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.ofalveoli(x10⁶)
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 1980
80% 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 & Luque trolley

46.5mm 27.3mm
Age at 3 yrs Age at 9 yrsAge 3
Spinal Growth 2 cm

2 cm growth for 6 years
27.3mm
46.5mm
Growth 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
VEPTER
Thoracic insufficiency
syndrome , cog chest
Wall deformities
Shilla
Construct
Hybrid
Construct
H Bar
Construct
Growing Rod Constructs
Vertical Expandable
Prosthetic Titanium Rib
Distraction Growth Guided

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

EONMS
Neuropathic
Lower Motor Neuron
 Spinal Muscular Atrophy
 Poliomyelitis
 Traumatic
 Dysautonomia
SMA

EONMS
Myopathic
 Muscular Dystrophy
 Congenital Hypotonia
 Myotonia Dystrophica
 Arthrogryposis
 Fibre Type Disproportion
CMD

Classification
Brown & 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 Principles
Neuromuscular
 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

Spinal Muscular Atrophy
 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 definitive
growing rod constructs based on sublaminar
wiring system
 These constructs have enabled me to achieve
and 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

90˚ 80˚
A D

65˚
Type 2
A R

07.10.2009
18.3 mm
19.03.2012
44.7 mm
26.4 mm  2½ years
Spinal Growth

50°
60°
80°
T C
Congenital Hypotonia

A & S B
Congenital Muscular dystrophy

AB

AB
70˚
60˚

SB

56⁰
70⁰
Congenital Muscular Dystrophy

07.10.2009 19.03.2012Spinal Growth
30 mm3 years

07.10.2009 15.09.2012
Spinal Growth
30 mm3 years
22mm 52mm

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
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 Segmented IncarceratedNonsegmented
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
Defects of Formation
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
Fully 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 by
skeletal maturity
 Two opposing fully segmented hemivertebrae
are more common

Cong Scoliosis
Semi-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
Non-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
Incarcerated 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
Wedge-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
Defects of Segmentation
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 Early Onset 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

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

Rhys Hart, 12 y/o M
K1738918
10 cm
T11 PVCR
T2–Ileum segmental
instrumented fusion

 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 Scoliosis
Lenke Classification
 The Lenke Classification is a complex,
accurate and reproducible . It relies on
measurements taken from standard x-rays
 X-rays of the patient from the AP, LAT,
and in bending positions are measured
and 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)

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
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Prader willi Syndrome

Early Onset
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