CONGENITAL SCOLIOSIS

Scoliosis– 3-dimensional deformity of the

spine affecting all the 3 planes.

– Can be difficult to visualize with 2-dimensional radiographs

– Scoliosis is a lateral deviation of the normal vertical line of the spine which, when measured by an X-ray, is greater than 10 degrees accompanied by vertebral rotation.

Scoliosis

“Normal” alignment• Spinous processes all line

up in a straight line over the sacrum

Scoliosis is a combination of• Angular displacement• Lateral displacement

Scoliosis

• Lateral displacement

Scoliosis

• Angular displacement

Scoliosis

Think in 3 dimensionsRotational displacementLateral displacementSagittal displacement

Genetics

• 11% incidence in first relatives of patients– Normal incidence < 3%

• Monozygote twins more common

• No gene identified to date

Degrees of Curvature

MILD MODERATE SEVERE

Types of Scoliosis• Congenital

• Idiopathic

• Neuromuscular

• Post Traumatic

• Infective

• Degenerative

• Inflammatory

• Tumor

CONGENITAL SCOLIOSIS

The critical time is the time of segmentation process (First Six weeks) and congenital anomalies develop during this period of time.

In the presence of vertebral anomalies, there is an imbalanced growth of spine resulting in congenital scoliosis.

Congenital Scoliosis

• Abnormal development of the spine resulting in:– A missing portion

– Partial formation

– Lack of separation of the vertebrae

ClassificationBy MacEwen et al. later modified by Winter, Moe, and Eilers

• FAILURE OF FORMATION

Partial failure of formation (wedge vertebra)Complete failure of formation (hemi vertebra)

• FAILURE OF SEGMENTATION

Unilateral failure of segmentation (unilateral un segmented bar)

Bilateral failure of segmentation (block vertebra)

Failure of Formation Failure of Segmentation

Congenital Scoliosis

Patient Evaluation Examine the skin of back for hair patches,

lipomata, dimples, and scars. Look for the evidence of neurological involvement, such as clubfoot, calf atrophy, absent reflexes and atrophy of one lower extremity compared with the other. Look for the other congenital anomalies.

Congenital Scoliosis Associations

• 60% OTHER ANOMALIES• 25% Cervical anomalies• 37% Genitourinary anomalies• 38% Intraspinal anomalies

Tethered Cord Diastamatomyelia Syringomyelia

• 7% Congenital Heart diseases

Scoliosis Screening Recommendations

• American Academy of Pediatrics

`- Screen at 10, 12, 14 and 16 years

• American Academy of Orthopedic Surgeons

- Screen girls at ages 11 and 13

- Screen boys once at age 13 or 14

Screening hints• Shoulders are different heights• Head is not centered directly

above the pelvis • Appearance of a raised,

prominent hip • Rib cages are at different

heights • Uneven waist • Changes in look or texture of

skin overlying the spine (dimples, hairy patches, color changes)

• Leaning of entire body to one side

Scoliometer

•The patient bends over, arms dangling and palms pressed together, until a curve can be observed in the upper back (thoracic area).

•The Scoliometer is placed on the back and measures the apex (the highest point) of the upper back curve.

•The patient continues bending until the curve can be seen in the lower back (lumbar area). The apex of this curve is also measured.

An inclinometer (Scoliometer) measures distortions of the torso.

Adam’s forward bend test

For this test, the patient is asked to lean forward with his or her feet together and bend 90 degrees at the waist. The examiner can then easily view from this angle any asymmetry of the trunk or any abnormal spinal curvatures.

Measure spinal curvature usingCobb method

- Choose the most tilted vertebrae above & below apex of the curve.

- Angle b/w intersecting lines drawn perpendicular to the top of the superior vertebrae and bottom of the inferior vertebrae is the Cobb angle.

Diagnosis

• Physician Physical Exam• Scoliometer measurements

• X Ray

• MRI

Scoliosis Treatment

• Observation Spinal curvature<25

• Brace Spinal curvature 25-40

• Surgery Spinal curvature >40

Observation

Non progressive curves and Minor curves (>20 degrees) and with other congenital anomalies.

Skeleton is close to maturity Exercises may help with surrounding muscular

strength. Limited value in patients with congenital

scoliosis.

Bracing

• Usually works on the vertebrae outside the actual congenital deformity.

• Compensatory curves also can be successfully managed for several years with orthotic treatment.

• Lumbar curves can be treated in a TLSO, but thoracic curves require a Milwaukee brace.

Bracing

• Duration and time in brace

– 23 hours per day

– Wear until skeletally mature

Bracing

Types– Milwaukee

– Thoraco-lumbar-sacral orthosis (TLSO or Boston brace)

– Charleston night time bending brace

Bracing

• TLSO Brace

Bracing

Milwaukee Brace

Bracing

Charleston night time bending brace

Bracing

Milwaukee brace

Three types of curves respond to brace management:

1- Long, flexible curves,

2- Curves that could be corrected either in traction or on side bending,

3- Curves with a mixture of anomalous and non-anomalous vertebrae.

Successful Bracing

• Prevent curve progression– Randomized study

• Braced 74% did not progress

• Not braced 34% did not progress

• Charleston brace still controversial

Problems with Braces

• Argued efficacy

• Narrow treatment window to initiate

• Poor compliance

• Must have good orthotist– Curves corrected by 20 degrees in brace do better

Surgery

- Surgery is the only truly effective way to CORRECT scoliosis as 75% of congenital curves are progressive.

- Only 5% to 10% can be treated with bracing,

Surgery

Indications:

1. Major curvatures (<45 degrees)

2. Rapid deterioration/progression

3. Generally spinal fusion

Goal of Surgery

• To produce safe maximal correction with anterior / posterior instrumentation / reconstruction

• To restore good frontal and sagittal balance

Surgery

Combined Team Approach involving

• Surgeons

• Anaesthetists

• Nurses

• Physiotherapists

• Orthotists

Surgical Treatment for Scoliosis

• Curves in growing children greater than 40 º require a spinal fusion

• Skeletally mature patients can be observed until their curves reach 50 º.

• Posterior spinal fusion is best choice for thoracic curves.

• Anterior spinal fusion is best treatment for thoracolumbar and lumbar curves.

Surgical Treatment for Scoliosis

• Spinal surgery with instrumentationsignificantly corrects deformity &usually stops curve progression

• Surgery is accompanied by spinalcord monitoring using somato-sensory & motor-evoked potentials (risk of neurologic injury is 1/7000)

Operative Treatment Options

• Posterior fusion without instrumentation

• Posterior fusion with instrumentation

• Combined anterior and posterior fusion

• Combined anterior and posterior convex hemiepiphysiodesis

• Hemivertebra excision

• Vertebrectomy

• Instrumentation without fusion

Post-Op Treatment & Long Term Consequences of Spinal Fusion

• If segmental instrumentation used, no post-op cast or brace required

• Post-fusion back pain does occur and is more common in distal spinal fusions

• Usually out of hospital in 4-5 days & back at school in 2 wks

• OK to participate in athletics after 9 – 12 months (should avoid contact sports)

Treatment Algorithm

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