CONGENITAL SCOLIOSIS

MODERATOR : DR
PRABODH
KANTIWAL ,ASSOCIATE
PROFESSOR,DEPT OF
ORTHOPAEDICS-AIIMS
JODHPUR

PRESENTER :DR SAGAR

SETIA
 INTRODUCTION
⮚ Scoliosis term derived from
Greek word meaning “crooked”
⮚ Congenital scoliosis - lateral
curvature of the spine due to
vertebral anomalies (due to
defect in formation and/or
segmentation) that result in an
imbalance of the longitudinal
growth of the spine.
 EPIDEMIOLOGY

Prevalence of 1 in 1000 live births(0.1%)

Female: Male=3:2.
The critical time in spine development is 5th - 6th week - time of
segmentation processes—and congenital anomalies develop
during the first 6 weeks of intrauterine life.
Risk Factors :
• Genetics (sonic hedgehog or homeobox genes)
• Environmental factors (during the prenatal period)
• Hypoxia
• Alcohol use
• Valproic acid
• Hyperthermia
• Maternal CO exposure
 WINTER’S CLASSIFICATION

Winter RB, Moe JH, Eilers VE. Congenital Scoliosis A Study of 234 Patients Treated and Untreated Part I: Natural History. J
Bone Joint Surg Am. 1968 Jan 01;50(1):1–15
 KAWAKAMI ET AL CLASSIFICATION
Classification of Congenital Vertebral Abnormalities (based on presence or absence
of abnormal formation) - three-dimensional (3D) computed tomography (CT) image
⮚ Type 3 : Complex (discordant) type
⮚ Type 1 : Solitary simple (unison) type
Mismatched complex
Hemivertebra
type
Wedge vertebra
Mixed complex type
Butterfly vertebra
Defect
⮚ Type 4 : No abnormal formation
Others
type
⮚ Type 2 : Multiple simple (unison) type
Pure segmentation
Combination of hemivertebra, wedge vertebra, or butterfly vertebra
failure
Discrete, adjacent, or others
Kawakami N, Tsuji T, Imagama S, et al. Classification of congenital scoliosis and kyphosis: a new approach to the three-dimensional classification for
progressive vertebral anomalies requiring operative treatment. Spine (Phila Pa 1976). 2009;34(17):1756-1765.
 WEDGE VERTEBRAE

❖ Partial unilateral failure of formation

❖ Wedged or trapezoid-shaped vertebra that

contains two pedicles, although one of them
may be hypoplastic

❖ Associated scoliosis worsens slowly

 HEMIVERTEBRA
❑ Hemivertebra is a wedged-shaped vertebra with 1
pedicle on 1 side i.e. Unilateral complete failure of
formation

❑ Highest likelihood of progressive deformity

❑ According to the relation with the cranial and caudal

adjacent vertebral bodies it can be :-
fully segmented, semi-segmented , incarcerated and
unsegmented,
FULLY SEGMENTED HEMIVERTEBRAE
❑ Unilateral complete failure of formation

❑ Separated from adjacent vertebra (both superior and inferior)

by a normal vertebral growth plate and disc
SEMI SEGMENTED HEMIVERTEBRA

⮚ Separated from one adjacent vertebra (superior or inferior)

by a normal vertebral growth plate and disc

⮚ Fused to the other adjacent vertebra

⮚ Treatment is necessary only if the deformity is progressive

INCARCERATED HEMIVERTEBRA.

❖Ovoid and smaller than a fully segmented

(nonincarcerated) hemivertebra
❖Vertebrae above and below compensate for
this hemivertebra
❖Minimal if any scoliosis is present
NONSEGMENTED HEMIVERTEBRAE

❖ Fused to both adjacent vertebrae (above and

below)

❖ No vertebral end-plates or adjacent discs

❖ No progressive spinal deformity

HEMI METAMERIC SHIFT

Defined as a hemi-vertebral deformation in which

1 or more hemivertebrae exists on each side of
the spine and are separated by at least 1 normal
vertebra.
BLOCK VERTEBRAE
❑ Circumferential, symmetric failure of segmentation leads to a block
vertebra.

❑ Jarcho Levin Syndrome (Spondylocostal dysostosis)

UNILATERAL BAR

⮚ Unilateral failure of segmentation two or

more vertebrae (unilateral bar)

⮚ Rib fusions or other rib abnormalities on the

concavity
MIXED TYPE

Unilateral failure of segmentation with one or

more hemivertebrae located on the opposite
(convex) side of the curve

Worst prognosis

Severe and rapidly progressive deformity

ASSOCIATED SYNDROMES

❑ Aicardi syndrome
❑ Cleidocranial dysostosis
❑ Gastroschisis
❑ Gorlin syndrome
❑ Fetal pyelectasis
❑ Jarcho-Levin syndrome
❑ OEIS complex
❑ VACTERLS association
❑ Goldenhar syndrome
VACTERLS

V: Vertebral
A: Anorectal
C: Cardiac
TE: trachea- esophageal
R: Renal
L: Limb defect
S: Single Umbilical Artery
 NATURAL HISTORY

⮚ Noticed by parents either at birth or usually around 2 years of age or during adolescence

⮚ May be associated with cyanotic spells, anorectal abnormalities, Limb abnormalities

(VACTERLS)

⮚ May or may not be associated with spinal cord abnormalities

 NATURAL HISTORY
⮚ Curve Progression:

Curve progression occurs more rapidly during the first 5 years of life and during
the adolescent growth spurt

Maximum in unilateral unsegmented bar with contralateral hemivertebra f/b unilateral

unsegmented bar

Maximum in Junctional defects (thoracolumbar region)

⮚ Thoracolumbar curves : 7 degrees per year up to 10 years

14 degrees per year during adolescent growth spurt
NATURAL HISTORY
❑ Severe congenital lumbar or thoracolumbar curves - notable pelvic obliquity and
apparent lower limb length inequality

❑ Compensatory curves in an otherwise normal spine develop more commonly in

patients with congenital scoliosis
i. Curve apex at T5, T6, or T7.
ii. As the congenital curve deteriorates, this secondary curve may worsen, become
inflexible, and require treatment.
Median yearly rate of deterioration without treatment for each type of single congenital scoliosis in each
region of spine.
Numbers on left in each column (patients seen before 10 years of age); numbers on right refer (patients
seen at age 10 years or older)
PATIENT EVALUATION
Through history taking

Physical and neurological examinations

Skin of back [hair patches, lipomas, dimples, and scars s/o underlying anomalous vertebra
or spinal cord anomalies

Neural Axis Abnormalities: 35%

Congenital Heart Disease: 25 – 54 %

Genitourinary anomalies: 20-40%

▪ Diastematomyelia was the most common intraspinal
abnormality (306/671, 45.60%).
▪ Syringomyelia (273/671, 40.69%), tethered cord
(190/671, 28.32%), low conus (58/671, 8.64%),
intraspinal mass (39/671, 5.81%)
▪ Chiari malformation (32/671, 4.77%), fatty filum
(27/671, 4.02%), spina bifida (occulta excluded)
(17/671, 2.53%), tumor (17/671, 2.53 %)
 CLINICAL EVALUATION

❑ CORONAL SPINE BALANCE (drop plumb line form spinous process of C7/ base of skull,
should not deviate more than 1-2cm from center of gluteal cleft)

❑ ADAMS FORWARD BENDING TEST

❑ SCOLIMETER in conjunction to AFBT to evaluate for truncal rotation

❑ LIMB LENGTHS should be measured because a discrepancy may cause a pelvic tilt and a
compensatory scoliosis.
ADDITIONAL EVALUATION

❑Syndromic associations

❑Pediatrician /Genetic Clinic

❑2D Echo

❑USG KUB

❑PFT
RADIOLOGIC EVALUATION

❑ X-ray whole spine : ap/lateral view/side bending

❑ NCCT Whole spine with pedicle cuts

❑ MRI WHOLE SPINE :

To look for associated spinal cord abnormalities
Prevalence rate of spinal dysraphism on MRI - 43%
 TREATMENT MODALITIES

⮚ Observation

⮚ Bracing

⮚ Operative treatment
OBSERVATION

• Limited value in patients with congenital scoliosis

• Nonprogressive curves require regular observation during

periods of rapid growth (0 to 5 years of age and 10 to 15

years of age)

• Radiographs twice a year

 BRACING
• Can be used to control secondary curves when the primary congenital curve
is being treated nonoperatively
• Prevent progression of a secondary curve that is causing balance problems
• Careful measurement and comparison of spine radiographs at 6-month
intervals must be made
BRACING
EDF (Elongation Derotation
Flexion) Casting

• Growth guidance

• Can be done under GA/ Awake

patient

• 3 dimensional Correction
attained over a Cortel frame
and stabilized with POP cast
 FULL-TIME BRACES NIGHT TIME BRACE

BOSTON BRACE

Milwaukee brace
 HALO GRAVITY TRACTION
❑ Used as an adjunct to surgery for rigid
curves

❑ Not always indicated

❑ Requires prolonged hospital stays

OPERATIVE TREATMENT
Why to operate ?
• Halt the progression
• Improve cardiorespiratory reserve
• Improve harmony & balance (improves long-term HRQOL outcomes)
• Cosmetic correction (improves self-image)
• As 75% of congenital curves are progressive, surgery remains the
fundamental treatment
• Procedures that prevent further deformity or procedures that correct the
present deformity (gradually or immediately)
SURGICAL METHODS
⮚ Prevention of future deformity
i. In situ fusion

⮚ Correction of deformity—gradual
i. Hemiepiphysiodesis and hemiarthrodesis
ii. Growing rod nonfusion
iii. Vertical expandable prosthetic titanium rib (VEPTR)

⮚ Correction of deformity—acute
i. Instrumentation and fusion
ii. Hemivertebra excision
iii. Vertebral column resection
iv. Osteotomy
INTRAOPERATIVE CONSIDERATIONS

❑ Continuous monitoring of BP: an intra-arterial cannula

❑ Urinary catheter for urinary output monitoring
❑ Electrographic leads
❑ Pulse oximeter as an adjunct to intra-arterial cannula
❑ Spinal cord monitoring using both spinal somatosensory and motor evoked
potential
❑ Triggered electromyographic monitoring is useful to detect a possible breach
in the pedicle wall by a pedicle screw
 NEURO MONITORING

Neuro-monitoring recorded during the surgery (MEP)

 Surgical intervention

Growth sparing Fusion/

surgeries Arthrodesis
 Growth
sparing

Convex
Growth rod
hemiepiphysiodesis

Shilla VEPTR
 GROWTH ROD

⮚ Growing rod instrumentation : posterior instrumentation that is

sequentially lengthened to allow longitudinal growth while still

attempting to control progressive spinal deformity

⮚ Usually is considered for patients younger than 10 years of age

who have a curve of 60 degrees or more

GROWTH ROD

⮚ Surgery typically is required every 6

months to lengthen the construct
⮚ Might need a definitive procedure after
skeletal maturity/growth arrest
⮚ Multiple surgeries
⮚ Prevent crankshaft phenomenon
10-year-old girl with congenital scoliosis resulting from failures of segmentation. The deformities of spine were
severe and showed a definitely progressive trend. She was treated with dual growing rod and underwent
5 lengthening in 30 months. The deformity was corrected and maintained well during the lengthening.
MAGNETIC CONTROLLED GROWTH RODS

⮚ MCGR can be lengthened using external remote controller on an outpatient

basis.
⮚ Reduce the risk of wound related complications, provides similar deformity
correction, but may not provide as much spinal growth.
⮚ Complications: Loss of distraction, screw pull out, junctional kyphosis ,
metallosis around the instrumentation (necessitates MCGR removal)
Eight-year-old boy with congenital scoliosis After insertion of MAGEC growing rods.
SHILLA GROWTH GUIDANCE
► Allows growth during treatment

► It consists of a nonlocking pedicle screw implant

► Dual rods are fixed posteriorly to the corrected

apex

► Limited fusion at the apex

► Sliding pedicle screws

 VEPTR
▪ Vertical Expandable Prosthetic Titanium Implant
▪ Rib abnormalities: Thoracic Insufficiency syndrome
▪ Congenital scoliosis associated with fused ribs on the
concave side of the curve
 CONVEX
HEMIEPIPHYSIODESIS

Transpedicular convex anterior hemiepiphysiodesis and

posterior arthrodesis
 CONSTRUCT ADVANTAGES DISADVANTAGES
Traditional growth rods Longest follow-up (time-tested Multiple surgical
(TGR) implants) procedures/anaesthetic risk

Low initial cost Law of diminishing returns

Better Cobbs correction, greater Hardware complications

spine length gains
Magnetic growth rods Avoids multiple surgeries Short follow-up
(MGR)
Psychological benefit High initial cost

Long-term cost effectiveness Loss of sagittal balance

Equivalent results to TGR Law of diminishing returns

Convex hemiepiphysiodesis Good results in less severe cases Limited indications

Preserves discs better Poor results

(experimental evidence)

Guided-growth procedures Mimics normal growth Lack of long-term studies

(Shilla) (theoretically)
Crankshafting
Avoids multiple surgeries
Inferior results to TGR in
comparative studies
 HEMIVERTEBRAE EXCISION

⮚ It produce immediate correction of a congenital

spine deformity
⮚ The L3, L4, or LS level, below the level of the
conus medullaris, is the safest level , thoracic
area has more risk as the spinal canal is the
narrowest and has the least blood supply
⮚ Optimal indication of hemivertebra resection is a
patient younger than 5 years with a TL, lumbar ,
or LS hemivertebra that is associated with truncal
imbalance or a progressive curve.
 HEMIVERTEBRAE EXCISION

Hemivertebra excision (combined Antero-posterior approach)

A, Patient positioning for anteroposterior excision
B and C, Resection of posterior hemilamina with Kerrison rongeur
D, Compression of laminar hooks with
closure of excision site
E, Anterior resection
F, Resection carried back to pedicle

(From Hedequist DJ,Emans JB: Hemivertebra excision. In Wiesel SW, editor: Operative techniques in orthopaedic
surgery,Philadelphia, 2011, Wolters Kluwer/Lippincott Williams & Wilkins, p 1466.)
HEMIVERTEBRAE EXCISION
Hemivertebra excision: Posterior approach
A, Pedicle is entered with standard pedicle probe and expanded
with series of increasing sized curets; this often creates a hole large
enough to see into.

B, Cross-section of hemivertebra. Medial wall of pedicle (black

arrow) is second-to-last thing to be removed. Posterior wall of
vertebral body is last thing to be removed by pushing anteriorly,
away from neural elements (white arrow).

In this case, not only are inferior disc and endplate removed, but
also some of vertebral body is removed to maximize correction.

(From Hedequist DJ, Emans JB: Hemivertebra excision. In Rhee J, Wiesel SW, Boden SD, et al, editors: Operative
techniques in spine surgery, Philadelphia, 2013, Lippincott Williams & Wilkins, pp 367-375.)
⮚ Similar correction of curves can be achieved with
single posterior and combined anterior and posterior
hemivertebra resection.
⮚ Benefits of the SPA are lower complication rate and
shorter recovery period.
 Arthrodesis

Anterior and
Posterior Insitu
Posterior
 ANTERIOR AND POSTERIOR FUSION
❑ Immature individuals, continued anterior growth: crankshaft phenomenon

(occurs when only posterior fusion is done in patients with a significant anterior

growth remaining)

❑ Entire spine and trunk gradually gets rotated and deformed as the anterior

portion continued to grow and twist around the axis of the fusion mass

❑ Prevention of crankshaft phenomenon:

Careful assessment of growth remaining prior to surgery

Combining anterior and posterior fusion in younger children

 POSTERIOR SPINAL FUSION

❑ Older children
❑ Neuromonitoring
❑ Correction through the flexible part of the curve (except
VCR)
❑ May be supplemented with Convex Rib resections
*Same midline incision
*Good bone graft source
*Good aesthetic reduction of the rib prominence.
❑ Crankshaft phenomenon prevented with modern implants
 OSTEOTOMIES
Smith-Peterson Pedicle Subtraction Vertebral column
Ponte’s osteotomy
osteotomy (SPO) Osteotomy (PSO) resection (VCR)

• Posterior Column • SPO + both superior • Closing wedge, • Maximum

Osteotomy and inferior facets, three column complication rates
the posterior
• Correction of 5 ligaments • Posterior elements • 40 degrees
degrees per level and a V-shaped correction per level
• Correction of 10-15 bony wedge of the
• Multiple levels degrees vertebral body are • Approximately 4
needed to achieve resected and the liters blood loss and
desired correction middle column is 8 hours per
shortened without osteotomy
lengthening the
anterior column

• 20-30 degree
correction per level
OSTEOTOMIES
THORACIC INSUFFICIENCY SYNDROME

• Thoracic insufficiency syndrome is defined as the inability of the thorax to support

normal respiration or lung growth
• Occurs in -
i. Hypoplastic thorax syndromes, such as Jeune and Jarcho-Levin syndromes
ii. Progressive infantile scoliosis with reductive distortion of the thoracic volume from
spinal rotation
iii. Congenital scoliosis associated with fused ribs on the concave side of the curve.
 LUNG DEVELOPMENT
At birth: 20 million
alveoli
Age 5: 300 million 300

No. of alveoli (x 10⁶)

alveoli
200 250x10⁶ 300x10⁶

100
20x10⁶

4 8
Birth Age(year)

Early growth disturbance compromises thoracic volume

TAKE HOME MESSAGE

Multiple types

Early diagnosis and early treatment

Surgical treatment is mainstay of treatment

Associated with multiple syndromes

THANK YOU