Congenital Kyphosis



Kyphosis due to congenitally anomalous vertebrae

- characterized by severe angular deformity

- prominent gibbus at the apex of the curve




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




Due to

- growth differential

- erosion of vertebral body from mechanical pressure




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)




Non operative not effective


Work up


MRI / Neurosurgery

- exclude intraspinal pathology



- cardiac abnormality


Abdominal US

- renal abnormality




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







Atlanto-axial Rotational Instability



Paediatric populations predisposed

- due to lateral mass anatomy and ligamentous laxity

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




Due to forced rotation and lateral tilt

Can be spontaneous




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






Klippel Feil








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




Widened atlanto - lateral mass interval


Dynamic CT


Head turned to left then to right

- demonstrate fixed subluxation




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




> 3/12

- consider fusion in situ




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



Cranial prominence of odontoid into region of foramen magnum

- upper cervical spine encroaches on brain stem




Congenital syndromes

- Klippel Feil Syndrome

- Achondroplasia

- OI

- Downs 

- Morquio


- NF


Congenital abnormalities

- atlas hypoplasia

- bifid posterior arch atlas

- occipitocervical synostosis




- Rickets

- RA


Clinical Features


Become symptomatic in third decade

- neurological compression 15%




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




Delineate pathology




Consider surgery if symptomatic




1.  Severe pain


2.  Neurology


3.  Progression

- basilar invagination > 5mm




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



Twisted / Wry neck secondary to fibromatosis in sternocleidomastoid




Packaging defect

- commonest first born 

- 75% on right




CDH 20%

Metatarsus adductus 15%

Breech presentation

Klippel - Feil Syndrome





Fibrosis of SCM on one side

Fails to grow & causes progressive deformity








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




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






Congenital fibrosis SCM


Congenital vertebral anomaly

- Klippel Feil

- os ondontoid

- C1-2 fusion

- unilateral C1 deficiency

- many others





- atlantoaxial rotatory subluxation

- # C1 /2


Grisel's syndrome

Ocular dysfunction

Infection / Discitis

SCM scar / tumour





To exclude congenital vertebral anomaly

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


Indicated with failure non operative management




Non operative


Stretching exercises


90% successful




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 






If persists past 1 year age chance of resolution very poor

- operate especially if > 30o limitation of movement




Ling et al Clin Orthop 1976

- 103 operations

- high rate scar tethering if released <1 year old




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




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





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




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




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







- ? 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




Open-mouth view



- > 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




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%




Failure of normal segmentation of mesodermal somites of neck

- during 3rd to 8th week

- may be AD inheritance






Stiff especially lateral movements

- usually hypermobile at unaffected levels




Short neck


Head arises from shoulders


Often associated with webbing of neck


Low posterior hairline

- on level with shoulders




Degenerative OA

- caused by hypermobility of adjacent segments

- leads to instability & degenerative OA

- presents 3rd decade



- associated abnormalities


Other congenital cervical spine abnormality

- occiptiocervical synostosis

- basilar impression

- odontoid anomalies



- 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



- 35%

- renal failure often occurs early



- 30%

- affects development of speech & language



- 20%

- involve paired movements of hands

- abnormal pyramidal tracts

- usually resolves



- 15%

- various congenital defects

- need preoperative investigations / echo




Vertebral fusions


Klippel Feil Xray


Thoracic abnormalities

- scoliosis

- kyphosis


CT scan


Body fusion / block vertebrae


Flattening & widening bodies

Hypoplasic discs

Cervical spina bifida


Klippel Feil CT SagittalKlippel Feil CT Coronal




1.  Manage associated conditions





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



Spondylosis / spondylolisthesis

- most common cause

- adolescent playing sport


Scheuermann kyphosis
- 1/3 of patients


HNP - rare



Apophyseal ring fracture

- traumatic fracture between vertebral body and cartilaginous ring

- apophysis displaced posteriorly with disc

- decompression if causes neurological symptoms


Discitis / Osteomyelitis



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

- malignant: Ewings / OS / Chordoma / Leukaemia




Juvenile Osteoporosis




Pain character

- aspirin relief / night pain

- osteoid osteoma


Associated neurology & deformity


Systemic features / Red flag signs

- fever / weight Loss




Standing PA & Lateral




For spondylithesis




Low threshold

- may need GA










Paediatric Discitis



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




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




1-12 years / symptoms age dependent


0 - 3

- refusal to walk / weight bear / limp

- irritability


3 - 9

- abdominal pain


> 9

- back pain



- stiff flexed spine 

- tight hamstrings & decreased SLR

- scoliosis




50% positive blood culture

66% positive CT guided biopsy

- usually not required



- Staph 60-70%

- Streptococcus

- Gonococcus > 12 years

- E Coli in neonates

- Atypicals (TB, Brucellosis)




Initial xray normal



- loss of disc height

- end plate irregularity & sclerosis

- disc can regain height, but endplate changes remain




Child may need sedation / GA





- leukaemia, metastasis (vertebral)

- EG (vertebra planar)

- OO, OB


Epidural abscess

Paraspinal abscess

SI joint septic arthritis






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 




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









Congenital Scoliosis



Lateral curvature of the spine 2° to vertebral anomaly

- causes an imbalance in longitudinal spine growth




True incidence unknown


F > M


Typically Thoracic




No association in twins / suggests not inherited

5% risk in family if complex multi-level




Occurs during mesenchymal development 4-8/52  

- unknown foetal insult

- high incidence other anomalies 



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





- 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



- radial club hand

- congenital amputations

- cavovarus foot / CTEV


Klippel-Feil 25%




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%)




Combination both / Commonest


Unclassifiable (20%)




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 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





- worst prognosis with clinical deformity in first year of life



- 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




Single hemivertebra unpredictable

- act as enlarging wedge

- often small with limited growth potential (incarcerated)




Lesion often difficult to interpret


See fused ribs


3D CT 


Excellent guide to anatomy




Should be performed before operative intervention

- assess for vertebral abnormality (40%)

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




Renal ultrasound





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




Bracing not indicated


Observe each 6/12

- look for progression

- examine for neurology

- continue until skeletal maturity





- progressive > 4-6o per year

- >40°

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

- unbalanced child e.g. L5 hemivertebrae



- 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




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



- coronal / scoliosis

- sagittal / thoracic lordosis

- transverse / rotational


General Categories




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




< 10o - 2.5 %

> 30o 4 / 1000 

> 40o 1/1000



- little difference overall

- females more likely to have larger curves

- females more likely to progress




Scoliosis Research Society (SRS)


Infantile: 0-3 years onset 


Juvenile: 3-10 (Puberty)


Adolescent: 10 - Cessation of Growth (20 years)




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




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 



- 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 




Scoliosis produced when the pineal gland removed from chickens 

-  transmitter found to date - ? melatonin




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




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 




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)


Double major (Lumbar dominant)

Left Lumbar 






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




Magnitude:  curve > 20o

Risser:  0 or 1

Sex:  Female

Menarche: premenarche

Age: < 12

Pattern:  Thoracic & double curves most


Growth Remaining



- 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



- 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 



- 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




Nachemson 1968

- not increased until curve of 100o noted


Cor Pulmonale 


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






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




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 



- 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 




If suspect intraspinal pathology 

- Brain + 3 level spine / neurocentral


Indications for MRI


Scoliosis Left Lumbar Curve


Left sided 



Rapidly progressive 

Neurological abnormality present 




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






3 areas of curve

- main thoracic: MT

- proximal thoracic: PT

- thoracolumbar / lumbar: TL/L



- 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



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 




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




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




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



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




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 




Once skeletal maturity / Risser 4 / full height 



- 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




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




Solid arthrodesis that prevents progression

Balanced spine

Correction of deformity

Prevent respiratory compromise




1.  Most curves 

- posterior instrumented fusion


2.  Lumbar curves

- anterior instrumented fusion


3.  Large curves > 70o / young patients

- anterior and posterior surgery




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



- 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



- 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





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


Pedicle screws / TP hooks / rods available

Post op ICU bed especially neuromuscular




4 Poster Bed

Protect eyes, knees, elbows

No pressure on abdomen / reduce venous bleeding




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



- 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




Technique Anterior Fusion




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




Fewer levels instrumented 

Better correction of rotation

Large surface for fusion

Fusion under compression

Use rib as bone graft




Requires anterior approach

Does not produce lumbar lordosis 

Respiratory problems (need chest drain)

Need to divide segmental vessels




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



- reduced blood loss and pain

- better scars and cosmesis



- technically difficult

- respiratory problems / deflate lung


Growing rods




Growing children / open triradiate cartilage

- avoid fusion / crankshaft phenomen

- biannual surgery

- high complication rate 50%

- hook dislocation

- rod breakage


Costoplasty / Thoracoplasty




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




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






0.32% in posterior corrections (SRS) in adolescents 

- 2% in adults 

- highest in congenital curves



- SSEP's monitoring in all idiopathic and congenital curves 

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



- 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%


- 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





- 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




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



< 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




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°




Exclude congenital scoliosis





- 20% incidence intraspinal pathology

- syrinx / tethered cord / diastematomyelia / ACM




Non operative




Curve < 25o and RVAD < 20o

- resolve spontaneously

- no need for treatment


Curve > 25o and RVAD > 20o

- brace




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






Curves > 35o

Progressive curves 5o in 6/12 




Patient < 10 years or before PHV

- high risk crankshaft effect 

- fusions must be anterior and posterior




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





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



- Lisch nodule (NF)

- blue sclera (OI)

- cloudy cornea (mucopolysaccharidases)

- dislocated lens (Marfan's)

- optic glioma



- abnormal teeth (OI)

- high-arched palate (Marfan's)



- pectus carinatum or excavatum (Marfan's)



- hemihypertrophy

- dolichostenomelia (long limbs)

- arachnodactyly (thumb in palm)

- clubfoot - often first sign of dysraphism

- cavovarus foot






Thoracic kyphosis - reduced / hypokyphotic


Lumbar lordosis - exaggerated, normal or reduced


Protruberant abdomen





- right or left


Balanced / Unbalanced 

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


Shoulder height


Scapular symmetry


Loin creases / lumbar fullness



- 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














- hamstring tightness



- reflexes UL / LL / Abdominal / Babinski

- sensation

- power UL / LL


Scoliosis + °Abdominal reflexes & °Axillary sensation 

- syrinx till proven otherwise


Abdominal reflexes disappear during teens








Ligamentous laxity / Wynne Davies criteria



- if forgotten 

- natal cleft / mouth / eyes / axillae


Cephalad joints

- ROM if deformity






"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



Prevalence of 25-100%



- walking decreases incidence



- worse with young onset




Exact biomechanical explanation of origin & progression of curves unclear


Associated with

- weakness 

- poor muscle control

- no proprioception




1. Neuropathic



- CP 

- Friedrich's

- cord tumour / trauma




- 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




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






Maintain cardiorespiratory function

Promote mobility & ambulation

Preserve sitting balance

Improve cosmesis




Brace till 12 years then fuse


Earlier if

- lose control with rapid progression

- large curve






Small curves < 30°

Large curves in patients with very poor prognosis




Best overall initial treatment

- rate of progression slowed

- allows further spinal growth before definitive treatment



- 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



- 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


- custom fitted support made from patient mould

- provides maximum sitting support with padded supports & belts




Neuromuscular Scoliosis Posterior Fusion




Solid arthrodesis of balanced spine over level pelvis




Long, solid fusion from upper thoracic to lower lumbar

Rigid instrumentation & massive bone grafting




Increased bleeding

Osteopenic bone / Poor hold

Fusion to pelvis

High pseudarthrosis rate

Poor medical state



- 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




General medical workup

- cardiopulmonary status

- especially Friedreich's & Duchenne MD

- FVC1, FEV, ABG's


Book ICU bed





- 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





- spinal cord monitoring if indicated

- cell saver

- post op ICU bed or HDU



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




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



- careful fluid balance 



- 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




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




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





- curves up to 30˚ in the growing child

- curves up to 50˚ in skeletally mature



- often only postpones arthrodesis until puberty

- seating most common form of non-operative treatment instituted

- TLSO effective in ambulatory patient




Fusion to sacrum essential with pelvic obliquity

- Galveston technique (iliac wing rod fixation)

- pedicle screws only viable in S1, not S2


Spina bifida




>60% of patients with myelomeningocoele develop scoliosis




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?





- often use peg

- tends to disappear into skin folds

- hygiene & technical difficulties

Acid reflux when hunched over

Only tolerate small feeds



Pressure areas

Ability to nurse

Takes arm off chair so can do ADL's

Allows stable sitting

Pelvic obliquity

Allows coverage of hips



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




Curve 20-40˚


- 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




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




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




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




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




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






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




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



- Short pedicles

- narrow interpedicular distance 

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

- most commonly L1-S1




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



Vertebral bodies grow axially by endochondral ossification. 

- radiation can inhibit this




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




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




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 Syndrome




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




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




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




Non operative


Brace treatment



- most patients fail brace treatment

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






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







- high incidence of malformations

- need good pre op work up


Superior mesenteric artery syndrome

- high incidence if put in jacket











2% scoliosis patients have NF


10 - 30 % NF patients have spinal deformity

- most common skeletal manifestation

- mostly non dystrophic variety




A.  Non dystrophic

- vertebral wedging, angulation and rotation

- very similar to idiopathic scoliosis

- right thoracic


B.  Dystrophic



- 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



- 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




Non Operative Management


Brace treatment


Not been effective in dysplastic curves

Can use early in non dysplastic


Operative Management


Non dystrophic




Recommend posterior fusion for progressive

- lower threshold for surgery than in idiopathic

- potential for progression much higher


Surgery as for idiopathic




Brace 20 - 35o

Surgery > 35o






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




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




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



- in dystrophic







Osteogenesis Imperfecta



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


Risk factors


Severe disease with nonambulatory status




Scoliosis in OI may progress after skeletal maturity 

- may be related to weakened osteoporotic bone 




Non operative




Poor results

- risk of rib fractures




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



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




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









- leg length discrepancy

- hip flexion contracture



- teenage females

- no rotation or wedging on XR



- painful condition of spine

- fracture, tumour, infection



- childhood

- slight correctable curve

- disappears on recumbency



- irritation of nerve root by HNP or tumour

- secondary to paravertebral muscle spasm




Idiopathic  75%


Commences before skeletal maturity

- diagnosis of exclusion


No features to categorise it as 

- Congenital

- Neuromuscular

- Other 



- Infantile < 3

- Juvenile 3 - 10

- Adolescent 10+


Neuromuscular 10%



- UMN:  CP, FA, trauma

- LMN:  Polio, SMA, Spina Bifida 



- Muscular Dystrophy

- Myotonica


Congenital (10%)


Failure of Formation

- wedge vertebra

- hemivertebra


Failure of Segmentation

- unsegmented bar

- block vertebra





Other (5%) NAOMI




Skeletal dysplasias

- Achondroplasia


- Mucopolysaccharidoses

- Diastrophic Dwarfism


Osteogenesis Imperfecta


Collagen disorders

- Marfan's

- Ehlers Danlos


Traumatic - fractures, surgical


Infective - vertebral osteomyelitis





- rickets, juvenile osteoporosis, osteogenesis imperfecta


Tumour - osteoid osteoma




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




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




Mainly in lumbar spine / can occur in thoracic




Can lead to cord tethering with associated neurological defects




Hyperreflexia / clonus / asymmetric abdominal reflexes




Inter-pedicular widening / bony bar / spina bifida occulta




May see 2 hemicords

- each within a single dural sac

- each within their own dural sac


Conus Medullaris often extends below L2










Should be resected



Spina Bifida General Principles



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




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 




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




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)



- 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





- without repair nearly 100% will die

- especially in pre-antibiotic day

- increase survival to 50% at 25 years

- antibiotics / selective closure / shunting



- 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



- only 30% are functionally independent as adults

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

- 65% will have normal intelligence



- 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



- 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



- incontinence 

- hydronephrosis 

- recurrent UTI



- 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 



- muscle imbalance 2° to UMN or LMN lesion

- intrauterine posture

- habitual posture after birth

- congenital malformation (eg club foot)

- arthrogryposis



- scoliosis

- hip FFD / Dislocation

- Knee hyperextension / flexion contracture / valgus

- teratological CTEV 

- cavus foot

- charcot foot

- ulceration






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 




L1 Level



- iliopsoas grade 2 or better / some weak hip flexion



- Hip flexed, abducted & ER



- HKAFO - hip knee ankle foot orthosis

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

- adults usually wheelchair bound 


L2 Level



- iliopsoas, sartorius & adductors grade 3 or better

- hip flexion & some adduction



- hip flexed and adduction 

- FFD of hip may develop 



- most ambulate as children in HKAFO

- FFD may need to be corrected first 

- adults usually choose wheelchair 


L3 Level



- quadriceps grade 3



- hip flexed & adducted

- knee extended 

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



- 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  



- medial hamstring grade 3

- Tibialis anterior 3 or better

- quadriceps grade 5



- hip flexed & adducted

- knee extended

- ankle in varus due to T anterior



- walk in AFO 


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


L5 Level



- lateral hamstrings >=3

- G medius >=2

- T posterior >=3



- calcaneovalgus or calcaneus foot 

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



- 95% community ambulators throughout life 


Sacral Level 



- Gastroc/Soleus >/=2 

- G medius >/=3

- G maximus >/=3



- clawing of toes & cavus foot from intrinsic minus



- 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




- no hip flexion

- no voluntary leg movement



- no ambulation

- hip flexed and ER

- feet equinovarus





- grade 2 psoas



- no ambulation

- hip adducted and flexed

- feet equinovarus





- grade 3 psoas



- hip adducted / flexed

- knee flexed

- feet equinovarus





- grade 3 quads



- hip adducted and flexed / highest risk hip dislocation

- household ambulator only with KAFO

- knee recurvatum / no hamstrings

- foot equinovarus





- > grade 3 quads

- medial hamstrings

- tibialis anterior grade 3




- hip adducted / flexed

- reasonable ambulation with AFO

- knee extended

- foot cavovarus





- lateral hamstrings

- grade 3 abductors



- hip flexed but not adducted

- community ambulator in AFO

- calcaneocavus foot





- grade 3 power T achilles



- 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





- 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 




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






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




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




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



- talectomy 

- triple arthrodesis near skeletal maturity




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




Usually less problem than varus 

- can be managed often with AFO

- may occurs in Ankle or Subtalar joint



- 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 




Pressure effects major problem 



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

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




< 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




Most common skeletal abnormality 80%

- more common in high lesions 




1. Congenital

- congenital spinal deformity 


2. Neuromuscular

- paralysis with nstability of posterior elements 


3. Neurological

- hydrosyringomyelia / malfunction of VP shunt

- tethered cord 



- temporary measure to delay fusion to allow trunk height development 

- pressure sore problems 




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




Specific problem in spine in spina bifida



- difficulty sitting in wheelchair 

- ulceration over kyphos 

- breathing difficulties 



- 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)




Usually corrected by FFD / hip correction




Spinal Dysraphism



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






Cord tethering


Cysts or lipoma of cord


Arnold Chiari Malformation

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




10-30% of population

- 25% no cutaneous stigmata

- clinically significant dysraphism is rare




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



- 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




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




Hemihypertrophy or hemiatrophy if small limb






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





- varying degrees of spina bifida

- vertebral anomalies








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









Progressive and  chronic disorder associated with cord cavitation & gliosis




Muscle wasting

Dissociate anaesthesia


Neuropathic arthropathy




M > F

No familial tendency




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




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




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




Cystic space

- lined by thick layer of glial tissue

- gliosis with tendency to infiltrate white matter




Craniovertebral anomalies





Cause of deterioration in Spina Bifida


Shunt malformation

Arnold Chiari

Cord Tether


Neuroenteric cyst



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





Base of skull




Most sensitive investigation

- main differential is oedema

- need T1 image


Look for obstructions


- spinal cord tumours




Amyotrophic Lateral Sclerosis / Motor Neurone Disease


Cervical Myelopathy

Thoracic Outlet Syndrome

Klippel-Feil Syndrome

Cord Tumour

Brainstem Infarct


Operative Management




Rapid progression of neurology

Short History

Respiratory compromise

Pseudobulbar Palsy

Pre-spinal corrective surgery





- 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



- infection

- peripheral neuropathy - DM / Leprosy / Tabes dorsalis




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



- 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



Conus medullaris below L2 / tethered

- unable to move freely with movement / growth




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

Intradural lipoma



Surgery to close myelomeningocoele

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




Stretches and damages the cord as child grows

- neurology

- pain

- difficulty walking

- foot deformity


Rapid progression of scoliosis




80 - 90% of children will have cutaneous manifestation






Open surgical release



- child

- neurological symptoms

- progressive scoliosis










Spinal Fractures

Cervical spine Fractures





- < 1% children's fractures


< 7

- upper cervical

- craniocervical junction


> 7

- lower C spine predominate




Very big heads

- will flex neck on spinal board

- need bump under T spine or

- cut out for head




Beware distracting injuries

- cannot clear C spine clinically



- normal mental state

- no distracting injuries

- not intoxicated

- able to adequately communicate

- no neck pain or tenderness

- Full ROM






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





- 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




Show disruption of endplate / disc junction

Ligamentous injuries





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




< 8

- mobile neck

- ligamentous laxity

- shallow facets

- big head


Usually falls and MVA





- 3 primary

- body and two arches

- fuse age 7



- 4 primary

- body, 2 arches, dens

- dens fuses age 6

- summit ossification appears 3-6, fuses 12




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



- 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



- occurs at the synchondrosis

- intact anterior periosteal sleeve



- MVA deceleration injury



- neurological defects rare



- anterior displacement



- reduce with extension and application HTB

- 50% apposition required

- non union rare


Lower C spine




Neurocentral synchondroses fuse 3-6

Bodies wedge shaped until become square at 7

Superior / inferior cartilage end plates attached to disc




Fractures occur between cartilaginous end plate and vertebral body

- between hypertrophic and calcified zones


Thoracic spine fracture



- protected by rib cage



- 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