Early onset scoliosis

H Mehdian MD , FRCS

Consultant Spinal Surgeon

The Centre for Spinal Studies and Surgery, Queen’s Medical

Centre, Nottingham

Management of Paediatric Spinal

Deformity

Paediatric Spinal Deformity

1) Early Onset Scoliosis

2) Late Onset Scoliosis

a) Idiopathic Scoliosis

b) Neuromuscular Scoliosis

c) Congenital Scoliosis

d) Syndromic Scoliosis

Classification

Idiopathic Scoliosis

Old Classification

Infantile Onset < 3 yrs Age

Juvenile Onset 3-10 yrs Age

Adolescent Onset > 10 yrs Age

New Classification

Early onset Onset < 8 yrs Age

Late onset Onset > 8 yrs Age

Approximately 80% of patients with scoliosis have

idiopathic scoliosis

Classification

Early Onset Scoliosis

Definition Curves greater than 10 degrees in a child under the age of five years are defined as early onset scoliosis, this umbrella term includes:

1) Idiopathic Scoliosis

2) Neuromuscular Scoliosis

3) Congenital Scoliosis

1) Syndromic Scoliosis

The first five years of life are crucial as the lungs

are still growing dramatically

Constriction of the chest cavity as a result of a

spinal deformity significantly restricts lung

growth and may contribute to serious pulmonary

complications



Scoliosis

New classification reflects the importance of

lung development up to age 7

At birth 20 million alveoli

Age 5 300 million alveoli

Early Onset

Lung development

4 8

300

250x10⁶200

100

Age(year)Birth

No

. of

alve

oli

(x 1

0⁶)

300x10⁶

20x10⁶

Early growth disturbance compromises thoracic volume



75% of the curves , resolve spontaneously

25% of the curves, progress very rapidly

(malignant)

• Identify the malignant curves, prevent

development of respiratory compromise

Features

• Male

• Left sided Thoracic curve


Early Onset

Early onset


Predicting Progression


Rib vertebral angle measurement and its significance

was brought in by Min Mehta

Measurement of this angle has an

important implication in infantile Idiopathic

scoliosis as it differentiates between

progressive and resolving type of scoliosis

A curve with an initial RVAD of 20°

or more is considered progressive

RVAD > 20°

Mehta 1972

Predicting Progression

RVAD > 20°

Mehta 1972

Convex RVA < 68°

Kristmundsdottir 198080% 85%

Observation• Differentiate between Resolving Curves, progressive

Bracing, serial casting• Is an attempt to stop progression of the curve but does

not improve the curve.

Early onset


Treatment


Operative Treatment

• Convex Stapling Smith 1954

• Posterior Fusion Scott 1956

• Unilateral Growth Arrest Roaf 1963

• Concave Costoplasty Piggott 1971

• Convex Epiphysiodesis Roaf 1977

• Segmental Instrumentation Luque 1980

Convex Epiphysiodesis


Convex Epiphysiodesis & Luque trolley

46.5mm27.3mm

Age at 3 yrs Age at 9 yrsAge 3

Spinal Growth 2 cm

2 cm growth for 6 years

27.3mm

46.5mmGrowth per year 3 mm

Growth per level 0.25 mm

Conclusion

• It appears that convex epiphysiodesis has

a tethering effect on growth phenomenon

and therefore should be avoided when

growing constructs are used.

Surgery

progressive curves (Growing construct)

Spinal fusion is not an option, because the thoracic height

would cease to develop and lung development would be

restricted

Early onset


Treatment


Consequences of Premature Spinal Fusion in a Case of Early Onset Scoliosis

Centre for Spinal Studies and Surgery Nottingham

15 year old female

4 months Serial Casting

3 years Uninstrumented

Convex Epiphysiodesis

7 years Post Fusion T3-L3

Residual spinal deformity

Back pain & costo-pelvic impingement

Breathlessness & difficulty of ambulation

Disproportionately small thorax

FVC 17% of predicted value


Main Complaints & Clinical Examination


Casting

Failed to control progression of the deformity

Premature fusion

Resulted in thoracic & respiratory insufficiency

Surgical options now

Too late for surgery to effect respiratory function?

Will respiratory function permit further surgery?

Outcome is premature death

• A

• SSSurgi Centre for Spinal Studies and Surgery Nottingham

What to do next, if anything?


Double Growing Rod Construct

VEPTERThoracic insufficiency syndrome , cog chest

Wall deformities

ShillaConstruct

Hybrid Construct

H Bar Construct

Growing Rod Constructs

Vertical ExpandableProsthetic Titanium Rib

Distraction Growth Guided

Early Onset


Early Onset


Early Onset


Early Onset


Early Onset

Neuromuscular Scoliosis

Neuromuscular

Spinal deformity develops in majority of the

Patients with neuromuscular disease

Deformity Early in life

Progression Common

Classification (SRS)EONMS

Upper Motor Neuron

Cerebral Palsy

Syringomyelia

Spinal Cord Trauma

Spinal Cord Tumour

Spinocerebellar Degeneration

Neuropathic

CP


Neuropathic

Lower Motor Neuron

Spinal Muscular Atrophy

Poliomyelitis

Traumatic

Dysautonomia

SMA


Myopathic

Muscular Dystrophy

Congenital Hypotonia

Myotonia Dystrophica

Arthrogryposis

Fibre Type Disproportion

CMD

ClassificationBrown & Swank

Location of the lesion in the neuromuscular system

Abnormal Muscle Activity

(Scoliosis)

Spastic

Brain, Cerebellum,

Upper motor neuron

Flaccid

Anterior horn cell,

Motor neuron , Primary

muscle diseases

EONMS

General PrinciplesNeuromuscular

Progression

Pulmonary dysfunction

Cardiomyopathy

Urinary tract disease

Pressure sore

Hip dislocation

Patients with neuromuscular scoliosis present

with significantly different and more complicated

problems than those with idiopathic scoliosis

Neuromuscular

Osteoporosis

External support

Post-operative ventilatory support

Post-operative management

Blood loss

The management of these patients by surgical

means may be associated with much greater risk

Surgical Risks

Neuromuscular

Correction of the spinal deformity

Maintain the correction during the growth period

Allow spinal growth and lung development

Prevent progressive deterioration of P.F.

Avoid the need for definitive fusion at an early age

Goals of Surgery

Neuromuscular

Observation

Orthosis

Operation

The modalities of treatment in neuromuscular

scoliosis are similar to those of idiopathic

scoliosis

Treatment

Neuromuscular

Attempts at prophylactic or early bracing

have not prevented curve development and

progression.

An immediate effect of this will be to cause

difficulty breathing and restrict growth

of the chest wall in the longer term.

Orthosis & Seating Systems

Arterial and central lines

Urinary catheter

Temperature probe

Blood warmer

Heating blanket

Controlled hypotension

Cell saver

Spinal cord monitoring

Experienced anaesthetic input is essential in

major spinal surgery

Surgery

Neuromuscular

Pre-op Assessment

Chest Physician

Neurologist

Anaesthetist

Physiotherapist

Spinal Surgeon

Multidisciplinary review

Early Onset

Neuromuscular Scoliosis


Autosomal recessive

Degeneration of anterior horn cells

Single gene responsible (chromosome 5)

Scoliosis is present in more than 70% of

patients with SMA. Those with type 2 and 3

are the most common group presenting to

spinal surgeons and often present at a

young age.

Neuromuscular

Type 1 : Acute Infantile (WH) Onset 6 months

Type 2 : Chronic Infantile (WH) Onset 4 years

Type 3 : Juvenile form (K W) Onset 2-15 years

Type 4 : Distal SMA Onset 7-15 years


Various surgical techniques have been employed

to treat EONMS

Limitations :

Lack of segmental control

Loss of sagittal balance

Multiple surgeries

High rate of complications

EARLY ONSET NEUROMUSCULAR SCOLIOSIS

I have been using two different definitivegrowing rod constructs based on sublaminarwiring system

These constructs have enabled me to achieveand maintain the correction during spinal growth.

Sliding H Bar Construct

Hybrid Construct

Definitive Self Growing Rod Constructs

Screw + Wire Construct

Sliding H Bar Construct

Self Growing Rod Constructs

Neuromuscular

Segmental Spinal Instrumentation using short closed wire loops

H Mehdian, Clinical Orthopaedics, 1989, 247

Sliding H-Bar Construct

Self Growing Rod Constructs (SMA)

1994


1996 1997

30°10°

Age 7

19981997 1999 2000

Age 7 Age 9Age 8 Age 10


2001 2002 2010 2010

Age 11 Age 20Age 12 Age 20

Post- op: 13 yrs

Spinal G: 12 cm

PJK: -

Preserved: TK, LL

Surgery: 1


19981997 1999 2000 2001 2002 2010 2010

Spinal growth ( 12cm)

Age 7 Age 8 Age 9 Age 10 Age 11 Age 12 Age 20 Age 20


Screw + Wire Construct


AD


90˚ 80˚

A D



AR


65˚

Type 2

A R


07.10.2009

18.3 mm

19.03.2012

44.7 mm

26.4 mm 2½ years

Spinal Growth

TC


T C


50°

60°

80°

T C


A & S B

Congenital Muscular dystrophy

AB


AB

70˚

60˚


SB


56⁰70⁰

Congenital Muscular Dystrophy

07.10.2009 19.03.2012Spinal Growth

30 mm 3 years

07.10.2009 15.09.2012

Spinal Growth

30 mm 3 years

22mm 52mm

4 Years 4 cm Growth

29.09.2008 20.01.2014

4 Years and 8 months 5 cm Growth

Definitive growing construct appears to be more

advantageous over other systems in patients with

early onset neuromuscular scoliosis, it eliminates the

need for further surgeries

The ideal design of implants for the treatment of

patients with EOS should have the following

characteristics:

Eliminates the need for recurrent lengthening

Provides good fixation

Maintains sagittal curvature of the spine

Conclusion

6th International Congress on

Early Onset Scoliosis & Growing Spine

November 15-16, 2012- Dublin, Ireland

Best Paper

Early Onset

Congenital Scoliosis

The spinal column develop at the same time as several

other major organ systems such as the bladder, kidneys

and heart.

Cong Scoliosis

Classification


Based on the embryological development

of the spine

Defects of Formation

Defects of Segmentation

Mixed

• MRI shows abnormalities in 26%

• Syrinx

• Chiari Malformation

• Tether cord

• Diastomatomyelia

• Single kidny

Imaging / Associations

Neuromuscular Congenital Scoliosis

Neuraxial Anomalies

Bracing

• Proven little value for congenital curves

Indication for Surgery

• Unacceptable deformity

• Bar + contralateral hemivertebra

• Progression >10° in one year

Surgical Options

• Posterior fusion

• Anterior and posterior fusion

• Convex hemiepiphyseodesis

• Posterior vertebral resection

Classification

Failure of formation:

Means one or more vertebrae become

partially or fully triangular

Failure of segmentation:

Means one or more vertebrae are

abnormally connected together

Failure of formation

Fully SegmentedIncarceratedNonsegmented

Semi

Segmented

Wedge Vertebra

Failure of Segmentation

Bilateral Failure of

Segmentation

Unilateral Failure of

Segmentation,unsegmented bar

Unilateral Failure of

Segmentation, unsegmented

bar + contralateral hemi

vertebra

Cong Scoliosis



A lateral defect of vertebral formation can vary from

mild wedging to the complete absence of half of the

vertebra (Hemivertebra)

A hemivertebra is one of the most common causes

of congenital scoliosis

Hemivetebra consists of half of the vertebral body, a

single pedicle, and hemi-lamina

Cong Scoliosis



Hemivertebra

Four different types of hemivertebrae

Fully Segmented Most common

Semi segmented Less common

Non-segmented Least common

Incarcerated Least common

Cong Scoliosis



Fully Segmented Hemivertebra

At the thoracolumbar region the deformity

can exceed 45⁰ at skeletal maturity

Cosmetic deformity is moderate

At the lumbosacral junction, cosmetic deformity

is major

Cong Scoliosis


Defects of FormationFully Segmented Hemivertebra

Two fully segmented hemivertebrae on the

same side of the spine are less common

All exceed 50⁰ by 10 years of age

Without treatment these could reach 70⁰ byskeletal maturity

Two opposing fully segmented hemivertebraeare more common

Cong Scoliosis


Defects of FormationSemi-Segmented Hemivertebra

Is synostosed to its neighbouring vertebra

Two growth plates are obliterated on this convexity

Can induce a slowly progressive scoliosis

Curves usually do not exceed 40⁰ at skeletal maturity

Treatment is required if hemivertebra occurs at the

lumbosacral junction

Cong Scoliosis


Defects of FormationNon-Segmented Hemivertebra

Is synostosed to both of its adjacent vertebrae

Has no growth potential

There is no cosmetic deformity

No treatment is required

Cong Scoliosis


Defects of FormationIncarcerated Hemivertebra

Ovoid in shape

Smaller than a fully segmented hemivertebra

The vertebrae above and below tend to compensate

for the hemivertebra

There is minimal scoliosis

Scoliosis rarely exceeds 20⁰ at skeletal maturity

Cong Scoliosis


Defects of FormationWedge-vertebra

Rare cause of congenital scoliosis

Partial failure of a vertebra to form on one side

Scoliosis deteriorates relatively slowly

Surgical treatment may occasionally be required

Cong Scoliosis


Associated Deforming Features

Upper thoracic curve:

Significant cosmetic deformity

A 30⁰ curve upper limit of acceptability

Cong Scoliosis



unilateral

Unsegmented bar

Cong Scoliosis



The unsegmented bar does not contain growth plates

Some degree of growth continues on the opposite side

Rib fusions are often seen adjacent to the unsegmented

bar

Unilateral unsegmented bar

Cong Scoliosis



Unilateral unsegmented bar

On average, these curves deteriorate at a

rate of 5⁰ a year

The great majority will exceed 50⁰ by 10

years of age

Cong Scoliosis



Unilateral unsegmented bar with contralateral

hemivertebrae

Is seen most clearly in the first few years of life

This type of anomaly produces the most severe

and rapidly progressive of all types of

congenital scoliosis

Cong Scoliosis



Unilateral unsegmented bar with contralateral

Hemivertebrae

On average, these curves deteriorate 6⁰ or

more a year

All exceed 50⁰ by 4 years of age

If untreated, at an early age can lead to

cor pulmonale

Cong Scoliosis


Treatment


Unilateral unsegemented bar with or without contralateral

hemivertebrae

Fusion should be performed as soon as the anomaly

is recognized

The best results are achieved when surgery is performed

before the age of 2 years

Cong Scoliosis



Bilateral

Block Vertebra

Congenital Kyphosis

• Type I: Failure of formation (hemivertebra)

• Rapidly progressive

• May produce paraplegia

• Type II: Failure of segmentation (bar)

• Less progressive

• Does not produce paraplegia

Congenital EO Scoliosis

Congenital Early Onset Scoliosis

Congenital EO Scoliosis

Congenital Kyphosis

Prognosis

• Most patients have slight short stature

• 70% risk of progression

• Progression greatest after 10 years of age

• May cause cor pulmonale

• Congenital kyphosis may cause paralysis

Congenital Kyphosis

Neuromuscular

Management of Kyphosis (Kyphectomy)

Kyphectomy

Congenital Early onset Scoliosis

Early onset


Spinal Growth

• Averages 0.07 cm/year per segment

• Anomalous segments will never have this

degree of growth

• Spinal growth is two thirds complete

by age 6

Early onset


Early onset


Rhys Hart, 12 y/o MK1738918

10 cm

T11 PVCRT2–Ileum segmentalinstrumented fusion

120º

72º

The Segmental Self Growing Rod Construct

is a powerful, definitive technique for the

management of early onset scoliosis

Construct Advantages:

Excellent correction

Maintain the correction during growth period

PJK is prevented

Sagittal contour of the spine is well preserved

Maximum spinal growth and thoracic

development is achieved just with one surgery

Conclusion

Adolescent Idiopathic Scoliosis

Natural History

• Progression related to maturity and curve size

• Risk of progression increases strongly at in an

immature patients

• Curves> 45° should be considered for surgery

• Pulmonary compromise > 75° to 100°

Initial Evaluation

Whole Spine X-rays (curve measurement)

Regular outpatient review

Progression more than 5° in curves between

20°-25° in patients with ( Risser 0-3)

Brace treatment for curves > 40° to 45° has very

lower success rate

Brace Indications

Brace Types

• Thoracolumbosacral (TLSO) TLC

• Milwaukee Brace high thoracic curves

• Charleston Night Brace Single curves 25°

Preoperative assessment

MRI indicated if:

Left Lower thoracic curve

Significant back or neck pain

Neurological abnormality

Less than 10 years of age

Late Onset ScoliosisLenke Classification

The Lenke Classification is a complex, accurate and reproducible . It relies onmeasurements taken from standard x-rays

X-rays of the patient from the AP, LAT,and in bending positions are measuredand evaluated

Each scoliosis curve is then classified in three ways


Curve type (1-6) : proximal thoracic, main thoracic and

thoracolumbar/lumbar

Lumbar modifier (A, B, C) lumbar

modifier based on the distance of

the center of the lumbar spine to

the midline


Sagittal thoracic modifier (-, N, +)

Sagittal thoracic modifier based on the

amount of thoracic kyphosis

The most common type is a 1AN curve

Type 1 B,N ( Main Thoracic)

Seg Screw Fixation

68º

Type 1 B,N (Main Thoracic)

Seg Screw Fixation

85º

Type 1C,N (Main Thoracic )

Seg Screw Fixation

90º

60º

Type 3C,N ( Double Major )Seg Screw Fixation

78º

72º

Type 3 C,N (Double Major)

3C

Seg Screw Fixation

78º

75º

Type 6C,N (Thoracolumbar)

Seg Screw Fixation

85º

75º

Image Free technique

Type 6C,N (Thoracolumbar)

Seg Screw Fixation Type 3C,N ( Double Major )

80º

78º

Seg Screw Fixation

Type 1 C,N (Main Thoracic)

Seg Screw Fixation

Seg Screw Fixation

Seg Screw Fixation

Paediatric spinal deformity surgery should be

performed in a specialist centre where a high

volume of procedures are performed

Good medical support staff, including

experienced paediatric anaesthetists, are an

essential part of the team dealing with these

children with deformity

Conclusion


EOS TO REMAIN NORMAL

• Weight: 40 kg

• T1-T12= 22 cm

• VC: more than 50%

Early Onset

Syndromic Scoliosis

Prader willi Syndrome

Early Onset

Syndromic Scoliosis

Early Onset

Syndromic Scoliosis

Early onset scoliosis

Health & Medicine

late onset scoliosis

yrs agelate onset onset

alveoliearly onset

type of scoliosis

yrs agejuvenile onset

yrs ageadolescent onset

infantile idiopathic

spinal growth