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 dimensions Rotational displacement Lateral displacement Sagittal 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

Classification
By 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)

Congenital Scoliosis
Failure of Formation Failure of Segmentation

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
An inclinometer (Scoliometer) measures distortions of the torso.
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.

Adams 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 using Cobb 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 Brace
Surgery

Spinal curvature<25 Spinal curvature 25-40

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 require a spinal fusion Skeletally mature patients can be observed until their curves reach 50 . 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 instrumentation significantly corrects deformity & usually stops curve progression Surgery is accompanied by spinal cord monitoring using somatosensory & 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

Thank You