Scoloisis

SCOLIOSIS
DR.ARJUN REDDY
(MS.ORTHOPAEDICS )
FINAL YEAR

INTRODUCTION
 The scoliosis research society defined scoliosis as lateral curvature of the spine
with an element of axial rotation when measured on radiograph should be
greater than 10 deg as measured using the cobb method on standing
radiograph
 Scoliosis is a complex three-dimensional deformity characterized by coronal
,sagittal and horizontal plane deviation
 Motion of the vertebra in one plane is associated with motion in another plane
,this coupling effect almost always results in rotation of vertebra within the
scoliotic curve

HISTORY
 The term scoliosis comes from a Greek word meaning “CROOKED”
 There are references of scoliosis in hindu mythology, in the BHAGAWADGITA
where LORD KRISHNA ,straightens the spine of kubja, his devotee,by pressing the
feet down and pulling her chin upwards(axial traction)
 Hippocrates coined the term scoliosis ,he did pioneering work in the field of
scoliosis ,including methods like application of axial traction and transabdominal
correction
 In,1510 ,French surgeon Ambriose pare advocated the use of extension and
pressure against the body to treat scoliosis
 In ,1839, Jules Guerin was the first to practice “myotomie rachidienne”, a
transsection of paraspinal muscles to treat scoliosis

 In 1878, Lewis A Sayre, an orthopaedic surgeon ,argued that the superior
treatment of the disease would be to have patients suspended themselves in a
jacket made out of POP
 In,1911,Fred Albee was the first to carry out a fusion surgery(spondylodesis
procedures)
 In,1962,Paul R Harrington ,an american orthopedic surgeon,developed and
introduced the Harrington rod system ,that revolutionized surgical scoliosis
surgery

ANATOMY
VERTEBRAL COLUMN
 Consists of
 7 cervical vertebrae
 12 thoracic vertebrae
 5 lumbar vertebrae
 5 sacral vertebrae(fused)
 4 coccygeal vertebrae(fused)

 Purpose of vertebral column:
 protect the spinal cord and spinal nerves
 support the body weight and head
 provide movement and posture

TYPICAL VERTEBRA
 Typical vertebra is by convention thoracic
 Parts of a vertebra
 A) body-anterior ,transmits weight
 B) vertebral arch –posterior ,surrounds vertebral canal,spinal cord and
consists of
 1)pedicles –project from the body
 2)laminae- unite to form arch posteriorly
 C) transverse an spinous process
 D) intervertebral foramina bordered by –superior and inferior vertebral
notches
 E) superior and inferior articular processes

 Cervical and lumbar vertebrae are
more similar to each other
 Both being lordotic
 Vertebral bodies are broader from
side to side/than from back to
back
 Described as kidney shaped
 Has a considerable range of
movement

 Thoracic vertebral bodies are:
 Longer from back to back
 Described as heart shaped
 Thoracic region is kyphotic
 Thoracic spine with its attached rib cage
is relatively rigid in flexion and extension

SPINAL CURVATURES
Provide a shock absorbing capacity to the spine
 PRIMARY CURVATURES
 Develop during fetal life
 Thoracic kyphosis-concave ant
 Sacral kyphosis-concave ant
 SECONDARY CURVATURES
 Develop during infancy
 Cervical lordosis-concave post
 Lumbar lordosis-concave post

CLASSIFICATION
 The great proponderence of scoliosis is idiopathic
 Scoliosis can be classified into 3 groups
A) NONSTRUCTURAL SCOLIOSIS
B) TRANSIENT STRUCTURAL SCOLIOSIS
C) STRUCTURAL SCOLIOSIS

A)NON STRUCTURAL SCOLIOSIS
1) Postural scoliosis- this is usually noted in the later years of the first
decade
The curves are always slight and disappear on lying down.
2) compensatory scoliosis-this is usually due to leg-length discrepancy
The pelvis dips down to the short side.
B)TRANSIENT STRUCTURAL SCOLIOSIS
1)Sciatic scoliosis –this is not true scoliosis. It is an irritative scoliosis due to
pressure on nerve roots from an herniated disc
2)Hysterical scoliosis-this is rare and usually requires psychiatric treatment

 3) inflammatory scoliosis-this is seen with perinephric abscess or similar
infection
C)STRUCTURAL SCOLIOSIS
1)Idiopathic scoliosis(about 70% of all scoliosis)
- the SRS recommends that idiopathic scoliosis be classifies according to
the age of the patient
a)infantile -under 3 yrs of age
b)juvenile -between ages 4 to 10 yrs
c)adolescent- between 10yrs to age of skeletal maturity

 2)Congenital scoliosis
 -a)vertebral
 1)open-with defect of posterior spine
 a)with neurologic defect(e,g. myelomeningocele)
 b)without neurologic defect(e,g .spina bifida occulta)
 2)closed-without defect of posterior spine
 a)with neurologic defect(diastomatomyelia without spina bifida)
 b)without neurologic defect(e,g. hemivertebra with unilateral
unsegmented bar)
 b) Extra vertebral (e,g. congenital rib fusions)

 3)Neuromuscular
a)neuropathic
1)Lower motor neuron(e,g.poliomyelitis)
2)Upper motor neuron(e,g. cerebral palsy)
b)Myopathic
1)progressive(e,g. muscular dystrophy)
2)Static(e,g. Amyotonia congenita)
3)others(e,g.Friedreich’s ataxia, unilateral amelia)
4)Neurofibromatosis(Von recklinghausens disease)

 5)Mesenchymal disorders
a)Congenital(e,g. marfans syndrome,arthrogyposis multiplex congenita,various
types of dwarfism)
b)Acquired(e,g. rheumatioid arthritis)
c)Others(e,g. scheuermann’s disease ,osteogenesis imperfecta)
6)trauma
a)Vertebral(fracture, irradiation , surgery)
b)Extravertebral(e,g. burns , thoracogenic)
7)secondary to irritative phenomina(e,g. spinal cord tumours)
8)others(metalbolic,nutritional,endocrine)

CLINICAL FEATURES
 One of the major complaints from parents and patients is cosmetic deformity
 Often painless
 Parents may notice abnormal posture in their growing child that includes
A tilted hed that doesnot line up over the hips
A protruding shoulder blade
One hip or shoulder higher than the other
An uneven neck line
Leaning more to one side than other
In developing girls, breasts appear to be of uneven size

EVALUATION OF SCOLIOSIS PATIENT
 HISTORY
 A history of the scoliosis patient includes the following
Chronologic age
Age at recognition of deformity
Impression of rate of progression of deformity
Associated symptoms : rate of growth , appearance of secondary sexual
characters
Genetic factors(e,g. similar deformity in other sibllings)

 PHYSICAL EXAMINATION
 A thorough physical examination is especially important in evaluating
cardiopulmonary function,studying growth ad skeletal age,and seeking out
underlying disease
Cardiopulmonary status is important in extreme thorasic curves,paralytic
curves ,and congenital scoliosis and includes a pulmonary study(ABG,lung
volume,breathing mechanics,blood-gas distribution)and a cardiac study
Growth factors include studies of the patient’s height compared with
parents and other sibblings,development of sec sexual characteristics,and of
dentition
Evidence of the underlying condition is sought.For example

 1)Spinal dysraphism may be suggested by cutaneous manifestations over
the lower back ,such as a pigmented hairy patch
 2)skin tumours and café au lait spots suggest neurofibromatosis
 3)congenital deformities must be considered
 EXAMINATION OF THE DEFORMITY
 The patient should be completely undressed and the following points
noted
 1)Trunk alignment
with the patient standing,the trunk is inspected from the posterior
aspect,and a plumb line is dropped.Normally,the head should be aligned
exactly over the guteal cleft

 Asymmetry of the body(e,g. in a single left thoracolumbar curve)shows
a)Left shoulder higher than right and carried forward
b)In folding of right loin and flattening of left
c)Prominent right iliac crest
d)thorasic cage lifted to left
e) Left side of chest more prominent than right
f)head to left of center of gravity
It should be noted that double major curves of equal degree,although
severe,produce balancing curves and therefore good alignment of the trunk

 2)symmetry of shoulder girdle
cervical or high thoracic curves cause asymmetry of the neck-shoulder angle
3)Assessment of specific curves: This includes following
a)Record the type of curve
b)Degree of flexibility and rigidity are noted when the patient bends to either
side.
c)Adams bending test: Prominances of thorasic and lumbar areas are
observed when the patient bends forward at right angle at the hips .This indicates
the degree of rotation and fixation of the curve

 Pelvic obliquity is either structural or nonstructural
The patient sits on a hard surface and any spontaneous correction of the
lumbar curve is noted
1)Non structural: due to habit pattern or a short leg on one side; lumbar
curve straightens when sitting
2)Structural: due to contractures above the crest;or to contractures below the
crest,either abduction or adduction contracture at hip causing apparent leg
lengthening or apparent shortening, respectively
4)Neurologic examination
Reflexes,sensation and motor power are recorded and repeated at
intervals to determine whether a neurologic defect exists and whether it is
progressing

SAGITTAL BALANCE
 Overall spinal sagittal balance is
determined by a plumb line dropped
from the dens
 This plumb line usually falls anterior to
the thoracic spine posterior to the
lumbar spine , and through the posterior
superior corner of S1
 On standing long lateral films generally
used in spinal deformity evaluation ,the
dens is not easily seen
 The plumb line therefore usually is
dropped from midline of C7 vertebral
body

ASSESMENT OF VERTEBRAL ALLIGNMENT AND
BALANCE
 Central sacral vertebral line(CSVL) is a
roughly vertical line drawn perpendicular
to an imaginary tangential line drawn
across the top of iliac crests on
radiographs.it bisects the sacrum
 CORONAL BALANCE is evaluated by
measuring the distance between CSVL
and plumb line.>2cm is abnormal
 SAGITTAL BALANCE is evaluated by
measuring the distance between
posterosuperior aspect of s1 and plumb
line

XRAY VIEWS
 Erect films include anteroposterior and lateral views of the spine from the occiput
to the sacrum
 Spot lateral and oblique views of the lumbosacral junction are taken to study this
area for spondylolisthesis or spondylosis
 Lateral bending films are taken to
1)determine the degree of flexibility of spine
2)to see how much each curve can be passively corrected
3)to distinguish structural curves from non structural

MEASUREMENT OF CURVES
 Measurement of curves is based on
determining the upper and lower end
vertebra
 The superior end vertebra is the last one at
the upper end of the curve that tilts
towards the concavity
 The inferior end vertebra is the last one at
the lower end that tilts towards the
concavity
 The apical vertebra is the one deviated
farthest from the vertebral column

 Two types of measurements are used
 COBBS METHOD;
 A horizontal line is drawn at the superior
border of superior end vertebra
 Another horizontal line is drawn at inferior
border of inferior end vertebra
 Perpendicular lines are then erected from
each of the horizontal lines ,and the
angles formed by intersecting lines are
measured

 RISSER-FERGUSON METHOD;
 A small dot is placed at the center of upper and
lower end vertebrae
 In addition ,a small dot is placed at the center of
the apical vertebra
 Straight lines are then drawn from the dot in
each end vertebra through the dot in the apical
vertebra , and the angle formed by intersecting
lines are measured

 The following groups have been standardized according to the degree of
curvature by SRS
GROUP DEGREE
1 0-20
2 21-30
3 31-50
4 51-75
5 76-100
6 100-175
7 126 AND UP

 DEGREE OF ROTATION;
 Rotation is an inherent part of , and reflects the degree of structural
change in, and the resistance to correction of the scoliotic curve,
 Two methods are used to interpret the amount of rotation
Displacement of spinous processes;
if the vertebra is rotated so that the spinous process is
displaces one “width” from the midline of the vertebra this is 1-plus
rotation

 Displacement of pedicles;
(Moe pedicle method)
When the vertebra rotates, one
pedicle moves towards the midline, and
its relationship to the midline that
determines the degree of rotation.
the other pedicle moves towards the
lateral border of the vertebral body
When one pedicle becomes situated
at the center of the vertebral body it is a
3 plus rotation

 DETERMINING MATURATION
 Scoliosis ceases to progress significantly when a patient is fully mature
 Therefore, determining maturation in boys and girls is essential in planning
treatment
 Girls generally cease growth and mature at about 16 ½ years of age
 Boys mature 1 o 1 ½ years later
 The following facts are indispensable when interpreting the age of maturity
Familial characteristics
study the parents and sibllings
secondary sexual characters
in girls,note menarche
bone age
compare a xray of the wrist with Greulich and Pyle atlas

 RISSER GRADING
(Excursion of Iliac apophysis)
ossification of the crest of the
ilium starts laterally and proceeds
posteriorly towards the sacrum
when the apophysis reaches
the sacro iliac junction and fuses to
the ilium,maturation is nearly
complete

 The peak height velocity (PHV) has been reported by
several authors to be a better maturity indicator than the Risser
sign, chronological age
PHV is calculated from serial height measurements and is
expressed as centimeters of growth per year. Average values of
PHV are 8 cm per year in girls and 9.5 cm per year in boys.
 The triradiate cartilage begins to ossify in the early stages of
puberty. In girls it is completely ossified after the period of
PHV and before Risser grade 1 and menarche.
 In boys it is in the early stages of ossification when puberty
begins.

IDIOPATHIC SCOLIOSIS
 Idiopathic scoliosis accounts for almost 70% of all the cases of scoliosis
The Scoliosis Research Society recommends that idiopathic scoliosis be
classified according to the age of the patient when the diagnosis is made
- Infantile scoliosis occurs from birth to 3 years of age
- juvenile idiopathic scoliosis, between the ages of 4 and 10 years
- adolescent idiopathic scoliosis, between 10 years of age and skeletal
maturity

INFANTILE IDIOPATHIC SCOLIOSIS
 Infantile idiopathic scoliosis is a structural, lateral curvature of the spine
occurring in patients younger than age 3 years.
 James, who first used the term infantile idiopathic scoliosis noted that these
curves occurred before 3 years of age, were more frequent in boys than in
girls, and were primarily thoracic and convex to the left.
 Wynne-Davies noted plagiocephaly in 97 children in whom curves developed
in the first 6 months of life ,the flat side of the head was on the convex side of
the curve. Other asossiated conditions that she found were intellectual
impairment in 13%, inguinal hernias in 7.4% of boys with progressive scoliosis,
developmental dislocation of the hip in 3.5%, and congenital heart disease in
2.5% of all patients.

 Most curves in infantile idiopathic scoliosis are self limiting and
spontaneously resolve (70% to 90%); however, some curves may be
progressive, usually increasing rapidly, are often difficult to manage, and
may result in significant deformity and pulmonary impairment.
 Mehta developed a method to differentiate resolving from progressive
curves in infantile idiopathic scoliosis based on measurement of the rib-
vertebral angle (RVA). She evaluated the relationship of the convex rib head
and vertebral body of the apical vertebra by drawing one line perpendicular
to the apical vertebral endplate and another from the midneck to the
midhead of the corresponding rib; the angle formed by the intersection of
these lines is the RVA
 The RVA difference (RVAD) is the difference between the values of the RVAs
on the concave and convex sides of the curve.

Mehta
reported that 83% of the curves resolved if
the RVAD measured
less than 20 degrees and that 84% of the
curves progressed
if the RVAD was greater than 20 degrees
She described
a two-phase radiographic appearance
based on the relationship
of the apical ribs with the apical vertebra. In
phase 1, the
rib head on each side of the apical vertebra
does not overlap
the vertebral body. In phase 2, the rib head
overlaps the
convex side of the vertebral body.

 Phase 2 curves are progressive, and therefore the measurement
of RVAD is unnecessary

 TREATMENT:
 Because of the favourable natural history in 70% to 90% of patients with
infantile idiopathic scoliosis, active treatment often is not required. If the initial
curve is less than 25 degrees and the RAVD is less than 20 degrees, observation
with radiographic follow-up every 6 months is recommended.
 Most resolving curves correct by 3 years of age; however, follow-up should
continue even after resolution because scoliosis may recur in adolescence

 CASTING
 Sanders et al. reported good results with early casting for progressive infantile
idiopathic scoliosis using the technique of Cotrel and Morel (extension, de
rotation, flexion) cast correction.
 Best results were achieved if casting was started before 20 months of age and in
curves less than 60 degrees.
 Cast correction in older patients with curves of more than 60 degrees frequently
resulted in curve improvement
 Casts were changed every 2 to 4 months based on age and growth of the child.
Once curves were corrected to less than 10 degrees, a custom-molded brace
was used.

 OPERATIVE TREATMENT:
 If a curve is severe or increases despite the use of an orthosis or casting, surgical
stabilization is needed.
 Ideally, surgery should not only stop progression of the curve but also allow
continued growth of the thorax and development of the pulmonary tree
 Growing rods may be used to control curve progression and still allow for
growth of the spine
 This usually requires surgery every 6 months to lengthen the rods
 the use of magnetically controlled growing rods , such as the MAGEC(magnetic
technology with adjustable growing rods and an external remote controller)
spinal bracing and distraction system ,avoids a return to surgery every 6 months

 VEPTR(vertical expandable prosthetic titanium rib) instrumentation has
been reported as another alternative to correct the curve and allow for
continued growth of the spine
 When surgical fusion is necessary, a relatively short anterior and
posterior arthrodesis should be considered, including only the
structural or primary curve.
 Combined anterior and posterior arthrodesis is necessary to prevent
the “crankshaft” phenomenon.
 The problem with this approach is that it leaves the child with a
straight, shortened spine rather than a deformed spine of near-normal
length

JUVENILE IDIOPATHIC SCOLIOSIS
 Juvenile idiopathic scoliosis appears between the ages of 4 and 10 years
 Multiple patterns can occur, but the convexity of the thoracic curve usually is to
the right.
 Juvenile idiopathic scoliosis comprises 12% to 21% of idiopathic scoliosis cases.
 The female-to-male ratio is 1 : 1 in children between 3 and 6 years of age. This
ratio increases with age, with the ratio of 4 : 1 from 6 to 10 years of age, and
reaches a female-to-male ratio of 8 : 1 by the time the children are 10 years of
age.
 Most juvenile curves are convex right thoracic curve or double thoracic curve
patterns and closely resemble those of adolescent idiopathic scoliosis

 Dobbs et al. modified the adolescent idiopathic scoliosis classification system of
Lenke for juvenile idiopathic scoliosis (There are the same six curve types, but
instead of using side-bending radiographs to distinguish structural from
nonstructural minor curves the authors used the deviation from the midline of the
apex of the curve from the C7 plumb line for thoracic curves and the center sacral
vertical line for thoracolumbar and lumbar curves.
 As in infantile idiopathic scoliosis, a high incidence of neural axis abnormalities
has been found on MRI in children younger than 11 years with scoliosis (26.7%).
Some may argue about the need for MRI in a routine preoperative workup, but
most would agree that specific factors indicating a need for further MRI evaluation
include pain, rapid progression, left thoracic deformity, neurological abnormalities
(alterations in the superficial abdominal reflex)

 TREATMENT:
 Although it is likely to progress and often
requires surgery, juvenile idiopathic scoliosis
is treated according to guidelines similar to
those for adolescent idiopathic scoliosis.
 For curves of less than 20 degrees,
observation is indicated, with examination
and standing posteroanterior radiographs
every 4 to 6 months. Evidence of progression
on the radiographs as indicated by a change
of at least 5 to 7 degrees warrants brace
treatment. If the curve is not progressing,
observation is continued until skeletal
maturity.

 Even if the curve progresses, bracing may slow progression and delay surgery
until the child is older, which may avoid a short trunk and lessen the possibility of
a crankshaft phenomenon.
 If orthotic treatment fails, operative management of the curve should be
considered.
 Important considerations in the operative treatment of patients with juvenile
idiopathic scoliosis are
1)the expected loss of spinal height and the limited chest wall growth and
lung development after spinal fusion.
2)important consideration is the crankshaft phenomenon.

 CRANKSHAFT PHENOMENON:
 With a solid posterior fusion, continued
anterior growth of the vertebral bodies
causes the vertebral body and discs to
bulge laterally toward the convexity and to
pivot on the posterior fusion, causing loss
of correction, increase in vertebral rotation,
and recurrence of the rib hump.

SURGICAL MANAGEMENT
 GROWING ROD INSTRUMENTATION:
 Growing rod instrumentation is a technique of posterior instrumentation that is
sequentially lengthened to allow longitudinal growth while still attempting to
control progressive spinal deformity
 Moe et al. described the use of a subcutaneous Harrington rod without fusion,
followed by a full-time external orthosis, in certain flexible curves in growing
children
 Complications, most frequently hook dislocation and rod breakage, occurred in
50% of patients.

 Currently ,growing rod techniques include,
1)a single growing rod
2)dual growing rods
3)VEPTR rods
4)Luque trolley
5)Shilla technique
Magnetically controlled growing rods ,such as MAGEC rods,provide a
noninvasive method for rod lengthening,avoiding further surgical procedures
 The growing rod techniques should be considered in a cooperative patient
with a stable family unit.
 This procedure usually is considered for patients younger than 10 yrs of age
who have a curve of 60 degrees

DUAL GROWING ROD INSTRUMENTATION
WITHOUT FUSION
 A multiple-hook segmental instrumentation that is appropriate for the child’s
size is used
 If the child weighs less than 30 lb, an infant spinal set may be necessary
 If the infant set is used, the rod is quite flexible, and therefore some additional
protection in the form of external immobilization is necessary until the system
can be converted to a pediatric rod system of a larger diameter

 POSTOPERATIVE CARE:
 The child is placed in an orthosis for the first 6 months.
 The rods routinely are lengthened every 6 months. Lengthening is performed
by exposing the connector and loosening the set screws.
 Distraction is applied, and the set screws are retightened.
 Lengthenings are stopped when no further distraction can be achieved.
 At that point, the patient then undergoes the final arthrodesis. The final
arthrodesis usually necessitates removal of the rods

SHILLA GUIDED GROWTH SYSTEM
 MCCARTHY ET AL.
 Careful assessment of upright coronal and sagittal films ,along with analysis of
the flexibility of the curve by bending or traction films,is necessary to
determine the location of apical vertebral segments
 The apical three or four vertebral segments that are least corrected through
flexibility testing are the apicals for fusion and maximal correction

 The most common complications of growing rods are:
1)hook displacement or failure of proximal or distal fixation
points
2)rod breakage
3)infection
4)skin breakdown over prominent rods
5)auto fusion of the spine

GROWING ROD ATTACHMENT USING RIB
ANCHORS

GUIDED GROWTH AND PHYSEAL STAPLING
 Growth modulation is an attempt to apply the principles of guided growth in the
lower extremities with physeal stapling
 Intervertebral stapling is used to produce a tethering effect on the convex side of
the spine
 This tether theoretically will allow for continued growth on the concave side of
the spine deformity and gradual correction of the deformity with growth.
 Devices that have been used for this growth modulation are a flexible titanium
clip,a nitinol staple,and an anterior spinal tether using anterior vertebral body
screws and a polypropelene cord

INTERVERTEBRAL STAPLING
 Current indications for vertebral body stapling for scoliosis include age younger
than 13 years in girls and 15 years in boys, skeletal maturity of Risser grade 0 or
1, with 1 year of growth remaining by wrist bone age, minimal rotation of both
the thoracic and lumbar curves of 45 degrees and flexibility to less than 20
degrees, and a sagittal thoracic curve of 40 degrees or less
 If the thoracic coronal curve is between 35 and 45 degrees and does not correct
on bending films to less than 20 degrees, adding a posterior rib-to-spine hybrid
construct may be considered

ANTERIOR VERTEBRAL TETHERING
 Advantages of anterior vertebral tethering include that it allows the spine to
grow and remain flexible,it is a one time surgery,and later fusion can be done
if needed
 The indications for this technique have not been well established,but it is
most likely beneficial in patients with enough growth remaining to
substantially alter the shape of the spine and is most suited for primary
thoracic curves
 Contraindications include
1)patients with no remaining growth
2)patients younger than 8yrs
3)patients with curves less than 40 deg or more than 65 deg
4)patients with left sided curves,pulmonary disease limiting single-lung
ventilation , previous ipsilateral chest surgery

INSTRUMENTATION WITH FUSION
 If a child is older than 9 or 10 years or is unable to cooperate with
the demands of growth rods, instrumentation and spinal fusion
should be considered.
 A combined anterior and posterior procedure should be considered
if the patient is deemed at risk for the crankshaft phenomenon
 Preferably, if an anterior procedure is performed, the anterior
release and fusion are done without sacrificing the segmental
vessels.
 Anterior instrumentation is not used if posterior instrumentation is
scheduled as a second procedure.

ADOLESCENT IDIOPATHIC SCOLIOSIS
 Adolescent idiopathic scoliosis is present when the spinal deformity is recognized
after the child is 10 years of age but before skeletal maturity.
 This is the most common type of idiopathic scoliosis.
 The characteristics of adolescent idiopathic scoliosis include a three-dimensional
deformity of the spine with lateral curvature plus rotation of the vertebral bodies.
 Most idiopathic curves are lordotic or hypokyphotic in the thoracic region.

 ETIOLOGY
 The exact cause of idiopathic scoliosis remain unknown
 There are many proposed etiologic factors, but these can be divided into six
general categories
1)genetic factors
2)neurologic disorders
3)hormonal and metabolic dysfunctions
4)skeletal growth
5)biomechanical factors
6)environmental and lifestyle factors

 CURVE PROGRESION:
 Once scoliosis has been discovered in a
child, the curve must be evaluated for the
probability of progression
 Most authors define progression as an
increase of 5 degrees or more measured
by the Cobb measurement over two or
more visits

CURVE PATTERNS
 PONSETTI AND FRIEDMAN CLASSIFICATION
 Idiopathic scoliosis curves were first classified by PONSETTI and FRIEDMAN into
five main patterns
 A sixth curve pattern was described by MOE
 1)single major lumbar curve:The lumbar curve has its apex between the
L1-2 disc and L4. These curves produce an asymmetry of the waistline with
prominence of the contralateral hip that parents often assume is caused by a
short leg on the side of the curve.
 2)Single major thoracolumbar curve: The thoracolumbar curve apex
is at T12 or L1. This curve tends to produce more trunk imbalance than other
curves. This decompensation from the midline often produces a severe cosmetic
deformity.

 3)Combined thoracic and lumbar curves(Double major curves):Symmetrical
double major curves generally cause less visible deformities because the curves
are nearly the same degree in size and the trunk usually is well balanced.
 4)Single major thoracic curve: This curve pattern generally is a convex right
pattern. Because of the thoracic location of the curve, rotation of the involved
vertebrae may be obvious. The curve produces prominence of the ribs on the
convex side, depression of the ribs on the concave side, and elevation of one
shoulder, resulting in an unsightly deformity.
 5)Single major high thoracic curve: There were only five patients with this
curve pattern in the series of Ponseti and Friedman; although none of these
curves became large, the deformity was unsightly because of the elevated
shoulder and the deformed thorax. The apex of the curve usually was at T3, with
the curve extending from C7 or T1 to T4 or T5.

 6)Double major thoracic curve: This pattern was described by
Moe and consists of a short upper thoracic curve, often
extending from T1 to T5 or T6, with considerable rotation of the
vertebrae and other structural changes in combination with the
lower thoracic curve extending from T6 to T12 or L1. The upper
curve usually is convex to the left, and the lower usually is
convex to the right. Deformities in patients with this curve
pattern usually are not as severe as in those with a single
thoracic curve, but because of asymmetry of the neckline
produced by the upper curve, this pattern is more deforming
than combined thoracic and lumbar curves.

 KING CLASSIFICATION
 The classification system of King et al. is used to describe thoracic curves.
 And aid in determining when the thoracic curve alone or both thoracic and
lumbar curves should be included in the instrumentation and fusion
 A King type I curve is recognized easily because the lumbar curve is larger than
the thoracic curve. On occasion, the thoracic and lumbar curves are nearly
equal, but the lumbar curve is less flexible on side bending
 As defined by King, type II thoracic scoliosis is a combined thoracic and lumbar
curve pattern. On radiographs, the thoracic curve is larger than or equal to the
lumbar curve. The lumbar curve must cross the center sacral line. On supine
sidebending radiographs, the lumbar curve is more flexible than the thoracic
curve.

 A type III curve is a thoracic scoliosis with the lumbar curve not crossing the
midline. The lumbar curve is very flexible on side-bending radiographs
 A type IV curve is a single long thoracic curve, with L4 tilted into the curve and L5
balanced over the pelvis
 type V curve is a double structural thoracic curve. On radiographs, the first
thoracic vertebra is tilted into the concavity of the upper curve, which is structural
on sidebending films.

 LENKE CLASSIFICATION
 Lenke et al. proposed a three-step classification system for adolescent
idiopathic scoliosis
 Measurements are obtained from standard posteroanterior, lateral, and right
and left bending radiographs.
 The three steps in this classification system are
(1) identification of the primary curve,
(2) assignment of the lumbar modifier, and
(3) assignment of the thoracic sagittal modifier.
 The first step is to identify the primary curve. These curves should be divided
by region: proximal thoracic, main thoracic, and thoracolumbar or lumbar.
 Curves are considered to be structural curves if they are more than 25 degrees
on posteroanterior radiographs and do not bend to less than 25 degrees on
side-bending radiographs.

 Based on these measurements the curve can be classified into six types
 The second step is to determine the lumbar spine modifier. This is determined
by drawing a vertical line upward from the center of the sacrum (center sacral
vertical line [CSVL]).
 The lumbar spine modifier
 is then determined by the relationship of the CSVL to the concave pedicle of the
apical lumbar vertebra and can be assigned into A, B, or C
 The third step is to determine the thoracic sagittal modifier. The sagittal
modifier is hypokyphotic (<10 degrees), normal (10 to 40 degrees), or
hyperkyphotic (>40 degrees)
 Forty-two discrete curve classifications can be identified by this three-step
process

 TREATMENT
 Any curve possesses the potential of progression,increasing deformity, and
interference with visceral function.
 Regardless of the known natural history of a certain curve or combination of
curves ,each curve should be closely observed until halt in progression can be
established with certainity.
 Thorough examination repeated at specific intervals is mandatory ,especially
during periods of growth spurts, until the following signs of growth stoppage
are clearly defined;
wrist bone age
Growth of iliac apophysis
growth of vertebral apophyseal rings
menarche
body growth

 There are 3 basic treatment
options available for adolescent
idiopathic scoliosis:
1)observation
2)nonoperative treatment such as
bracing
3)surgical intervention

 OBSERVATION:
 Observation is always employed in a growing child with mild curve.
 Growing children who have curves less than 20 deg are observed for
progression.
 They are usually examined at 4 to 6 month intervals.
 Children who when first seen to have curves in the 20 deg range are observed
for an additional 4 to 6 months.
 They should show 5 deg of progression before bracing is instituted.
 If a child who is growing presents with a curve of greater than 30 deg, a brace is
immediately prescribed,and there would be no observation period.

 NON-OPERATIVE TREATMENT:
 The mainstay of nonoperative treatment is bracing.
 The original successful brace treatment for scoliosis was a standard MILWAUKEE
BRACE or cervical thoracic lumbosacral orthosis(CTLSO).
1)the basic concept of bracing is prevention of curve progression rather than
correction of the curve.
2)it will only prevent curves from progressing as the child continues to grow.
3)braces are applied when a curve is progressing and is between 20 deg and
40 deg.
4)in addition,a brace is usually prescribed in premenarchal girls if their curve
measures greater than 30 deg at the time of first evaluation .
5)rarely does a brace work after a curve is 40 deg or greater.

 OTHER ORTHOSES:
 These are primarily underarm braces or what is called as thoracolumbosacral
orthosis(TLSO).
 There are numerous types of braces,such as
-underarm Milwaukee brace
-the DuPont brace
-the Boston brace
-Risser brace
All are basically similar to standard Milwaukke brace but cosmetically are much
more appealing. they are usually as effective as standard Milwaukee brace.

 Three factors are essential for successful bracing:
1)someone must manage the brace and know when it is and is not to be
used.
2)the brace must be well fitting, comfortable and effective.
3)the patient must be willing to wear the brace conscientiously.
DURATION OF BRACING:
The orthosis were originally intended to be worn 23 hours a day but most part
time bracing protocols call for approximately 16 hours or less of brace wear each
day.
if the curve is less than 35 deg and does not show significant vertebral
wedging, part-time brace wear is certainly reasonable to attempt first.
If significant progression of the curve is noted during use of the part time
protocol ,full time bracing can then be instituted.

 HALO-TRACTION DEVICE:
 A spinal skeletal traction and fixation device,the halo traction device is attached
to the skull and is connected to a plaster body cast by an adjustable steel frame.
 It is used particularly to exert traction on and immobilize the cervical and upper
thoracic spine for prolonged periods of time after fusion when the spine has
been rendered unstable by paralysis or fracture dislocation.
 Spinal skeletal traction is especially valuable in scoliosis when cardiopulmonary
insufficiency contraindicates the use of corrective cast.
 In this situation,the halo-skull traction is used with distal traction through pins
inserted in the distal femurs or proximal tibias,permitting the use of respiratory
aids and surgery while in traction.
 This method is suitable for the prolonged fixation necessary following extensive
fusions.

 SURGICAL MANAGEMENT:
 Indications for surgery
-a progressive curve
-Deformity of the trunk regardless of whether the spinal growth has ceased
-pain,particularly in older patients
-decreasing cardiopulmonary functions
-hereditary history of severe scoliosis
-thoracic lordosis

APPROACHES TO THE SPINE
 POSTERIOR APPROACH
 It has been the gold standard for decades and is generally used with Harrington
instrumentation.
ADVANTAGES
-Surgeons are familiar with it so fusion rates are excellent.
-curve correction is good,and it has few complications.
-usually required in patients with double or triple curves or curves associated with
significant kyphosis.
DISADVANTAGES
-Preadolescent children are at risk for the crankshaft phenomenon.
-doesnot always correct hypokyphosis in the thoracic spine.
-this procedure is not always effective for curves in the thoracolumbar region and
may cause spinal abnormalities there.

 ANTERIOR APPROACH:
 Increasingly,surgeons are using the anterior approach
 With the anterior approach,the surgeon makes an incision in the chest,deflates
the lung, and removes a rib in order to reach the spine.
 This rib can be used during the operation as a strut to support the spine
 It also may be repositioned within the patient until it is used for bone grafting
during fusion.
ADVANTAGES:
1)a more complete three-dimensional correction of the deformity because of
thorough disc and annular excision.
2)curve correction by convex compression that shortens the spinal column and
avoids distraction of neural elements
3)fusion of end vertebra,resulting in a shorter fusion construct that would be
required posteriorly

4)possible reduction of the number of upper and midlumbar fusion levels.
5)avoidance of a crankshaft phenomenon in skeletally immature patients.
6)decreased frequency of decompression in primary thorasic scoliosis with
compensatory lumbar curves.
7)avoidance of problems with prominent posterior instrumentation that occurs
in thin patients even with the newer,low profile instruments.
DISADVANTAGES
1)possible chest cage disruption ,which can effect pulmonary function.
2)an increases risk of progressive kyphosis because of posterior spinal growth
in skeletally immature patients(riser 0).

 THE COMBINED ANTERIOR-POSTERIOR APPROACH:
 The combination approach uses an anterior approach first , wich allows better
correction of the problems.
 The fusion part of the operation is done with the posterior approach.
 This a very long ang complex procedure.
 It appears to be safe ,and is proving to be useful , even in very young patients ,
for preventing crankshaft phenomenon.
 it also may correct large rigid curves and specific severe curves in the thoracic
spine.

 Two surgical approaches are used in treating idiopathic curves:
1)posterior spinal instrumentation and fusion
2)anterior spinal instrumentation and fusion
THE OBJECTIVE IS: to partially correct the deformity and to permanently stabilize the
area of the spine.
it takes a two-part process to accomplish these goals:
a)Fusing(joining together) the vertebrae along the curve.
b)supporting these fused bones with instrumentation(steel rods,hooks ,and
other devices attached to the spine.

 The ideal spinal instrumentation system is:
-safe and reliable ,with infrequent instrument failure and breakage.
-it should be strong enough to resist load from all directions without external
support.
-be easy to use with little increase in operative time , and restore normal spinal
contours in the coronal , sagittal and transverse planes.
-it should not create new deformities as the instrumentation is applied.
-it also should e a cost-effective system.

 POSTERIOR FUSION:
 The long term success of any operative procedure for scoliosis depends on a
solid arthrodesis.
 The classic extra articular hibbs technique has been replaced by intraarticular
fusion techniques that include the facet joints.
 Autogenous bone graft from iliac crest remains the “gold standard” for graft
material , combining osteogenic , osteoconductive and osteoinductive
properties.
 Another excellent source of autogenous bone is rib obtained from a
thoracoplasty.
 Allografts provide osteoconductive and osteoinductive properties , they are
used where large amounts of bone graft are needed and the iliac crests are
small or are used for instrumentation.

 POSTERIOR SPINAL INSRTUMENTATION:
 The goals of instrumentation in scoliosis surgery are to correct the deformity as
much as possible and to stabilize the spine in corrected position while the fusion
mass becomes solid.
 The fusion mass in a well corrected spine is subjected to much lower bending
moments and tensile forces than the fusion mass in an uncorrected spine.
 HARRINGTON introduced the first effective instrumentation system for scoliosis.
 Two hooks were inserted at either end of a scoliotic curve and a metal rod was
placed between these hooks.
 Distraction was then accomplished similar to the way one would jack up a car.
 This would partially correct the deformity.

 The hook and rod system was used to hold the curve in the corrected position
until a bony fusion has developed.
 Use of the Harrington distraction rod , combined with a thorough posterior
arthrodesis and immobilization in a cast or brace for 6 to 9 months ,has been
the standard surgical procedure of adolescent idiopathic scoliosis.
 The major correcting force with the Harrington instrumentation is distraction.
 DISADVANTAGES:
 As the curve is corrected with distraction,the efficiency of correction is
decreased.
 The distraction forces are applied only at the end of the construct where the
hooks are seated.

 If the load exceeds the strength of the lamina , fracture and loss of
correction can result.
 With distraction , the spine is elongated and loss of sagittal contour occurs.
 Finally,distraction doesnot deal with rotation component of idiopathic
adolescent scoliotic curve.

 These systems provide multiple points of fixation to the spine and apply
compression ,distraction and rotation forces through the same rod.
 These systems do not require any postoperative immobilization.
 They provide somewhat better coronal plane correction and better control in the
sagittal plane.
 3 kinds of devices are available for fixation of posterior segmental
instrumentation
1)wires or cables
2)hooks
3)Screws
POSTERIOR SEGMENTAL SPINAL INSTRUMENTATION:

 THT LUQUE TECHNIQUE:
 The luque technique consists of placing two rods along the posterior elements of
the spine and passing wires beneath the lamina and tying them to the rod.
 This procedure has a certain risk factor , because passing the wires under the
lamina into the spinal canal can cause neurologic deficits
Other segmental techniques have been developed using hooks,these
techniques include Cotrek-Dobousset , Texas Scottish rite hospital ,Isola and Mod-U-
Lock systems. All have the same basic principle ; segmental fixation with hooks

 MULTIHOOK SEGMENTAL INSTRUMENTATION :
 PRINCIPLES OF FUSION LEVELS
1)AP , lateral and bending films are essential. Supine bending films are
always recommended.
2)In the sagittal plane , all pathological curves must be included. The
instrumentation should not be stopped in the middle of a pathological curve , such
as a thoracolumbar kyphosis. The upper hook should not stop at the apex of the
kyphosis proximally.
3)The instrumentation should extend to a rotational neutral vertebra.

4) The instrumentation should be
stopped at the level above disc space
neutralization. The disc height should
be equal on the right and left sides
and should open on both the right
and left sides with supine bending
films.
5)The distal level should fall within
the stable zone of Harrington.

 PRINCIPLES OF HOOK PATTERN AND SITE PLACEMENT:
1) Distraction forces decrease lordosis or contribute to kyphosis.
2)Compression forces decrease kyphosis or create lordosis.
3)to create kyphosis,the concave side should be approached first.
4)To create lordosis , the convex side must be approached first , and the forces
must be directed towards the apex of the curve.

 There are basically two types of hooks
1)Pedicle
2)Laminar
-The pedicle hooks are designed for secure fixation in the thoracic spine by
insertion into the facet with impingement on the thoracic pedicle
-Pedicle hooks are used in an upgoing direction at T10 or higher.
-The laminar hooks are for most other areas of the spine.
-These can be placed around either the superior or inferior edge of the lamina or
transverse process , according to the designed direction and point of application of
forces.

 SUBLAMINAR WIRES AND SUBLAMINAR CABLES:
 Sublaminar wires generally are not used alone as anchors at the upper or lower
instrumented vertebrae because they provide no axial stability.
 Sublaminar wires and cables are useful in and around the apex of curves to aid
in the translation maneuver, in which the spine can be pulled to a pre contoured
rod , thus minimizing the need for derotational maneuvers.
 The more rigid the curve , the more helpful these sublaminar wires or cables are.
 Use of sublaminar cables instead of monofilament stainless steel wire because
wire breakage and migration have been serious complications of sublaminar
wiring.
 Cable flexibility prevents repeated contusions to the spinal cord that can occur
during insertion of the rod and tightening of the wire.

 PEDICLE FIXATION:
 Instrumentation that uses the pedicle as a source of purchase for bone screws
from the posterior approach into the vertebral body has become an increasingly
popular form of spinal fixation
 Pedicle screws on the convex side of the lumbar spine improved coronal and
sagittal correction , allowed the lower instrumented vertebra to be translated to
the midline and bought to a horizontal position , and allowed improved
restoration of segmental lordosis.
 Thoracic screws have a tendency to produce more thoracic hypokyphosis than
with posterior segmental hook instrumentation.

 Basic steps of pedicle fixation:
1)Clearing the soft tissue
2)Exposing the cancellous bone of the pedicle canal by decortication at the
intersection of the base of the facet and the middle of the transverse process
3)probing of the pedicle
4)verifying the four walls of the pedicle canal by probing or obtaining
radiographic confirmation
5)tapping the pedicle
6)placing the screw

 ADVANTAGES OF THORACIC PEDICLE SCREWS:
 when optimally placed , the screws are completely external to the spinal canal
(supralaminar and infra laminar hooks , in contrast are within the canal).
 Stronger fixation is possible than with hook implants.
 The screws are attached to all three columns , providing a rigid triangular cross
linked construct than with a posterior only implant.
 Facet joints , laminae and transverse processes are free of implants , and
therefore theoretically there is more surface area for decortication.
 There is superior coronal correction and axial derotation.
 Most studies have shown slightly shorter fusion lengths than with hook
constructs.
 With improved correction , there is decreased need for anterior procedures and
thoracoplasties.

 DISADVANTAGES OF THORACIC PEDICLE SCREWS:
 The implants add significantly to the cost of the procedure
 The potential complications in insertion of thoracic pedicle screws include injury
to the spinal cord , nerve roots , pleural cavity and the aorta.
 Radiation exposure is significant to the surgeon and patient if routine
fluoroscopy is used

 COMPLICATIONS OF POSTERIOR SCOLIOSIS SURGERY :
EARLY COMPLICATIONS:
1)Neurological injury:The most feared and unpredictable complication in scoliosis
surgery remains neurological injury
2)Infection: The most common organism associated with postoperative infection
is Staphylococcus aureus.
3)Ileus:Ileus is a common complication after both anterior and posterior spinal
fusion. Oral feedings are resumed slowly after surgery. Intravenous fluids are
continued until a full diet is tolerated.
4)Atelectasis:Atelectasis is a common cause of fever after scoliosis surgery .
Frequent turning of the patient and deep breathing and coughing usually control
or prevent serious atelectasis.

5)Pneumothorax: At the time of subperiosteal posterior spine exposure, the pleura
may be entered inadvertently between the transverse processes on the concave side
of the scoliosis. If a thoracoplasty is done at the same time, a pneumothorax is more
likely to occur.
6)Dural tear:If a dural tear occurs during removal of the ligamentum flavum or
insertion of a hook or wire, repair should be attempted.
7)Wrong levels:Care should be taken in the operating room to correctly identify
the vertebral levels.
8)Urinary complications:The syndrome of inappropriate antidiuretic hormone
secretion develops in the immediate postoperative period in a high percentage of
patients undergoing spinal fusion .
9)Vision loss:Postoperative loss of vision has an incidence of 0.02% to 0.2%. Prone
positioning, particularly in the Trendelenburg position, has been noted to increase
intraocular pressure. Most deficits are permanent

 LATE COMPLICATIONS:
 1)Pseudarthrosis:In adolescents with idiopathic scoliosis, the pseudarthrosis
rate is approximately 1%; the rate is higher in patients with neuromuscular
scoliosis.
The most common areas of pseudarthrosis are at the thoracolumbar
junction and at the distally fused segment.
2)Loss of lumbar lordosis:If distraction is applied across the lumbar spine,
normal physiological lumbar lordosis may be diminished or eliminated, causing the
patient to stand with a forward tilt that results in upper back pain, lower back pain,
and even pain in the hips.
3)Crankshaft phenomenon

4)Superior mesenteric artery syndrome:Rarely, superior mesenteric artery
syndrome may cause small bowel obstruction after spinal fusion.
The transverse portion of the duodenum crosses the midline anterior to
the spine and the aorta and posterior to the superior mesenteric artery. As the
space between these structures decreases, obstruction of the duodenum can
occur.
5)Trunk decompression: Problems with trunk decompensation have been
noted with the newer segmental instrumentation systems in the treatment of King
type II curves.
Decompensation can be prevented and a well-balanced correction
obtained if the preoperative ratio of the thoracic to lumbar curve magnitude is
greater than 1.2, the apical vertebral rotation ratio of the thoracic to the lumbar
curve is greater than 1.0, and the apical vertebral translation of the thoracic to
lumbar curve is greater than 1.2.

 6)Late infection:Delayed deep wound infections requiring removal
of instrumentation because of pain, swelling, and spontaneous
drainage have been reported.

 ANTERIOR INSTRUMENTATION FOR IDIOPATHIC SCOLIOSIS:
 Anterior instrumentation and fusion for idiopathic scoliosis is now a well-
accepted procedure for certain thoracolumbar and lumbar curves
 A lenke type 4 pattern in which the thoracolumbar or lumbar curve is the
structural component and the main thoracic or proximal thoracic curves are non
structural is the ideal candidate for this type of procedure
 Anterior instrumentation provides excellent derotation and correction of the
curve in the coronal plane

 Two major instrumentation systems are used for anterior spinal instrumentation
and fusion, the DWYER and ZIELKE SYSTEMS .
 A thoracolumbar approach is used for both
 The approach is through the convex side of the spine
 The intervertebral discs are removed and screws are placed in the vertebral bodies
as fixation points
In the DWYER procedure, a cable is threaded through the screw heads
and as the cable is tightened , the vertebral bodies approach each other because the
discs have been removed. Thus , partial correction of the curve can be obtained
With the ZIELKE procedure , a threaded rod is used instead of a cable .
This allows better positioning of the vertebrae as the rod is tightened.

ANTERIOE ZIELKE AND POSTERIOR HARRINGTON
INSTRUMENTATION AND FUSION

 COMPLICATIONS OF ANTERIOR INSTRUMENTATION:
 Pitfalls and complications may be related to poor patient selection, poor level
selection, or instrument technical difficulties.
 A common technical problem is failure of the most proximal screw , which can be
prevented by watching this screw carefully during the derotation maneuver.
 At any sign of screw loosening, the correction maneuver should be stopped
 Another technical problem is encountered if the screw heads are not aligned
properly and one screw head is offset from the others. If one screw is off just
slightly, rod placement can be difficult.

 A number of studies have emphasized the potential complications associated
with an anterior approach to the spine, including respiratory insufficiency
requiring ventilatory support, pneumonia, atelectasis, pneumothorax, pleural
effusion, urinary tract infection, prolonged ileus, haemothorax, splenic injury,
retroperitoneal fibrosis, and partial sympathectomy.
 Neurological injury can occur during discectomy or screw insertion. The screws
should be placed parallel to the vertebral endplates.
 When the segmental vessels are ligated, the anastomosis at the intervertebral
foramina should be avoided to minimize the chance of injury to the vascular
supply of the spinal cord.

 Video-assisted Thoracoscopic surgery (VATS)-
 Video-assisted thoracoscopic surgery is the treatment of paediatric spinal
deformity is a commonly used procedure for anterior release
ADVANTAGES OF THORACOSCOPIC SURGERY OVER OPEN THORACOTOMY:
-Better illumination and magnification at the site of surgery.
-Less injury to the latissimus muscle and chest wall with less long-term pain ,
decreased blood loss , better cosmesis , shorter recovery time , improved
postoperative pulmonary function , and potential shorter hospital stays.
The primary Disadvantage of thoracoscopy are related to steep learning curve and
technical demands of the procedure.

INDICATIONS FOR VATS:
1)Rigid curves requiring anterior release and posterior fusion.
2)Skeletal immaturity in patients whom anterior surgery is needed to
prevent crankshaft phenomenon.
CONTRAINDICATIONS FOR VATS:
1)Inability to tolerate single lung ventilation.
2)Severe or acute respiratory insufficiency.
3)High airway pressure with positive-pressure ventilation , emphysema ,
and previous thoracotomy.

CONGENITAL SCOLIOSIS
 Congenital scoliosis is a lateral curvature of the spine caused by the presence
of vertebral anomalies that result in an imbalance of the longitudinal growth of
the spine.
 The critical time in the development of the spine embryologically is the fifth to
sixth week – the time of segmentation process – and congenital anomalies of
the spine develop during the first 6 weeks of intrauterine life.
 No genetic etiology has been found for isolated congenital abnormalities of
the spine such as hemivertebrae.
 Research suggests that maternal exposure to toxins, such as carbon monoxide,
may cause congenital scoliosis.

 CLASSIFICATION OF CONGENITAL SCOLIOSIS:
Congenital scoliosis can be classified as
-Failure of formation
Partial failure of formation(Wedge vertebra)
Complete failure of formation(hemivertebra)
-Failure of segmentation
Unilateral failure of segmentation(unilateral segmental bar)
Bilateral failure of segmentation(block vertebra)
-Miscellaneous

 PATIENT EVALUATION:
 In , addition to the routine spinal evaluation , some specific physical findings
should be sought in patients with congenital scoliosis.
 The skin of the back should be carefully examined for signs such as hair patches ,
lipomata , dimples , and scars which may indicate an underlying anomalous
vertebra.
 Evidence of neurological involvement , such as clubfoot , calf atrophy , absent
reflexes , and atrophy of one lower extremity compared with the other , should
be noted carefully.
 Many children with congenital scoliosis have other anomalies.

 TREATMENT:
 Treatment of congenital scoliosis is based on the anticipated natural history and
follow-up documentation based on standing x-rays.
 Large series of congenital scoliosis have shown that about 11% of patients will
have no progression , 14% will have slight progression , and about 75% of
patients will have progression greater than 30% and will require surgical
intervention.
 Treatment consists firstly of observation at 6-monthly intervals
 In an infant , follow-up at 4 monthly intervals for the first 2 to 3 years of life may
be indicated to determine if a rapidly progressive deformity is present.
 The frequency of observation depends on the type of congenital deformity
present and its clinical course.

 NONOPERATIVE TREATMENT:
 Nonoperative treatment is of limited value in patients with congenital scoliosis.
 Bracing sometimes can be used to control secondary curves when the primary
congenital curve is being treated nonoperatively.
Also, bracing may prevent progression of a secondary curve that is causing
balance problems.
 If orthotic treatment is elected, careful measurement and comparison of spine
radiographs at 6-month intervals must be made.
 Because of the slow progression of some curves, it is important to compare
current radiographs with all previous films, including the original films, to detect
curve progression.

 Because 75% of congenital curves are progressive, surgery remains the
fundamental treatment.
 Congenital spinal deformity can be treated by procedures that prevent further
deformity or procedures that correct the present deformity.
 If treatment is aimed at correcting the present deformity, the curve can be
corrected gradually or immediately. The surgical methods available for
treatment of congenital scoliosis are:
FOR PREVEMTION OF FUTURE DEFORMITY:
-Posterior fusion with instrumentation
-Posterior fusion without instrumentation
-Combined anterior and posterior fusions

 GRADUAL CORRECTION OF DEFORMITY:
TRANSPEDICULAR CONVEX ANTERIOR HEMIEEPIPHYSIODESIS AND
POSTERIOR ARTHRODESIS:
-King et al. described a technique of transpedicular convex anterior
hemiepiphysiodesis combined with posterior arthrodesis for treatment of
progressive congenital scoliosis.
-In effect, a combined anterior and posterior fusion can be done through a
single posterior approach.
-The average age of patients at surgery was 9 years.
-Their technique is based on the work of Michel and Krueger, who described
a transpedicular approach to the vertebral body, and Heinig, who described the
“eggshell” procedure, so called because the vertebral body is hollowed out until it is
eggshell thin before it is collapsed.

 COMBINED ANTERIOR AND POSTERIOR CONVEX
HEMIEPIPHYSIODESIS(GROWTH ARREST):
 Gradual correction of congenital scoliosis may be obtained through the use of
a convex hemiepiphysiodesis.
 This technique is used for curves that are the result of failure of formation.
 There is no role for this technique in failures of segmentation.
 Correction of deformity relies on the future growth of the spine on the concave
side
 In deformities caused by failure of segmentation, there is really no growth
potential on the concave side.
 This technique is best for treating single hemivertebrae that have not resulted
in a large curve at the time of surgery.

 This technique is appropriate in children younger than 5 years who meet certain
criteria:
- a documented progressive curve
- a curve of less than 50 degrees
- a curve of six segments or less,
-concave growth potential, and no pathological congenital kyphosis or
lordosis
Epiphysiodesis of the entire curve , not merely the apical segment , should be
done.
Rigid spinal immobilization is used until the fusions are solid, usually at least 6
months after surgery.

 Growing rods and VEPTR instrumentations that have been used to treat early
onset scoliosis also have been used for gradual correction and stabilization of
progressive congenital curves.
 Several authors have reported good results with these techniques , with
acceptable complication rates.
 The growing rod technique is suggested for patients with primary vertebral
anomalies.
 Patients with rib fusions or associated thoracic insufficiency syndrome with
congenital scoliosis usually are treated with a VEPTR.

 ACUTE CORRECTION OF DEFORMITY:
 HEMIVERTEBRA EXCISION:
 Hemivertebra excision can produce immediate correction of a congenital spine
deformity.
 This technique will remove the cause of and prevent further worsening of the
deformity.
 Hemivertebra excision usually is reserved for patients with pelvic obliquity or with
fixed, lateral translation of the thorax that cannot be corrected by other means.
 At the lumbosacral area, excision of the hemivertebra can improve trunk
imbalance.
 Hemivertebra excision in the thoracic area has more risk because this area of the
spinal canal is the narrowest and has the least blood supply

 The curves best managed by hemivertebra excision are angular curves in which
the hemivertebra is the apex.
 The optimal indication of hemivertebra resection is a patient younger than 5
years with a thoracolumbar, lumbar, or lumbosacral hemivertebra that is
associated with truncal imbalance.
 Winter emphasized that hemivertebra excision should be considered a convex
osteotomy at the apex of the curve.
 The entire curve front and back must be fused.
 Neurological risk is inherent in hemivertebra excision because the spinal canal is
entered both anteriorly and posteriorly.

 TRANSPEDICULAR EGGSHELL OSTEOTOMIES WITH FRAMELESS STEREOTACTIC
GUIDANCE:
 Mikles et al. described a technique for transpedicular eggshell osteotomies for
congenital scoliosis with frameless stereotactic guidance.
 This technique is recommended in older patients who have congenital scoliosis
with multiplanar spinal abnormalities.
 The guidance system was used to locate the pedicles intraoperatively for accurate
screw placement.
 They thought that screw placement was difficult because of the abnormal
anatomy, and they found the use of the guidance system to be helpful in
obtaining screw placement proximally and distally and, therefore, a rigid
instrumentation construct.

 EXPANSION THORACOPLASTY:
 The vertical expandable prosthetic titanium rib (VEPTR) comes in two forms.
 The device with a radius 220mm is most commonly used in the treatment of
fused ribs and scoliosis.
 The titanium alloy permits the use of MRI postoperatively.
 There are 3 anchors available: RIB , SPINE ,and PElVIS.
 The rib anchor consists of two C-shaped clamps that , when locked , form a
loose encirclement around the rib to avoid vascular compromise of the
underlying rib.
 Lateral stability is provided by the surrounding soft tissues.
 The spine anchor consists of a low-profile closed laminar hook.
 The pelvic anchor consists of a n S-shaped modified McCarthy hook that is
placed over the iliac crest.

 The central portion of the device consists of two sliding rib sleeves.
 The superior sleeve is attached to the cranial anchor , which is usually a rib , and
the inferior sleeve is attached to the caudal anchor , which can be a rib , the
spine , or the pelvis.
 The device is locked inferiorly by a peg-type lock through one of two holes ,
5mm apart in the distal sleeve , into partial-thickness holes in the inferior rib
cradle post.
 This provides variable expandability for the device in increment of 5mm.

NEUROMUSCULAR SCOLIOSIS
 The specific causes of neuromuscular scoliosis are unknown, but several
contributing factors are well known.
 Loss of muscle strength or voluntary muscle control and loss of sensory
abilities, such as proprioception, in the flexible and rapidly growing spinal
column of a juvenile patient are believed to be factors in development of these
curves.
 Neuromuscular curves develop at a younger age than do idiopathic curves, and
a larger percentage of neuromuscular curves are progressive.
 Unlike idiopathic curves, even small neuromuscular curves may continue to
progress beyond skeletal maturity.
 Many neuromuscular curves are long, C-shaped curves that include the sacrum,
and pelvic obliquity is common.

 Patients with neuromuscular scoliosis also may have pelvic obliquity from
other sources, such as hip joint and other lower extremity contractures, all of
which can affect the lumbar spine.
 The progressing neurological or muscular disease also can interfere with trunk
stability.
 These patients generally are less tolerant of orthotic management than are
patients with idiopathic scoliosis, and brace treatment often is ineffective in
preventing curve progression.
 Spinal surgery in this group is associated with increased bleeding and less
satisfactory bone stock, and longer fusions, often to the pelvis, are needed.

 TREATMENT;
 Goals for treatment in neuromuscular scoliosis are
-to prevent severe deformity.
-to prevet progressive impairment of pulmonary function.
Other common problems are decubitus ulcers , back pain , hip dislocation
associated with pelvic obliquity.
Treatment options are:
1)observation
2)Nonoperative treatment
3)Operative treatment

 OBSERVATION:
 Not all neuromuscular spinal deformities require immediate treatment.
 Small curves of less than 20 to 25 degrees can be observed carefully for
progression before treatment is begun.
 Similarly, large curves in severely mentally retarded patients in whom the curve is
not causing any functional disability or hindering nursing care can be observed.
 Progressive neuromuscular scoliosis in very young patients can be treated with
an orthosis.
 The scoliosis often continues to progress despite orthotic treatment, but the rate
of progression can be slowed, and further spinal growth can occur before
definitive spinal fusion.

 The brace also can provide flaccid patients with trunk support, allowing the use
of the upper extremities.
 A custom-molded, total-contact TLSO usually is required for these children
because their trunk contours do not accommodate standard braces.
 Most patients with neuromuscular scoliosis lack voluntary muscle control,
normal righting reflexes, and the ability to cooperate with an active brace
program; therefore, passive-type orthotics have been more successful in
managing neuromuscular curves.
 Patients with severe involvement and no head control frequently require
custom-fabricated seating devices combined with orthoses or head-control
devices

 The goal of fusion in patients with neuromuscular scoliosis is to produce solid
arthrodesis of the spine, balanced in both the coronal and sagittal planes and
over a level pelvis.
 In doing so, the surgery should maximize function and improve the quality of
life.
 To achieve this goal, a much longer fusion is necessary than usually is indicated
for idiopathic scoliosis.
 Because of a tendency for cephalad progression of the deformity when fusion
ends at or below the fourth thoracic vertebra, fusion should extend to T4 or
above.
 The decision on the distal extent of the fusion generally is whether to fuse to the
sacrum or to attempt to stop short of it.

 PREOPERATIVE CONSIDERATIONS:
 Patients with neuromuscular scoliosis must have complete medical
evaluations, including cardiac, pulmonary, and nutritional status.
 Many conditions, such as Duchenne muscular dystrophy and Friedreich ataxia,
are associated with cardiac involvement.
 Most patients with neuromuscular scoliosis have diminished pulmonary
function, and careful preoperative evaluation is essential.
 Patients with neuromuscular scoliosis often have suboptimal nutrition because
of gastrointestinal problems, such as a hiatal hernia or gastroesophageal
reflux.
 Surgery intensifies the preexisting state by raising the patient’s metabolic
requirements.
 Seizure disorders are common in patients with neuromuscular scoliosis, and
preoperative anticonvulsant levels should be optimized within a therapeutic
range.

 Most patients with neuromuscular disease have insufficient autogenous bone;
allograft bone usually is used to obtain a fusion and is an acceptable alternative.
 As in other scoliosis surgery, the fusion levels and instrumentation must be
determined preoperatively.
 The source of pelvic obliquity must be determined.
 Combined anterior and posterior arthrodeses may be required for severe pelvic
obliquity.
 Other indications for a combined anterior and posterior approach include
necessity for an anterior release for further correction of severe kyphosis, severe
and rigid scoliosis that cannot be corrected by bending or traction to less than 60
degrees, and deficient posterior elements, such as those in patients with
myelomeningocele.

 LUQUE ROD INSTRUMENTATION WITH SUBLAMINAR WIRING:
 Eduardo Luque is credited with popularizing the use of long L-shaped rods and
sublaminar wires in the surgical treatment of spinal deformity.
 The rods can be contoured, and the spine is corrected as the wires are tightened.
 The neurological complications from sublaminar wires are of three types: cord
injury, root injury, and dural tears.
 Root injuries are the most common and lead to hyperesthesia, but these
generally resolve within 2 weeks.
 Delayed paraplegia and neurological deficits have been reported in patients
months after sublaminar wiring techniques.

 SACROPELVIC FIXATION:
 Many patients with neuromuscular problems
require instrumentation and fusion to the
sacrum.
 O’Brien described three fixation zones for
sacropelvic fixation.
 Examples of zone I fixation include S1 sacral
screws and a McCarthy S-rod. Zone II fixation
includes S2 screws and the Jackson intrasacral
rod technique (see later section on combined
anterior and posterior fusion for scoliosis in
patients with myelomeningocele). Zone III
fixation includes the Galveston L-rod technique
and sacroiliac screws.

If fixation to the pelvis is necessary , McCarthy
has described an S-rod technique
These two rods are crosslinked at the
lumbosacral junction and then fixed with a
combination of hooks , pedicle screws , and
sublaminar wires or cables bilaterally. throughout
the lumbar and thoracic spine.
The rods generally are crosslinked below the
upper fixation to provide further stability against
migration or rotation of the rods.
The advantages of the S-rod are that firm
fixation is provided around the sacral ala without
crossing the sacroiliac joint and the harvesting of
bone graft from ilium is not a problem because
the ilium is not violated.

 GALVESTON SACROPELVIC FIXATION:
 Another popular method for achieving sacropelvic fixation is the Galveston
technique described by Allen and Ferguson in which the pelvis is stabilized by
driving a segment of the L-rod into each ilium.
 The rod is inserted into the posterior iliac crest and rests between the cortices
above the sciatic notch.
 This fixation allows immediate firm stability and is biomechanically a stable
construct.
 There are potential disadvantages, however, because the rod crosses the
sacroiliac joint.
 It is postulated that motion in the sacroiliac joint is responsible for a “halo” that
is often seen around the end of the Galveston rod in the iliac wing.

CEREBRAL PALSY
 The prevalence of spinal deformities in patients with cerebral palsy varies
according to the degree of neuromuscular involvement.
 Less than 10% of ambulatory patients with spastic hemiplegia have scoliosis;
however, of those, 21% have scoliosis greater than 10 degrees and 6% have
scoliosis greater than 30 degrees.
 Scoliosis in patients with cerebral palsy can continue to progress into the third
decade.
 The greatest progression has been noted in patients who are unable to walk and
have thoracolumbar or lumbar curves.
 Scoliosis in patients with cerebral palsy is best managed by early recognition and
control of the curve before the deformity becomes severe.
 Unlike idiopathic scoliosis, scoliosis caused by cerebral palsy can be painful.

 Bonnett et al. listed the following seven goals of scoliosis treatment in patients
with cerebral palsy:
1. Improvement in assisted sitting to make positioning and transfer
easier for nursing attendants and family.
2. Relief of pain in the hips and back.
3. Increased independence because of decreased need for assistance,
both for the positioning required to relieve pain and to prevent pressure areas and
for feeding.
4. Improvement in upper extremity function and table-top activities by
eliminating the need to use the upper extremities for trunk support.
5. Reduction of the equipment needed, making possible the use of
other equipment.
6. Placement of the patient in a different facility, one in which less care is
provided.
7. Improved eating ability made possible by a change in position

 CLASSIFICATION:
 Lonstein and Akbarnia classified cerebral palsy curves into two groups.
Group I curves—double curves with both thoracic and lumbar
components—occurred in 40% of their patients.
These curves, which are similar to curves of idiopathic scoliosis, occurred
more commonly in patients with only intellectual impairment who were ambulatory
and lived at home.
Group II curves were present in 58% of patients.
These curves were more severe lumbar or thoracolumbar curves that
extended into the sacrum , with marked pelvic obliquity.
Patients with these curves usually were nonambulatory with spastic
quadriplegia, generally were not cared for at home, and were more likely to have
the classic form of cerebral palsy rather than intellectual impairment alone.

 TREATMENT:
 If the curve is small, careful observation is indicated.
 If the curve progresses or is more than 30 degrees in a growing child who is an
independent ambulator or sitter, treatment should be instituted.
 If a child is skeletally mature, bracing is not likely to be effective and surgery is
indicated if the curve is 50 degrees or more.
 If neurological involvement is extreme in a patient who is severely mentally
retarded and the curve is not causing any significant functional problems or
pain, observation is appropriate.
 Most nonambulatory patients with cerebral palsy do not have head or neck
control during the first years of life.

 Custom seating may be effective in providing these patients with a straight spine
and a level pelvis.
 Custom seating also can effectively accommodate severe spinal deformities and
allow an upright posture in severely involved individuals.
 If the curve is progressive, an orthotic device may be helpful as a temporizing
device but will not provide permanent control of the curve.
 Orthoses generally are used for curve control during growth in a child who is
ambulatory or who has independent sitting ability.
 The orthosis often provides enough trunk support to free the upper extremities
for functional use.
 The orthosis of choice is a passive, totalcontact TLSO with either a one-piece
front-opening or a two-piece bivalved design or the soft Boston orthosis.

 The operative treatment of scoliosis in cerebral palsy is complex.
 Determining which type of surgery is needed, and even whether any surgical
procedure is warranted, is difficult.
 The indications for surgical stabilization depend on the degree of mental
involvement and the functional state of the individual with cerebral palsy.
 Determining which functional abilities would be helped by surgery is not
always easy.
 Certainly, in ambulatory children and in those of near-normal intelligence,
operative indications are similar to those for idiopathic scoliosis (curves of 40
to 45 degrees or more during adolescence; curves of more than 50 to 60
degrees in young, skeletally mature adults; and progressive curves that do not
respond to nonoperative treatment).

 The decision concerning treatment of a child with total body involvement,
seizure disorder, no head or trunk control, and major nutritional problems is
difficult. If a practical, functional gain is not likely, the risks of surgery may not
be acceptable.
 The surgical techniques available for scoliosis in patients with cerebral palsy
have improved significantly.
 A pseudarthrosis rate of 20% has been reported in patients with posterior
spinal fusion and Harrington instrumentation.
 Combined anterior and posterior procedures with anterior and posterior
instrumentation result in adequate correction with a low incidence of
pseudarthrosis.
 The introduction of Luque rods and segmental instrumentation also has greatly
improved the results of surgery for scoliosis in cerebral palsy patients.

 The type of surgery also depends on the type of scoliosis.
 According to Lonstein and Akbarnia, patients with group I curves usually require
only a posterior fusion, with fusion to the sacrum rarely needed.
 A combined anterior and posterior approach to group I curves is needed only
when there is a significant lumbar component, in which case an anterior release
and fusion and posterior instrumentation add correction and reduce the rate of
pseudarthrosis.
 Group II curves usually require a long fusion to the sacrum because the sacrum
is part of the curve and pelvic obliquity is present.

 COMPLICATIONS:
 Improved techniques of instrumentation and preoperative and postoperative
management have decreased complications, but a much higher complication
rate should be expected after surgery for this type of scoliosis than after that
for idiopathic curves.
 Complications in patients with cerebral palsy have been reported in up to
81%, including infection in 15% to 19%.
 Patients with cerebral palsy are believed to be at an increased risk for
infection.
 Pulmonary complications often develop in these patients because they
cannot cooperate in deep breathing and coughing exercises, and appropriate
prophylactic pulmonary measures are needed.

 If the upper limit of the fusion is not selected carefully (above T4), kyphosis
cephalad to the upper limit of the fusion can occur.
 Pseudarthrosis is less frequent with newer instrumentation systems.
 Other possible complications are those inherent in any spinal operation, such
as urinary tract infection, ileus, and blood loss.

FRIEDREICH ATAXIA
 Friedreich ataxia is a recessively inherited condition characterized by
spinocerebellar degeneration.
 The genetic cause has been found to be a flaw within the frataxin gene on
chromosome 9q13.
 The clinical onset takes place between the ages of 6 and 20 years.
 Primary symptoms include progressive ataxic gait, dysarthria, decreased
proprioception or vibratory sense, muscle weakness, and lack of deep tendon
reflexes.
 Secondary symptoms include pes cavus, scoliosis, and cardiomyopathy.
 Labelle et al. evaluated 56 patients with a diagnosis of Friedreich ataxia and
found that all 56 patients had scoliosis.
 The most common pattern was double structural thoracic and lumbar curves
(57%).

 TREATMENT:
 Most authors have not found bracing to be useful for progressive curves in
patients with Friedreich ataxia.
 The orthosis fails to control the curve, and by the time scoliosis develops, the
patients often have a significant degree of ataxia and the restriction of a spinal
orthosis makes ambulation more difficult.
 Curves of less than 40 degrees should be observed, curves of more than 60
degrees should be treated operatively.
 Curves of between 40 and 60 degrees should be observed or treated
operatively, depending on the age of the patient, the onset of the disease, and
such characteristics of the scoliosis as the patient’s age when it is recognized
and evidence of progression of the curve.

 Cardiology evaluation is mandatory before any surgery is considered in these
patients.
 Patients with Friedreich ataxia frequently are unable to walk with
postoperative immobilization.
 Prolonged bed rest postoperatively must be kept to a minimum, or weakness
can increase rapidly.
 For these reasons, the ideal instrumentation for these patients is segmental
spinal instrumentation with multiple fixation devices, such as hooks,
sublaminar cables, or pedicle screws, that do not require external support
postoperatively.
 In general, these patients require a long fusion with attention to sagittal
contours to prevent later problems with thoracic kyphosis.

CHARCOT-MARIE-TOOTH DISEASE
 Classic Charcot-Marie-Tooth disease is a demyelinating neuropathy.
 The condition is dominantly inherited, with considerable variation in severity.
 The reported incidence of spinal deformity in Charcot-Marie-Tooth disease
varies from 10% to 26%.
 Some authors have found brace treatment to be well tolerated, whereas others
have had little success, with curve progression reported in 71% and with 33%
requiring instrumentation and fusion.
 The sagittal plane deformity accompanying this scoliosis most frequently is
kyphosis, and fusion to the pelvis generally is not necessary unless pelvic
obliquity exists.

SYRINGOMYELIA
 Syringomyelia is a cystic, fluid-filled cavitation within the spinal cord.
 Scoliosis may be the first manifestation of a syringomyelia.
 Syringomyelia can exist with or without Chiari I malformations.
 The proposed etiology of syringomyelia associated with Chiari I malformation is
disturbed or obstructed cerebrospinal fluid flow.
 Syringomyelia without associated Chiari I malformation is described as a
noncommunicating syrinx.
 Scoliosis has been reported in 63% to 73% of children with syringomyelia.
 Physical findings that may indicate syringomyelia include neurological deficits
and pain associated with the scoliosis, intrinsic muscle wasting of the hands,
cavus deformity, asymmetrical muscle bulk, occipital and upper cervical
headaches, and loss of superficial abdominal reflexes.

 Radiographic features suggestive of syringomyelia include Charcot changes in
joints and a left thoracic curvature.
 Patients with syringomyelia and scoliosis have been found to have thoracic
kyphosis (>40 degrees) instead of thoracic hypokyphosis seen with idiopathic
scoliosis.
 Cervical lordosis also is increased in this patient population.
 TREARMENT:
 The association of syringomyelia with scoliosis may have a significant influence
on treatment.
 Paraplegia and rupture of a large cyst in the cord resulting in death have been
reported in patients with syringomyelia who had instrumentation and fusion.
 Because of the possibility of these complications, surgery for scoliosis in
patients with syringomyelia should be approached cautiously.

 Drainage of the cyst, followed by observation to determine if the subsequent
curve stabilizes, has been recommended as initial treatment.
 Drainage of the syrinx allow use of distraction-type instrumentation without
complications.
 If instrumentation is necessary in these patients, distraction should be avoided if
at all possible.
 This can be accomplished by either anterior instrumentation or posterior thoracic
pedicle instrumentation with a direct vertebral rotation technique

SPINAL CORD INJURY
 Several series in the literature have reported an incidence of spinal deformity in
99% of children with spinal cord injuries before the adolescent growth spurt.
 Spinal deformity is much more common and the rate of curve progression much
greater in preadolescents than in older patients.
 Increasing curvature with pelvic obliquity in a child with a spinal cord injury can
lead to a loss of sitting balance that requires the use of the upper extremities for
trunk support rather than for functional tasks.
 Pressure sores may occur on the downside of the ischium, and hip subluxation
can occur on the high side of the pelvic obliquity.

 TREATMENT:
 ORTHOTIC MANAGEMET:
 Although some authors believe that alteration of the natural progression of
scoliosis in these patients is impossible with devices such as braces and corsets,
other authors indicated that orthotic treatment does have a place in the
management of scoliosis in preadolescent patients with spinal cord injuries.
 Orthotic treatment is difficult because of potential skin problems, but effective
slowing of progression has been noted.
 The use of an orthosis may delay the need for surgery in preadolescent patients
until longitudinal growth of the spine is more complete.
 A customfitted, well-padded, plastic total-contact TLSO generally is used.
 Close attention must be paid to any evidence of pressure changes on the skin.
The brace can be removed at night and used only during sitting

 Most preadolescent children with spinal cord injuries ultimately require surgical
stabilization of their scoliosis (50% to 60%).
 If the curve progresses despite orthotic treatment, operative intervention is
indicated.
 If the curve is more than 60 degrees when the child is first seen, surgery should
be considered.
 Segmental instrumentation allows more rigid fixation, and postoperative
immobilization can be avoided.
 Complete urinary tract evaluation should be done before surgery because urinary
tract infections are common in patients with spinal cord injuries.
 If possible, surgery should be delayed until the patient weighs more than 100 lb.
 This allows the use of larger rods and more stable fixation.

 With the increased use of thoracic pedicle screws and lumbar pedicle screws,
anterior release is becoming less necessary.
 It still should be considered, however, in a patient who has a large, rigid curve.
 If a definitive fusion is required in a young child at risk for future crankshaft
problems, a first-stage anterior release and fusion should be considered,
followed by posterior segmental spinal instrumentation and fusion.
 If laminectomy was used to treat the initial spinal cord injury, an increased
incidence of kyphosis can be expected.

POLIOMYELITIS
 The basic principles of
treatment, are no different
from those of treatment of
spinal deformities resulting
from other neuromuscular
diseases.
 Bonnett et al. outlined the
indications for correction
and posterior spine fusion
inpatients with poliomyelitis.

 As in any other neuromuscular curve, the length of fusion is much greater in
patients with poliomyelitis than in those with idiopathic scoliosis.
 Segmental instrumentation is recommended.
 In evaluation of the distal extent of the fusion in a patient with poliomyelitis, it
must be determined whether the pelvic obliquity is caused by the spinal
curvature itself or by other factors, such as iliotibial band contractures.

SPINAL MUSCLE ATROPHY
 Spinal muscle atrophy is an autosomal recessive condition in which the anterior
horn cells of the spinal cord, and occasionally the bulbar nuclei, atrophy.
 Daher et al. proposed that this disorder is caused by one episode of neural
destruction at different times in childhood.
 Children affected earlier in life have more severe involvement than do those
affected later.
 Spinal muscle atrophy can be classified into three types based on the severity of
disease and the age of patient at the time of clinical onset:
Type I, or acute infantile Werdnig-Hoffmann disease, is the most
severe form and is usually diagnosed within the first 6 months of life.
The course of the disease is progressive, with most of these children dying
within the first 2 to 3 years of life

Children with type 2 spinal muscular atrophy (chronic or intermediate
form) manage to achieve normal motor milestones until 6 to 8 months of age.
They often are very weak but can usually sit without support.
Patients will usually survive into the third or fourth decade.
Type III, or Kugelberg-Welander disease, usually is seen after 2 years of
age.
It is more slowly progressive, and most patients are able to ambulate
independently.
 On clinical examination, children with spinal muscle atrophy have severe
weakness of the trunk and limb muscles.
 Fasciculations of the tongue and tremors of the extremities are frequent.
 Reflexes are diminished.
 Most patients have normal intelligence, and the heart is unaffected by the
disease process.

 TREATMENT:
 Bracing has been reported to slow progression of the curve and allows sitting
for longer periods.
 When the scoliosis in a skeletally immature patient reaches 20 degrees in the
sitting position, orthotic treatment should be considered, usually with a total-
contact TLSO.
 This is used only during sitting to minimize progression of the curve and to
provide an extremely weak child with a stable sitting support.
 Severe chest wall deformities can occur from bracing, and developing chest wall
deformities are a contraindication to brace treatment.
 Although bracing may not eliminate the need for surgical stabilization, it may
delay surgery until closer to the end of growth.

 Surgical treatment of the spinal deformity is posterior spinal fusion with
posterior segmental instrumentation and adequate bone grafting.
 Because fusion to the sacrum is needed for many of these patients, fixation to
the pelvis can be obtained by the Galveston or iliac screw technique.
 Augmentation of the fusion with bone-bank allograft bone usually is
necessary.
 If the vertebrae are extremely osteoporotic, external support, such as a
bivalved body jacket, can be used.
 For a severe fixed lumbar curve with pelvic obliquity, anterior release, and
fusion may be needed in addition to posterior instrumentation.
 It should be understood, however, that anterior surgery in patients with severe
pulmonary compromise carries a great risk, and this risk must be evaluated
carefully before surgery.

 If possible, endoscopic techniques of anterior release and fusion should be
considered.
 Preoperative traction offers an excellent method to improve the flexibility of
the spine and also improves pulmonary function before posterior fusion and
instrumentation.
 Complications should be expected in this group of patients.
 Pseudarthrosis, atelectasis, pneumonitis, and death have been reported.

FAMILIAL DYSAUTONOMIA
 Familial dysautonomia (Riley-Day syndrome), is a rare autosomal recessive
disorder.
 Its clinical features include absence of overflow tears and sweating, vasomotor
instability that often leads to hyperthermia, and relative indifference to pain.
 Other frequent findings include episodic hypertension, postural hypotension,
transient blotching of the skin, hyperhidrosis, episodic vomiting, disordered
swallowing, dysarthria, and motor incoordination.
 Death is caused most often by pulmonary disease.
 Scoliosis is the major orthopaedic problem in patients with this disease.
 The scoliosis may be progressive and may be large enough to contribute to
early death because of kyphoscoliotic cardiopulmonary decompensation.

 Kyphosis also is a frequent sagittal plane deformity in these patients.
 If surgery for the scoliosis is considered, however, features of the syndrome such
as vasomotor and thermal instability can cause troublesome and sometimes fatal
operative or postoperative complications.
 Brace treatment, although beneficial in some patients, often is complicated by
the tendency for pressure ulcers to develop.
 Posterior spinal fusion with instrumentation was required in 13 of 51 patients in
the Israeli series of Kaplan et al.
 All children undergoing surgery had severe pulmonary problems.
 Intraoperative and postoperative respiratory and dysautonomic complications
were frequent.

 Because of osteopenic bone, only minor improvement of the spinal deformity
was possible, and a small loss of correction was common; however, those
surviving noted a marked decrease in the frequency of pneumonia and, for
some reason, an improvement in the degree of ataxia.
 Intraoperative complications included transient hypertension, failure of the
lamina because of osteopenia, and an endotracheal tube that was plugged by
thick secretions after the lung was collapsed for an anterior approach.

ARTHROGRYPOSIS MULTIPLEX
CONGENITA
 Arthrogryposis multiplex congenita is a syndrome of persistent joint
contractures that are present at birth.
 A myopathic subtype is characterized by muscle changes similar to those found
in progressive muscular dystrophy.
 In the neuropathic subtype, anterior horn cells are reduced or absent in the
cervical, thoracic, and lumbosacral segments of the spinal cord.
 In the third subtype, joint fibrosis and contractures alone are the main problems.
 Scoliosis is common in patients with arthrogryposis multiplex congenita (20% to
66%).

 A single thoracolumbar curve is the predominant curve pattern.
 The scoliosis usually is detected at birth or within the first few years of life.
 Brace treatment rarely is successful and should be used only with small, flexible
curves (<30 degrees) in patients who were ambulators.
 In patients who are nonambulatory or have curves of more than 30 degrees, the
brace is ineffective in controlling the curve.
 The onset of pelvic obliquity is a serious problem.
 If treatment of the pelvic obliquity by release of the contractures in the hip area
does not halt progression of the curve, spinal fusion to the sacrum may be
necessary.
 The onset of thoracic lordosis also requires prompt treatment.

 Because of the severity and rigidity of the curves, postoperative complications
are frequent.
 The connective tissue is tough, and the bones are osteoporotic.
 An average blood loss of 2000 mL has been reported, and Herron et al. obtained
a maximal correction of only about 25% with Harrington instrumentation and
posterior fusion.
 Combined anterior and posterior spinal arthrodesis has been recommended in
these patients because it is more effective in terms of curve correction and in the
least loss of correction at follow-up.
 If patients have less than 90 degrees of passive flexion of the hip, caution should
be exercised in extending spinal arthrodesis to the pelvis because this is likely to
make sitting difficult.

DUCHENNE MUSCULAR
DYSTROPHY Duchenne muscular dystrophy is an inherited X-linked recessive condition
caused by a frameshift mutation in the dystrophin gene at the Xp21.2 locus of
the X chromosome.
 The clinical course is of progressive weakness, losing the ability to walk between
10 to 14 years of age, and becoming wheelchair dependent.
 Death from pulmonary or cardiac compromise usually occurs in the second or
third decade of life.
 Scoliosis develops in most patients with Duchenne muscular dystrophy, although
the use of corticosteroids may decrease the development of spinal deformity in
these patients.
 Spinal deformity usually occurs after the patient becomes confined to a
wheelchair, although very early scoliosis has been detected in some ambulatory
patients.

 The curves are predominantly long thoracolumbar curves with pelvic obliquity,
the collapse of which is caused by absence of muscles and not by asymmetrical
muscle activity or contracture.
 Once a curve develops, it generally is progressive and cannot be controlled by
braces or wheelchair seating systems.
 In patients with Duchenne muscular dystrophy, pulmonary function deteriorates
approximately 4% each year after the age of 12 years.
 If orthotic treatment is continued while pulmonary function deteriorates
significantly, surgical stabilization may become impossible.
 When the forced vital capacity decreases to less than 35%, surgery probably is not
advisable because of the potential pulmonary problems postoperatively.

 Lonstein and Renshaw’s indications for spinal fusion in patients with Duchenne
muscular dystrophy:
-curves of more than 30 degrees
-forced vital capacity of more than 30% of normal
-prognosis of at least 2 years of life remaining
 Because the scoliosis invariably increases, many authors recommended spinal
fusion at the onset of the deformity in patients who use a wheelchair full time
even when the curves are as small as 20 degrees or less.
 Treatment of scoliosis in patients with Duchenne muscular dystrophy consists of
segmental instrumentation with sublaminar wires or cables, hooks, or pedicle
screws and fusion from T2 to the pelvis.
 In patients with smaller curves and no fixed pelvic obliquity, the fusion and
instrumentation can end at L5.

 Fixed pelvic obliquity is more than 15 degrees, fusion to the pelvis with iliac
screw or S-rod pelvic fixation is indicated.
 Lonstein and Renshaw listed the following benefits of spinal fusion in patients
with Duchenne muscular dystrophy:
-preserves sitting balance
-prevents back pain
-improves spinal decompensation
-frees the arms of the necessity of trunk support
-improves body image
-possibly slows the deterioration of pulmonary function

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