Approach to a case of Scoliosis

Scoliosis Abnormal lateral curvature of spine in which there is deformity in the coronal plane. May alter sagittal plane as well Thoracic kyphosis normally = 30-35 degrees Range 10-50 degrees Lumbar lordosis normally = 50-60 degrees Range 35-80 degrees Spinal rotation causes posterior prominence

Upto 10 degrees is normal. Can be seen as C- curve or S-curve. S- curve is usually compensatory.

Demographics : Occurs in 2-3% of population below the age of 16 years. 0.1% have a curve greater than 40 degrees. Girls are more affected than boys. Those with a curve of more than 30 degrees are generally girls, outnumbering boys by 10:1. Generally progresses during the period of ‘growth spurts’. Adolescents are more routinely tested for this.

Anatomy All bony elements are altered Discs are wedged as well Vertebra are wedge shaped Rib vertebral angle altered Pedicles rotated Discs are wedged as well

Types of Scoliosis Congenital Neuromuscular Syndrome related Cerebral palsy Syndrome related Marfan’s syndrome Idiopathic 80% are this

Etiological Theories Genetic Tissue deficiencies Growth abnormalities Central nervous system alteration

Genetic 11% incidence in first relatives of patients Normal incidence < 3% Monozygote twins more common No gene identified to date

Tissue Deficiencies Marfan’s syndrome deficient fibrillin Osteopenia noted in girls Elevated calmodulin Involved in contractile properties thru actin & myosin Elevated in platelets No consistent findings to date

Growth Abnormality Asymmetrical vertebral growth Hueter-Volkman effect is suppression of growth on concave side Hypokyphosis during growth spurt No increased incidence with growth hormone No initiating factor identified

Central Nervous System Different size cerebral cortices Altered equilibrium Primary or secondary Deficient melatonin Chicken model Inconclusive in humans

Terminology Named by apex Primary vs secondary Cervical if between C2-C6 Cervicothoracic if between C7-T1 Thoracic if between T2-T11 Thoracolumbar if between T12-L1 Lumbar if between L2 and below Primary vs secondary Structural vs non-structural

Classification Infantile: 0-3 years old (.5%) Juvenile: 4-11 years old (10.5%) Adolescent: 10-17 years old (89%) Adult: >18 years old

History Family history Recent growth history Sexual maturity Pain Affected sibling 7 times more frequent Affected parent 3 times more frequent Recent growth history Sexual maturity Pain ‘Fatigue pain’ Post diagnostic pain ‘Severe pain’

Physical Exam Iliac crest height Shoulder height Arm trunk space Leg length discrepancy Shoulder height Arm trunk space Scapular position Trunk shift Inspection of skin Café au lait spots

Physical Examination: Features suggestive of polio, neurofibromatosis, Von Reclinghausen syndrome, Down’s, Marfan’s, Hurler’s syndrome, neural tube defects and osteogenesis imperfecta. Forward protrusion of chest wall on affected side. Increased flank creases on opposite side. Higher ASIS and PSIS on concave side. Spinous process turned into concave side.

Tests of flexibility of spine: Adam’s forward bending test. Pushing the curve from convex side and noting the correction. Lifting the patient up from head. Lateral bending.

Forward Bend Test Adam’s sign

Neurologic Exam Observe gait Hop test Heel and toe walk Reflexes

Early Detection: Visual examination of gait, posture, limb length and lateral curvature of spine. A posterior view taken, bent at 90 degrees at hips. Can also be detected accidently when radiographs are taken to rule out other pathologies.

Once scoliosis is suspected: A scoliosis series is ordered. AP cervical, thoracic and lumbar spine Xrays collimated to soft tissues needed. Sometimes lateral views may also be necessary.

Imaging Plain x-rays Note rotation Measure deformity by Cobb method Need standing 36 inch cassette Posterior to anterior Decrease thyroid and breast exposure 3-7 fold Note rotation Measure deformity by Cobb method Skeletal maturity

Cobb Method Choose the most tilted vertebrae above and below the apex of the curve. Draw a line perpendicular to that vertebrae. The angle created between these intersecting lines is the Cobb angle.

Rotation Spinous process rotates into concavity Pedicle position

Skeletal Maturity Triradiate cartilage fusion Risser sign

MRI Neurologic deficit Infantile and juvenile curves Spinal cord abnormality in younger children Infantile idiopathic scoliosis 50% Juvenile 20%

Who needs an MRI: A thoracic curve to the left. Painful scoliosis. Abnormal neurological findings. Untoward stiffness. Deviation to one side during the bend test. Sudden rapid progression of a previously stable curve.

Will the curve progress? Three factors involved in progression patient’s gender future growth potential curve magnitude at time of diagnosis Females are 10 times more likely to have progression than males. The greater the growth potential and larger the curve = more likely to progress

Curve Progression Curves 30 to 50 degrees progress an average of 10 to 15 degrees over a lifetime. Curves > 50 at maturity progress steadily at a rate of 1 degree per year. Curves less than 30 at bone maturity are unlikely to progress.

Medical complications: At 100 degrees or greater: increased potential for life threatening effects on pulmonary function. Psychologic illness: seen in up to 19% of females with curves great than 40 degrees as adults.

Treatment principles: Orthotic braces - 74% success rate at halting progression Must be worn 20 hours a day, but most pts are not compliant. Braces do not correct scoliosis. Surgical therapy is definitive, but indicated only for those at 40 degrees or above

Infantile Treatment Must prove idiopathic 90% are left thoracic 3 female : 2 male 90% resolve spontaneously Predict progression by RVAD < 20 degrees 83% resolve >20 degrees 84% progress

Juvenile Treatment Younger onset likely to progress >30 degree curve almost always progress Some adolescent curves are missed juvenile

Adolescent Treatment Most curves <10 degrees Boys = girls for these curves Usually don’t progress More sever curves (>30 degrees) 8 girls : 1 boy Predicting who will progress

Risk for Progression Younger onset Female more likely than male Skeletal age Risser 0-1 at presentation 60-70% progress Risser 3 only 10% risk Menses starts after growth spurt Female more likely than male Curve pattern Apex above T12 Degree at presentation 20-29 degrees 68% risk for progression 30-59 degrees 90% risk for progression

Natural History If curve <30 degrees at maturity No adult consequences Unlikely to ever progress Curves >45 degrees may progress a degree/year Mortality not increased unless curve >90 degree Right heart failure Decreased pulmonary function

Treatment : 10 degrees curve or less This curve is considered normal. No action is taken. Follow up appointments are prescribed to monitor the patient. Usually done every 3-6 months, but at the physician discretion.

Treatment:10 to 25 degree curve Sometimes no treatment needed, if no progression. Begins with simple orthotics(very effective) daytime/nighttime braces. Shoe lifts for leg length discrepancies. Stretches, exercises.

Shoe Lifts: Used for leg length discrepancies. Worn in regular shoes. Places opposing pressure on scoliosis curvatures. Must be worn during every scoliosis radiograph.

Treatment: 25 to 35 degree curve Day and night brace worn 20+ hours/day. Shoe lifts may also be needed. Stretches and exercises to loosen muscles and to relieve pain if present.

Treatment: 45 degree + curve Almost always treated with surgery. Vertebrae are fused using- Bone grafts. Hardware(metal splints) Still require braces to be worn in post op period. Causes growth to stop. Can cause nerve damage, infection and other problems.

Left untreated: If progressing, can worsen upto 70 degrees + curve. Places pressure on vital organs. Can cause cardio-respiratory problems. Can eventually become untreatable.

Non-Operative Treatment <25 degrees monitor every 4-12 months Depends on skeletal maturity >25 degrees monitor every 3-6 months >30 degrees in skeletally immature brace Curve change by 10 degrees brace Curve >40-45 degrees surgery

Braces : Made of polypropylene. Contoured to size and shape of body. Curved to oppose specific points of scoliosis curvature. Flexible and comfortable. Worn under clothing. Nighttime/daytime use. Must be worn faithfully.

Bracing Duration and time in brace Types Electrical stimulation 23 hours per day Wear until skeletally mature Types Milwaukee Underarm orthosis Electrical stimulation

Braces

Successful Bracing Prevent curve progression Electrical stimulation Randomized study Braced 74% did not progress Not braced 34% did not progress Electrical stimulation 33% 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

Treatment Algorithm

Surgery Failed bracing Curves >45 degrees Unbalanced curves >40 degrees Surgery is fusion with instrumentation

Surgical Options: Infantile and juvenile scoliosis: <8 yrs- instrumentation without fusion. After 8 years- anterior and posterior spinal fusion. After 11 years- posterior spinal fusion.

Surgical Options: Adolescent scoliosis: Posterior spinal fusion with instrumentation. Anterior spinal fusion if younger than 11 years and with open triradiate cartilage.

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