2. HISTORY AND PHYSICAL EXAMINATION IN
PEDIATRIC
SPINAL DEFORMITIES
• It is important to assess children with possible spinal deformities in
relation to their age and development to formulate an appropriate
differential diagnosis.
• The history in younger patients or in preverbal children is often obtained from
the parents, but older children and adolescents should be able to give a
useful history of their symptoms.
• A detailed birth history should be obtained for most pediatric patients. This is
most important in the evaluation of children for spinal deformities during the
neonatal or infant periods
3. • Specific history points should include
• gestational age,
• exposure to illicit drugs or toxins during pregnancy, whether routine prenatal care
was available and obtained,
• infections during pregnancy and delivery,
• metabolic abnormalities after birth,
• cardiopulmonary status,
• the history of seizures
• detailed family history should be obtained for any neonate or infant presented for
assessment of spinal deformity. This can aid in the diagnosis of inherited disorders
that portend spinal deformity in children (Friedreich's ataxia, spinal muscular
atrophy, Duchenne's or Becker's muscular dystrophy) or strengthen the case for
adolescent idiopathic scoliosis.
• History of any birth defects should also be included.
• All children should be evaluated for development by assessment of milestone
achievement based on age.
4. • Delays in milestone achievement
• more importantly, regression of previously gained function should be particularly
noted.
• Evaluation of patients' major presenting symptoms is important in the evaluation of
children with spinal deformities.
• if a child complains of back pain, it should be paid special attention because the
likelihood of an underlying abnormality is usually much greater.
• The examination of patients with possible spinal deformities should not be limited
to the neurological or orthopedic system.
• Children should have their skin examined for café-au-lait spots, neck webbing, low-
set hairline, midline cutaneous lesions such as dimples, hemangiomas, hairy patches,
and appendages, or subcutaneous lipomas.
• Most important of these neurological evaluations is motor testing, with specific
attention paid to individual muscle group testing and tone within the extremity
musculature.
5. • Examination of the spine should then include palpation for tenderness, spinal
column deficits, and abnormal prominence of one side of the spine.
• The Adam's forward bend test should be performed to assess for any
thoracic or lumbar prominence on one side of the body, which can be
quantified by a scoliometer.
• When a spinal deformity is suspected, the flexibility of the curve should be assessed
by having the child bend laterally to both sides and the spine manipulated by the
examiner to document flexibility.
• Pulmonary function can be assessed with formal pulmonary function tests in the
appropriate clinical setting.
7. RADIOLOGICAL PARAMETERS OF
SCOLIOSIS
• The major imaging modalities in the pediatric population for spinal deformity
include plain films,
computed tomography (CT),
magnetic resonance imaging (MRI),
ultrasonography (US).
• A biplane x-ray imaging system called EOS (EOS Imaging, Paris) allows the
simultaneous acquisition of weight-bearing full-body posteroanterior (PA), lateral,
and three-dimensional views of the spine with minimal radiation dosage to the
patient.
• In the sagittal plane, there is approximately 40 degrees (range 30-50 degrees) of
cervical lordosis, 30 degrees (range 10-50 degrees) of thoracic kyphosis, and 50
degrees (range 35-80) of lumbar lordosis.
8. PLAIN FILMS
• Initially, standing (or seated, if necessary, because of patient age or severe
neuromuscular disability) 36-inch plain films (PA and lateral) remain the most ideal
means of assessing spinal deformity.
• When evaluated along with a detailed history and physical examination, plain
radiographs can aid in the decision to obtain more advanced imaging.
• Hyperextension films for assessing patients with kyphosis can also be obtained with
use of a bolster. The push-prone radiographs are obtained with the patient prone
and a technician wearing a lead glove pushing on the patient's side to try to correct
the deformity.
• These types of radiographs aid in assessing the degree of rigidity of the deformity
and help with surgical decision making
9. • The initial PA view is assessed for skeletal maturity utilizing the
• Risser stage (progression of iliac crest ossification), the major and minor curvature
with
• Cobb angle,
• coronal balance,
• shoulder balance,
• Pelvic obliquity
11. COBB ANGEL
• Cobb angle is measured by defining the angle formed between a line drawn parallel
to the superior end plate of the most tilted rostral vertebrae and the line parallel to
the inferior end plate of the most tilted caudal vertebrae.
12. CORONAL BALANCE
• The coronal balance is measured by dropping a plumb line from the centroid of C7
and measuring the difference between this line and the central sacral vertical line
(positive balance is defined to the right, negative balance to the left).
13. • The shoulder balance can be measured in multiple different ways.
• clavicle angle as described by Kuklo and associates.
• In this method the angle is defined as the intersection of a horizontal line and the
tangential line connecting the highest two points of each clavicle
• Perfect shoulder balance is defined as 0 degrees.
14. • In a similar manner, pelvic obliquity is determined by the intersection of a horizontal
line and the tangential line connecting the highest two points on each iliac crest
15. MAGNETIC RESONANCE IMAGING
• MRI should be obtained in any patient with abnormal neurological findings on the initial
evaluation, atypical features of scoliosis (acute curve; apex to the left; or
• significant kyphotic component), or rapidly progressive curves.
• Patients with juvenile or infantile idiopathic scoliosis, congenital scoliosis, and neuromuscular
scoliosis have a higher incidence of neural axis abnormalities than patients with adolescent
idiopathic scoliosis.
• The most MRI useful sequences include sagittal T1- and T2-weighted imaging of the entire
spine from the posterior fossa through the coccyx.
• These sagittal images in conjunction with axial images should be utilized to assess for
• Chiari I malformation,
• syringomyelia or hydromyelia,
• level of termination of the conus, and filum terminale
• thickening or lipoma.
• Contrastenhanced imaging may be useful in assessing for neoplastic and inflammatory
• conditions.
16. • Coronal T2-weighted imaging is helpful in assessing for spinal column abnormalities
(hemivertebrae, block vertebra) and spinal cord abnormalities (split cord
malformation).
• Nonspinal disorders such as renal anomalies may also be observed
incidentally and aid in the overall diagnosis
17. COMPUTED TOMOGRAPHY
• CT is especially useful in assessing for bone abnormalities, including hemivertebrae,
block vertebrae, deficits in the neural arches, neural foramina, and articular facets
• Advances in CT technology have allowed detailed two-dimensional reconstructions
in all planes (sagittal, coronal, and axial) to give the surgeon a complex
understanding of the anatomy.
• This modality is particularly useful in
• preoperative planning for more complex scolioses, such as congenital deformities,
by allowing precise measurements of pedicle size, orientation, and anatomic
relationships between bony structures.
• CT also enables the surgeon to assess rigid or fused areas of the spine and thereby
to be prepared to release these areas, if necessary, during correction.
18. ULTRASONOGRAPHY
• Ultrasonography is being used more and more to screen young neonates and
infants younger than 4 months for intraspinal pathologic conditions that can lead to
spinal deformity.
• This modality can be used to assess the level and thickness of the conus, any
abnormal spinal lesions, movement of the cauda equina, or abnormal spinal cord
mobility that may be a contributing factor to the patient’s spinal deformity.
• Its major advantage is the limitation of irradiation and/or sedation in young patients
in comparison with CT and MRI
19. ETIOLOGY AND CLASSIFICATION OF PEDIATRIC SPINAL
DEFORMITIES
• The most common etiology, however, is idiopathic scoliosis,
• which is diagnosed only after exclusion of these other predisposing diseases.
Conditions known to be associated with scoliosis include neuromuscular disorders,
congenital anomalies, syringomyelia, Chiari malformation, connective tissue
disorders, neurofibromatosis, dwarfism syndromes, and other skeletal dysplasias.
• These conditions are important to exclude because their specific features and
resultant effects on the spinal column are influenced by the precise disease
pathophysiology; therefore, management strategies for treatment may differ.
20. INFANTILE IDIOPATHIC SCOLIOSIS
• Infantile idiopathic scoliosis is defined as deformity occurring before 3 years of age
without an underlying abnormality.
• This term was introduced by Harrenstein
• In 1965, Lloyd-Roberts and Pilcher suggested that intrauterine molding—similar to
skull remodeling—may play a greater impact than infant positioning.
• As mentioned previously, scoliosis tends to progress significantly during periods of
rapid spinal growth.Because the most rapid rate of postnatal spinal growth occurs
between 0 and 3 years of age, infantile scoliosis has a high risk of progression.
• However, previous studies have demonstrated two specific types of infantile
scoliosis, progressive and resolving.
21. • Older children had a significantly greater likelihood of having progressing idiopathic
scoliosis
• Mehta, in 1972, demonstrated that evaluation of plain radiographs
• for the apical rib–vertebra angle could offer significant prognostic ability for curve
progression or resolution of curves in infants, with high intrarater and interrater
reliability.
• Mehta introduced the rib vertebra angle difference (RVAD): the difference
between the apical-rib vertebra angle on the convex side (lesser magnitude) and that
on the concave side (greater magnitude)
• If the vertebra covered the rib head, the patient was very likely to have
a progressive curve.
Management of children with progressing infantile scoliosis is predicated on
preventing pulmonary compromise and obtaining an appropriate cosmetic
appearance.
22. • If a patient has an RVAD less than 20 and no overlap of the apical rib and vertebra
on AP radiographs, the current recommendation is for observation with radiographs
at 4- to 6-month intervals until skeletal maturity.
• greater than 5 to 10 degrees of change in the Cobb angle in the observation period,
or a baseline Cobb angle greater than 35 degrees signals a requirement for non
observational management
• No matter the type of brace used, the goal should be to halt curve progression until
more definitive treatment can be undertaken after the
adolescent growth spurt. However, curves that continue to progress in spite of
bracing may require surgical fusion.
23. JUVENILE IDIOPATHIC SCOLIOSIS
• Juvenile idiopathic scoliosis is defined as a lateral curvature of the spine greater
than 10 degrees without a known underlying abnormality diagnosed between 4
and 10 years of age.
• Craniospinal imaging with MRI is important to exclude predisposing conditions
such as Chiari malformation, congenital vertebral malformation,
neurofibromatosis, tethered cord, spinal cord tumors, and syringomyelia, which
have been reported in up to 15% to 20% of cases.
• After exclusion of these predisposing conditions, patients with true juvenile
idiopathic scoliosis need to be monitored closely because the progression rate is
typically much higher than that with adolescent idiopathic scoliosis.
• Natural history of juvenile idiopathic scoliosis is much more aggressive than that of
the adolescent form;
24. • observation is indicated for patients with curves less than 20 degrees at
presentation, bracing for patients presenting with 25- to 30-degree curves,
and surgical fusion for patients with curves greater than 30 to 40 degrees
25. ADOLESCENT IDIOPATHIC SCOLIOSIS
• Adolescent idiopathic scoliosis (AIS), or a lateral curvature of the spine of greater
than 10 degrees without an underlying abnormality, is the most common pediatric
spinal deformity in patients 10 to 17 years of age, with a female sex predilection
tenfold greater than that in males.48 The prevalence of AIS has been reported to
range from 0.5 to 5 per 100 adolescents.
• Classification and surgical management of AIS have been addressed by two
major systems.
• First, in 1983, King and associates devised a treatment algorithm–
based classification system that depended on five curve types.
However, major limitations of this system included inclusion of only thoracic curves,
its assessment of scoliosis as a purely coronal deformity as opposed to the three-
dimensional deformity inherent in scoliosis, and its poor interrater and intrarater
reliability.
• In 2001, a new AIS classification system was developed by Lenke and colleagues.
26.
27. • Recommendations for general management are for observation of curves with
magnitudes between 1 and 25 degrees, bracing for curves between 25 and 45
degrees, and surgical management for curves greater than 45 to 50 degrees.
• Observation at 6-month intervals with clinical and radiographic examination is
appropriate for patients with curves measuring less than 25 degrees.
• Bracing for 23 hours per day has been suggested for children who have not finished
growth (Risser sign stage 3 or less) with curve magnitudes of 25 to 45 degrees.
28. NEUROMUSCULAR SCOLIOSIS
• Neuromuscular scoliosis refers to a spinal deformity that results from either
abnormal nervous innervation (neuropathic type) to the axial musculature or a primary
muscle disorder (myopathic type) affecting the musculature adjacent to the spine.
• Both these types of disorders may be developmental or acquired.
• Conditions Associated with Neuromuscular Scoliosis
• Neuropathic
• Cerebral palsy
• Friedreich's ataxia
• Charcot-Marie-Tooth disease
• Roussy-Levy disease
• Syringomyelia
• Spinal cord tumor
• Spinal cord trauma
• Poliomyelitis
30. • The treatment paradigms are not as consistent as with other types of scoliosis
• Important, and different from other types of scoliosis, is the propensity for
neuromuscular scoliosis to progress after skeletal maturity.
• neuromuscular scoliosis often have a large pelvic obliquity in addition to the
thoracolumbar curve.
• Management of neuromuscular scoliosis is generally surgical,
• In addition, treating the common long C-shaped curves with significant
pelvic obliquity abnormalities often requires long segment fusion involving
pelvic fixation.
• Timing of surgery is a balance between performing surgery early enough so as not
to allow progression to a severe deformity and performing it late enough to reduce
the risk of restricting lung and thorax development.
31. CONGENITAL SCOLIOSIS
• Congenital scoliosis refers to spinal deformity caused by an error in the
embryologic formation of the vertebral column. The error usually occurs during
the first 6 weeks of embryonic formation and is estimated to appear in
approximately 1 in 1000 births.
• Possible neurological associations include
• Chiari malformation,
• hydrocephalus,
• split-cord malformation,
• tethered filum terminale or lipoma
• syringomyelia.
32. McMaster and Ohtsuka82 separated congenital scoliosis into
• failure of formation,
• failure of segmentation, and
• mixed deficits.
• Failure of formation deformities refer to the absence or maldevelopment of one or
multiple parts of the spine that results in deformity. Included in this subtype of
congenital scoliosis are hemivertebra, wedge vertebrae (partial hemivertebra), and
absence of or bifid posterior elements.
• Failure of segmentation describes partial or complete failure of separation of
adjacent vertebral elements and includes block vertebrae and unilateral bar
deformity
33.
34. • Therefore, surgery should be aimed at causing growth arrest on the convex side and
limiting curve progression with the understanding that these goals will provide a
better overall status for the patient than allowing growth to complete before
surgical management.
• The old dogma that “later surgery is better” should not be employed in patients
with congenital scoliosis.
35. SCHEUERMANN'S KYPHOSIS
• Scheuermann's kyphosis was initially described in 1921 as a rigid spinal kyphosis
that could not be corrected with postural movement.96 This disease was then
defined radiographically by Sorensen97 as greater than or equal to 5 degrees of
anterior wedging of at least three adjacent vertebrae, end plate abnormalities, and
Schmorl nodes .
• The apex is often between T7 and T9.
• Currently, the disease is reported to have a prevalence of 0.5% to 8.3%, with a
slightly higher incidence in males.
• increase in incidence in monozygotic twins
• the natural history of Scheuermann's kyphosis is poorly understood and not well
studied.
• Full-time bracing in the skeletally immature child for 1 to 1.5 years is the initial
modality of treatment.
36. • patients with at least 40% passive correction of curve magnitudes
between 50 and 75 degrees had a favorable outcome with
bracing, whereas patients with greater than 75 degrees of
curvature tended to require surgery for an optimal outcome
