Scoliosis

Aka: Idiopathic Scoliosis, Adolescent Scoliosis

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I. Epidemiology

A. Prevalence: 2% of adolescent population

B. Age

1. Girls: After 9-10 years old

2. Boys: After 11-12 years old

C. Gender

1. Boys and girls affected equally

2. Girls are much more likely to significantly progress

II. Causes

A. Idiopathic scoliosis (85%)

B. Congenital Causes

1. Failed vertebral development (e.g. Hemivertebra)

2. Developmental failure of vertebrae to segment

C. Neuromuscular disorders

1. Neurofibromatosis

2. Syringomyelia

3. Diastematomyelia (congenital spinal cord splitting)

4. Cerebral Palsy

5. Muscular Dystrophy

6. Myelomeningocele

7. Spinal muscular atrophy

8. Friedreich ataxia
 9. Tethered cord

10. Syrinx

D. Miscellaneous Causes

1. Asymmetric Pelvis

2. Spinal cord or vertebral tumor

3. Vertebral infection

E. Connective Tissue Disease

1. Marfan Syndrome

2. Ehlers-Danlos Syndrome

3. Homocystinuria

II. Pathophysiology

A. Lateral curvature of the spine

1. Rotation of vertebrae about vertical axis

B. Idiopathic scoliosis is inherited

1. Autosomal dominant inheritance (variable penetrance)

2. Concordance in monozygotic twins: 73%

3. Risk in first degree relatives: 11%

II. History

A. Age of onset, progression and prior management

B. Back pain or stiffness symptoms

II. Signs

A. Scoliosis screening should begin at age 6 years

B. Right thoracic and left lumbar curvature is the norm

C. Landmarks

1. Shoulder height
 2. Scapular prominence

3. Flank crease

4. Pelvic symmetry

5. Leg Length Discrepancy

D. See Scoliosis Examination

1. Forward Bending Test

2. Scoliometer (measures trunk rotation)

3. Adam's Test

E. Determine growth spurt

1. Assessment Tools

2. Measure Sitting Height (Truncal Height) q3 months

3. Obtain Risser Grading (Iliac XRay)

F. Functional exam

1. Neurologic Exam

2. Gait

G. Red Flags

1. Left thoracic curve (possible spinal cord lesion)

2. Neurofibromatosis stigmata

3. Marfan's Syndrome stigmata

II. Radiology

A. Thoracic Spine XRay (may require full spine)

1. See Scoliosis XRay (Cobb Angle)

2. Images
 a.

B. Spine CT or MRI for atypical scoliosis

. Left thoracic curve

. Onset of scoliosis before age 8 years

. Rapid curve progression >1 degree per month

. Neurologic deficit or pain

II. Differential Diagnosis

A. Nonstructural Scoliosis

. Leg Length Discrepancy

. Local inflammation

B. Structural Scoliosis

. See Causes above

II. Course: Curves at skeletal maturity

A. Curves <20 degrees: Resolve spontaneously 50% of cases

B. Curves <30 degrees: Progress minimally

C. Curve 40-50 degrees: 10-15 degree lifetime progression

D. Curve >50 degrees: Progresses 1-2 degrees per year

II. Course: Curves before skeletal maturity

A. Spinal Curvature 20-29 degrees

. Risser Grade 0 to 1: 68% probability of progression

. Risser Grade 2 to 4: 23% probability of progression

II. Progression risk factors

A. Females
 B. Higher apex vertebral level

C. Thoracic or thoracolumbar curve (70% progression)

D. Double major curves (70% progression)

E. Young children at beginning of growth curve

F. Larger curves progress more severely

II. Management

A. Treatment based on progression risk

. See Progression risk factors above

B. Orthopedic referral indications

. Cobb Angle

a. Angle exceeds 20 degrees

. Scoliometer

a. Angle of trunk rotation exceeds 7 degrees

B. Observation protocol (curves <10 to 15 degrees)

. Observe for progression until stable or maturity

. Examine every 3-4 months

. Indications to Repeat Thoracic XRay every 6 months

a. Curve increasing

b. Child has growth spurt

B. Management Strategies

. Cobb Angle greater than 20 degrees

a. Bracing is controversial and noncompliance is high

b. Bracing options

i. Thoracolumbar-Sacral Orthosis (TLSO)

ii. Cervicothoracolumbar-Sacral Orthosis (CTLSO)

 . Cobb Angle greater than 45 to 50 degrees

a. Surgery (rod placement, bone grafting)

Idiopathic Scoliosis:
This is the most common type of scoliosis, and constitutes 80% of the cases of scoliosis. There is no
known cause of idiopathic scoliosis.
It is subdivided into 3 categories:
1)Infantile Scoliosis: Birth to 3 years old
2)Juvenile Scoliosis: 3 - 10 years old
3)Adolescent Scoliosis: 10 years old to skeletal maturity

Congenital Scoliosis:
This is a rare type of scoliosis. It is often due to failures of formation or segmentation of the spine. People
with congenital scoliosis will develop lateral spinal curves in infancy.

Neuromuscular Scoliosis:
In this type of scoliosis, a lateral curvature of the spine occurs due to muscular weakness or neurological
disorders.

Degenerative Scoliosis:
Degenerative scoliosis occurs in adults and is due to degeneration of the spine that occurs with aging.

Description

 Scoliosis is a sideways curvature of the spine that makes the spine look more like an "S" or "C" than a
straight "I".
 Scoliosis can cause the bones of the spine to turn (rotate) so that one shoulder, scapula (shoulder blade), or
hip appears higher than the other.
 The term "idiopathic" means that the cause of this disorder is not known (in most cases).

Understanding the spine can help you better understand scoliosis. Learn more about spine anatomy at Spine Basics

Left, Clinical photograph of an adolescent girl with right thoracic idiopathic scoliosis. Middle, Her rib prominence is most
obvious when bending forward. Right, X-ray clearly demonstrates right thoracic scoliosis.

Courtesy of Texas Scottish Rite Hospital for Children

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Statistics

 Frequency of scoliosis:
o Scoliosis curves measuring at least 10° occur in 1.5% to 3.0% of the population
o Curves exceeding 20° occur in 0.3% to 0.5% of the population
o Curves exceeding 30° occcur in 0.2% to 0.3% of the population
 Small spinal curves occur with similar frequency in boys and girls, but girls are more likely to have a
progressively larger scoliotic curve that will require treatment.
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Cause

 In most cases of scoliosis, the exact cause is not known (idiopathic). However, scoliosis can occur in several
people within a family. When it does, there is probably a genetic component to its cause.

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Classification

 Scoliosis can occur at any age.

o Adolescent idiopathic scoliosis occurs after the age of 10 years. It is the most common type.
o Infantile scoliosis occurs in children less than 3 years old. It may result from abnormally shaped
vertebrae at birth (congenital), various syndromes, neurologic disorders, or unknown reasons
(idiopathic).
o Juvenile scoliosis occurs in children between the ages of 3 and 10 years. It is not common

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Symptoms

 Scoliosis does not usually cause any pain, neurological dysfunction, or respiratory problems. The concern
over the cosmetic appearance of the back often is the primary concern of the patient and parents.

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Examination, Signs

 The doctor will ask your child to bend forward, which will show any deformities (see the image above). This
is called the "Adam's forward bend test." He or she will also check for any limb-length discrepancies,
abnormal neurological findings, or other potential causes.

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Investigations, Tests

Many schools regularly conduct scoliosis screenings of students. These screenings usually occur during the middle
school years. Your child may receive a referral for scoliosis to a doctor based on the results of a school screening.

Scoliosis is confirmed with an x-ray of the spine. Your doctor will measure the degree of the curve, as shown in the
accompanying x-ray.

This x-ray of a patient's scoliosis measures 82° in the upper curve, and 75° in the lower curve.

Courtesy of Texas Scottish Rite Hospital for Children

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Natural History

If left untreated, curves exceeding 50° can lead to problems in the long term. Progressive deterioration of the scoliotic
curve can occur, which in some patients can lead to diminished lung capacity and the development of restrictive lung
disease. Cosmetic concerns are significant to many patients. The incidence of back pain among patients with
scoliosis is similar to that of the general population.

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Treatment - Indications

 The type of treatment required depends on the kind and degree of the curve, the child's age, and the
number of remaining growth years until the child reaches skeletal maturity.

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Nonsurgical Treatment

Observation
This option is appropriate when the curve is mild (less than 20°) or if the child is near skeletal maturity. However, the
doctor will want to recheck the curve on a regular basis to see that it is not progressively getting worse. You may be
asked to return every 3 to 6 months for re-examination. Most instances of scoliosis identified by school screening will
fall into this category.

Bracing
The goal of bracing is to prevent scoliotic curves from getting worse. Bracing can be effective if the child is still
growing and has a spinal curvature between 25° and 45°. There are several types of braces, most being the
underarm type.

This underarm brace is intended to prevent a scoliotic curve from worsening to the point of needing surgery.

Courtesy of Texas Scottish Rite Hospital for Children

Your orthopaedist will recommend a brace and tell you how long it should be worn each day. Wearing a brace does
not affect participation in sporting activities. Time out of the brace is allowed for these activities.

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Surgical Treatment

If the curve is more than 45° and the child is still growing, the doctor may recommend surgery. If the patient has
reached skeletal maturity, surgery may still be recommended for scoliotic curves that exceed 50° to 55°.

Procedure
Before surgery, your child may be asked to donate blood (which will be used during the surgery, if needed).

An implant made up of rods, hooks, screws, and/or wires is used to straighten the spine (Figure C). Bone graft from
the bone bank, or from the patient's hip region, is also used to help the operated portion of the spine heal solid.

This is an x-ray of the same patient shown in the x-ray above, but with the implant used to correct the scoliosis.

Courtesy of Texas Scottish Rite Hospital for Children

Following surgery, patients are usually walking by the second day without the need for a brace, are discharged from
the hospital within 1 week, and can rapidly resume their daily activities.

Long-Term Outcome Following Surgery

Patients usually don't experience much pain once they have recovered from surgery. A return to most sporting
activities is possible in 6 to 9 months after surgery. However, due to permanent limitation of some spine movement
following surgery, participation in contact sports, such as football or rugby, is discouraged.

The spine fusion should not interfere with girls' future pregnancies or deliveries.

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Research

At present, the main research focus in idiopathic scoliosis is investigation into genetic factors as a cause of scoliosis.

Scoliosis: Types list

The list of types of Scoliosis mentioned in various sources includes:

 Types of scoliosis based on age:

o Adolescent idiopathic scoliosis - most common type; over 10 years old.
o Infantile idiopathic scoliosis - when aged under 3.
o Juvenile idiopathic scoliosis - occurs ages 3-10
 Types of scoliosis based on the cause of scoliosis:
o Nonstructural (functional) scoliosis - a temporary condition caused by some underlying
condition.
o Structural scoliosis - a fixed curve not always caused by an underlying condition.
 Types of scoliosis based on the apex of the spinal curvature:
o Thoracic curve scoliosis
o Lumbar curve scoliosis
o Thoracolumbar curve scoliosis

Types discussion:
Questions and Answers about Scoliosis in Children and Adolescents: NIAMS (Excerpt)
Adolescent idiopathic scoliosis (scoliosis of unknown cause) is the most common type and occurs
after the age of 10. Girls are more likely than boys to have this type of scoliosis. Since scoliosis can run
in families, a child who has a parent, brother, or sister with idiopathic scoliosisshould be checked
regularly for scoliosis by the family physician.

Idiopathic scoliosis can also occur in children younger than 10 years of age, but is very rare. Early
onset or infantile idiopathic scoliosis occurs in children less than 3 years old. It is more common in
Europe than in the United States. Juvenile idiopathic scoliosis occurs in children between the ages of
3 and 10. (Source: excerpt from Questions and Answers about Scoliosis in Children and Adolescents:
NIAMS)

Questions and Answers about Scoliosis in Children and Adolescents: NIAMS (Excerpt)
Causes of curves are classified as either nonstructural or structural.

 Nonstructural (functional) scoliosis--A structurally normal spine that appears curved. This is a
temporary, changing curve. It is caused by an underlying condition such as a difference in leg
length, muscle spasms, or inflammatory conditions such as appendicitis. Doctors treat this type of
scoliosis by correcting the underlying problem.

 Structural scoliosis--A fixed curve that doctors treat case by case. Sometimes structural
scoliosis is one part of a syndrome or disease, such as Marfan's syndrome, an inherited
connective tissue disorder. In other cases, it occurs by itself. Structural scoliosis can be caused by
neuromuscular diseases (such as cerebral palsy, poliomyelitis, or muscular dystrophy), birth
defects (such as hemivertebra, in which one side of a vertebra fails to form normally before birth),
injury, certain infections, tumors (such as those caused by neurofibromatosis, a birth defect
sometimes associated with benign tumors on the spinal column), metabolic diseases, connective
tissue disorders, rheumatic diseases, or unknown factors (idiopathic scoliosis).
(Source: excerpt from Questions and Answers about Scoliosis in Children and Adolescents: NIAMS)
Questions and Answers about Scoliosis in Children and Adolescents: NIAMS (Excerpt)
Doctors group curves of the spine by their location, shape, pattern, and cause. They use this
information to decide how best to treat the scoliosis.

 Location--To identify a curve's location, doctors find the apex of the curve (the vertebra within the
curve that is the most off-center); the location of the apex is the "location" of the curve. A thoracic
curve has its apex in the thoracic area (the part of the spine to which the ribs attach). A lumbar
curve has its apex in the lower back. A thoracolumbar curve has its apex where the thoracic and
lumbar vertebrae join (see "Normal Spine" diagram ).

 Shape--The curve usually is S- or C-shaped.

 Pattern--Curves frequently follow patterns that have been studied in previous patients (see
"Curve Patterns" diagram). The larger the curve is, the more likely it will progress (depending on
the amount of growth remaining).
Symptoms of Scoliosis

The list of signs and symptoms mentioned in various sources for Scoliosis includes the 7 symptoms
listed below:

 Spinal curvature
 Sideways curvature of the spine
 Sideways body posture
 One shoulder raised higher than the other
 Clothes not hanging properly
 Local muscular aches
 Local ligament pain

Tests and diagnosis discussion for Scoliosis:

The doctor takes the following steps to evaluate a patient for scoliosis:

 Medical history--The doctor talks to the patient and the patient's parent or parents and reviews
the patient's records to look for medical problems that might be causing the spine to curve, for
example, birth defects, trauma, or other disorders that can be associated with scoliosis.

 Physical examination--The doctor looks at the patient's back, chest, pelvis, legs, feet, and skin.
The doctor checks if the patient's shoulders are level, whether the head is centered, and whether
opposite sides of the body look level. The doctor also examines the back muscles while the patient
is bending forward to see if one side of the rib cage is higher than the other. If there is a significant
asymmetry (difference between opposite sides of the body), the doctor will refer the patient to an
orthopaedic spine specialist (a doctor who has experience treating people with scoliosis). Certain
changes in the skin, such as so-called café au lait (coffee-with-milk-colored) spots, can suggest
that the scoliosis is caused by a birth defect.

 X-ray evaluation--Patients with significant spinal curves, unusual back pain, or signs of
involvement of the central nervous system (brain and spinal cord) such as bowel and bladder
control problems need to have an x ray. The x ray should be done with the patient standing with his
or her back to the x-ray machine. The view is of the entire spine on one long (36-inch) film.
Occasionally, doctors ask for more tests to see if there are other problems.

 Curve measurement--The doctor measures the curve on the x-ray image. He or she finds the
vertebrae at the beginning and end of the curve and measures the angle of the curve (see "Curve
Patterns" diagram ). Curves that are greater than 20 degrees require treatment.
Treatment List for Scoliosis

The list of treatments mentioned in various sources forScoliosis includes the following list. Always seek
professional medical advice about any treatment or change in treatment plans.

 Watchful waiting - mild conditions may require no treatment other than monitoring for worsening.
 Treatment of any underlying cause of scoliosis
 Bracing
o Spinal cast
o Spinal brace
o Milwaukee brace
o Thoracolumbosacral orthosis (TLSO)
 Surgery
 Other treatments that have not been successful
o Chiropractic manipulation
o Electrical stimulation
o Nutritional supplementation
o Exercise - not successful in correcting scoliosis but desirable to maintain mobility.
 Supportive treatments to cope with the spinal curvature:
o Built-up shoe - if one leg is longer than the other