- consider fusion in situ

 

Grisel's

 

Usually will reduce spontaneously

 

First week

- NSAIDS and hard collar

 

Failure or > 1 week symptoms

- soft halter traction

 

> 4 weeks

- skeletal traction and HTB once reduced

 

 

Basilar Invagination

Definition

 

Cranial prominence of odontoid into region of foramen magnum

- upper cervical spine encroaches on brain stem

 

Aetiology

 

Congenital syndromes

- Klippel Feil Syndrome

- Achondroplasia

- OI

- Downs 

- Morquio

- SED

- NF

 

Congenital abnormalities

- atlas hypoplasia

- bifid posterior arch atlas

- occipitocervical synostosis

 

Acquired

- JRA

- Rickets

- RA

 

Clinical Features

 

Become symptomatic in third decade

- neurological compression 15%

 

Xray

 

McRae's line 

- line along foramen magnum 

- Basion to Opisthion

- dens above line = basilar invagination

 

MacGregor' s Line

- hard palate to inferior aspect occiput

- < 4.5mm above line

 

Ranawat Criterion

- distance between centre of C2 pedicle & transverse axis of C1

- men < 15mm

- women < 13mm

 

MRI 

 

Delineate pathology

 

Management

 

Consider surgery if symptomatic

 

Indications

 

1.  Severe pain

 

2.  Neurology

 

3.  Progression

- basilar invagination > 5mm

 

Technique

 

Surgery complex & fraught with complications

 

1.  Fusion

- Gallie C1/2 fusion if reducible

- occipitocervical fusion if not with C1 laminectomy

 

2. Excision of odontoid

 

 

 

 

Congenital Muscular Torticollis

Definition

 

Twisted / Wry neck secondary to fibromatosis in sternocleidomastoid

 

Epidemiology

 

Packaging defect

- commonest first born 

- 75% on right

 

Associations

 

CDH 20%

Metatarsus adductus 15%

Breech presentation

Klippel - Feil Syndrome

Arthrogryposis

 

Aetiology

 

Fibrosis of SCM on one side

Fails to grow & causes progressive deformity

 

Pathogenesis

 

Unknown

 

Theories

 

1. Ischaemia secondary to position in utero 

- compartment syndrome SCM

 

2. Birth injury with haemorrhage

 

Natural History

 

Many resolve spontaneously

 

However if untreated get permanent facial asymmetry

 

Clinically

 

Lump may be noticed in first few weeks of life 

- often disappears

 

Head tilted to one side so ear approaches shoulder

 

Head turned towards other shoulder

 

Associated facial asymmetry

 

DDx

 

Primary

 

Congenital fibrosis SCM

 

Congenital vertebral anomaly

- Klippel Feil

- os ondontoid

- C1-2 fusion

- unilateral C1 deficiency

- many others

 

Secondary

 

Trauma

- atlantoaxial rotatory subluxation

- # C1 /2

 

Grisel's syndrome

Ocular dysfunction

Infection / Discitis

SCM scar / tumour

HNP

 

X-ray 

 

To exclude congenital vertebral anomaly

- 17 cases of unilateral C1 deficiency with wry neck in literature

 

Indicated with failure non operative management

 

Management

 

Non operative

 

Stretching exercises

 

90% successful

 

Techniques

 

1.  Parents taught to carry child with their arm under flexed side of neck 

- stretches SCM whilst carrying

 

2.  Passive stretching exercises

- lateral head bend away from affected side

- head rotation towards affected side

- 90% success 

 

Operative

 

Indications

 

If persists past 1 year age chance of resolution very poor

- operate especially if > 30o limitation of movement

 

Timing

 

Ling et al Clin Orthop 1976

- 103 operations

- high rate scar tethering if released <1 year old

 

Options

 

1.  Distal release first

- if not successful proximal release also

 

2.  Distal and proximal release

- often at age 4-6

- expose both and mark with sling (more difficult to isolate after one end divided)

- complete release both

 

Technique

 

Distal release 

- 5cm transverse incision 1cm above medial end clavicle

- incise tendon sheath

- draw tendons out (NB sternal and clavicular heads)

- divide / Z plasty / excise 2 cm of both tendons 

- explore wound digitally for any fibrous bands and divide these

- if release incomplete perform proximal release

 

Proximal release

- incision immediately behind & below ear

- divide SCM transversely just distal to tip of mastoid process

- NB spinal accessory nerve at risk

 

Post operative

- manual stretching 3x /d starts at 1 week post-op

 

 

 

Congenital Odontoid Abnormalities

Ossification

 

Atlas

 

Three 1° Ossification centres

- two lateral masses with posterior arch in between

- anterior arch which appears at 1 year

- posterior arch fuses at 4 years

- anterior & posterior fuses to body at 7 years

 

Axis

 

Five 1° Ossification centres

- two neural arches / lateral masses

- two 1/2 dens

- body

 

Five 2° Centres

- Os Terminale (Appears 3, fuses 12)

- inferior ring apophysis

- 2 transverse processes

- 1 spinous process

 

Body of odontoid fuses by birth from two centres

- failure = Dens Bicornis

 

Dens separated from body by Dentocentral Synchondrosis

- present at birth

- fuses by 6 years

 

Tip of Odontoid not ossified at birth

- ossifies by age 3

- fuses by age 12

- Ossicum Terminale Persistens if doesn't fuse

 

Deformities

 

1. Os Odontoideum

- failure of fusion of base of odontoid

- normally fuses by age 6

- appears like Type II fracture

 

2. Odontoid Hypoplasia

- congenitally small odontoid

- asssociated with Morquio Syndrome & SED

- can get AAI 2° Hypoplasia 

 

3. Odontoid Aplasia

- absent odontoid

 

4. Os Terminale Persistens

- unfused terminal apophysis

- normally fuses by age 12

 

Os Ondontoideum

 

Theories

 

Congenital

 

Developmental

- ? os odontoideum is a traumatic event

 

Clinical Features

 

Usually asymptomatic

 

May present in adulthood with

- pain (Usually dull ache)

- neurologic symptoms

- Lhermitte sign / lightning pain

- long tract signs

- risk of sudden death

 

X-ray

 

Open-mouth view

 

Instability

- > 3.5 mm translation on FE views

- ADI > 5 mm

- SAC also important if < 14 mm

 

Surgical Indications

 

Non syndromic

 

1.  > 10 mm FE motion

2.  Painful neck with instability 

3.  Neurology

 

Prophylactic neck fusion is controversial

- if asymptomatic avoid contact sport

 

SED /  Morquio

 

ADI > 5mm = Fusion

 

Technique

 

Gallie Fusion

- involves using notched bone graft

- sitting it between the posterior arch of C1 and the spinous process and medial lamina arch of C2

- secured by sublaminar wires 

- this is not inherently rotationally stable

- usually requires halo immobilisation

 

 

 

 

 

 

 

Klippel Feil Syndrome

DefinitionKlippel Feil

 

Congenital synostosis of cervical vertebrae

- refers to patients with any element of cervical spine fusion

- ranges from 2 vertebrae to whole spine

 

Classic triad 

 

1. Short neck

2. Low posterior hairline

3. Stiff neck

 

Triad present in <50%

 

Aetiology

 

Failure of normal segmentation of mesodermal somites of neck

- during 3rd to 8th week

- may be AD inheritance

 

Clinical

 

Neck

 

Stiff especially lateral movements

- usually hypermobile at unaffected levels

 

Torticollis

 

Short neck

 

Head arises from shoulders

 

Often associated with webbing of neck

 

Low posterior hairline

- on level with shoulders

 

Problems

 

Degenerative OA

- caused by hypermobility of adjacent segments

- leads to instability & degenerative OA

- presents 3rd decade

 

VECTRAL

- associated abnormalities

 

Other congenital cervical spine abnormality

- occiptiocervical synostosis

- basilar impression

- odontoid anomalies

 

Scoliosis

- 60%

- congenital type

- associated respiratory problems

- need to consider neck problems when treating scoliosis

 

Sprengel's Deformity

- 30%

- same insult that causes KF halts descent of scapulae

 

Renal

- 35%

- renal failure often occurs early

 

Deafness

- 30%

- affects development of speech & language

 

Synkinesia

- 20%

- involve paired movements of hands

- abnormal pyramidal tracts

- usually resolves

 

Cardiac

- 15%

- various congenital defects

- need preoperative investigations / echo

 

Xray

 

Vertebral fusions

 

Klippel Feil Xray

 

Thoracic abnormalities

- scoliosis

- kyphosis

 

CT scan

 

Body fusion / block vertebrae

Hemivertebrae

Flattening & widening bodies

Hypoplasic discs

Cervical spina bifida

 

Klippel Feil CT SagittalKlippel Feil CT Coronal

 

Management

 

1.  Manage associated conditions

 

Torticollis

Scoliosis 

 

2.  Neck Deformity

 

Little can be done for neck deformity

 

Avoid contact sports as sudden death & neurology reported 

- worse if small mobile segment adjacent to long fused segment

 

 

 

Paediatric Back Pain

DDx

 

Spondylosis / spondylolisthesis

- most common cause

- adolescent playing sport

 

Scheuermann kyphosis
- 1/3 of patients

 

HNP - rare

Fracture

 

Apophyseal ring fracture

- traumatic fracture between vertebral body and cartilaginous ring

- apophysis displaced posteriorly with disc

- decompression if causes neurological symptoms

 

Discitis / Osteomyelitis

 

Tumour

- benign: OO / OB / ABC / Giant cell / EG

- malignant: Ewings / OS / Chordoma / Leukaemia

 

RA

Diastematomyelia

Juvenile Osteoporosis

 

Clinical

 

Pain character

- aspirin relief / night pain

- osteoid osteoma

 

Associated neurology & deformity

 

Systemic features / Red flag signs

- fever / weight Loss

 

X-ray

 

Standing PA & Lateral

 

CT

 

For spondylithesis

 

MRI

 

Low threshold

- may need GA

 

Bloods

 

WCC / ESR / CRP

RF, ANA, HLA B27

 

 

 

 

Paediatric Discitis

Aetiology

 

1.  Infection

- usually haematogenous 

- some direct spread from vertebral osteomyelitis

- usually frankly infective with marked vertebral body end plate changes

- elevated temperature & ESR

 

2.  Inflammatory 

- probable auto-immune picture with minimal bone changes

- no need for antibiotics

 

Anatomy 

 

Blood supply

- nucleus pulposis never has blood supply

- annulus fibrosis has blood supply until age 20

- primary infection possible in infants

 

Natural History

 

60% resolve

20% auto-fuse

20% chronic pain

 

Presentation

 

1-12 years / symptoms age dependent

 

0 - 3

- refusal to walk / weight bear / limp

- irritability

 

3 - 9

- abdominal pain

 

> 9

- back pain

 

Also

- stiff flexed spine 

- tight hamstrings & decreased SLR

- scoliosis

 

Microbiology

 

50% positive blood culture

66% positive CT guided biopsy

- usually not required

 

Types

- Staph 60-70%

- Streptococcus

- Gonococcus > 12 years

- E Coli in neonates

- Atypicals (TB, Brucellosis)

 

X-ray

 

Initial xray normal

 

Later

- loss of disc height

- end plate irregularity & sclerosis

- disc can regain height, but endplate changes remain

 

MRI

 

Child may need sedation / GA

 

DDx

 

Tumour

- leukaemia, metastasis (vertebral)

- EG (vertebra planar)

- OO, OB

 

Epidural abscess

Paraspinal abscess

SI joint septic arthritis

 

Management

 

Antibiotics

 

Controversial as whether to treat with antibiotics or not

- most authors agree that there is a bacterial component to the process

- most recover with or without antibiotics

 

Appropriate ABx (broad spectrum)

- bed rest

- brace

 

Usually rapid response

- CT guided biopsy if fails to resolve with above 

 

Results

 

Kayser et al Spine 2005

- 25 patients with spondylodiscitis

- most had long delays to treatment due to non specific symptoms

- inflammatory markers usually only mildly elevated

- 48% had evidence of vertebral body destruction

- 60% healed with disc narrowing, 40% with fusion

 

Garron et al J Paediatr Orthop 2002

- 35 needle aspirations of the disc

- 55% Staph

- 27% Kingella Kingae

 

 

 

 

 

 

 

Scoliosis

Congenital Scoliosis

Definition

 

Lateral curvature of the spine 2° to vertebral anomaly

- causes an imbalance in longitudinal spine growth

 

Epidemiology

 

True incidence unknown

 

F > M

 

Typically Thoracic

 

Inheritance

 

No association in twins / suggests not inherited

5% risk in family if complex multi-level

 

Aetiology

 

Occurs during mesenchymal development 4-8/52  

- unknown foetal insult

- high incidence other anomalies 

- VECTRAL"

 

V vertebral and spinal abnormalities

E endocrine

C cardiac

T tracheo-esophageal fistulas

R renal

R radial club hand

A anus imperforate/ urogenital (cloacae abn)

L limb Sprengel shoulder, club hand, CTEV

S single umbilical artery

 

Associations

 

Referrals

- neurosurgery

- cardiology

- urology

 

Spinal Dysraphism (25%)

- failure of neural tube closure

- diastematomyelia , syrinx, tethered cord, arnold chiari malformation, fibrous dural bands, intradural lipoma, SB

- clinically associated with hair patches, dimples, lipomas, tails

- all should have MRI especially presurgery

- can have rib abnormalities / fusions

- can have posterior element abnormalities

 

Genitourinary 25%

- 6% have obstructive uropathy

- horse shoe kidneys

- lifethreatening

- all have ultrasounds +/- urology opinion

 

Cardiac 10%

- all murmurs should be investigated

- ASD, VSD, PDA, Tetralogy Fallot

- all patients have echo prior to surgery

 

Limbs

- radial club hand

- congenital amputations

- cavovarus foot / CTEV

 

Klippel-Feil 25%

 

Classification 

 

International  Spine Society / MacEwen

- failure formation

- failure of segmentation

- mixed

- unclassifiable

 

Most common

- unilateral bar

- fully segmented hemivertebrae

- semi segmented hemivertebrae

 

Failure of Formation

 

A.  Partial / Wedge Vertebra (5%)

 

B.  Complete / Hemivertebra (30%)

 

Fully Segmented

- common

- open disc above & below

- greatest growth disturbance

 

Semi Segmented

- 1 growth plate (either above or below)

 

Non Segmented / Incarcerated

- no disc above or below

- minimal growth potential

 

Failure of segmentation

 

A.  Unilateral / Unilateral Unsegmented Bar (50%)

 

B.  Bilateral / Block Vertebra (5%)

 

Mixed

 

Combination both / Commonest

 

Unclassifiable (20%)

 

NHx

 

Anomaly present at birth but may not become evident until later life

 

Diagnosed < 3 or 9-14 years old

 

Associated with most rapid growth periods for the spine

- in utero

- from birth to 5 years

- puberty

 

Progression

 

Progression occurs if differential growth

- if more physis on one side 

- count number of physes

 

25% show no progression

 

50% progress rapidly

 

25% progress slowly

 

Prognostic Features

 

Type

 

Age

- worst prognosis with clinical deformity in first year of life

 

Location

- higher in the spine, less likely to progress

- upper thoracic curve best

- lumbar worst

 

Worst  10° / year

 

Unilateral unsegmented bar + fully segmented contralateral hemivertebrae

- over 14 years can get 140 - 180o curve

 

Intermediate 5˚

 

Unilateral unsegmented bar 

 

Fully segmented hemivertebra

- most common

- usually a problem

- 2 growth plates

- 2 consecutive hemivertebra a problem

 

Semisegmented hemivertebrae

- second most common

- single growth plate

- curve at maturity usually < 40o

 

Consecutive Hemivertebrae

 

Best < 2˚

 

Block vertebra best

- bilateral growth impairment

 

Non segmented / incarcerated hemivertebrae

- may produce slowly progressive curve

 

Unpredictable

 

Single hemivertebra unpredictable

- act as enlarging wedge

- often small with limited growth potential (incarcerated)

 

Xray

 

Lesion often difficult to interpret

 

See fused ribs

 

3D CT 

 

Excellent guide to anatomy

 

MRI

 

Should be performed before operative intervention

- assess for vertebral abnormality (40%)

- exclude Diastematomyelia / Tethered cord / Syrinx / Arnold Chiari Malformation

 

Other

 

Renal ultrasound

Echo

 

Management

 

Key is identification of curves likely to progress to prevent severe deforrmity

 

Non-operative

 

Bracing not indicated

 

Observe each 6/12

- look for progression

- examine for neurology

- continue until skeletal maturity

 

Operative

 

Indications

- progressive > 4-6o per year

- >40°

- high likelihood severe deformity i.e. unilateral unsegmented bar

- unbalanced child e.g. L5 hemivertebrae

 

Options

- fusions (posterior, anterior/posterior)

- hemi-epiphysiodesis

- hemivertebrae excision

- corrective osteotomy + instrumentation

- growing rods

- expansion thoracoplasty

 

Growth Inhibition

 

Each vertebrae contributes 1 cm in height in normal spine

 

Not true in abnormal spine

- delaying fusion actually makes patient shorter due to increasing deformity

- best to operate early

- unbalanced growth centres don't contribute to vertical height

 

Principle 

 

Fully segmented hemivertebrae

 

1.  Stop growth on affected side

- segments above & below fused unilaterally on side of hemivertebra

- hemi-epiphyseodesis performed anterior & posterior

- effectively produce contralateral unsegmented bar above & below

 

2.  Excision of hemivertebrae

- more common

- especially at lumbosacral junction

 

Unilateral bar

 

Stop growth on other side

- hemi-epiphyseodesis anterior / posterior on  convex side 

- < 5 years

- segment effectively converted to block vertebra

 

Other options

 

Growing Rods

 

For patient < 5

- long segment involved

 

Expansion Thoracoplasty

 

Fused ribs common

- excise fusion

- vertical expandable titanium rib

- allows chest expansion and normal development of ribs

 

 

Idiopathic Scoliosis

Adolescent Idiopathic Assessment

DefinitionScoliosis Thoracic Major

 

Lateral curvature of the spine with vertebral rotation

- defined as > 10o coronal plane deformity

- occurs at or near the onset of puberty 

- no cause is established

 

Planes of Deformity

 

Triplanar

- coronal / scoliosis

- sagittal / thoracic lordosis

- transverse / rotational

 

General Categories

 

Structural

 

Fixed lateral curvature with rotational deformity

- intrinsic anatomical change

 

1.  Idiopathic 75%

2.  Neuromuscular 10%

3.  Congenital 10%

4.  Other 5%

 

Non structural

 

Reversible, non rotational and disappears with sitting

- nil intrinsic anatomical change

 

Compensatory / Hysterical / Irritative / Postural / Sciatic

 

Incidence

 

< 10o - 2.5 %

> 30o 4 / 1000 

> 40o 1/1000

 

Gender

- little difference overall

- females more likely to have larger curves

- females more likely to progress

 

Classification

 

Scoliosis Research Society (SRS)

 

Infantile: 0-3 years onset 

 

Juvenile: 3-10 (Puberty)

 

Adolescent: 10 - Cessation of Growth (20 years)

 

Alternative

 

Early Onset - < 5 years 

- rare and severe

- male 2:1

- left sided

- if less than 1, 90% resolve

- >1, 20% resolve

- many other congenital anomalies

 

Late Onset  - > 5 years

- Adolescent Idiopathic

- females 6:1

- right thoracic

- nil associations

- FHx common

 

Aetiology

 

Structural Differences

 

Intervertebral Disc

- decreased GAG in Nucleus and increased collagen content 

 

Paravertebral Muscles 

- differences in muscle fibres on either side of curve 

- more type I fibres on the convex side of curve 

 

Ligaments and Tendons 

- PLL thickened 

 

Endocrine 

- patients with idiopathic scoliosis often taller 

- normal GH but altered Somatomedin levels - ? significance 

 

Vertebral Body

- structures on concave side hypoplastic 

- structures on convex side hyperplastic

- due to persistent asymmetrical loading

 

Postural Equilibrium

- abnormality in vestibular system in brainstem 

- scoliosis induced in bipedal rats with destruction of brainstem 

- not conclusive - ? effect rather than cause 

 

Neurotransmitter

 

Scoliosis produced when the pineal gland removed from chickens 

-  transmitter found to date - ? melatonin

 

Genetics 

 

Increased incidence in affected relatives 

 

Mother with scoliosis

- 10% chance for female child

 

Sister with scoliosis

- 20% chance for female child

 

Mother and father with scoliosis

- 80% chance for female child

 

Lordosis

 

Biomechanical initiator of the deformity

- thoracic lordosis normally lies in front of the normal axis of rotation

- causes the lumbar lordotic section to rotate in flexion 

- the tethering of the posterior elements (thickened PLL) also contributes to rotation in flexion

- explains the Crank Shaft Phenomena 

 

Adolescent Idiopathic Scoliosis 

 

Epidemiology

 

Prevalence dependant on the size of the curve 

 

As the curve increases in magnitude the female preponderance increases as well 

 

Overall is 3.6:1  F:M

 

Curve Patterns

 

There are five major curve patterns in decreasing order they are

 

Right Thoracic

Double major (Thoracic dominant)

Thoracolumbar 

Double major (Lumbar dominant)

Left Lumbar 

 

Progression 

 

Definition

 

Absolute increase in Cobb angle of 10o

- or 5o over two consecutive visits 

 

Remember the interobserver error of Cobb angles is +/- 4o

- can vary with the time of day (increases in the pm)

 

Factors related to progression

 

MR Sex MAP

 

Magnitude:  curve > 20o

Risser:  0 or 1

Sex:  Female

Menarche: premenarche

Age: < 12

Pattern:  Thoracic & double curves most

 

Growth Remaining

 

Menarche 

- 66% prior to and 33% after menarche 

- most growth is 1 year before and 1 year after menarche

- have on average 2 years growth left

- have passed PHV

 

PHV

- peak height velocity / most important factor

- adolescent growth spurt

- girls 8 cm / year

- boys 9.5 cm / year

- before menarche / at Risser 0 / open triradiate cartilage

- PHV generally over 2 years

 

Tanner sign 

I - Pre-pubertal 

II - Breast buds  - related to adolescent growth spurt

III- Pubic hair 

IV- Menarche 

 

Triradiate cartilage

- may be more sensitive in judging the maturity 

- Risser 0 + open triradiate cartilage indicates a lot of growth to go

- closes in the middle of the PHV

 

Risser sign 

 

Risser grade relies on ossification of the iliac apophysis from lateral to medial and is completed with maturity

- Grade 0 to 5 

- Grade 0 means no ossified apophysis present

- Grade 1 means appearance of apophysis laterally / after menarche

- Grade 5 is fusion of the apophyseal cap to ilium / little growth remaining / 14-16 Boys and 11-13 Girls

- can be difficult to distinguish between 0 and 5

 

Curve Pattern 

 

Double curves have higher incidence of progression than single curves

- single thoracic > single lumbar

- lumbar the least

 

Curve Progression Studies

 

1.  Lowenstein Study of Curve Progression

 

Looked at Risser sign + intial curve in regards to curve progression

 

Risser   0 - 1 2 - 5
Initial curve 0 - 19o 22% 2%
Initial curve 19 - 290 66% 22%

 

2.  Weinstein and Ponsetti 

 

Looked at the progression after maturity  / 30 year study

- curves less than 30o as rule DO NOT progress after maturity 

- 50 - 75o progressed most ( 1o/ year ) 

- this is the basis for surgery for curves 450 plus

 

Slowed over 100o with costopelvic impingement 

 

Findings

- mortality 2x expected

- high percentage disability pension

- none in heavy work

- nil increase incidence LBP

 

Natural History of Untreated Scoliosis

 

Back Pain 

 

Most studies suggest that the incidence of back pain is no higher than in general population 

 

Back pain seen in thoracolumbar or lumbar curves of > 45o

- particularly if large apical rotation or imbalance 

 

Pulmonary Function 

 

Affect on pulmonary function not seen until curves of 80o reached  

- restrictive pattern

- linear relationship between FVC and PaO2 and curve size 

- nil effect with curve < 60o

- 1/3 with curve 60-100o

- 1/2 with curve > 100o

 

Mortality

 

Nachemson 1968

- not increased until curve of 100o noted

 

Cor Pulmonale 

 

Seen at 40 and 50 years of age if curve > 80o

 

Assessment

 

History 

 

How detected 

Presence of progression 

Associated complaints 

- pain 

- neurological symptoms 

- respiratory symptoms 

 

Status of growth 

- growth spurt

- menarche

- changes in puberty 

 

Want to ensure is idiopathic

- normal delivery / normal milestones

- bladder troubles (NM)

- Marfinoid / OI / NF

 

Examination 

 

See the section on examination for detail

 

AP Film

 

Standing AP or PA films of whole spine including the iliac crests 

- PA has less radiation to ovaries and breasts 

- AP has less magnification 

 

1.  Neutral / end vertebrae

 

End vertebra is the last vertebra that tilts into the concavity of the curve

- when the end plates are parallel, the one furthest from the apex of the curve is the end vertebra

 

Scoliosis End Vertebrae

 

2.  Cobb Angle 

 

Detect the end vertebrae where the end plates are last to converge 

- line drawn along upper plate of the upper end vertebrae and lower plate of the lower end vertebrae 

- perpendiculars to these lines 

- intersection angle measured 

 

If double curve

- one vertebrae is upper end vertebrae for the lower curve 

- lower end vertebrae foe the upper curve 

 

Measurements all taken from same vertebrae in future 

 

Scoliosis Cobb Angle

 

3.  Identify Apical Vertebrae

 

In centre of curve

- furtherest from central sacral line

- not tilted / most horizontal

- maximum rotation

 

T10 above apical - Thoracic

T11 - L1 apical - Thoracolumbar

L2 down - Lumbar

 

4.  Stable Vertebrae

 

Central sacral line

- lowest vertebrae this bisects or

- line between 2 pedicles

- lowest vertebrae instrumented in surgery

 

Scoliosis Central Sacral Line

 

5.  Look at shoulders

 

Important in double thoracic major

- high structural thoracic curve

- if left shoulder high in right thoracic curve

- need to instrument to T2 to correct this

 

Lateral Films 

 

Standing 

- measure the kyphosis and lordosis via Cobb method

- important presurgery

- want to correct this intra-operatively

- usually need to recreate thoracic kyphosis

 

Lateral Bend Films 

 

Push prone

- supine with maximal voluntary bend 

- differentiates structural from compensatory curves 

 

MRI 

 

If suspect intraspinal pathology 

- Brain + 3 level spine / neurocentral

 

Indications for MRI

 

Scoliosis Left Lumbar Curve

 

Left sided 

Male 

Painful 

Rapidly progressive 

Neurological abnormality present 

 

Findings 

 

Right sided curve: 20% have pathology 

Left sided curve: 80% have pathology 

 

Assessment of Rotation 

 

Rib Hump / Scoliometer 

 

Adams forward bend test

< 5o tilt = < 30o rotation

> 7o tilt = > 30o rotation

 

Rotation of Pedicles

- indicates the structural curve

 

Classification

 

Lenke

 

3 areas of curve

- main thoracic: MT

- proximal thoracic: PT

- thoracolumbar / lumbar: TL/L

 

Assess

- curve location

- lumbar modifier

- thoracic sagittal profile

 

Type 1 Main Thoracic

- MT structural

- PT non structural

- TL/L non structural

 

Scoliosis Main Thoracic

 

Type 2 Double Thoracic

- MT and PT structural

- TL/L non structural

 

Type 3 Double Major

- MT and TL/L structural

- PT non structural

 

Type 4 Triple Major

- all 3 structural

 

Type 5 Thoracolumbar / Lumbar

- only TL/L

 

Scoliosis Lumbar

 

Type 6

- TL/L and MT structural

- TL > MT by more than 10o

 

King-Moe  Classification

Very poor inter observer reproducibility

 

Type I -  Lumbar Dominant Double T + L

- both the thoracic and lumbar curves cross the midline 

- lumbar curve larger and more rigid 

 

Type II - Thoracic  Dominant Double

- both the thoracic and lumbar curves cross the midline 

- thoracic curve larger and more rigid 

 

Type III -  Short Thoracic 

- thoracic curve 

- lumbar curve doesn't cross the midline 

- lumbar curve not structural

 

Type IV - Long thoracic 

- long thoracic curve extends to lumbar spine 

- L5 over the sacrum 

- L4 tilted into the curve (stable vertebrae)

 

Type V -  Double structural thoracic 

- double thoracic curve with L upper, R lower

- tilting of T1 into the upper curve / elevation of L first rib

- cervical extension

- compensatory lumbar curve with upper curve structural

 

 

 

Adolescent Idiopathic Management

Observation

 

Curves < 20o observation only at 3-6 month intervals depending on growth rate 

 

Non Operative Management / Bracing 

 

Never brace curves if patient Risser 4 or 5 

 

Indications

 

1.  Risser 0-2 (growth potential)

 

2.  Curve >30o adolescent

 

3.  Curve >25o with progression (5o in six months)

 

4.  < 10 years old

- very young with high progression potential

- high risk crankshaft if operate

 

5.  Willingness to comply

 

Guidelines

 

Angle                      High Growth Potential                Lowth Growth Potential

<20°                                   observe                               observe or DC

20°-30°                               observe/brace                     observe

30°-45°                    brace                       observe

>45°                                   surgical                              surgical / observe

 

Effect

 

Will control curve only

- end result is initial curve + 5o

 

Brace should be customised to patients curve 

- designed to prevent progression NOT to achieve correction 

- generally see a moderate amount of correction when using the brace

- then slow steady progression of curve back to original magnitude during weaning 

- best curves to brace are those < 40o

 

Bracing complications

 

Failure to prevent progression

Skin irritation

Pressure areas

Abdominal discomfort, eating habit disruption

Cast syndrome - SMA / duodenal obstruction

Psychological

 

Milwaukee Brace  / CTLSO

 

Best for curves with apex above T8 

- three point fixation technique 

- less efficacious for curves > 40o

 

Consists of 

- well moulded pelvic piece above the iliac crests (most important)

- two posterior uprights and one anterior upright 

- neck piece with plastic throat mould anteriorly and two occipital pads posteriorly         

- thoracic pad placed over the apex of convexity of curve 

- lumbar pad over TP between lowest rib and iliac crest on concave side 

- active correction by muscle contraction pulling body away from pads

 

Protocol

 

23 out of 24 hours a day

- result dependant on time in brace

 

Need to check regularly and readjust after 1-2 weeks 

- Xray on 6 month basis 

- if progresses > 45o then surgery

 

Aim for 30-50% correction in first 6 months 

- if not achieved consider surgery 

 

Weaning

 

Once skeletal maturity / Risser 4 / full height 

 

Wean 

- 20 hours for 4 months 

- 16 hours for 4 months 

- 12 hours for 4 months 

- night time only for 4 months 

 

TLSO (Under arm or Boston Brace)

 

If apex < T8 

 

Higher compliance 

May not be as efficacious in holding correction 

Made from cast

 

Operative Management

 

Indications 

 

1.  Immature / Risser 1 /2

- Cobb > 40o with documented progression

- peak height velocity

- will progress 1o per month

- need to stabilise early

 

2.  Mature

- T > 45 - 50o

- TL or L > 30o with marked rotation

- double major > 50o

- significant coronal imbalance

- cosmetic deformity

- failure bracing

 

This curve will progress slowly

- patient has time to make up mind

 

Goals

 

Solid arthrodesis that prevents progression

Balanced spine

Correction of deformity

Prevent respiratory compromise

 

Options

 

1.  Most curves 

- posterior instrumented fusion

 

2.  Lumbar curves

- anterior instrumented fusion

 

3.  Large curves > 70o / young patients

- anterior and posterior surgery

 

Principle

 

Fuse the structural curve with minimum segments

- to stable vertebra

- minimise the levels (preserve motion segments)

- avoid to L5 and above T1 (may increase pain)

- if fuse to L5, only 1 motion segment left, risk LBP

 

Correct curve in sagittal and coronal planes

 

Best to wait til 10 - 12 years to avoid crankshaft

 

Structural Curve

 

1.  Largest curve

2.  One to which trunk shifted

3.  Least correction on AP lateral bending Xray

4.  Pedicles rotated

 

Posterior Instrumented fusion

 

Multisegmental Hook and Pedicle screw systems

- allows for correction via Compression / Rotation / Distraction

 

Crankshaft phenomenon

 

Concept

- seen in young child with high growth potential

- pre PHV surgery or with open triradiates

- pivot on posterior fusion

- vertebral bodies and discs bulge towards convexity

 

Problem

- get loss of correction, increase in rotation, recurrence of rib hump

 

At risk

- Risser 0

- girls < 10

- boys < 13

 

Specific Surgery

 

Lenke Type 1

- main thoracic

- posterior stabilisation

- usually limit to T4 as shoulders equal

 

Scoliosis Fusion Long Thoracic

 

Lenke Type 2

- double thoracic / MT and PT

- need to instrument to T2

- equalise shoulders

 

Scoliosis Fusion Double Thoracic

 

Lenke Type 3

- double major / MT and TL/L

- long posterior instrumented fusion

 

Lenke Type 4

- triple major

- very long posterior instrumented fusion

 

Lenke Type 5

- thoracolumbar / lumbar curve

- can fuse short curve this through bed of T9 / T10 rib

- otherwise posterior instrumented fusion

 

Scoliosis Lumbar Curve Fusion

 

Lenke Type 6

- TL > MT structural

- long posterior instrumented fusion

 

Technique Posterior Instrumented Fusion

 

Pre-operative

 

Consent

Cell saver

- accumulate large blood loss

- often large exposure

Xmatch blood

2 x milled femoral head allograft 

Spinal monitoring / SSEP's

- needles scalp / hands / feet

- begin pre-op once asleep as baseline

IDC

Pedicle screws / TP hooks / rods available

Post op ICU bed especially neuromuscular

 

Position

 

4 Poster Bed

Protect eyes, knees, elbows

No pressure on abdomen / reduce venous bleeding

 

Dissection

 

Posterior approach

- betadine pack buttocks

- midline incision

- divide thoracolumbar fascia midline

- split apophysis with knife (if present)

- subperiosteal elevation strap muscles

- use diathermy, cobb

- sequentially pack with rolled up packs to control bleeding

 

Lumbar spine

- expose facet joints and transverse processes

- don't go between transverse processes laterally as nerve roots here

- pedicle screws inserted bilaterally bottom 3 pedicles

 

Thoracic

- TP hooks above

- pedicle hooks below

- compress

 

2 x rods prebent in sagittal plane

- correct coronal malignement and rotation as able

- may use sublaminar wires if large long curve

- midsection of curve in concavity

- tie over rod and tighten to correct

 

Decorticate lamina, add bone graft along each side

 

Closure

 

Technique Anterior Fusion

 

Indications

 

Large lumbar curve in young patients 

- skeletally immature patient to achieve growth arrest and prevent crankshaft

 

Any lumbar curve to decrease fusion length

- this is debatable

 

Large / rigid curve to achieve mobility 

- severe curves >70o

- supplement posterior fusion

 

Advantages

 

Fewer levels instrumented 

Better correction of rotation

Large surface for fusion

Fusion under compression

Use rib as bone graft

 

Disadvantages 

 

Requires anterior approach

Does not produce lumbar lordosis 

Respiratory problems (need chest drain)

Need to divide segmental vessels

 

Technique

 

Supine, rolled

- curved right sided approach

- remove 9th rib (save for bone graft)

- through bed of rib

- identify peritoneum, stay outside

- take down diaphragmatic crura

- divide segmental vessels, remove discs

- unilateral screws and rod

- repair diaphragm, close over ICC

 

Endoscopic Anterior Instrumentation

 

Advantages

- reduced blood loss and pain

- better scars and cosmesis

 

Disadvantages

- technically difficult

- respiratory problems / deflate lung

 

Growing rods

 

Indications

 

Growing children / open triradiate cartilage

- avoid fusion / crankshaft phenomen

- biannual surgery

- high complication rate 50%

- hook dislocation

- rod breakage

 

Costoplasty / Thoracoplasty

 

Technique

 

Partial excision of 5 or 6 ribs from the TP to posterior axillary line 

 

Advantage

 

Corrects the rib hump 

Cosmetic procedure

Good source of graft 

Does not affect the post op morbidity or pulmonary function

 

Complications G. Coe SRS Report 2006

 

Early

 

Neurological

 

0.32% in posterior corrections (SRS) in adolescents 

- 2% in adults 

- highest in congenital curves

 

Prevention

- SSEP's monitoring in all idiopathic and congenital curves 

- wake - up test in suitable patients (difficult in children)

 

SSEP's

- stimulate in legs, readings in cortex

- avoid inhalation anaesthetics

- time delay as must average amplitudes and reduce background noise

- issue if lose > 50% amplitudes

 

If lose SSEP's

- avoid hypotension

- transfuse Hb if low

- check electrodes

- wake up test

- give steroids

- reverse correction

- remove instrumentation

 

Infection 1.35%

 

Prophylaxis warranted 

Late chronic infection with Proponiobacterium acnes 

 

Respiratory 1.6%

- PTX

- atelectasis

 

PE 0.02%

 

Death 0.03%

 

Ileus - very common

 

Blood Loss 

 

Avoided with

- autologous blood 

- cell savers 

- hypotensive anaesthesia

- autotransfusion

- often blood loss that contributes to neurological compromise 

 

Incorrect fusion levels / wrong level surgery

 

SIADH secretion 

- decrease UO night of surgery

- steady improvement 2-3/7

 

Late

 

Pseudoarthrosis

- 1% overall

- instrument failure 

 

Crank shaft Phenomena 

 

In rapidly growing child after posterior fusion

- spine will rotate as the bodies grow anteriorly

- thus if child with significant growth then add anterior discectomies and fusion

 

Other solutions

- posterior growing rods

- anterior staples / guided growth

 

Sagittal malalignment 

- loss of lumbar lordosis

- flat back / loss of thoracic kyphosis

 

Back Pain 

 

Related to fusion below L4 and loss of lumbar lordosis 

 

Levels and back pain

- L5 - 80%, L4 - 60%

- L3 - 40%, L2 - 20%

 

Late infection - low virulence organism

 

Results

 

Gothenburg Sweden 1968

- 23 year follow-up post fusion with instrumentation

- preop Cobb 62°; postop 33°; last followup 37°

- same series had 127 patient braced

- prebrace 33°, best brace position 30°, last followup 38°

 

 

 

Infantile Idiopathic

Epidemiology

 

< 4 years by definition

 

M : F

 

75% left thoracic

 

Actually very uncommon 

- likely most patients once had spinal dysraphism (Arnold-chiari / syrinx / tethered cord)

- reduced by prenatal folate

 

Progression

 

Age < 1

- 90 % spontaneously regress

- very important

- 10% progress to severe deformities

 

Age > 1

- 80% progress

- more likely to cause cardio / respiratory compromise

- alveoli not developed til age 8

- lungs need room to develop

- high associated with other abnormalities

 

Progression Risk

 

1.  Mehta Angle / Rib-vertebral angle difference / RVAD

 

Difference in angle at which rib meets spine at Apex of curve on either side on AP x-ray

-  > 20° likely to progress 

 

Phase one and phase two

- phase 2 : rib head overlaps and angle cannot be measured

- risk of progression high

- indicative of rotation

 

2.  Degree of Curve

 

Likely to progress if curve > 25°

 

CT

 

Exclude congenital scoliosis

 

MRI

 

Mandatory

- 20% incidence intraspinal pathology

- syrinx / tethered cord / diastematomyelia / ACM

 

Management

 

Non operative

 

Indications

 

Curve < 25o and RVAD < 20o

- resolve spontaneously

- no need for treatment

 

Curve > 25o and RVAD > 20o

- brace

 

Bracing

 

Serial cast < 1 year

 

Brace > 1 year 

- Milwaukee brace can be curative

- younger age group more likely to tolerate this brace than adolescents

- brace must be worn until curve maximally & permanently corrected

- infantile growth spurt continues till ~ age 4-5

 

Operative

 

Indications

 

Curves > 35o

Progressive curves 5o in 6/12 

 

Issue

 

Patient < 10 years or before PHV

- high risk crankshaft effect 

- fusions must be anterior and posterior

 

Options

 

1.  Posterior growing rods

- obtain correction whilst maintaining truncal growth 

- subcutaneous Harrington rod / Growth Rods / Luque rods

- spine exposed at ends for hook insertion with submuscular or subcutaneous rod insertion

- requires surgical adjustment every year (can be done up to 5 times)

- surgery each time adds to scar and increases risk of problems including infection and cut out

- posterior fusion at later age 

 

2.  Hemiepiphyseodesis

- fusion of convex side of apical vertebrae

- is difficult to obtain half disc fusion

- may be used supplementary to growing rods

 

3.  Staples / guided growth hemiepiphyseodesis

- can be done endosopically

 

4.  Posterior instrumented fusion

- not indicated in young children as causes crankshaft effect due to anterior growth continuing

- can be safely performed at age 12 (girls) or 14 (boys)

 

5.  Anterior & Posterior instrumented fusion

- avoids crankshaft effect

- inhibits truncal growth

 

 

 

Scoliosis Examination

Aims of Examination

 

1.  Identify cause

- Marfan's / Neurofibromatosis / Skeletal Dysplasia

 

2.  Balance & body asymmetry

 

3.  Exclude LLD as cause

- correct with blocks or sit patient

 

4.  Forward flexion / Adams forward bending

- look for rotation / rib hump

 

5.  Assess flexibility if considering surgery

 

Typical curve

- the right shoulder is raised

- the right scapula is prominent

- the loin creases are asymmetrical

- the pelvis is level

- there is flattening of the normal thoracic kyphosis

- there is a normal lumbar lordosis

- on forward bending, there is a (mild/moderate/severe) (well rounded/angular) rib hump and a mild left lumbar fullness

 

Front

 

Skin

- cafe-au-lait spots / axillary freckling / neurofibromas

 

Eyes

- Lisch nodule (NF)

- blue sclera (OI)

- cloudy cornea (mucopolysaccharidases)

- dislocated lens (Marfan's)

- optic glioma

 

Mouth

- abnormal teeth (OI)

- high-arched palate (Marfan's)

 

Trunk

- pectus carinatum or excavatum (Marfan's)

 

Limbs

- hemihypertrophy

- dolichostenomelia (long limbs)

- arachnodactyly (thumb in palm)

- clubfoot - often first sign of dysraphism

- cavovarus foot

 

LLD

 

Side

 

Thoracic kyphosis - reduced / hypokyphotic

 

Lumbar lordosis - exaggerated, normal or reduced

 

Protruberant abdomen

 

Back

 

Curve 

- right or left

 

Balanced / Unbalanced 

- alignment of C7 over gluteal cleft (ask for plumb bob)

 

Shoulder height

 

Scapular symmetry

 

Loin creases / lumbar fullness

 

Pelvis 

- level or not (pant line or PSIS)

 

Spinal dysraphism

- hyperpigmentation / hairy patch / dimple / lipoma / tail

 

Leg length 

- if abnormal use blocks & reassess curve

 

Scoliosis can cause apparent LLD

- true curves rotate into their convexity

 

A long leg will elevate the hemipelvis / convexity will be to opposite side

- fixed left scoliosis will give apparent left leg shortening

 

True LLD -> Apparent scoliosis

True scoliosis -> apparent LLD

 

Adam's test 

 

Hands together & bend forwards to touch floor

- mild / moderate / severe rib hump

- well rounded or angular

- satisfactory unroll

 

Meaure rib hump with scoliometer

 

Gait / STRAWS

 

Short

Trendelenburg

Rigid

Antalgic

Weak

Spastic

 

Supine

 

SLR

- hamstring tightness

 

Neurological

- reflexes UL / LL / Abdominal / Babinski

- sensation

- power UL / LL

 

Scoliosis + °Abdominal reflexes & °Axillary sensation 

- syrinx till proven otherwise

 

Abdominal reflexes disappear during teens

 

Prone

 

Correctable

 

Collagen

 

Ligamentous laxity / Wynne Davies criteria

 

Concealed

- if forgotten 

- natal cleft / mouth / eyes / axillae

 

Cephalad joints

- ROM if deformity

 

Circulation

 

Xray

 

"This is a PA spine radiograph of a __ old skeletally mature / immature Risser __ male/ female with scoliosis"

 

"There is a R/L typical/atypical curve thoracic/lumbar curve ±  a R/L T/TL/L lower curve"

 

"The spine is/isn't balanced, the pelvis is/isn't level & the curve has a rotational component"

 

"The curve appears to be Idiopathic / Congenital / NF / NM

 

Don't mention which is 1°/ 2° or postural or structural

 

Neuromuscular Scoliosis

Epidemiology

 

Prevalence of 25-100%

 

Ambulation

- walking decreases incidence

 

Age 

- worse with young onset

 

Aetiology

 

Exact biomechanical explanation of origin & progression of curves unclear

 

Associated with

- weakness 

- poor muscle control

- no proprioception

 

Classification

 

1. Neuropathic

 

A. UMN

- CP 

- Friedrich's

- cord tumour / trauma

- CMT

 

B. LMN

- Polio

- Spinal muscle atrophy

- Spina Bifida

 

2. Myopathic

- Arthrogryposis

- Muscular Dystrophy

- Myotonia Dystrophica

 

NHx compared with Idiopathic 

 

Onset younger

More rapid progression

Progression after skeletal maturity / throughout life

Longer curve

Cervical involvement

Pelvic obliquity

Pulmonary complications common

 

Curve

 

Long C shaped curve

- compensatory curve uncommon but possible

 

Curve associated with

- pelvic obliquity

- hip contractures / dislocation

- cervical involvement

 

Other problems

 

Lung disease common (decreased FVC, LRTI) 

Poor nutrition 

Pressure areas

CRF in Spina bifida

 

Management

 

Aims

 

Maintain cardiorespiratory function

Promote mobility & ambulation

Preserve sitting balance

Improve cosmesis

 

Principles

 

Brace till 12 years then fuse

 

Earlier if

- lose control with rapid progression

- large curve

 

Nonoperative

 

Indications

 

Small curves < 30°

Large curves in patients with very poor prognosis

 

Orthoses

 

Best overall initial treatment

- rate of progression slowed

- allows further spinal growth before definitive treatment

 

TLSO

- 2 piece custom moulded

- used to control & correct scoliosis or kyphosis in growing years

- preferable to Milwaukee brace in NM conditions

- easier to make and wear with reduced incidence of press sores

- worn during day while child is upright

- off at night

 

Seating

- very important

- aims to give trunk & spine control

- able to correct postural curves

- no effect on structural curves

- significantly decreases nursing & handling time

 

1. Tumbleform Seat

- moulded seat for infants

- 3 sizes

 

2. Commercial Chairs

- pelvic support, abduction pillow, thoracic support, head support

 

3. Moulded Sitting Support Orthosis

- AKA SSO

- custom fitted support made from patient mould

- provides maximum sitting support with padded supports & belts

 

Surgery

 

Neuromuscular Scoliosis Posterior Fusion

 

Aim

 

Solid arthrodesis of balanced spine over level pelvis

 

Requirements

 

Long, solid fusion from upper thoracic to lower lumbar

Rigid instrumentation & massive bone grafting

 

Issues

 

Increased bleeding

Osteopenic bone / Poor hold

Fusion to pelvis

High pseudarthrosis rate

Poor medical state

 

Ambulation

- fusion may eliminate ability to ambulate

- trunk movement may be important for ambulation

- obviously lose any spinal movement with flexion

- should test in brace first

- may need to limit extent of surgery

 

Preoperative

 

General medical workup

- cardiopulmonary status

- especially Friedreich's & Duchenne MD

- FVC1, FEV, ABG's

 

Book ICU bed

 

Approach

 

Posterior

- most common especially in CP

- moderate curve

 

Staged Anterior / Posterior

- severe kyphosis / severe rigid scoliosis (> 45°)

- posterior fixation compromised (SB)

- < 9 years to stop crankshaft deformity

 

Technique

 

Require

- spinal cord monitoring if indicated

- cell saver

- post op ICU bed or HDU

- IDC

 

Instrumented fusion

- proximally from T3 or T4 to prevent later subsequent deformities

- caudally to pelvis if >15° pelvic obliquity

- to L4 or L5 if balanced

- often need sublaminar wires for large C shaped curve

- place in midcurve to pull spine to the rods

- large fusions require allograft

 

Blood Loss

- issue because extensive approach / small  blood volume / osteopenic bone bleeds+++

- addressed with meticulous haemostasis / hypotensive anesthesia / haemodilution / cell saver

 

Complications

 

Pulmonary complications

- most common cause of death

- nearly all CP patients get pneumonia post op 

 

Neurological injury

- especially if correcting curve > 90°

- spinal monitoring

- if damaged when wake up, remove metalwork

 

SIADH

- careful fluid balance 

 

Infection

- bacteraemia from remote sites especially  UTI

- increased incidence of wound infection

 

Loss of ability to ambulate

- due to loss of trunk flexion and extension

 

Cerebral Palsy

 

Most common neuromuscular cause of scoliosis in western world

 

Principles

 

Professor Bruce McPhee, Royal Brisbane Hospital

- key is that they all continue to progress past maturity

- says as general rule all need operation so can pick your timing

- try not to fuse too early as get very short trunk

- no longer goes to pelvis 

- feels that stopping at L5 is adequate and pelvis is not worth the morbidity

- insists CP patients have their operation at 50o

- if families decline he won't operative at 70-80o because then they need 2 stage

- high associated morbidity

- follow up every 6 or 12 months

 

Incidence

 

10% in ambulatory patients

70% spastic quadraplegics

 

Lonstein Classification

 

Group 1 curves (A&B) have level pelvis

- double thoracic curve

- little pelvic obliquity

- fuse as for idiopathic

 

Group 2 curves (C&D) have pelvic obliquity

- large lumbar or thoracolumbar curve

- marked pelvic obliquity

- fuse long (T4) to pelvis

 

Algorithm

 

Observation

- curves up to 30˚ in the growing child

- curves up to 50˚ in skeletally mature

 

Orthoses

- often only postpones arthrodesis until puberty

- seating most common form of non-operative treatment instituted

- TLSO effective in ambulatory patient

 

Surgery

 

Fusion to sacrum essential with pelvic obliquity

- Galveston technique (iliac wing rod fixation)

- pedicle screws only viable in S1, not S2

 

Spina bifida

 

Incidence

 

>60% of patients with myelomeningocoele develop scoliosis

 

Principles

 

The higher the level the more severe

- T12 - 100%

- L1 - 90%

- L2 - 80%

- L3 - 70%

- L4 - 60%

- L5 - 25%

- S1 - 10% 

 

40% incidence of spinal dysraphism

- may cause scoliosis progression

- hydrocephalus, syringomyelia, tethered cord, Arnold Chiari

 

20% have congenital component

 

Most require surgery

 

Why fuse wheelchair bound Spina Bifida children?

 

Physiological

RFT's

Feeding

- often use peg

- tends to disappear into skin folds

- hygiene & technical difficulties

Acid reflux when hunched over

Only tolerate small feeds

 

Physical

Pressure areas

Ability to nurse

Takes arm off chair so can do ADL's

Allows stable sitting

Pelvic obliquity

Allows coverage of hips

 

Psychosocial

Self-esteem both patient & carer

Allows better interaction with environment

 

Poor prognostic signs

 

Early age of onset

Asymmetrical motor paralysis

Presence of spasticity

10% - 20% have congenital scoliosis

 

Algorithm

 

Curve 20-40˚

- TLSO

- but bracing complicated by pressure sores

 

Curve > 40˚

- anterior and posterior fusion

- need anterior as posterior elements deficient

- pelvis may be deficient

- attempt to preserve lumbosacral segments to aid wheelchair transfers

 

Duchenne's muscular dystrophy

 

Incidence

 

Usually lose ability to walk during age 9-12 years

- due to progressive weakness

- 80 - 100% develop severe collapsing scoliosis

 

May progress rapidly (2-3˚/ month)

- can progress to 100o

- 10o per year once in chair

 

Issues

 

Affects ability to sit

 

Scoliosis is life threatening

- progressive respiratory compromise

- due to respiratory muscle weakness and curve

- with curve > 35˚, vital capacity 40% of predicted

- further compromises respiratory problems

- death occurs in late teens to early twenties

- may be delayed by curve correction

 

Bracing

 

Orthotic use little success

- almost all progress

- slows progression (15˚ / year comparted with 30˚ / year)

 

Surgery Indications

 

Curve greater than 30o

FVC < 30% predicted

Life expectancy > 2 years

 

Surgery

 

Only posterior

- anterior compromises respiratory function too much

 

Spinal muscular atrophy

 

Most patients develop scolioses

- onset by age 7

- usually progressive

- consider surgical treatment when curve reaches 40o

- large C shaped curves

- T2 to sacrum

 

Friedreich's ataxia

 

All have scoliosis

- 50% have hyperkyphosis

- curve patterns resemble idiopathic rather than neuromuscular

- curves appear later

- late teens or early 20's

- consider surgical treatment when curve reaches 40o

 

Paraplegia and quadriplegia

 

Incidence

 

Age related with risk of spinal deformity

- 90% if cord injury preadolescent

- due to loss of sensory / proprioceptive / motor functions supporting the spinal column

 

Posttraumatic angular deformities related to crush fractures

 

 

 

Other Causes Scoliosis

 

 

Achondroplasia

Background

 

Among the skeletal dysplasias, spinal deformity is seen most commonly in patients with achondroplasia. 

 

Issues

 

1.  Thoracolumbar kyphosis

 

Occurs in almost all patients prior to walking

- non rigid, likely due to hypotonia

- resolves with ambulation

 

Bracing may help to prevent clinically significant kyphosis

- persists in approximately 15% to 20% of patients

 

2.  Spinal Stenosis

 

Pathology

- Short pedicles

- narrow interpedicular distance 

- Stenosis may be present at any level from the foramen magnum down

- most commonly L1-S1

 

Symptoms

 

Over 50% of patients with achondroplasia have back pain

- other symptoms may include leg pain, claudication, paresthesias, weakness, and paraplegia. 

- Bowel and bladder difficulties may occur with stenosis in the lower thoracic spine

 

Symptoms usually occur in early adulthood and are slowly progressive

- Occasionally, symptoms may arise in childhood

 

Surgical decompression

 

Wide, multilevel laminectomies

- Disc removal usually is not indicated 

- unless radiculopathy is present

- Early surgical intervention leads to a better outcome

 

Postlaminectomy instability may occur

- fusion probably should be added in cases of multilevel decompression

 

3.  TL kyphosis + stenosis

 

Anterior decompression and strut grafting should be added

 

Irradiation Induced

Mechanism

 

Vertebral bodies grow axially by endochondral ossification. 

- radiation can inhibit this

 

Incidence

 

Spinal deformity is the most common side effect of abdominal irradiation

- Children irradiated at age 2 years or younger are most affected

 

Study of Wilm's tumour patients

- 7 x incidence scoliosis

 

Reported 10 to 100%

 

May get LLD

- femoral head

- ilac crests

 

Effects

 

Irradiation of the entire vertebra

- produces a symmetric hypoplastic vertebral body

- without significant malalignment

 

Irradiation of a portion of the body

- causes segmental loss of growth 

- resultant scoliotic deformities

 

Soft-tissue fibrosis and contracture may be a cause of spinal curvature

 

Management

 

Milwaukee brace treatment 

- indicated in a young child with a flexible curve caused by irradiation

 

Early posterior fusion and instrumentation is recommended

- Repeat bone grafting may be necessary

- need longer duration of postoperative immobilization 

- several authors recommend a duration of up to 1 year

 

 

Marfan's

Marfan's Syndrome

 

Epidemiology

 

Spinal involvement occurs in approximately 75% of patients with Marfan's syndrome

- scoliosis is the most common spinal deformity

- the cervical spine is almost always normal

 

Those who develop scoliosis do so by 9 years of age

- one half of the patients develop it by age 6 years

 

Pathology

 

Curve patterns resemble idiopathic scoliosis

- frequent progression during adolescence

 

Despite the patients' generalized ligamentous laxity, many of the curves are quite rigid

 

Thoracic lordosis

- second most common spinal deformity

- associated with loss of normal lumbar lordosis

 

Progression

 

These curves also tend to be painful, progressive, and often cause respiratory problems

 

Curve progression rates average 7 - 10o per year

- with the most rapid increase occurring during the early adolescent period

 

Management

 

Non operative

 

Brace treatment

 

Controversial

- most patients fail brace treatment

- orthotics may be used as a holding device for patients who eventually will undergo fusion

 

Operative

 

Technique

 

Posterior spinal instrumentation and fusion 

- mainstay of treatment

- a high rate of pseudarthrosis can be expected

- may lower with addition of an anterior fusion, abundant bone grafting, and postoperative immobilization

 

Complications

 

Pseudoarthrosis

 

Cardiac

- high incidence of malformations

- need good pre op work up

 

Superior mesenteric artery syndrome

- high incidence if put in jacket

 

 

 

 

 

 

 

NF

Epidemiology

 

2% scoliosis patients have NF

 

10 - 30 % NF patients have spinal deformity

- most common skeletal manifestation

- mostly non dystrophic variety

 

Types

 

A.  Non dystrophic

- vertebral wedging, angulation and rotation

- very similar to idiopathic scoliosis

- right thoracic

 

B.  Dystrophic

 

Cause

- may be due to intraspinal lesions such as tumours, meningoceles and dural ectasia

- can simply be bony dysplasia

 

Characterised by

- short segments, sharp angulation with severe apical rotation

- scalloping posterior margins

- widening of the spinal canal

- enlargement of neural foramina

- widened interpedicular distance

- thinned defective pedicles and lamina

- paraspinal mass

- rotation of ribs - 'pencilling'

 

Other problems

 

Cervical spine kyphosis

 

Atlantoaxial dislocation 

- has been reported in five patients

- flexion and extension views are important in assessing cervical instability

- needed pre-op

 

Kyphosis

- marked acute posterior angulation

- can develop myelopathy and even paralysis

 

Intraspinal Tumours

 

Dural Ectasia

- circumferential dilatation of dural sac

- contains CSF and brown material

- erodes osseous structures

- very thin lamina

- can cause great angular deformity

- may have multiple dumbbell appearances

 

Dumbbell lesion

- single neurofibroma

 

Meningocoeles / pseudomeningocoeles

 

Management

 

Non Operative Management

 

Brace treatment

 

Not been effective in dysplastic curves

Can use early in non dysplastic

 

Operative Management

 

Non dystrophic

 

Issues

 

Recommend posterior fusion for progressive

- lower threshold for surgery than in idiopathic

- potential for progression much higher

 

Surgery as for idiopathic

 

Idiopathic

 

Brace 20 - 35o

Surgery > 35o

 

Dystrophic

 

Issues

 

A.  All dystrophic curves need MRI to assess intraspinal lesion

 

B.  All need C spine screening before surgery

- these patients also often have cervical spine abnormalities and instability

 

C.  Incidence of pseudoarthroses ranges from 6% to 38% with isolated posterior fusion

- recomment addition of anterior fusion for dysplastic scoliosis is therefore recommended

- anterior release then posterior fusion

 

Indications

 

Anterior release and fusion followed by posterior fusion if > 50o

 

Kyphoscoliosis

 

Posterior fusion alone is usually insufficient in patients with kyphosis

 

Decompression of the cord with laminectomy 

- contraindicated because the lesion is usually anterior

- removal of the posterior elements predisposes the patient to further postlaminectomy kyphosis.

- need decompression and fusion

 

> 50o

- brace early

- need anterior and posterior fusion

- may even need brace post surgery

- if have myelopathy symptoms may also need decompression

 

Lordoscoliosis

- in dystrophic

 

 

 

 

 

 

Osteogenesis Imperfecta

Incidence

 

Spinal deformities are found in 20% to 80% of patients with OI

 

Risk factors

 

Severe disease with nonambulatory status

 

Progression

 

Scoliosis in OI may progress after skeletal maturity 

- may be related to weakened osteoporotic bone 

 

Management

 

Non operative

 

Bracing

 

Poor results

- risk of rib fractures

 

Operative

 

Posterior segmental instrumentation / arthrodesis 

 

A progressive curve that has reached 35 - 40o should be fused

- regardless of the patient's age

- significant correction cannot be expected

 

Augmentation with segmental sublaminar wiring

- to improve purchase in the osteoporotic bone

 

OI Scoliosis

 

 

Scoliosis Classification

Definition

 

Lateral curvature of the spine with coronal plane deformity > 10°

 

Types 

 

1.   Structual

 

Passively non-correctible deformity of a spinal segment 

- vertebral rotation at the apex

 

2.   Nonstructural (CHIPS)

 

Secondary or compensatory scoliosis

- no rotational component

- fully passively correctable to the midline

 

Compensatory

Hysterical

Irritative

Postural

Sciatic

 

Compensatory

- leg length discrepancy

- hip flexion contracture

 

Hysterical

- teenage females

- no rotation or wedging on XR

 

Irritative

- painful condition of spine

- fracture, tumour, infection

 

Postural

- childhood

- slight correctable curve

- disappears on recumbency

 

Sciatic

- irritation of nerve root by HNP or tumour

- secondary to paravertebral muscle spasm

 

Structural

 

Idiopathic  75%

 

Commences before skeletal maturity

- diagnosis of exclusion

 

No features to categorise it as 

- Congenital

- Neuromuscular

- Other 

 

Types

- Infantile < 3

- Juvenile 3 - 10

- Adolescent 10+

 

Neuromuscular 10%

 

Neuropathic

- UMN:  CP, FA, trauma

- LMN:  Polio, SMA, Spina Bifida 

 

Myopathic 

- Muscular Dystrophy

- Myotonica

 

Congenital (10%)

 

Failure of Formation

- wedge vertebra

- hemivertebra

 

Failure of Segmentation

- unsegmented bar

- block vertebra

 

Mixed

Complex

 

Other (5%) NAOMI

 

Neurofibromatosis

 

Skeletal dysplasias

- Achondroplasia

- SED

- Mucopolysaccharidoses

- Diastrophic Dwarfism

 

Osteogenesis Imperfecta

 

Collagen disorders

- Marfan's

- Ehlers Danlos

 

Traumatic - fractures, surgical

 

Infective - vertebral osteomyelitis

 

Irradiation

 

Metabolic

- rickets, juvenile osteoporosis, osteogenesis imperfecta

 

Tumour - osteoid osteoma

 

Screening

 

Incidence of curve >30° is 3/1000 in USA

 

Screening controversial

- cost vs benefit

- increases health costs by 20% vs late fusion for severe disease only

- if believe that bracing works, then should believe in screening before Peak Height Velocity to decrease severe scoloiosis

- if don't believe in bracing, treat scoliosis as it comes later

- it is debatable whether condition is common enough to merit screening

 

WHO 5 Features of Screening Programme

1. Condition should be important problem

2. NHx should be known

3. An acceptable screening test

4. Acceptable treatment available

5. Programme should be cost effective

 

Screening Methods

 

1. Adam's Test

- bend forward with arms free

- Interobserver error

- no threshold value

 

2. Inclinometer / most common

- assess angular rib hump

- 7° threshold

- will miss 12% 20° curve, but decreases referral rate to 3%

- less sensitive

- more specific

 

 

 

Spinal Dysraphism

Diastomatomyelia

Definition

 

Fibrous / cartilage / bony bar creating a longitudinal cleft in the cord

 

Site

 

Mainly in lumbar spine / can occur in thoracic

 

Symptoms

 

Can lead to cord tethering with associated neurological defects

 

Signs

 

Hyperreflexia / clonus / asymmetric abdominal reflexes

 

Xray

 

Inter-pedicular widening / bony bar / spina bifida occulta

 

MRI

 

May see 2 hemicords

- each within a single dural sac

- each within their own dural sac

 

Conus Medullaris often extends below L2

 

Management

 

Asymptomatic

 

Observe

 

Neurology

 

Should be resected

 

 

Spina Bifida General Principles

Definition

 

1. Spinal dysraphism

 

Failure of the neural tube to close (i.e. spina bifida + failure of neural tube development)

 

May see one of 5 telltale skin defects 

- tail

- skin dimpling 

- sacral pit 

- hair tuft 

- lipoma 

 

Myelodysplasia: Any developmental defect of the spinal cord 

 

2. Spina Bifida Cystica

 

Vertebral laminae absent with prolapse of neural elements 

 

A.  Myeloschisis / Myelocoele

- neural plate material spread out over the surface (most severe form)

 

B.  Myelomeningocoele

- prolapse of cord & dura in defect (commonest)

 

C.  Meningocele

- prolapse of dura only

- usually no neurological deficit 

 

3. Spina Bifida Occulta

 

Congenital spinal disorder where the two halves of posterior arches fail to fuse leading to bony abnormality

- defect is hidden 

- may have diastematomyelia 

- seen in 5-10% of radiographs in normal population 

- not really related to spina bifida cystica and probably not prevented by folate

 

Epidemiology

 

1 / 1000 live births 

- varies with geographic location 

- Ireland, England, Wales - 5/1000

- Australia & USA 1/1000

 

1:20 with one affected sibling 

1:10 with second affected sibling

 

Girls > boys 

 

Aetiology

 

Multifactorial nature 

- polygenic inheritance with teratogenic enviromental factors 

 

Decreased RBC folate associated with increased risk 

- avoid folate antagonists

- folate taken prior to conception reduces rate of spina bifida by 70%

- need to take before first month of pregnancy

- 0.4 mg per day normally

- 4mg per day if have a previous child with SBC

 

Pathogenesis

 

Early problem 26th gestational day (24-28)

 

Two theories

A. Failure of neural tube to close  

B.  Initially closed tube which reopens due increased intraluminal pressure

 

The latter theory is favoured & explains other defects such as diastematomyelia etc

 

Prenatal Screening

 

Should be able to detect 90% fetus with an exposed neural defect

- small skin covered defects may be missed

 

Maternal alphafetoprotein increased 

- test at 16-20 weeks

- detects 90% of spina bifida

- will justify other investigations

- amniocentesis (spontaneous abortion risk 1:200)

 

Ultrasound

- vaginal at 10-12 weeks will identify anencephaly & some spina bifida

- standard 16-18 weeks identify anencephaly & 80% spina bifida

 

Management of early detection

- deliver by C section to avoid contamination by vaginal flora

- secondary infection which then can lead to loss of level

 

NHx

 

Survival

- without repair nearly 100% will die

- especially in pre-antibiotic day

- increase survival to 50% at 25 years

- antibiotics / selective closure / shunting

 

Neurology

- not static & there is often a decline during childhood especially first 4 years

- 60% have neurological deficiency in upper limb

- this is due to syrinx / Arnold Chiari / hydrocephalus

 

Function

- only 30% are functionally independent as adults

- only 30% will have full or part-time jobs

- 65% will have normal intelligence

 

Wheelchair

- 100% if L1 or L2 or higher

- 80% if L3, L4, L5

 

Modified Asher & Olson Classification

 

Lowest level at which Grade 3 power is available i.e. antigravity power

 

Thoracic: no grade 3 strength in lower leg muscles

L2: hip flexion

L3:      knee extension

L4:      knee flexion

L5:      ankle dorsiflexion

Sacral: ankle plantarflexion

 

i.e. L4 and below are most likely to walk

 

Clinical Features

 

1. Hydrocephalus 

 

Incidence 90% 

- of these 80% will require shunt

- inserted at time of the defect closure

 

IQ normal if no hydrocephalus 

 

Must constantly be on lookout for blocked shunt 

- deteriorating neurological function

- bulging fontanelle

- poor muscle function

 

2. Hydromyelia / Syringomelia / Syrinx 

 

Incidence 50%

- related to hydrocephalus 

- fourth ventricle communicates with the central canal of cord 

- increased hydrocephalus pushes fluid into cord 

 

Leads to

- increased lower limb paralysis & back pain

- progressive scoliosis

- weak upper extremities with increased tone

 

Usually settles with V-P shunt 

 

May need drainage prior to spinal procedure 

 

3. Arnold - Chiari Malformation: (Type II in 90%)

 

Displacement of medulla into cervical canal

- cerebellar tonsils herniated below foramen magnum on MRI

 

Symptoms   

- periodic apnea 

- stridor 

- weak / absent cry

- nystagmus 

- upper extremity spasm & weakness 

 

May resolve with shunt / if not then surgically decompress 

 

4. Tethered Cord    

 

Attachment of cord to meningocoele sac 

- prevents normal upward migration of the cord during growth

- almost universal to some degree

 

Only small number have symptoms

- buttock & posterior thigh pain 

- increased spasticity & weakness in lower extremities 

- progressive scoliosis the classic presentation is progressive 

- cavus foot deformity in young child 

- rapid deterioration in quadriceps power in teenager

 

Surgical release will halt progression but not restore function

 

5. Urinary Complications

 

One of major causes of morbidity & mortality in childhood from renal failure

 

Causes

- incontinence 

- hydronephrosis 

- recurrent UTI

 

Management

- intermittent catheterisation 

- bladder augmentation

 

6. Skin anaesthesia

 

Pressure sores major problem with casts

- hip spicas should always include feet

 

7. Latex Allergy

 

IgE mediated

 

Should always provide latex free operating environment 

- assume allergy and prevent development

 

Can screen with RAST (radioallergosorbent test) / skin test / IgE assay

- IgE assay most sensitive & specific

 

8. Fractures

 

Classic exam scenario / clinical photo

- supracondylar femoral fracture is commonest

 

Often present late 

- picture of infection (redness, swelling, warmth)

 

Treat with removable splints for short time as get very stiff

 

9. Upper Extremity Function

 

60% have abnormality of UL function

- usually weakness with increased tone

 

Risk factors

- high level

- multiple shunt revisions (ie cerebral damage from hydrocephalus)

- syrinx etc

 

10.  Orthopaedic Deformity 

 

Causes

- muscle imbalance 2° to UMN or LMN lesion

- intrauterine posture

- habitual posture after birth

- congenital malformation (eg club foot)

- arthrogryposis

 

Problems

- scoliosis

- hip FFD / Dislocation

- Knee hyperextension / flexion contracture / valgus

- teratological CTEV 

- cavus foot

- charcot foot

- ulceration

 

Orthopaedic

 

Goals

 

1.  Ambulation

- level dependent

 

2. Hips

- reduce hips if unilateral / low level / walk

- different to CP as not painful

 

3. Foot 

- keep shoeable & plantigrade 

- even if in wheelchair

- avoids ulcers and amputation

 

4.  Spine

- 80% develop scoliosis

 

5.  Prevent pressure areas

 

Always manage in spina bifida clinic

 

Muscle charting 

- should start 24 hours after birth

 

1/3 Complete LMN & loss of sensation & bowel control below affected level 

 

1/3 Complete lesion at some level but distal segment of cord preserved 

- mixed picture of intact DTR & spasticity 

 

1/3 Incomplete & some movement & sensation preserved 

 

Levels

 

L1 Level

 

Muscles

- iliopsoas grade 2 or better / some weak hip flexion

 

Position

- Hip flexed, abducted & ER

 

Ambulation

- HKAFO - hip knee ankle foot orthosis

- RGO - reciprocating gait orthosis, when one hip flexes the other extends

- adults usually wheelchair bound 

 

L2 Level

 

Muscles

- iliopsoas, sartorius & adductors grade 3 or better

- hip flexion & some adduction

 

Position

- hip flexed and adduction 

- FFD of hip may develop 

 

Ambulation

- most ambulate as children in HKAFO

- FFD may need to be corrected first 

- adults usually choose wheelchair 

 

L3 Level

 

Muscles

- quadriceps grade 3

 

Position

- hip flexed & adducted

- knee extended 

- Hip subluxation & dislocation common due to unopposed hip adduction & flexion 

 

Ambulation

- usually household ambulators 

- grade 4 quadriceps - 80% community ambulators / 98% household ambulators 

- grade 3 quadriceps -  need KAFO to walk & 88% in wheelchair 

- patients with quads & good abductors can ambulate without aids 

 

L4 Level  

 

Muscles

- medial hamstring grade 3

- Tibialis anterior 3 or better

- quadriceps grade 5

 

Position

- hip flexed & adducted

- knee extended

- ankle in varus due to T anterior

 

Ambulation

- walk in AFO 

 

Surgery often required to maintain hip & knee extension / foot plantigrade

 

L5 Level

 

Muscles

- lateral hamstrings >=3

- G medius >=2

- T posterior >=3

 

Position

- calcaneovalgus or calcaneus foot 

- need T anterior to T Achilles transfer age 3 to prevents foot drop gait

 

Ambulation

- 95% community ambulators throughout life 

 

Sacral Level 

 

Muscles

- Gastroc/Soleus >/=2 

- G medius >/=3

- G maximus >/=3

 

Position

- clawing of toes & cavus foot from intrinsic minus

 

Ambulation

- usually brace free but may need special shoes 

- ie arch supports

 

At high risk of heel ulcers due to foot imbalance / calcaneus / nil S1 protective sensation

 

 

 

 

Spina Bifida Levels

Thoracic

 

Function

- no hip flexion

- no voluntary leg movement

 

Effect

- no ambulation

- hip flexed and ER

- feet equinovarus

 

L1

 

Function

- grade 2 psoas

 

Effect

- no ambulation

- hip adducted and flexed

- feet equinovarus

 

L2

 

Function

- grade 3 psoas

 

Effect

- hip adducted / flexed

- knee flexed

- feet equinovarus

 

L3

 

Function

- grade 3 quads

 

Effect

- hip adducted and flexed / highest risk hip dislocation

- household ambulator only with KAFO

- knee recurvatum / no hamstrings

- foot equinovarus

 

L4

 

Function

- > grade 3 quads

- medial hamstrings

- tibialis anterior grade 3

- AFO

 

Effect

- hip adducted / flexed

- reasonable ambulation with AFO

- knee extended

- foot cavovarus

 

L5

 

Function

- lateral hamstrings

- grade 3 abductors

 

Effect

- hip flexed but not adducted

- community ambulator in AFO

- calcaneocavus foot

 

S1

 

Function

- grade 3 power T achilles

 

Effect

- claw toes, poor sensation

- still get calcaneus as weak T Achilles

 

 

 

 

Spina Bifida Management

Closure of defect

 

Selection of patients for closure of the defect is controversial 

- if not treated most die of meningitis

- remainder have early closure - 50%

- closure of defect within 24 hours with VP shunt insertion

- early closure & shunting survival is 50% at 25 years

 

Most centres avoid urgent operation if

- level above L1 

- severe deformity 

- marked hydrocephalus 

 

Principles Orthopedic Management / Menelaus

 

1. Always manage in Spina Bifida clinic 

2. Select surgery appropriate to future demands 

3. Perform minimal surgery 

4. Condense management 

5. Correct muscle imbalance 

6. Consider absent sensation / bone fragility / infection 

7. Minimise immobilisation time to prevent bone loss & pathological fractures

8. Promote walking to

- allow normal bone development 

- prevent contractures

- psychological advantages

9. Surgery is soft tissue releases and tendon transfers 

10. All surgery at one sitting / SEML

11. Wait until 12 months

- spinal level & deformity evident & assessable

- most shunt & closure problems sorted 

 

Potential walkers are likely to need more sophisticated surgery

 

Ambulation Requirements

- extended hips & knees 

- plantigrade feet 

- straight spine

- strong quadriceps as general rule

- strong abductors / if no hip abductors usually need crutches or stick)

 

Sitting Requirements

- flexed knees 

- feet that are shoeable & placeable in wheelchair 

- straight spine - releases the hands for other activities other than truncal support

 

Hips

 

Concepts

- hip dislocation does not alter level of mobility only neurosegmental level does

- a painless dislocated hip is preferable to a stiff, painful, reduced hip

- reduction is dealt with as for DDH hip

 

Indications / Menelaus

 

1.  Non walkers

- no quadriceps & bilateral - never 

- no quadriceps & unilateral - sometimes 

- usually doesn't affect ability to sit in wheelchair

 

2.  Walkers

- quadriceps and bilateral - sometimes 

- strong quadriceps & unilateral - always 

 

Management

 

1.  Sharrard procedure 

- soft tissue procedure to prevent and reduce hip dislocation

- transfer psoas to GT / adductor tenotomy

- limited benefit and can compromise walking

- iliopsoas is the major driver of walking

 

2.  Girdlestone's procedure

- rarely done in spina bifida as not painful

- indicated for painful, arthritic subluxed or dislocated hips

- combine with valgising osteotomy / Schanz

 

3.  Osteotomy age 1 year 

- varus shortening osteotomy of femur 

- pelvic osteotomy if acetabular dysplasia present

 

Knees

 

Concept

 

Walker (L3 or better)

- aim for extended knee that is braceable 

- extended knee is stable position 

 

Non walker

- need knee flexion for wheelchair use

 

Extension Contracture 

 

May interfere with walking 

- serial casting best 

- if fails then V-Y quads lengthening 

 

Flexion Contracture

 

Hamstrings are usually not tight

- more common with CP

- check popliteal angle in HE/HF

 

Options

 

1.  Posterior capsulotomy and gastrocnemius releases 

 

2.  Anterior hemiepiphysiodesis with Stephens 8 plates

- treat FFD with hyperextension

 

3.  Extension distal femoral osteotomy

- near skeletal maturity with large FFD

 

Ankles & Feet

 

Concepts

 

1. Majority have deformity

- 50% equinovarus / CTEV like 

- 20% calcaneus / L5 level

- 20% normal 

- 10% planovalgus, equinovalgus, cavus & claw toes 

 

2. Aim for braceable plantigrade foot 

- almost all require a brace 

 

3.  Multiple deformites seen

- very difficult to predict the deformity

- not just due to the muscle imbalance

- can be abnormal secondary to hydrocephalus, tethered cord etc

- may be a mixture of upper and lower cord signs

- due to lesions at various levels / cerebral from ICP / high or low spinal lesions

 

Equinovarus / CTEV like

 

Varies from flexible to very rigid 

 

Splintage & casting initially 

- is not the same as CTEV

- the tendons etc are not just tight

- there is a muscle imbalance

 

All require OT / PMR at 6 - 12 months

- high risk recurrence

- may be better to resect 1 - 2 cm of tendon

 

Recurrence

- talectomy 

- triple arthrodesis near skeletal maturity

 

Calcaneus

 

L5 level most common 

 

Causes heel ulcers

 

Surgery at 3 years

1.  Divide the EDL, EHL  +/- anterior AKJ capsule release

2.  Transfer Tibialis Anterior to heel if normal & no tendo achilles function 

 

>6 years

- deformity osseous

- posterior displacement osteotomy calcaneum

 

>10 years

- triple arthrodesis for severe deformity

 

Planovalgus

 

Usually less problem than varus 

- can be managed often with AFO

- may occurs in Ankle or Subtalar joint

 

X-ray

- Cobey view

- assesses alignment of AKJ & STJ in weight bearing

- can see where malalignment is

 

Lateral tilt of ankle in mortise 

- TA to fibular tenodesis - in young child causes overgrowth of fibula due to increased blood supply

- 8 plates medially/ guided growth if sufficient growth remaining  (< 6 years of age)

- supramalleolar osteotomy with medial closing wedge if teenager

 

Subtalar joint

- Grice arthrodesis

- calcaneal siding osteotomy

- lateral calcaneal lengthening

- triple arthrodesis 

 

Cavus

 

Pressure effects major problem 

 

Options

- plantar release / metatarsal osteotomies / calcaneal osteotomy  / Jones procedure / claw toe management

- if close to maturity & significant deformity then look at Triple Arthrodesis 

 

CVT

 

< 2% of children with spina bifida

- manage as per all CVT

 

External Rotation of Tibia

 

Commonly associated with valgus ankle 

 

Requires supramalleolar osteotomy > 8 years of age if severe

 

Scoliosis

 

Most common skeletal abnormality 80%

- more common in high lesions 

 

Aetiology

 

1. Congenital

- congenital spinal deformity 

 

2. Neuromuscular

- paralysis with nstability of posterior elements 

 

3. Neurological

- hydrosyringomyelia / malfunction of VP shunt

- tethered cord 

 

Orthotics

- temporary measure to delay fusion to allow trunk height development 

- pressure sore problems 

 

Surgery

 

Indications for intervention

- failure of orthotic management to maintain curves <45°

- severely affected children will have to support trunk with upper limbs

- makes walking & sitting very difficult

 

Anterior release & fusion 

- posterior elements usually very deficient & not suitable for bone graft

- posterior approach may be difficult exposure due to previous sacral repair & skin flap

 

Kyphosis

 

Specific problem in spine in spina bifida

 

Issues

- difficulty sitting in wheelchair 

- ulceration over kyphos 

- breathing difficulties 

 

Management

- excision of kyphosis & osteotomy of spine 

- excision of distal cord (roll up procedure)

- may need extensive anterior release & full length fusion to pelvis (loss of correction is common)

 

Lordosis

 

Usually corrected by FFD / hip correction

 

 

 

Spinal Dysraphism

Definition

 

Spina Bifida is a congenital disorder in which the 2 halves of posterior vertebral arch fail to fuse

 

Dysraphism is maldevelopment of neural tube and skin

 

Cutaneous signs

 

Tuft of hair

Pigmented naevus

Scarred area

Post midline dimple / sinus

Lipoma

Tail

 

Associations

        

Cord tethering

Diastematomyelia

Cysts or lipoma of cord

Hydrocephalus

Arnold Chiari Malformation

- distal tethering may cause herniation of brain stem / cerebellum obstructing CSF flow

 

Epidemiology

 

10-30% of population

- 25% no cutaneous stigmata

- clinically significant dysraphism is rare

 

Embryology

 

Germ cell problem

- can get deformity in all three germ cell lines

 

1.  Somatic Ectoderm 

- cutaneous dysplasia

- hairy patch, naevus, dermal sinus

 

2.  Mesoderm

- vertebral dysplasia

- split in spinous process and laminal defects

 

3.  Neuroectodermal

- neural tube

- myelodysplasia, intramedullary and extramedullary growths associated with dysraphia

 

Commonest forms

 

Diastematomyelia

- spinal cord / filum terminale / both

- split sagittally by bony or fibrocartilaginous septum

 

Lumbosacral lipoma

 

Meningocele manque 

- loop of nerve root or trunk becomes adherent to dura

- then returns to cord or cauda close to origin

 

Arachnoid / Dermoid cyst

 

Tethered Cord

- tight filum terminale leading to tethered cord

- conus at level of coccyx in fetus

- upper border of L3 at birth

- upper border L2 by 5 years

 

Hydromyelia

 

Clinical Presentation

 

Vast majority asymptomatic

 

Any age from birth to maturity with

- short, wasted leg

- small foot

- cavovarus deformity

- paralytic valgus foot deformity

- trophic ulceration

 

DDx

 

Hemihypertrophy or hemiatrophy if small limb

CMT

CP 

 

Ultrasound 

 

Investigation of choice < 6 /12 presenting with midline lumbar dermal anomalies

 

X-ray

 

>6/12  

- varying degrees of spina bifida

- vertebral anomalies

 

MRI 

 

Management

 

Principles

 

Recognise the condition and arrest any further neurological deterioration

- MRI spine

- If find tethered cord etc needs neurosurgical opinion on whether release of cord worthwhile

 

Address foot deformity along typical lines once neurological deformity stabilised

 

 

 

 

 

Syringomyelia

Definition

 

Progressive and  chronic disorder associated with cord cavitation & gliosis

 

Effects

 

Muscle wasting

Dissociate anaesthesia

Scoliosis

Neuropathic arthropathy

 

Epidemiology

 

M > F

No familial tendency

 

Aetiology

 

1. Idiopathic

 

2. Hindbrain Herniation / Arnold- Chiari Malformation

- abnormal pressure & circulation of CSF

 

3. Post-Traumatic

- occurs in 3%

- ? due to subarachnoid block 2° to adhesive arachnoiditis

- more common in complete tetraplegics

- delayed presentation of deterioration

- 3/12 - 30 years

 

4. Arachnoiditis

5. Intramedullary Tumour

6. Extrinsic Compression With central cord necrosis

7. Necrotic Myelitis

 

Arnoldi-Chiari Malformation 

 

Congenital abnormality of Cerebellar Tonsils 

- herniation of medullary tonsils through foramen magnum into cervical canal

- block CSF flow through foramen Lushka & Malgagne blocked

- often need AV shunt

 

Type I  

- communicating

 

Arnold Chiari Type 1 MRI

 

Type II  

- non communicating

- usually larger and associated with spina bifida

 

Arnold Chiari Malformation with syrinx

 

Pathology

 

Cord damage

- anterior dissection of cavity with interruption of decussating spinothalamic fibres

- effects pain & temperature 

- extension into lateral corticospinal tracts 

 

Most common in lower C Spine

- may progressively extend up to medulla & down to lumbar 

- fluid-filled tubular cavity

- separate from but usually communicating with central canal

 

Spastic Paraparesis

- involvement of 2° order neurones

 

Pain with causalgia

 

Consequences

 

Dissociative sensory loss

Spastic paraparesis

Neuropathic joints especially GHJ

Scoliosis as syrinx is irritative

- scoliosis not due to muscle imbalance as neurology most commonly normal

 

Histology

 

Cystic space

- lined by thick layer of glial tissue

- gliosis with tendency to infiltrate white matter

 

Associations

 

Craniovertebral anomalies

Platybasia

Hydrocephalus

Myelomeningocoele

 

Cause of deterioration in Spina Bifida

 

Shunt malformation

Arnold Chiari

Cord Tether

Diastomatomyelia 

Neuroenteric cyst

Syrinx

 

Clinical Features

 

Classic Triad

 

1.  Dissociate sensory loss in cape distribution

- loss of pain and temperature

- preservation of light touch (dorsal columns) 

- arms and trunk with cervical

- legs with lumbar

 

2.  Muscle wasting / weakness with diminished reflexes 

- in UL due to direct pressure on anterior horn cells

 

3.  Atypical high thoracic kyphoscoliosis

 

Site of syrinx

 

1.  Cervico-Thoracic

- dissociate sensory loss in arms & trunk

- weakness & wasting of arms

- absent abdominal reflexes

 

Syrinx Thoracic MRI SagittalSyrinx Thoracic MRI Axial

 

2.  Lumbar

- dissociate lumbosacral sensory loss

- weakness & wasting of legs & pelvic girdle

- diminished reflexes / Babinski negative

- impaired bowel & bladder function

 

3.  Cervico-Medullary 

- AKA syringobulbia

- palatal & vocal paralysis / dysarthria

- nystagmus / dizziness / tongue weakness

 

Cervicomedullary Syrinx 1Cervicomedullary Syrinx 2Cervicomedullary Syrinx 3

 

X-ray

 

Spine

Base of skull

 

MRI

 

Most sensitive investigation

- main differential is oedema

- need T1 image

 

Look for obstructions

- ACM

- spinal cord tumours

 

DDx

 

Amyotrophic Lateral Sclerosis / Motor Neurone Disease

MS

Cervical Myelopathy

Thoracic Outlet Syndrome

Klippel-Feil Syndrome

Cord Tumour

Brainstem Infarct

 

Operative Management

 

Indications

 

Rapid progression of neurology

Short History

Respiratory compromise

Pseudobulbar Palsy

Pre-spinal corrective surgery

 

Technique

 

Shunt

- Syringo-Cisternal

- Syringo-Peritoneal

 

Suboccipital decompression & C1-2 laminectomy

 

Orthopaedic Implications

 

Neuropathic Joints

 

Syrinx is most common cause of UL charcot joint

- shoulder

- elbow

- hot swollen joint may be first presentation of syrinx

 

DDx

- infection

- peripheral neuropathy - DM / Leprosy / Tabes dorsalis

 

Scoliosis

 

Scoliosis is usually 1° Presentation of syrinx

- curve is idiopathic type

- often left sided thoracic

- corresponds with level of syrinx

 

Males > Females

- 25% if < 10 year old

- 20% males with scoliosis have syrinx compared with 2% of girls

 

Neurological deficit usually subtle or absent

- axillary sensation absent

- absent abdominal reflexes

 

Treatment

- must correct syrinx first

- malignant progression if syrinx untreated

- cyst rupture during scoliosis surgery may be fatal

- progresses like idiopathic once syrinx treated

 

 

 

Tethered Cord

Definition

 

Conus medullaris below L2 / tethered

- unable to move freely with movement / growth

 

Aetiology

 

Thickened filum terminale (> 2mm at L5/S1)

Intradural lipoma

Diastomatomyelia

 

Surgery to close myelomeningocoele

- almost all will have signs of tethered cord at site of surgery

 

Issue

 

Stretches and damages the cord as child grows

- neurology

- pain

- difficulty walking

- foot deformity

 

Rapid progression of scoliosis

 

Clinical

 

80 - 90% of children will have cutaneous manifestation

 

MRI

 

Management

 

Open surgical release

 

Indication

- child

- neurological symptoms

- progressive scoliosis

 

 

 

 

 

 

 

 

 

Spinal Fractures

Cervical spine Fractures

 

Incidence

 

Rare

- < 1% children's fractures

 

< 7

- upper cervical

- craniocervical junction

 

> 7

- lower C spine predominate

 

Immobilisation

 

Very big heads

- will flex neck on spinal board

- need bump under T spine or

- cut out for head

 

Clinical

 

Beware distracting injuries

- cannot clear C spine clinically

 

Clearance

- normal mental state

- no distracting injuries

- not intoxicated

- able to adequately communicate

- no neck pain or tenderness

- Full ROM

 

X-rays

 

Issues

 

Soft tissue swelling

- unreliable in crying child

 

Paeds C spine

- not well cleared by X ray

- much of cervical spine cartilaginous

- CT invaluable

 

Normal findings

 

C2/3 pseudosubluxation

- up to 4 mm

- common / seen in 40% < 8 years old

- reduced in extension

- Swischuk's line:  posterior arch C1 - C3, C2 shoulde be within 1.5 mm

 

ADI > 3 mm in 20% children

 

Vertebral bodies classically wedged

 

CT

 

Fast

- may not need sedation

 

Essential for C0-2 if intubated

- include in any child having CT head

 

Will pick up vast majority of unstable fractures

 

MRI

 

Show disruption of endplate / disc junction

Ligamentous injuries

SCIWORA

 

SCIWORA

 

Spinal cord injury without radiographic abnormality

- C spine very flexible

- traction injury with normal X-ray

- usually upper C spine < 8

 

Immature C spine can stretch 5 cm without fracture

- spinal cord ruptures with 5 mm traction

- it is less elastic and tethered

 

Must be aware of possibility especially with GCS <3

 

Management of obtunded patient

- unable to clear C spine

- Aspen collar will cause pressure areas / increase ICP

- MRI within 12 hours

 

Upper C spine Fractures

 

Aetiology

 

< 8

- mobile neck

- ligamentous laxity

- shallow facets

- big head

 

Usually falls and MVA

 

Ossification

 

Axis

- 3 primary

- body and two arches

- fuse age 7

 

Atlas

- 4 primary

- body, 2 arches, dens

- dens fuses age 6

- summit ossification appears 3-6, fuses 12

 

Problems

 

1.  Os ondontoid

 

Thought to be related to previous trauma

- can give C1 / 2 instability

 

2.  C0/1 dislocation

 

Terrible injury

- quadriplegia

- can be fatal

 

C0-C1

- Basion axial / Basion Dens interval

- each less than 12 mm

 

Powers ratio

- tip of basion to posterior arch (BP)

- tip of opisthon to anterior arch (AO)

- BP / AO

- > 1 anterior dislocation

- < 1 posterior dislocation

 

3.  Ondontoid Fractures

 

Pathology

- occurs at the synchondrosis

- intact anterior periosteal sleeve

 

Mechanism

- MVA deceleration injury

 

Clinical

- neurological defects rare

 

Xray

- anterior displacement

 

Management

- reduce with extension and application HTB

- 50% apposition required

- non union rare

 

Lower C spine

 

Anatomy

 

Neurocentral synchondroses fuse 3-6

Bodies wedge shaped until become square at 7

Superior / inferior cartilage end plates attached to disc

 

Pathology

 

Fractures occur between cartilaginous end plate and vertebral body

- between hypertrophic and calcified zones

 

Thoracic spine fracture

 

Uncommon

- protected by rib cage

 

Cause

- MVA, falls

- osteopenia ( OI, chemo, leukaemia)

 

Fracture / dislocations / Chance fractures

 

High energy

- usually TL junction

- lap belt injuries

- high association with intra-abdominal injuries

 

Apophyseal ring injuries