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Adolescent Idiopathic Scoliosis

January 21, 2011 Meghan N Imrie, MD Pediatric Orthopaedic Surgery Lucile Packard Childrens Hospital

Disclosures

none

Introduction

Definition Etiology Prevalence/Natural History Screening/Evaluation Treatment


Non-operative Operative

On the Horizon

Definition

Scoliosis
Derived from Greek for crooked Coronal plane deformity > 10o
< 10o = spinal asymmetry Measured by the Cobb method

3-dimensional deformity Clinical sign, not an outright diagnosis


Four main categories
Congenital Degenerative Neuromuscular/syndromic Idiopathic

Definition - continued

idiopathic
What must be ruled out?
Neurofibromatosis Marfan syndrome Ehlers Danlos syndrome Intraspinal abnormalities
Tumors Tethered cord Syrinx

Varies by age:
Infantile - birth to age 2 years Juvenile - from about 2 to 8 or 9 years Adolescent - greater than 9 or 10 years, but not an adult

Overview

Definition Etiology Prevalence/Natural History Screening/Evaluation Treatment


Non-operative Operative

On the Horizon

Etiology

Numerous theories, none proven Answer is there


Male: female ratio1
1:1 for minor curves 1:8 for treatable curves

Family history
27% prevalence of scoliotic curves >15 degrees in daughters of scoliotic mothers2

Curve type
Majority right thoracic (about 98%)
Left thoracic is red flag for possible intraspinal abnormality
1. 2. Bunnell WP Spine 1986;11:773-6. Harrington PR Clin Orthop 1977;126:17-25.

Etiology

Overview

Definition Etiology Prevalence/Natural History Screening/Evaluation Treatment


Non-operative Operative

On the Horizon

Prevalence

Varies significantly based on degree of curvature:


>10o = 0.5-3% >30o = 1.5-3/1000

Male to female ratio:


1:1 for minor curves 1:8 for those requiring treatment1
1. Bunnell WP Spine 1986;11:773-6.

Natural History

Progression
Before skeletal maturity After skeletal maturity

Natural History

Progression before skeletal maturity


Many unknowns Some knowns
Growth remaining Size of curve female

From Lonstein JE and Carlson JM JBJS Am 1984;66:1061-71.

Natural History

Risser sign
Radiographic measurement based on ossification of iliac apophysis

Natural History

Clues to skeletal maturity and peak height velocity (PHV)


Radiographic measures
Risser sign Triradiate cartilage (TRC) (Elbow ossification)

Menarche in girls Tanner staging

Natural History

From Lonstein JE and Carlson JM JBJS Am 1984;66:1061-71.

Natural History

Progression after skeletal maturity


Some variability Weinstein and Ponseti data most frequently used1
Curves <30o do not progress Thoracic curves >500 progress average 10 per year
50o at 18 -> 90o curve at 58

Lumbar curves >30o progress about 0.5o per year


1. Weinstein SL and Ponseti IV JBJS Am 1983;65:447-55.

Natural History

Adults with untreated AIS:


No increased rate of mortality (all comers with AIS)1 Respiratory failure if curve >110 degrees2
Increased risk of shortness of breath, especially if thoracic curve >80 degrees3

Chronic back pain4


Common Not related to size or location of curve Usually does not interfere with ability to work
1. Pehrsson K et al Spine 1992;17:1091-6. 2. Pehrsson K et al Thorax 1991;46:474. 3. Weinstein SL et al JAMA 2003;289:559-567.

4. From Tachdjians Pediatric Orthopaedics 269.

Natural History

Back pain in adults with untreated AIS:

Weinstein SL et al JAMA 2003;289:559-567.

Natural History

Adults with untreated AIS:


Demographics similar to control group
Education level Marriage Children
Lower C section rate

Rate of depression similar Body satisfaction


AIS slightly dissatisfied to slightly satisfied Control slightly satisfied to satisfied

Perception of limitation due to scoliosis


32% reported such issues as difficulty in purchasing clothes, decreased physical capacity, and self-consciousness
Weinstein SL et al JAMA 2003;289:559-567.

Natural History

In summary:
Trying to prevent curves from reaching at skeletal maturity:
>50 degree thoracic >40 or 45 degree lumbar

Because these curves continue to progress in adulthood Adults with untreated, more mild scoliosis do well in adulthood (at least in Iowa)

Overview

Definition Etiology Prevalence/Natural History Screening/Evaluation Treatment


Non-operative Operative

On the Horizon

Screening

Somewhat controversial
AAOS, SRS, POSNA and AAP currently recommend1:
Females screened twice at ages 10 and 12 Males screened once, at age 13 or 14

British Orthopaedic Association and British Scoliosis Society advise against screening United States Preventive Services Task Force (USPSTF) in 2004 recommended against screening
AAOS, SRS, POSNA, AAP responded with 2008 information statement1
1. Richards BS and Vitale MG JBJS Am 2008;90:195-8.

Screening - controversy

In summary:
Screening is fairly reliable to detect curves (though not terribly accurate) Early detection could result in improved health outcomes (by potentially avoiding surgery) Brace therapy is likely effective in altering natural history for many patients (but not all)

Most organizations continue to recommend screening

Evaluation

History
Reason for presentation (in patient or parents own words Pain
Red flag warnings: positive finger test, night pain, nonactivity related pain

Age Family history Pubertal status Rate of progression Any neurologic complaints
Radicular symptoms Bowl/bladder incontinence

Evaluation

Back pain and AIS


23% have pain at presentation1
Only 9% of these had underlying pathological condition

9% have pain during course of observation1 Significant association if1:


> 15 years Risser 2 or more Post-menarchal status History of injury

Painful left thoracic curve or abnormal neuro exam more likely to have neuro-axis problem

1. Ramirez et al JBJS Am 1997;79:364-8.

Evaluation - radiographs

Cobb Angle inter/intra observer error 5o

Evaluation

Indications for MRI


Atypical, specific pain Neuro abnormality
Abnormal reflexes Ataxia weakness Progressive foot deformity (cavus feet)

Left thoracic curve Rapidly progressive curve ?males


Routinely recommended, but minimal data to support1
1. Nakahara D et al Spine 2010 (epub ahead of print)

Evaluation

Classification systems
King-Moe Lenke

Evaluation

Classification systems
King-Moe Lenke

Overview

Definition Etiology Prevalence/Natural History Screening/Evaluation Treatment


Non-operative Operative

On the Horizon

Treatment

A lot of information on the internet Three main treatments:


Observation Bracing
Daytime Nighttime Spine-Cor

Surgery

Treatment
11-25o 25-45o >40-50o

Skeletal maturity?*

Skeletal maturity?

yes

no

yes

no Consider surgical intervention


*skeletal maturity = Risser 3 or greater

F/u as needed

F/u every 46 mos until skeletal maturity

F/u q5 yrs to assess progression

Consider bracing, f/u q 4-6 mos

Natural History

From Lonstein JE and Carlson JM JBJS Am 1984;66:1061-71.

Treatment - bracing

Types
Full-time bracing
Boston Milwaukee (if apex higher than T7)

Night-time bending brace


Charleston Providence

Others
Spine-Cor

Treatment - bracing

TLSO (Boston-type)
Worn as much as possible
Can take off for sports, sleepovers etc

Want at least 50% correction in brace

Treatment - bracing

Milwaukee brace
For refractory curves Apex >T7 Not really tolerated in our patient population

Treatment - bracing

Night-time bending braces:


Overcorrects the curve Only worn at night Probably not as efficacious
Price et al1 - 66% success, only 17% requiring surgery Randomized study Charleston vs Boston brace2
41% vs 61% success (<5 degrees progression) 31% vs 19% required surgery

1. 2.

Price CT et al JPO 1997;17:703-707 Katz DE Spine 1997;22:1302-12.

Treatment - bracing

SpineCor
Well advertised Soft straps, so more easily worn under clothers/during physical activity Originators report only 40% progressed, only 23% needed surgery1
Other authors have found no better results with SpineCor2

1. 2.

Christine C et al. Stud Health Technol Inform 2008;135:341-55. Wong MS et al Spine 2008;33:1360-5.

Treatment - bracing

Brace efficacy Data all over the place, both for and against Meta-analysis by Rowe et al1
1910 patients in 22 studies on non-operative treatments
Weighted mean proportion of success 0.93 for bracing 0.49 for observation 0.39 for electrical stimulation

Prospective international study2


Boston brace in girls with 25-35 degree curves
74% success vs 34% with observation alone

Prospective study of Boston brace with heat sensor (compliance)


>12 hours/day: 82% success <7 hours/day: 31% success Patients who went on to surgery: 24% compliance Patients who did not progress to surgery: 42% compliance
1. 2. Rowe DE et al JBJS Am 1997;79:664-74. Nachemson AL et al JBJS Am 1995;77:815-22.

3.

Katz DE et al JBJS Am 2010;92:1343-52.

Treatment - bracing

Best for:
Girls1
Boys only 38% compliant with brace wear 30 degree curve 50% chance of surgery BMI >85th %ile --> 2.5x risk of failure, double surgical rate

Lower BMI2

More flexible curves Younger patients


Many studies Higher rate of progression by natural history
1. Karol LA Spine 2001;26:2001-5.

2.

ONeill PJ et al. JBJS Am 2005;87:1069-74.

Treatment - bracing

Summary:
Probably alters natural history in some but not all
Especially if patient young, thin, and compliant Curve can definitely still progress

Only tool we have to prevent progression Success = prevent progression


Never corrects the curve!

Prospective, blinded, randomized controlled study needed


BRAiST underway Enrollment has been difficult

Treatment - surgery

Indications for surgery:


Thoracic curve >40-45 degrees in skeletally immature patient Thoracic curve >50 degrees in skeletally mature patient Lumbar numbers: usually around >40 degrees Double curves more well tolerated

Goals of surgery:
Achieve solid fusion SAFELY! (improve cosmesis, body image)

Treatment - surgery

Treatment - surgery

No long-term, prospective controlled studies to support hypothesis that surgery for AIS is superior to natural history
Reliably prevents progression Achieves permanent correction Improves appearance

Not a small undertaking


4-7 day hospital stay 6 months out of contact sports Complications:1
Infection 0-6% Pseudarthrosis 2-7% Reoperation rate 5-7% Possibility of permanent neurologic injury
1. Westrick ER and Ward T JPO 2011;31:S61-8.

Expensive to health care system

Treatment - surgery

A brief history of correction:


All methods at core the same:
Expose the spine (facet joints) Get correction
Coronal plane (Cobb angle) Axial plane (rotation) Sagittal plane (maintain normal kyphosis/lordosis relationship)

Wait for the fusion

Treatment - history of surgery

(Brief) history of correction


1958, Moe
Risser cast, bed rest, no instrumentation Ave correction 43%

Treatment - history of surgery

(Brief) history of correction


1958, Moe
Risser cast, bed rest, no instrumentation Ave correction 43%

1964, Moe
Harrington rods, Risser cast Ave correction 55% --> 41%

Treatment - history of surgery

(Brief) history of correction


1958, Moe
Risser cast, bed rest, no instrumentation Ave correction 43%

1964, Moe
Harrington rods, Risser cast Ave correction 55% --> 41%

1992, Lenke
CDI - Cotrel Dubousset instrumentation Ave correction 48%

Treatment - history of surgery

(Brief) history of correction


2004, Luk et al
Comparative studies of 4 different systems (CD Horizon, Moss-Miami, TSRH, Isola) Ave correction: 63% for CD Horizon, Moss-Miami vs 58% for TSRH, Isola Equal when matched against bending films

Treatment - history of surgery

(Brief) history of correction


2004, Luk et al
Comparative studies of 4 different systems (CD Horizon, Moss-Miami, TSRH, Isola) Ave correction: 63% for CD Horizon, Moss-Miami vs 58% for TSRH, Isola Equal when matched against bending films

2005, Suk
Introduction of pedicle screw Ave correction 62%

Other pedicle screw correction rate: 6276%

Treatment - current techniques

Approach:
Posterior vs Anterior
Open vs Thoracoscopic

Combined approach
For very big, stiff curves Younger patients
To prevent crankshaft

Treatment - current techniques

Standard posterior approach


Positioning
Prone neuromonitoring

Approach
Posterior exposure Careful at most cephalad and caudal ends to avoid unnecessary exposure +/- Use of C-arm

Treatment - current techniques

Preparing the spine for fusion


Facetectomies

loosening up the spine


Ponte osteotomies Allow for better Cobb correction and kyphosis creation

Treatment - current techniques

Screw placement
Free-hand With C-arm With O-arm Definitely dealers choice

Correction
In 3 planes
Coronal (straight rods) Axial (derotation) Sagital (rod contour)

Treatment - current technique

Screw types
Monoaxial Uniplanar Polyaxial

Ways to reduce the screws to the rods


Reduction screws Reduction tools
Each company has their specific types

Treatment - current techniques

Preparation for fusion


Facetectomies Decortication
Various techniques

Addition of bone graft


Autograft (iliac crest) Allograft
Many options!

Closure!
+/- drain +/- brace

Treatment - current techniques

MIS scoliosis surgery


Applying some adult techniques to pediatric scoli At cephalad levels mostly ? Fusion rates?

Overview

Definition Etiology Prevalence/Natural History Screening/Evaluation Treatment


Non-operative Operative

On the Horizon

On the Horizon

Scoli score = developed by Axial Biotech/Jim Ogilvie and crew


Saliva test Predictive of progression For Caucasian girls, age 9-13, with curves between 10 and 25 degrees
Likelihood will progress to surgical curve

Based on 53 genetic markers Log scale from 0 to 200 Very high and very low scores helpful, middle score unclear Not widely used or accepted Expensive May be more helpful in research
Ie are curves with high scores those that progress despite a brace?

On the Horizon

Fusionless techniques:
Compressing anterior overgrowth
Vertebral stapling Spinal tethering
Newton et al Encouraging results in animal model Human trials just starting

Summary

AIS
3-dimensional deformity Defined as 10 degrees of curve on PA xray Exact cause unknown; watch for red flags Screening controversial but still recommended by most
Girls at 10 and 12 years (younger better) Boys at 13 or 14 years

> 7 degrees on Adams forward bend, consider xray vs referral to orthopaedist Treatment
Based on age and size of curve Includes observation, bracing, and surgery

Thank you