CHAPTER I

INTRODUCTION

1.1 Background
The spine (spine) is a column of bone in the back of the body, which supports the entire
upper body, protects the spinal cord, and the place where the muscles attach. The vertebral
column is composed of 33 bones, consisting of 5 parts, there are: cervical, thoracic, lumbar,
sacral, and coccygeus. Each vertebra in the spine is separated from each other by a small pillow
of cartilage known as an intervertebral disc, which makes it movable. Because of many functions
of this spine, when there is an abnormality occurs it can affect the activity of the body. One of
the spinal disorders that mostly occur is scoliosis. (1) (2)
Scoliosis comes from a Greek word which means curved or crooked. This word describes
the most common type of curvature of the spine and is pathological. (3) Scoliosis is defined as
deviation from the normal vertical line of the spine, which consists of lateral curvature with
rotation of the spine in the curve. Usually, to be considered as scoliosis, there must be at least
10° angulation of the spine on the posterior-anterior radiograph associated with spinal rotation.
In addition, the spinal curve of scoliosis is often associated with torso and extremity asymmetry
(4) (5) Patients with scoliosis will sometimes experience back pain and, posterior chest wall pain
on the side of the rib. (5)
Scoliosis can be divided into two: structural and non-structural scoliosis. Structural
scoliosis is permanent, and may be caused by other conditions. Structural scoliosis deformity
cannot be corrected in the affected spinal segment. Non-structural scoliosis is temporary and
tends to disappear with time. Deformity is secondary or as compensation for some conditions
outside the spine, for example with short legs, or pelvic tilt due to hip contractures, if the patient
sits or is flexed the curve disappears. An important component of the deformity is rotation of the
vertebrae; the spinous process rotates towards curve concavity. (4) (6)
The most common type of scoliosis is Idiopathic scoliosis reaching 80%, that is caused by
unknown factors, but some cases are related to cerebral palsy, muscular dystrophy, spina bifida,
or birth defects. (5) Scoliosis can be suffered by people of all ages, but it usually occurs in
children aged 10 to 12 years old and in their early adolescents, and affects more women than
men in a ratio of 8-10: 1, which is commonly called idiopathic Adolescent (3) (6). Based on a
study, the prevalence of scoliosis is increasing, which is around 3% in the world and 4-5% in
Indonesia. (7).
The diagnosis of scoliosis will begin with a history of medical history, physical
examination, and imaging (X-rays, CT scans and MRI) can help assess the shape, direction,
location, and angle of the spinal curve. Scoliosis with a primary (non-idiopathic) diagnosis must
be recognized immediately to identify the cause, which may require intervention. People with
mild scoliosis only need to visit their doctor for routine examinations, as well as do some
non-operative procedures. Some people who suffer from serious scoliosis and interfere with
daily activities require treatment, such as braces or surgery (5) (6). Because of this backround,
we will discuss more about this disease so we can prevent it and can decrease the prevalence.

1.2 Problem Identifications

The problem identifications of this Student Project is as follows:
1.2.1 What is the definition of Scoliosis?
1.2.2 What is the epidemiology of Scoliosis?
1.2.3 What is the etiology of scoliosis?
1.2.4 What is the pathophysiology of Scoliosis?
1.2.5 How is the diagnosis of scoliosis enforced?
1.2.6 What is the differential diagnosis of Scoliosis?
1.2.7 What is the management of Scoliosis?
1.2.8 What is the prognosis of Scoliosis?
1.2.9 What are the complications of scoliosis?

1.3 Writing’s Purpose

The purpose of writing this Student Project is as follows:
1.3.1 To find out the definition of Scoliosis
1.3.2 To find out the epidemiology of scoliosis.
1.3.3 To find out the etiology of scoliosis.
1.3.4 To find out the pathophysiology of Scoliosis.
1.3.5 To find out the diagnosis of scoliosis.
1.3.6 To find out the differential diagnosis of scoliosis.
1.3.7 To find out the management of scoliosis.
1.3.8 To find out the prognosis of scoliosis.
1.3.9 To find out complications from scoliosis.

1.4 Benefits of Writing

1.4.1 General Benefits
This Student Project is expected to be able to provide additional insights regarding the
definition, etiology, epidemiology, pathogenesis, diagnosis, differential diagnosis, management,
to the prognosis of scoliosis for readers, so that in the future readers in particular and the public
in general can prevent this condition.
1.4.2 Special Benefits
The writing of this Student Project is the fulfillment of the fourth semester students'
assignments in the Bachelor of Medicine and Doctor of Professional Studies Program, Faculty of
Medicine, Udayana University in the block of the Musculoskeletal System & Connective Tissue
Disease.
CHAPTER II

2.1 Definition of Scoliosis

Scoliosis comes from a Greek word which means curved or crooked. This word describes
the most common type of curvature of the spine and is pathological. Scoliosis is defined as a
three-dimensional (3D) structural deformity of the spine and is diagnosed on the basis of a
measurement of the major curves comprising the deformity. ​Scoliosis is a sideways curvature of
the spine that occurs most often during the growth spurt just before puberty. While scoliosis can
be caused by conditions such as cerebral palsy and muscular dystrophy, the cause of most
scoliosis is unknown.
This measurement is traditionally done using the Cobb method and gives the Cobb angle The
measurement is carried out in the coronal plane using a standard posteroanterior radiograph, and
the Cobb angle is formed between a line drawn parallel to the superior endplate of the upper
vertebra included in the scoliotic curve and a line drawn parallel to the inferior endplate of the
lower vertebra of the same curve. Scoliosis is diagnosed if the Cobb angle is ≥10o. In addition to
spinal curves, scoliosis is frequently associated with asymmetries of the trunk and the
extremities.
Most cases of scoliosis are mild, but some children develop spine deformities that continue to get
more severe as they grow. Severe scoliosis can be disabling. An especially severe spinal curve
can reduce the amount of space within the chest, making it difficult for the lungs to function
properly.

Children who have mild scoliosis are monitored closely, usually with X-rays, to see if the curve
is getting worse. In many cases, no treatment is necessary. Some children will need to wear a
brace to stop the curve from worsening. Others may need surgery to keep the scoliosis from
worsening and to straighten severe cases of scoliosis.(8)

The Scoliosis Assocition of the United Kingdom ​describes five main types of
scoliosis:
 ● Congenital scoliosis, when the spine does not form correctly before
birth
● Early-onset scoliosis appears between birth and 10 years
● Adolescent idiopathic scoliosis, which occurs as the child grows,
leading to a curving and twisting of the spine
● Degenerative scoliosis can affect adults due to wear and tear of the
skeletal system, whether or not they already have scoliosis
● Neuromuscular scoliosis stems from a problem with the muscles or
nervous system
● Scheuermann's kyphosis, where the front sections of the vertebrae
grow more slowly than the back sections, making them smaller
● Syndromic scoliosis is linked to one of a range of syndromes, including
Marfan's syndrome and trisomy 21

2.2 Epidemiology

There are not many studies that provide data of high relevance regarding prevalence of
Scoliosis. Data collected regarding this disease were from children in Germany, Korea, Brazil,
Singapore, Turkey and Greece. The results show that 0.47 – 0.52 % of the children are affected
by scoliosis and 97% of patient with scoliosis have genetic factors influencing the progression of
this disease. Scoliosis has been suggested to frequently affect children born to mothers who are
aged 27 years or older. It is assumed that gene fragility might be involved, for example, a higher
rate of infants with Down syndrome born to older mothers. The precise explanation as to why
this might be the case has not been explained. In Prader–Willi syndrome patients, scoliosis is
found up to 40 % among these patients. In Germany shows a percentage of 6.5% in the 11-13
age group and a percentage of 11.1% in the 14-17 age group. Genetic factors do influence the
and progression of scoliosis. The influence of genetic factors in scoliosis is also apparent in
epidemiologic studies. Scoliosis is more frequently found in Europeans than in Polynesians.
Several study reports show higher Cobb angles and a higher grade in severity off scoliosis in
girls than in boys. For patients with a Cobb angle of more than 30° the prevalence ratio gets as
high as 10:1. Moreover, the female to male ratio increases with increasing age of the children.
(9)

2.3 Etiologi

Scoliosis is defined as lateral curvature of the spine which is also associated with a change in the
curves in sagittal plane and vertebral rotation. Scoliosis is diagnosed as idiopathic in 70 % of
structural deformities affecting the spine in children and adolescents. Many abnormalities
associated with this condition have been described, and the debate on whether IS is a primary or
secondary disorder is still open. These abnormalities include disorders of neurologic system,
tissues (such as elastic and collagen fibers found in ligaments), muscles, bones, biology
molecular (such as genetic, melatonin, calmodulin, platelet, retinoid acid) and also biomechanics
system. ​(10. Janicki AJ, Alman B. Scoliosis : Review of Diagnosis and Treatment. NCBI.
2007;12(9):771-776)
1. Neurologic System
A comparative MRI study of the craniocervical junction demonstrated that 42 % of AIS subjects
had cerebellar tonsillar tip positioning 1 mm below the basion-opsithion line and that the
patient’s cerebellar tonsillar level was significantly lower than in normal controls. Magnetic
resonance imaging (MRI) with multiplanar reconstruction also revealed a significantly reduced
spinal cord-to-vertebral column length ratios in patients with severe curves, suggesting a
disproportional growth between the skeletal and the neural systems ​(11. Dayer R, Haumont T, et
al. Idiopathic Scoliosis : Etiologycal Concepts and Hypotheses. NCBI. 2013;7(1):11-16)
2. Biology Molecular
a. Melatonin : Pinealectomy in chicken has been shown to be consistently associated with
secondary development of a scoliotic deformity when no substitutive treatment was given
postoperatively, thereby suggesting a possible role of melatonin deficiency in the etiology of
experimentally induced scoliosis.
b. Calmodulin : as a second messenger of melatonin and because of its effects on muscle
contractility, is considered as another potential key molecule in the etiology of scoliosis.
Increased calmodulin levels in platelets have been shown to be associated with the progression of
AIS.
c. Platelet and cytosolic acid : Structural and functional platelets anomalies, including
increased cytosolic calcium and phosphorus levels, were initially reported in patients with AIS
by many studies. However, this is debated and some authors report contradictory results with
unimpaired platelet function.
d. Retinoic acid is believed to be an important factor during somitogenesis and left-right
patterning (i.e., the control of the symmetrical arrangement of the left and right body sides during
development) in chicken and mice embryos. Blocking its production in chicken embryos has
been shown to induce a desynchronization of somite formation between the two embryonic sides,
leading to a shortened left segmented region.
3. Biomechanichs
atient-specific finite element models have been used to explore the effects of biomechanical
factors on curve progression in AIS. An anterior spinal overgrowth combined with gravity and a
pre-existent curve in the spine was identified as possible promoting factors for thoraco-lumbar
scoliosis progression. As opposed to a common belief, lumbar curves appeared to behave
differently from other curve types using this numerical model, with fewer and slower curve
progression

2.4 Patofisiologi Scoliosis

There are three types of scoliosis, there are idiopathic scoliosis, congenital scoliosis and
neuromuscular scoliosis. Mainly most of scoliosis cases causes are idiopathic. Idiopathic
scoliosis could affect both children and adolescent. Genetic factors and people lifestyle are
believed to take role in idiopathic scoliosis ​(11)
 Congenital scoliosis is caused by a defect that was present when the baby is born. In
congenital scoliosis, there are failure of formation, failure of segmentation or both failure of
formation and segmentation of the vertebrae that could lead to scoliosis. Failure of formation of
vertebrae also called as hemivertebrae that cause congenital scoliosis. Baby with congenital
scoliosis that treated well will have outstanding results and achieve normal function although
there is shortening the spine. if not treated, congenital scoliosis will develop into severe curve
because children are growing fast ​(12)

Neuromuscular scoliosis is caused by abnormal muscle forces acting on the spine. There are
disorders of the brain, spinal cord and muscular system which causes the spinal curvature.
Cerebral palsy, Rett Syndrome, Spinal muscular atrophy, muscular dystrophy, spinal bifida, and
polio are examples of diseases that could lead people to have neuromuscular scoliosis. Abnormal
muscle forces that act at spines will pull the spine to curvate. Neuromuscular scoliosis could lead
to pulmonary complication that result in death of the patient ​(13)

2.5 diagnosis

Scoliosis can be hard to diagnosed just throughout anamnesis, so to diagose scoliosis physical
examination and imaging tests is needed.

a. Patient History

Usually, patients present spinal deformity or, more likely, chest wall and back
asymmetry. Whether identified by the patient, their parents, or through school or
physician screening programs, posterior chest wall prominence is the most outward
manifestation of spinal curvature. With more significant scoliosis, adolescent girls
sometimes notice a difference in their breast sizes. Other body characteristics may
include shoulder asymmetry and overall posture imbalance in the coronal plane. While
not typically the presenting symptom, back pain is not unusual, and ealth care provider
must be on the watch for neurological problems also. A complete neurological history
should include inquiries of weakness, sensory changes, problems of balance, gait and
coordination, as well as bowel and bladder difficulties such as incontinence. There is a
genetic component to this condition with siblings (seven times more frequently) and
cousins.
 b. Physical examination

Physical examination for scoliosis mainly consists of height measurement, gait check,
foot shape, skin inspection, assessment of pubertal development, neurological
examination( including motor, sensory and reflex tests), symmetry of shoulders and iliac
crest, deformity evaluation,limb length inequality and a special test the Adam’s forward
bend test.
The Forward bending test assesed by the patient stands and bends forward at the waist,
with the examiner assessing for symmetry of the back from behind and beside the patient.
Patient with possible scoliosis will have a lateral bending of the spine, nut the curve will
cause spinal rotation and eventually arib hump [14] (Horne JP, 2014)

c. Imaging

Scoliosis usually confirmed through imaging tests such as X-Ray, spinal radiograph, CT
Scan or MRI [15] (AANS). Things that have to be assessed through imaging are end
vertebra, apical, curve pattern, curve magnitude, risser sign, structural curve, and
nonstructural curve.

2.6 Differential Diagnosis of Scoliosis

The most common cases of scoliosis are idiopathic. But the signs and symptoms may be the
same as other diseases. To determine the correct diagnosis, differential diagnosis and
distinguishing signs, symptoms, and tests must be known. Scoliosis has many differential
diagnoses related to neuromuscular disorders and spinal disorders. The following will be
explained about the differential diagnosis of scoliosis:

1. Syringomyelia
Syringomyelia can present similar sign and symptoms to idiooathic scoliosis but the earlier onset
may present subtle neurologic abnormalities of assymetic or hyperflex deep tendon reflexes ,
clonus, positive Babinski, and abnormal or asymmetric abdomen reflex. The test that can be
performed to differentiate is MRI to evaluate the entire spinal cord to evaluate the cause of
abnormalities that may cause deformity. (16)

2. Spina bifida

Severe cases of spina bifida can cause spinal deformity. Sensory and motoric deficit
corresponding to the level of deformity usually present together. Plain film and MRI can be the
differentiating test to reveal incomplete union of the posterior vertebral elements that
demonstrate various degrees of failure of neural tube closure (17).

3. Leg-length discrepancy

This deformitiy may present as a result of development of compensatory spinal curvature to

balance the body and lower limb. Physical eamination on sitting positions can show the balance
position of spine and pelvis without the influence of the leg-length disparency and can correct
the observed spine deformity. This condition is not present in scoliosis case (16)

2.7 Management of scoliosis

Treatment of scoliosis depends on the severity of the curve and the odds of the curve
getting worse. Particular types of scoliosis have a greater possibility of getting worse, so the type
of scoliosis also helps to determine the proper treatment (18). There are two main categories of
treatment: bracing and surgery(19).
Casting

Casting instead of bracing is sometimes used for infantile scoliosis to help the infant's spine to go
back to its normal position as it grows. This can be done with a cast made of plaster of Paris.

The cast is attached to the outside of the patient's body and will be worn at all times. Because the
infant is growing rapidly, the cast is changed regularly.
Braces

If the patient has moderate scoliosis and the bones are still growing, the doctor may recommend
a brace. This will prevent further curvature, but will not cure or reverse it. Braces are usually
worn all the time, even at night. The more hours per day the patient wears the brace, the more
effective it tends to be.

The brace does not normally restrict what the child can do. If the child wishes to take part in
physical activity, the braces can be taken off.

When the bones stop growing, braces are no longer used. There are two types of braces:

● Thoracolumbosacral orthosis (TLSO) - the TLSO is made of plastic and designed to

fit neatly around the body's curves. It is not usually visible under clothing.
● Milwaukee brace - this is a full-torso brace and has a neck ring with rests for the chin
and the back of the head. This type of brace is only used when the TLSO is not possible
or not effective.

One study found that when bracing is used on 10-15 year olds with idiopathic scoliosis, it
reduces the risk of the condition getting worse or needing surgery.

Surgery involves correcting the curve back to as close to normal as possible and
performing a ​spinal fusion to hold it in place. This is done with a combination of screws, hooks,
and rods that are attached to the bones of the spine to hold them in place (20).
Scoliosis surgery involves the following:
● Bone grafts - two or more vertebrae (spine bones) are connected with new bone grafts.
Sometimes, metal rods, hooks, screws, or wires are used to hold a part of the spine
straight while the bone heals.
 ● Intensive care - the operation lasts 4-8 hours. After surgery, the child is transferred to
an ICU (intensive care unit) where they will be given intravenous fluid and pain relief.
In most cases, the child will leave the ICU within 24 hours, but may have to remain in
hospital for a week to 10 days.
● Recovery - children can usually go back to school after 4-6 weeks, and can take part in
sports roughly 1 year after surgery. In some cases, a back brace is needed to support the
spine for about 6 months.

The patient will need to return to the hospital every 6 months to have the rods lengthened - this is
usually an outpatient procedure, so the patient does not spend the night. The rods will be
surgically removed when the spine has grown.

People with degenerative scoliosis will often have more complaints of back pain and leg
pain. This is related to the arthritis in the back and possible compression of the nerve roots that
lead to the legs. Non operative treatment including physical therapy, exercises, and gentle
chiropractic can help relieve these symptoms in some cases (20). People who fail to improve
with these treatments may benefit from surgery. X-rays and possible ​MRIs ​will be obtained to
plan for surgery. The surgery could include only a decompression or removal of bone spurs that
are compressing the nerves (21).

2.8 Prognosis
The prognosis of scoliosis can vary immensely, according to the degree of the spinal deformity.
Upon initial diagnosis in childhood, it is difficult to predict the future growth of the spine as the
child grows and the expected deformity cannot be known with certainty.
However, there are some indicative factors that can help to speculate the prognosis of the
condition for the individual. Thoracic spine anomalies and multiple fully segmented
hemivertebrae have a tendency to progress more.

The majority of the spinal growth occurs in the first five years of life and in the adolescent
growth spurt just before puberty. These two time periods, therefore, are the most critical times
and the spinal growth of children with scoliosis in these age groups should be monitored closely.
If interventions are made to promote healthy spinal growth and reduce curvature, the prognosis is
usually improved. (22)

● Mild Scoliosis

A curve of fewer than 20 degrees can be described as mild scoliosis. The prognosis for this
condition is positive and most individuals with the form of the condition do not require and
specific treatment.

Instead, they should be monitored for signs of worsening of the condition, particularly as
children grow to prevent the degree from increasing. (22)

● Moderate Scoliosis

A curve between 25 and 70 degrees can be described as moderate scoliosis. Current research has
not drawn strong conclusions about the outcomes or resulting health complications of this group
of individuals with the condition.

For this reason, the utility of surgical intervention is not clear and recommendations are currently
made on a case-by-case basis. (22)

● Severe Scoliosis

A curve greater than 70 degrees can be described as severe scoliosis. This often causes the ribs to
press against the lungs, leading to a restriction of breathing and overtime hypoxia. The reduce
levels of oxygen in the blood can have implications on the function of other organs in the body.
Additionally, the spinal curvature may cause structural changes in the heart with dangerous
outcomes.

Intervention to promote healthy spinal growth is routinely recommended for individuals with
severe scoliosis. (22)

● Very Severe Scoliosis

A curve greater than 100 degrees can be described as very severe scoliosis. This commonly leads
to repercussions for both the heart and the lungs, due to the changes in structural space in the
chest. Individuals with severe scoliosis are more susceptible to lung infections and pneumonia.

This level of deformity is rare among developed countries but is associated with significantly
increased mortality rates in respect to other severities.(22)

● Lifelong Health

Medical studies have shown that for individuals with scoliosis, the overall physical and mental
health over their lifetime is comparable to the general population.

Even without treatment, most patients with scoliosis are able to lead functional and productive
lives, with nine out of ten patients displaying normal cardio and pulmonary function.

It is worth noting that some experts have voiced concern over using the measurement of the
spinal curvature as the sole point of reference to identify patients at risk of cardiopulmonary
complications. They argue that other factors should also be considered, including spinal
flexibility and the asymmetry of the ribs and vertebrae.(22)

2.9 Complications of Scoliosis

This scoliosis complication occurs when people suffering from scoliosis are not treated properly
and correctly. Scoliosis complications that are often found include:
· Heart and lung problems. ​Scoliosis sufferers experience the curvature of their spine, this
curve indicates abnormalities of the spine shape which can lead to disruption of heart and lung
function, as a result scoliosis sufferers can experience things like shortness of breath and chest
pain due to circulatory processes to the heart and lung area - the lungs are experiencing
inhibition. This is what causes patients with scoliosis to be easily attacked by heart disease and
lung disease.

· Back problems. ​Scoliosis sufferers will experience back pain that is continuous or we can say
in chronic conditions, all of this is due to disruption of the nervous system on the back and back
holding only one side.

· Nerve problems. ​People with scoliosis will experience nervous system disorders that are in the
spine because of the uneven position of the backbone of the patient. The severity of this problem
is seen from the position of the spine that has been bent, and the nerves affected. One of the
complications of the disease is back pain, incontinence of urine and feces, weakness of the legs
can hardly / difficult to walk, so that impotence can occur in men.

CHAPTER III

3.1 Conclusion
Scoliosis is a sideways curvature of the spine that occurs most often during the growth spurt just
before puberty. ​There are three types of scoliosis, there are idiopathic scoliosis, congenital
scoliosis and neuromuscular scoliosis. The most common type of scoliosis is Idiopathic scoliosis
reaching 80%, that is caused by unknown factors, but some cases are related to cerebral palsy,
muscular dystrophy, spina bifida, or birth defects.

Many abnormalities associated with scoliosis include disorders of neurologic system, tissues ,
muscles, bones, biology molecular and also biomechanics system.

Treatment of scoliosis depends on the severity of the curve and the odds of the curve getting
worse. ​There are two main categories of treatment: bracing and surgery. The prognosis of
scoliosis can vary immensely, according to the degree of the spinal deformity.
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