DEFORMITIES OF SPINE

ANATOMY OF SPINE

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KYPHOSIS

A. DEFINITION

Kyphosis is the normal spine profile of the thoracic region of the

spinal column. It originates from the Greek word kyphos, meaning

“humpbacked,” and refers to a curve pointing backward (that is, the apex

of the curve is posterior). The normal range of thoracic kyphosis is 20° to

50° (Cobb angle from T3 to T12), and values greater than 50° are

considered hyperkyphotic. 1

Postural kyphosis and Scheuermann disease are the most common

causes of hyperkyphosis, and both present during adolescence. Postural

kyphosis is a flexible deformity that is more common in girls, and patients

are able to correct the kyphosis voluntarily. Scheuermann disease, in

contrast, is more common in boys and is not passively correctable.

Scheuermann disease is diagnosed radiographically as a hyperkyphosis

associated with anterior wedging of more than 5° in at least three

successive vertebrae, usually with disk space narrowing, irregularities of

the vertebral end-plates, and Schmorl nodes (herniations of disk material

through the vertebral end plates). The etiology is unclear, but a familial

incidence is noted in 30% to 48% of cases. 1,3

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Figure 1. Scheuermann thoracic kyphosis: clinical photograph.2

Congenital kyphosis implies a structural defect in vertebral

formation, and may become apparent in infants and toddlers; the other

forms of kyphosis may be diagnosed throughout childhood and

adolescence.1

B. CLASSIFICATION

1. Postural kyphosis

The most common type, normally attributed to slouching, can

occur in both the old4 and the young. In the young, it can be called

'slouching' and is reversible by correcting muscular imbalances. In the

old, it may be a case of hyperkyphosis and called 'dowager’s hump'.

About one third of the most severe hyperkyphosis cases in older

people have vertebral fractures.5 Otherwise, the aging body does tend

towards a loss of musculoskeletal integrity,6 and hyperkyphosis can

develop due to aging alone.

2. Scheuermann's kyphosis

Significantly worse cosmetically and can cause varying degrees of

pain, and can also affect different areas of the spine (the most common

being the midthoracic area). Scheuermann's kyphosis is considered a

form of juvenile osteochondrosis of the spine, and is more commonly

called Scheuermann's disease. It is found mostly in teenagers and

presents a significantly worse deformity than postural kyphosis. A

patient suffering from Scheuermann’s kyphosis cannot consciously

correct posture.8

Clinically, it is possible to distinguish two forms of juvenile

kyphosis. Thoracic Scheuermann’s disease has an apex of the curve at

T7–9, and thoracolumbar Scheuermann’s disease has an apex at T11-

12. Cosmetic deformity is often the chief complaint. Pain is usually

aching and occurs more commonly with the thoracolumbar form.3

3. Congenital kyphosis

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It can result in infants whose spinal column has not developed

correctly in the womb. Vertebrae may be malformed or fused together

and can cause further progressive kyphosis as the child develops.9

C. PATHOPHYSIOLOGY

The pathophysiology of kyphosis depends on the etiologic factor.

The exact cause of Scheuermann disease is still imprecisely defined.

Scheuermann postulated that the condition resulted from avascular

necrosis of the apophyseal ring. Other theories include histologic

abnormalities at the endplate, osteoporosis,10 and mechanical factors that

affect spinal growth.11 A Danish study demonstrated an important genetic

component to the entity.12

D. DIAGNOSIS

1. Radiographic findings

a. Excessive kyphosis

b. Three sequential vertebra with more than 5 degrees of wedge

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Anterior wedging of thoracic spine in a 15-year-old boy withScheuermann kyphosis

2. Clinical characteristics

a. More common in boys

b. Affected patients usually overweight

c. Kyphosis is not postural: does not complete correct with

hyperextension

d. Neurologic changes are rare: MRI indicated if they are

present13

E. Physical Examination

Hyperkyphosis is apparent when viewing the patient from the side,

and the deformity is increased on the Adam forward bend test (Figure 1).

Adolescents with Scheuermann disease and other pathologic causes of

kyphosis usually have a sharp angulation at the apex of the kyphosis,

whereas patients with postural kyphosis have a more gentle curvature.

Patients with postural kyphosis have normal flexibility (ie, the spine

flattens in the supine position). The neurologic examination is usually

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normal in postural hyperkyphosis and Scheuermann disease but may be

abnormal with other forms of kyphosis, especially when the deformity is

severe. Hamstring spasm and/or contracture are common with pathologic

forms of hyperkyphosis.

F. Diagnostic Tests

AP and lateral radiographs of the entire spine should be obtained

with the patient standing. The reviewer should look for congenital

abnormalities as well as irregularities of the vertebrae, disk spaces, and the

end plates. The curve magnitude is measured on the lateral radiograph

using the Cobb method. When the upper thoracic vertebrae cannot be well

visualized, kyphosis should be measured from T5 to T12. The angle is

formed by the intersection of a line drawn along the superior end plate of

T5 and a line drawn on the inferior end plate of T12. Any curvature

exceeding 50° is considered abnormal.

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G. Complication

The natural history of flexible or postural hyperkyphosis is benign,

and these curves are unlikely to progress, but other forms of kyphosis

present a significant risk for progression. The consequences of a

progressive kyphosis may include back pain; rarely, neurologic symptoms,

usually seen with congenital kyphosis; and a decrease in pulmonary

function, seen with curvatures greater than 90° to 100° (restrictive

pattern).

H. Treatment

Postural kyphosis can be observed or treated with an exercise

program. Progressive curvatures due to Scheuermann disease may be

treated by a bracing program in patients who are skeletally immature (full-

time wear, Milwaukee brace or equivalent), and a subset of patients with

progressive and symptomatic curvatures greater than 70° are candidates

for a posterior or an anterior/posterior spinal fusion with instrumentation.

Congenital kyphosis is much more likely to require surgical intervention,

sometimes in the first few years of life. Bracing is not effective in patients

with congenital deformities, and progressive deformities often require

surgical stabilization.

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SCOLIOSIS

A. Introduction

Spinal deformity may affect the sagittal, coronal, and axial planes. Sagittal

plane deformity or kyphotic deformities of the spine are best seen on physical

examination from the side with

the patient bending forward. Sagittal plane malalignment of the spine may

involve any region of the spine but is most common in the thoracic and

thoracolumbar spine and is common with aging. Scoliosis is defined by

coronal plane deformity, but there is a concordant rotational deformity that is

apparent on examination. Shoulder asymmetry, pelvic tilt, and asymmetric

abdominal or flank creases are signs of deformity in the coronal plane. Trunk

rotation or rib prominence on forward bending (Adam forward bend test) is a

critical measure of axial plane deformity and may be apparent earlier than

coronal deformity in patients with scoliosis. In younger patients, scoliosis is

usually idiopathic, but in older patients it also may occur as a result of

degenerative changes. Patients with a new onset of scoliosis or rapid

progression of deformity should be evaluated for syndromes or diseases that

may be a cause of spinal deformity such as neurofibromatosis, spinal cord

lesions, or a tethered spinal cord. Spondylolisthesis (isthmic) usually occurs at

the lumbosacral joint and is accompanied by tight hamstring muscles (inability

to toe-touch). Onset of a spinal deformity in adulthood, or de novo

degenerative scoliosis, is common and may be associated with aging,

segmental instability, and osteoporosis. A rapid progression of deformity in

the adult may be an indication of neoplasm or infection. These conditions may

be accompanied by compromise of the spinal nerve roots and/or the spinal

cord.

Scoliosis is primarily a lateral curvature of the spine, but there is often a

degree of associated kyphosis. Mathematical analysis of the curves has

revealed several distinct patterns, which are dependent on the relative

contributions of these two deformities. In the management of any case, the

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first and most important decision to make is whether there is any deformity of

the vertebrae (structural scoliosis). If the vertebrae are normal (non-structural

scoliosis) the deformity is usuall due to one of the following conditions: it

may be compensatory, resulting from tilting of the pelvis from real or apparent

shortening of one leg. It may be sciatic and due to unilateral protective muscle

spasm, especially that accompanying a prolapsed intervertebral disc. Postural

scoliosis occurs most commonly in adolescent girls and generally resolves

spontaneously.

B. Defenition

Scoliosis is a lateral curvature of the spine. In adults, the condition is

classified as either a deformity that developed during childhood or a deformity

that developed after skeletal maturity, usually secondary to degenerative

spondylosis and/or degenerative spondylolisthesis. Changes that occur with

aging, including osteoporosis, degenerative disk disease, spinal stenosis, and

degenerative spondylolisthesis, may contribute to and/or confound the

symptoms and progression of either condition.

C. Epidemiology

Scoliosis is present in 2 to 4 percent of children between 10 and 16 years

of age. The ratio of girls to boys with small curves of 10 degrees is equal but

increases to a ratio of 10 girls for every one boy with curves greater than 30

degrees. Scoliosis in girls tends to progress more often and, therefore, girls

more commonly need treatment than boys. The prevalence of curves greater

than 30 degrees is approximately 0.2 percent, and the prevalence for curves

greater than 40 degrees is approximately 0.1 percent. Improved understanding

of the natural history and prognosis of this disease can help the physician

predict the patients with scoliosis who need treatment.

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D. Pathogenesis

Many studies have attempted to uncover the pathophysiologic process

underlying idiopathic scoliosis. Multiple abnormalities have been found, yet

none has been conclusively linked to all cases. Studies of twins have given the

firmest indication that the most significant factor is genetic. Indeed, a recent

meta-analysis showed that not only is the risk for scoliosis greater in

monozygotic twins than in dizygotic twins, the rate of curve progression is

nearly identical among twins subjected to a variety of environmental

influences. Current theorists believe that scoliosis is a multigene dominant

condition with variable phenotypic expression. Therefore, even though

scoliosis is typically present in most members of the same family, its severity

can vary widely from parent to child and sibling to sibling. When both parents

have scoliosis, the risk that their children will require treatment is 50 times

that in the general population

E. Clinical Findings

The most common presenting symptom is pain localized to the region of

the deformity. The most common overlapping syndrome is degenerative

spondylosis, which may also cause lower lumbar pain. Because age-related

changes in the spine are present in nearly everyone, a thorough evaluation is

required to identify the most likely source of pain. Radicular pain is most

commonly associated with compression of the L4 or L5 nerve root due to

asymmetric hypertrophy of the facet joints, asymmetric disk degeneration, and

mild rotatory subluxation.

Neurologic changes are infrequent but most commonly involve the

extensor hallucis longus muscle. In some patients, the chief presenting

symptom is a progressive spinal deformity Some report that they are “getting

shorter.” These patients may also report that the “hump” on their back is

getting bigger, or that they are leaning to the side more. Cardiopulmonary

decompensation rarely is evident in adultonset scoliosis. Symptoms related to

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pulmonary compromise are associated with more severe thoracic curves,

including both idiopathic and neuromuscular curves.

F. Classification

Congenital Scoliosis

A. Defenition

Congenital scoliosis is a progressive three-dimensional deformity of the

spine caused by congenital anomalies of the vertebrae that result in an

imbalance of the longitudinal growth of the spine. To understand their natural

history and their treatment, it is important to understand the embryologic

development of vertebrae.

B. Classification

Two types of basic vertebral anomalies can occur: failures of formation

and failures of segmentation.

a) Failure of vertebral formation

Failure of vertebral formation (type I deformity) can be partial, causing

a wedged vertebra with intact pedicles (Fig. 22–2), or complete, causing a

hemivertebra with a unilateral pedicle (Fig. 22–3). Hemivertebrae are

classified according to their longitudinal growth potential, which in normal

vertebrae is provided by growth apophyses on both vertebral ends.

a. Segmented hemivertebra: Both the superior and the inferior ends of the

hemivertebra have growth potential. The shape of adjacent vertebrae is

normal.

b. Semisegmented hemivertebra: Either the superior or the inferior end of

the hemivertebra has growth potential. The other end is fused with the

adjacent vertebra.

c. Incarcerated hemivertebra: Both the superior and the inferior ends of

the hemivertebra have growth potential, but the adjacent vertebrae

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compensate for it. The hemivertebra is “carved into” the adjacent

levels.

d. Nonsegmented hemivertebra: There is no growth potential. The

hemivertebra is completely fused with vertebrae above and below.

b) Failures of Segmentation

Failure of segmentation (type II deformity) can be partial, causing

a bar (Fig. 22–4), or complete, causing a block vertebra. A congenital bar

can be anterior, posterior, lateral, or mixed. In many cases, vertebral

anomalies owing to failures of formation and failures of segmentation

coexist, occasionally on several levels, and form a mixed deformity (type

III deformity).

C. Etiology

Congenital scoliosis is uncommon in the general population. Its true

incidence is unknown, but the familial incidence in the congenital scoliosis

population is typically 1% to 5%. It is slightly more common in girls than in

boys, with a ratio of 3 : 2. The precise etiology of congenital scoliosis is

unclear. Although most cases seem to be sporadic, in contrast to idiopathic

scoliosis, the role of genetic and environmental factors is often reported. The

genetic role has been reported in cases of congenital scoliosis in twins, but,

more recently, several studies have isolated gene mutations. Environmental

factors have also been implicated in the genesis of congenital scoliosis.

Maternal acute carbon monoxide exposure during somite formation induces

vertebral anomalies in the offspring of mouse and rabbit models. The

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mechanism of carbon monoxide action remains vague, however. Carbon

monoxide could act directly on the cartilaginous spine via resulting hypoxia or

a gene mutation. The etiologic theories are clouded further by the finding of

an increased incidence of idiopathic scoliosis in families with congenital

scoliosis.

D. Diagnosis

Anamnesis

As with scoliosis of any etiology, congenital scoliosis progresses in 70%

of patients during growth. The potential for increase in curvature is related to

imbalances in the number of growth apophyses and the location of vertebral

anomalies. Without any treatment, about 85% of patients with congenital

scoliosis have a curve greater than 41 degrees by maturity. Curves with

segmented hemivertebrae are at risk for progression during growth because

segmented hemivertebrae act as enlarging wedges (Fig. 22–6). The most

progressive anomaly is a convex segmented hemivertebra associated with a

concave unilateral bar because there is absolutely no growth potential on the

side of the bar. Conversely, a wedge vertebra has only a slight risk of

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worsening, whereas a complete block or an incarcerated hemivertebra does

not cause any progressive scoliosis. The location of the anomaly also plays a

part in the evolution of scoliosis. The most severe anomalies are those located

at the thoracolumbar region, whereas the least severe are located at the upper

thoracic spine. The natural history of congenital scoliosis has to take several

factors into account:

Type of anomaly

Location

Number of anomalies

Initial severity of the scoliosis

Global growth potential balance between each side of the Spine

Physical Examination

The physical examination of a patient with congenital scoliosis is guided

by the knowledge of a heightened incidence of other structural and neural

anomalies. The examination should begin with an assessment of a patient’s

existing balance: sagittal plane balance and coronal balance, shoulder

malalignment, and any deviation of head and trunk from the center of the

pelvis. In addition, it is crucial to assess and document the neurologic status,

including strength, reflexes, and presence of any atrophy. Flexibility of the

deformity, gait, and limb-length inequality should be checked. Pain, if present,

should be localized and quantified. The presence of a dimple or any cutaneous

mark on the back should be noted. The examiner should search for other

anomalies of the extremities (particularly radial malformation) and range of

neck motion.

Radiology

CT Scan

MRI

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A and B, It is easier to analyze segmented hemivertebra (A) or unilateral bar (B) when films are taken before 4 years of age than after.

C, Lumbar segmented hemivertebra in a 9-year-old child

FIGURE 22–5 Congenital scoliosis with VACTERL association managed by observation since birth. A and B, Right thoracic curve and compensatory lumbar curve remain relatively unchanged from birth at

1 year of age (A) and at 8 years of age (B).

E. Treatment

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Non Operative

Congenital vertebral anomalies require close clinical monitoring at

periodic intervals during growth. Consistent observation allows for assessment

of the evolution of spinal curves. In complex malformations, early treatment is

often more straight forward and safer.

In contrast to idiopathic scoliosis, nonoperative treatment has little value

in congenital scoliosis. The only potentially useful treatment is bracing of the

noncongenital components of a flexible curve. For a few cases with long and

flexible curves, progression of scoliosis can be slowed by bracing. Spinal

curves in congenital scoliosis are often short and rigid, however Given the

significant time period remaining before skeletal maturity, bracing is rarely, if

ever, more than a temporizing solution. Treatment of congenital scoliosis

consists of two options: (1) clinical monitoring of static vertebral anomaly and

(2) operative treatment of worsening scoliosis.

Operative

Congenital scoliosis develops because one side of the spine is growing

faster than the other. The main principle of operative treatment is to balance

growth, with or without deformity reduction. Five major operations have been

described: posterior spine fusion, combined anterior and posterior spine

fusion, convex hemiepiphysiodesis, hemivertebra excision, and guided growth

by vertical expandable prosthetic titanium rib (VEPTR) or growing rods.

Idiopathic Scoliosis

A. Defenition

Idiopathic scoliosis is the most common cause of spinal deformity 80% of

all scoliosis cases are due to idiopathic scoliosis. Before arriving at the

diagnosis of idiopathic scoliosis in a patient, other causes, such as congenital,

neuromuscular (developmental or acquired), functional, inflammatory or

infectious, pathologic, and intraspinal, have to be discounted. Ponseti and

Friedman1 first described early-onset scoliosis in 1950. Dickson expounded

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further on that concept and proposed that idiopathic scoliosis be divided into

early (0 to 5 years old) and late onset (>5 years old), based on spinal growth

velocity noted in these two age groups. Presently, idiopathic scoliosis is

divided into three categories based on chronologic age: infantile (birth to 2

years + 11 months), juvenile (3 years to 9 years + 11 months), and adolescent

(10 years to 17 years + 11 months). The radiographic diagnosis necessitates

measuring the coronal plane angle, using the Cobb method, as equal to or

greater than 10 degrees. Patients with curves less than 10 degrees are

considered to have spinal asymmetry.

B. Epidemiology

Infantile and juvenile scoliosis are less prevalent than adolescent

idiopathic scoliosis. Infantile idiopathic scoliosis is more common in Europe,

constituting less than 1% of idiopathic scoliosis cases in the United States, and

tends to comprise leftsided thoracic curves, typically occurring in boys. More

recent reviews suggest that there might be a decline in its incidence. Juvenile

cases are typically diagnosed at age 7 years in girls and 5 years in boys and

account for about 10% to 20% of idiopathic scoliosis cases.3 In contrast to

infantile idiopathic scoliosis, juvenile cases tend to occur predominantly in

girls and tend to comprise right-sided curves. Between the ages of 3 and 6

years, there seems to be a similar distribution between boys and girls,

however, again becoming predominant in girls after age 6 years.

Adolescent idiopathic scoliosis is more prevalent than other types of

idiopathic scoliosis. Among adolescents, the prevalence of 10-degree curves is

less than 3%, with about 5% of curves showing progression of greater than 30

degrees.

This prevalence decreases as a function of curve magnitude, however, to

about 0.3% to 0.5% and 0.1% in curves measuring 20 degrees and 40 degrees.

The prevalence of curves greater than 10 degrees is higher among girls, with a

4 : 1 ratio of girls to boys.

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C. Etiology

Infantile scoliosis occurs roughly in 1 of 10,000 births. Possible causes are

thought to occur from intrauterine molding or postnatal pressure on the spinal

column from supine positioning

while sleeping. Other etiologic factors that have been considered in idiopathic

scoliosis include dysfunction in proprioception to maldevelopment in central

pattern generators in the spinal cord9-11 and connective tissue, hormonal, and

muscle structural changes.12 More recent reports in the literature strongly

suggest a genetic link. The growth spurt noted among adolescents seems to

play a role in progression, as a critical buckling load is reached on the existing

curve as the spine grows. In a review of the literature, Kouwenhoven and

Castelein13 concluded many factors may play a role in the initiation and

progression of adolescent idiopathic scoliosis at a certain age. The literature

suggests, however, that in the observed deformation of the spine, genetics and

the unique mechanics of the fully erect posture, which is exclusive to humans,

play an important role.

D. Diagnosis

Anamnesis and Physical Exam

A complete history and physical examination is completed, and any family

history of scoliosis is noted. With infantile and juvenile cases in particular, a

thorough prenatal and birth and developmental history is obtained. In

adolescent cases, growth spurt history, if any at the time of presentation, is

noted. This information is imperative in determining peak growth velocity and

its implications on curve progression. Symptoms of or weakness and how the

patient perceives his or her appearance relative to the deformity are especially

important with adolescent idiopathic scoliosis. Age at onset of menarche and

voice changes in boys are noted as well because they are likely predictors of

growth potential and possible curve progression.

During the examination, height, weight, and age (years plus months since

last birthday) are recorded. The head is examined with special attention to

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torticollis and plagiocephaly because the latter has been associated with higher

incidence in infantile scoliosis. Possible conditions and anomalies that might

be present are buccal and palatal anomalies; café au lait spots; and midline

dimples or hair patches or both over the lumbodorsal spine, which can be

important clinical clues that an intraspinal pathologic process might be

present. Limb laxity is also checked, and genetic counseling and testing is

requested when laxity is present. Trunk shift is evaluated with the patient

standing and the hips and knees fully extended. The relationship of the

patient’s head to the pelvis is also noted in evaluating the overall coronal and

sagittal balance. Any shoulder, breast, or pelvic asymmetry is noted. Curve

rotation is assessed by performing an Adams forward-bend test and is

quantified with a scoliometer. This assessment is modified in infants by laying

the patient on the examiner’s knee. This test also helps assess the rigidity of

the curve, which is an important factor in terms of prognostication. Leg-length

discrepancy and pelvic obliquity are evaluated. Alternatively, a sitting forward

test can be performed. The latter maneuver can also help rule out

plagiocephaly and developmental hip dysplasia, especially in infants. When

leglength discrepancy is the likely cause of the deformity, a shoe lift is used to

reevaluate the patient to determine if the curve corrects.

A thorough neurologic examination is performed. The neurologic

examination includes all cranial nerves; motor strength; reflexes (including

abdominal reflexes), often associated with Chiari malformations; sensory

modalities; and gait.27 Finally, other possible causes of scoliosis, such as

congenital, neuromuscular, and syndromic types, must be ruled out. Infection,

neoplasms, and spondylolisthesis also must be discounted.

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FIGURE 3. Adam’s forward bend test. (Left) As the patient bends over, the examiner looks from behind and from the side, horizontally along the contour of the back.

(Right) A rotational deformity known as a rib hump (arrow) can be easily identified.

Radiographic

Posteroanterior and lateral 36- × 14-inch long cassette views including

bending films with appropriately placed bolsters for further curve

classification and planning bracing or surgical intervention are obtained, and

the Cobb angles are measured. Curves greater than 20 degrees in infants and

children, neurologic symptoms in all patients with idiopathic scoliosis, and

left-sided, sharp angular or irregular curve patterns require further

investigation, including screening total spine magnetic resonance imaging

(MRI). When anomalies of the nervous system are present on MRI, a

neurosurgical consultation is indicated.

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FIGURE 23–3 A and G, A girl age 2 years + 9 months who presented with severe infantile-onset idiopathic scoliosis

FIGURE 23–4 A and B, A boy age 7 years + 9 months who presented with progressive juvenile-onset right thoracic idiopathic scoliosis

E. Treatment

Non operative

Observation

Most infantile curves are left-sided; these curves have been known to

resolve spontaneously up to 90% of the time, but they can progress.41

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Deciphering which curves will progress can be guided by the RVAD and the

relationship of the apical rib head to the vertebral body (i.e., phase I or II).22

Typically, curves less than 20 degrees are expectantly followed every 6 to 8

months. Infants with curves less than 25 degrees and RVAD less than 20

degrees and children with curves less than 25 degrees should be followed

clinically and radiographically every 6 months. Treatment is instituted for

curves greater than 25 degrees. Treatment is also started for a progression of 5

degrees or greater in two consecutive visits or 10 degrees or greater in one

follow-up visit. Juvenile curves more often require operative intervention,

however.

Bracing and Casting

Bracing is the nonoperative treatment of choice in small but progressive

scoliosis in growing children and teens. In about 75% of cases, bracing can

control the curve and avoid progression, rendering the curve small enough so

that the risk of progression after growth is unlikely. In a younger child, whose

growth potential remains a significant issue, bracing allows for continued

growth until the patient requires eventual operative treatment because of curve

progression. With infantile cases, molded casting followed by bracing used to

be the mainstay in nonoperative management. Bracing and casting of these

patients comes with potential consequences, however, that include pulmonary

restriction, which can have future ramifications. Sanders and colleagues found

serial casting to be beneficial in the treatment of infantile scoliosis. They

reported that curves less than 60 degrees often fully corrected in infants if

casting was started before age 20 months.

Adolescents with curves less than 20 degrees at presentation are observed

and followed at 4-month and 6-month intervals. For curves between 20

degrees and 30 degrees, bracing is started if a curve progresses 5 degrees or

more in two consecutive visits or 10 degrees or more in one visit.

Bracing is usually started the first office visit when the patient is skeletally

immature (Risser ≤2) and presents with a 25- to 40-degree curve. Several

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brace options exist, and deciding which brace to use depends on the apex of

the curve and physician preference. Curves with an apex above T6 would

likely require the use of a Milwaukee (cervicothoracolumbosacralorthosis)

brace. Conversely, curves with apices at T7 or below and above L2 do well in

a Boston underarm thoracolumbosacral orthosis brace, and these braces are

more socially acceptable because of the lack of a cervical extension. The

Charleston bending brace is an option if the child is noncompliant to brace-

wear. This brace is typically worn at night, and some studies have shown its

efficacy.49,50 The efficacy of a brace seems to depend on the length of time

the brace is worn. When bracing is initiated and pad placement is deemed

appropriate, patient follow-up occurs every 4 to 6 months, with in-brace

radiographic evaluation and appropriate fitting adjustments made when

necessary.

Operative

Operative intervention is usually recommended for patients whose curves

progress despite nonoperative management. In infants, operative intervention

is controversial; it is occasionally performed in infants with curves greater

than 45 degrees or thoracolumbar/lumbar curves greater than 40 degrees.

Children are typically more prone to curve progression and are more likely to

require operative intervention. Other patients who are likely to benefit from

operative intervention are skeletally immature patients with adolescent

idiopathic scoliosis with a greater than 40- to 45-degree curve and mature

patients with curves greater than 50 degrees.

Neuromuscular Scoliosis

A. Introduction

Neuromuscular disorders commonly lead to spinal deformities that are

some of the most challenging treatment dilemmas addressed by spine

surgeons. Despite the various conditions that fall in this category,

neuromuscular disorders involve neurologic or muscular deficiencies that

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produce progressive multiplanar skeletal deformities. Common features of

neuromuscular scoliosis include the following:

Large curves early in life: Early neuromuscular insult predisposes patients

to rapidly progressive scoliosis.

Stiff curves: These patients are more likely to develop stiff curves because

of the early onset of neuromuscular deficiency resulting in limited

mobility and secondary contractures.

Progressive curves: As in idiopathic scoliosis, the potential for curve

progression is greatest during rapid growth and with loss of ambulation.

Increasing weakness or persistent muscle imbalance around the spine in

patients with neuromuscular disorders can cause progression of scoliosis

independent of growth, however.

Long curves: Less severely affected individuals may have an S-shaped

curve with well-balanced double curves. Long C-shaped curves are more

likely in severely affected patients with resultant sitting imbalance.

Pelvic obliquity: Lower extremity contractures and imbalanced spinal

deformity cause pelvic obliquity, which may impair comfortable sitting for

these patients.

Sagittal plane deformity: Gravity and muscular deficiency can also lead to

sagittal plane deformity, including thoracic or lumbar hyperkyphosis or

lumbar hyperlordosis. Patients with neuromuscular disorders are

challenging because of the complexity of their deformity and fragility of

their overall health and are best treated by an experienced surgeon with

support from a multidisciplinary team.

B. Classification

The classification of neuromuscular scoliosis can be based on the

underlying disorder: neurologic (e.g., cerebral palsy) or muscular (e.g.,

muscular dystrophy). Neurologic deficiencies can be broken down further into

upper motor neuron dysfunction, as seen in myelomeningocele, or lower

motor neuron dysfunction, as seen in spinal muscular atrophy (SMA).

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C. Natural History

Neuromuscular scoliosis generally begins early in life, is rapidly

progressive, and causes significant morbidity. Some patients are capable of

ambulation, although many lose their ability to walk early in life or never

achieve ambulatory status at all. The use of a wheelchair affords these patients

educational and social opportunities that enrich their lives. Spinal deformity

can impair comfortable sitting and dramatically reduce the individual’s quality

of life. Unbalanced curves and significant pelvic obliquity make wheelchair

positioning difficult and may cause uneven distribution of weight that may

lead to pressure sores. Prominences created by the convexity of a curve may

result in skin breakdown; creases within the concavity of the trunk deformity

are susceptible to skin maceration and infection. Majd and colleagues showed

a correlation between deformity size, functional decline, and decubitus. Large

rigid curves restrict lung volume and impair respiration in patients who often

already have limited pulmonary capacity. Treatment of neuromuscular

scoliosis can also help the caretakers of these patients, improving the ease of

transfers, positioning, feeding, and hygiene. The ultimate goal of treatment of

patients with neuromuscular scoliosis is the maintenance of as much

independence and function as possible. When patients with neuromuscular

scoliosis lose the ability to sit comfortably, their quality of life is dramatically

decreased. The natural history for a given patient is largely determined by the

specific underlying neuromuscular condition and the degree of involvement.

D. Treatment

The basic principles of observing or bracing smaller, flexible curves and

surgically fusing larger, more rigid curves in adolescent idiopathic scoliosis

apply to the treatment of neuromuscular scoliosis, although with less

aggressive parameters.

Observation alone is employed until curves begin to cause functional

impairment. Bracing can be a temporizing measure, used primarily to provide

sitting support while the patient grows. Eventually, many of these patients

26

require surgical stabilization with a spinal instrumentation and fusion

procedure.

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LORDOSIS

A. Definition

Lordosis is defined as an excessive inward curve of the spine. Some might

also called lordosis as swayback. The spine naturally curves at the neck, upper

back, and lower back to help absorb shock and support the weight of the head,

as well as the body itself. Lordosis occurs when the natural arch in the lower

back, or lumbar region, curves more than normal. This can lead to excess

pressure on the spine, causing pain.17

B. Etiology

Lordosis is found in all age group. It primarily affects the lumbar spine,

but can occur in the neck (cervical). When found in the lumbar spine, the

patient may appear swayback, with the buttocks more prominent and the

stomach seems to be sticking out, and in general an exaggerated posture.18

C. Cause

Up till now, the exact cause of lordosis in childhood remains unknown.

However, lordosis can affect people of any age. Other potential causes of

lordosis include:17,18,19

1. Obesity. Most researchers agree that obesity, especially central

(abdominal) obesity, with Body Mass Index (BMI) more than 24 kg/m2

might increase the lumbar lordosis angle. It is because some overweight

people lean backward to improve balance, and this leads to the change in

lumbar angle over time.

2. Discitis. An inflammation of intervertebral disc space that leads to

changing of lumbar curvature.

3. Osteoporosis. A decrease in bone density may affects the strength of the

bone, compromising the spine’s structural integrity.

4. Spondylolisthesis. This condition occurs when one vertebra slips forward

in relation an adjacent one, usually in the lumbar spine.

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5. Poor posture. Some studies showed that a posture affects the angle or

vertebra over time. People who spend more time standing in excessive

straight position is said has more lordosis angle than people who are not.

It is hypothetically said that it is due to the prolong tilt of the pelvis by

abdominal muscles.

6. Kyphosis. Kyphosis is a condition where there is an excessive outward

curvature at the mid-back. Kyphosis may force the low back to

compensate for the imbalance created by a curve occurring at a higher

level of the soine.

7. Achondroplasia. A form of dwarfism that affects the alignment of the

vertebra.

D. Diagnosis

Apart from the complaints from patient, a thorough physical examination

needs to be performed in diagnosing a lordosis. The physician will want to

know when the curvature was first noticed, past progression, and other related

symptoms the patient experiences. A simplest way to check for lordosis is to

ask the patient to lie on his back on a hard surface. The physician should be

able to slide his hand under the patient’s back, with little space to spare. In

lordosis, there will be extra space between the hand and the back.17 Other than

that, the patient will be asked to bend forward and to the side to see whether

the curve is flexible or fixed, and to see for any asymmetrical appearance, also

the range of motion to measure the degree to which a patient can perform

movement of flexion, extension, lateral bending and spinal rotation.18 A

neurological evaluation for the symptoms of pain, tingling or numbness,

muscle spasm, weakness and bowel/bladder change will be evaluated to see

how much lordosis has affects the quality of life of the patient.18 Last but not

least, radiographic image will be taken to reveal the entire length of spine.

Radiographic examinations that were asked in lordosis are anterior/posterior

(AP) and lateral x-ray of lumbosacral. Side bending AP x-rays are sometimes

29

used to evaluate the flexibility of the spine, and MRI may be ordered if the

spinal cord has been compromised (or suspected).18

E. Treatment

Treatment for lordosis consists of non-surgical treatment and surgical

treatment. Non-surgical conservative treatment may include:17,18

Analgesics and anti-inflammatory medication.

Physical therapy to help build strength in core muscles, as well as to

enable the patient to build flexibility and increase range of motion.

Bracing maybe used to control curve progression in adolescents.

Reduction of body weight to ideal.

In severe cases, surgical intervention is needed. Surgical intervention is

considered if the lordotic curve is severe, when neurologic involvement exists,

or non-surgical conservative treatment has failed.18

DAFTAR PUSTAKA

1. Sarwark J, editor: Essential of Musculoskeletal care, ed 4, Rosemont, 2010,

AAOS;p.1118

2. Herring JA, editor: Tachdjian’s pediatric orthopaedics, ed 4, Philadelphia,

2008, WB Saunders

30

3. Taylor R, editor : Handbook of Musculoskeletal Problems and Injuries, New

York, 2006, Springer;p.175

4. Annals of Human Biology, Volume 1, Number 3 / July 1974.

5. Kado DM, Prenovost K, Crandall C (2007). "Narrative review: hyperkyphosis

in older persons". Ann. Intern. Med. 147 (5): 330–8. doi:10.7326/0003-4819-

147-5-200709040-00008. PMID 17785488.

6. Keller TS, Harrison DE, Colloca CJ, Harrison DD, Janik TJ (2003).

"Prediction of osteoporotic spinal deformity". Spine 28 (5): 455–62.

doi:10.1097/00007632-200303010-00009. PMID 12616157.

7. Osteopathy: A Complete Health Care System, by Leon Chaitow N.D., D.O.,

M.R.O.

8. http://www.back.com/back-pain/conditions/scoliosis/index.htm

9. Natural History of Congenital Kyphosis and Kyphoscoliosis. A Study of One

Hundred and Twelve Patients – MCMASTER and SINGH 81 (10): 1367 –

Journal of Bone no People and Joint Surgery

10. Bautmans I, Van Arken J, Van Mackelenberg M, Mets T. Rehabilitation using

manual mobilization for thoracic kyphosis in elderly postmenopausal patients

with osteoporosis. J Rehabil Med. Feb 2010;42(2):129-35. [Medline].

11. Tribus CB. Scheuermann's kyphosis in adolescents and adults: diagnosis and

management. J Am Acad Orthop Surg. Jan-Feb 1998;6(1):36-43. [Medline].

12. Damborg F, Engell V, Anderson M, et al. Prevalence, concordance, and

heritability of Scheuermann kyphosis based on a study of twins. J Bone Joint

Surg Am. 2006;88(10):2133-6. [Medline].

13. Miller MD, Thompson SR, Hart JA. Review of Orthopaedics, ed 6,

Philadelphia, 2012, Elsevier Saunders;p.253, miller's review 253

14. Sarwark, John F. Essentials of Muskuloskeletal Care. 4th Edition. P. 1118-

1121

15. Reamy Brian. Adolescent Idiopathic Scoliosis : Review and Current Concepts.

Volume 64. July 2001

16. Rothman-simeone The Spine. 6th edition. Volume 1. Chapter Scoliosis

31

17. Gabbey A.E., Krucik G. Lordosis. [online] Downloaded at: http://www.healthline.com/symptom/lordosis (13th January 2015)

18. Regan J.J., Herkowitz H.N. A Closer Look at Lordosis. Updated 9 th July 2012. [online] Downloaded at: http://www.spineuniverse.com/conditions/spinal-disorders/closer-look-lordosis (13th January 2015)

19. Been E., Kalichman L. Perspective: Lumbar Lordosis. The Spine Journal.

Elsevier Inc. 2013 [online] Downloaded at: https://lumbar%20lordosis1-s2.0-

S1529943013013855-main.pdf (13th January 2015)

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