Mohammed Attiah, MB,Ch.B. FRCSC
Assistant Professor,Orthopaedic Surgery UQU
Orthopaedic Specialty Hospital
Jeddah - Saudi Arabia
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Congenital Scoliosis: Treatment Options and Challenges
Review
• Definition
• Spine Growth Facts
• Associated Anomalies
• Natural History
• Challenges
• Decision making
• Treatment Options
• Controversies
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Spinal Deformity with the Presence of Vertebral-Anomalies
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Congenital Scoliosis
Big Deal, Why?
• Osseous Development
• Neural Element
• Sagittal Natural Curves
• Symmetric Growth
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Spine Growth
• Longitudinal GrowthChondro-epiphyseal portion of the end plateEndo-chondral Ossification
Circumferential growth
– Posterior growth ( 5-8 y)• Laminar growth• Pedicular growth
– Anterior Growth(Pre-Pub yrs)
• Latitudinal Growth
Perichondral & Periosteal apposition
Taylor,J. Anat. (1975), 120, 1, pp. 49-68QuickTime™ and a
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Spine Growth Facts ?
In utero = Extremely Accelerated
Birth -2y = Increased Rate
2y - 10 y = Steady Rate
Pre-pub = Increased Rate
Thoracic Vertebra = 0.8 mm /y
Lumbar Vertebra = 1.1 mm /y
Thoracic Disc = 0.2-0.6 mm /y
Lumbar Disc = 0.3-0.8 mm/y
Hefti, JBJS Br, 1983;65:247-54
Dimeglio, Acta Orthop Belg,1990Taylor, J Anat,1975
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Thoracic Spine = 1.2 cm/ Year Lumbar Spine = 0.7 Cm / year
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Spine Growth Facts ?
Dimeglio, Acta Orthop Belg,1990Taylor, J Anat,1975
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Campbell, JBJS AM, 2004
Wezeka, Spine, 2004
Meehan, J Ped Ortho,1985
Associated Anomalies
• Pulmonary Compromise– Rib Deformity
– Spinal Deformity
– Lung Congenital Abnormality
• Congenital Heart Disease 15 %
– Echo / Cardiology Consult
• CT chest measurement
• PFT volume depletionVC < 50% = Post Op Pulmonary complication
Ferguson, J Ped Orthop ,1996Reckles, JBJS, 1975
• Renal Anomalies 20-40 %
– Unilateral kidney
– Ureteric duplication
– Ureteric obstruction
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Associated Anomalies
Renal U/S
Hensinger, JBJS,1974MacEwen, JBJS, 1972
• Spinal Cord Anomalies 20%
– Tethered cord
– Diastemetomyelia
– Fibrous Dural Band
– Intradural Lipoma
– Syringomyelia
– ACM
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Associated Anomalies
Spine MRISpine CT scan
McMaster, Spine,1998
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Natural History
McMastar, Spine, 1998Winter, Ortho Clin North Am,1998
• Type of Vertebral Anomalies
• Growth Potential
• Site of Anomalies
Type of Vertebral Anomalies
– Uni Un-segmented Bar+
– Hemi-vertebra
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Natural History
McMastar, Spine, 1998Winter, Ortho Clin North Am,1998
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Natural History
Growth Potential
– First 2 years
– Adolescent Growth Spurt
McMastar, Spine, 1998Winter, Ortho Clin North Am,1998
Site of Anomalies
Thoracic > Lumbar
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Natural History
McMastar, Spine, 1998Winter, Ortho Clin North Am,1998
• Early stage of life
• Cause large deformity
• Rigid Curve
• Resistant to correction
• Progressive
• Associated anomalies
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Congenital Curves Challenges
McMaster, JBJS.1982Fernandes, JBJS,2007
Prediction about what will happen with growth is very difficult
Decision Making
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Early Treatment Decision
Magnitude of Curve
Age
Type / Location of Anomaly
Diagnosis
Full Work Up
Consultation
• Balanced Spine
• Stop progression
• Deformity Correction
• Growing Vertebra
• Growing Neural Element
• Torso - Leg ratio
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Treatment Goal
What do you want? : What does the spine do?:
• Low Risk for Progression– Cobb angle < 25˚
– RAVD < 20 ˚
• High Risk for Progression– Cobb Angle > 25˚
– RAVD > 21˚
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Initiation of Treatment
Mehta, JBJS,Br, 1972
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When to start Treatment
• Anticipated or actual curve progression
– Curve progression 10 ˚
– Initial curve > 30 ˚ at growth spurt stages
Mehta, JBJS,Br, 1972
Treatment Option
• Bracing– Mixed anomalies– Progressive secondary curve
Controlling long,flexible compensatory curve below congenital anomalies
Not Successful Treatment Option
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Prevent Future Deformity
In Situ Fusion
Correct Present Deformity
Gradual Correction• Hemiepiphysiodesis• Growing Nonfusion Rod
Acute Correction• Instrumentation & Fusion• Hemivertebra Excision• Osteotomy
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Surgical Treatment Options
In situ Fusion
• No hope to get congenitally
fused side growing again
• Simplest & Safest solution
• Stop growth on convex side
– Unilateral Unsegmented Bar
– Balanced
– Not large curve < 40 ˚
– Early stages of life
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McMaster, Spine, 1998Dubousset, J Pedi Orthop,1998Keller,lindesth, spine.1994
In situ Fusion Controversy
• Age– Very early years of life?
• Anterior & Posterior fusion– ? Combined
– Less potential anterior growth
– Abnormal anterior vessels
• Trans-pedicular approach
• Decorticating the spine
• Facet Fusion
– One level cephalad
– One level caudad
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McMaster, Spine, 1998Dubousset, J Pedi Orthop,1998Keller,lindesth, spine.1994
Prevent Future Deformity
In Situ Fusion
Correct Present Deformity
Gradual Correction• Hemiepiphysiodesis• Growing Nonfusion Rod
Acute Correction• Instrumentation & Fusion• Hemivertebra Excision• Osteotomy
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Surgical Treatment Options
FAILURE OF FORMATION
CONCAVE FUTURE GROWTH
I. Single Hemi-vertebra
II. Curve < 50 ˚
III. Age < 5 years
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Hemiepiphysiodesis & Hemiarthrodesis
Keller,J Ped Orthop B,1994Winter,J Ped Orthop,1981Andrew, JBJS Br,1985
• Failure to achieve correction
• High failure rate
– 30 % improvement ?
– 40 % no change
– 20 % progressed
Average correction 10˚
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Hemiepiphysiodesis Controversy
Roaf, JBJS Br,1963Keller, Spine,1994
Growing Non-Fusion Rod
• Very Young child
• Treat the extended Secondary Curve
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Akbarnia,McCarthy,SRS,1994
Growing Non-Fusion Rod
• Primary curve should be addressed:
– In Situ fusion
– Hemiepiphysiodesis
– Excision
– Osteotomy
• Not commonly used technique
• Lack of strong evidence & F/U
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Hemivertebra Excision
• Popular procedure
– Immediate
– Excellent – Bradford,JBJS Am,1990
• Remove the Etiology
• Prevent worsening
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Correction
Hemivertebra Excision
• Ideal indication
– Hemivertebra LS junction
– Ignored large curve
• Combined Vs Staged
– Anterior Approach
– Posterior Approach
Leatherman,JBJS
Am, 1996
• Single Approach
– Posterior Excision
– Eggshell procedure
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Hemivertebra Excision
Single Stage Posterior Approach
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Complication
• Blood loss
– Segmental
– Epidural
• Neurological injury
– Cord injury
– Root injury
Winter,spine, 1989
Wiles, JBJS Am,1951
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Instrumentation & Fusion
• Safe correction
• Balanced spine
• Spinal cord status
• Fusion level selection
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8 yrs 9 Yrs
40 80
Downside of Long Segment Fusion
• Crankshaft Phenomenon
– Less anterior growth rate
– No absolute grantee technique
Sanders, JBJS
Am,1995
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Spine Osteotomy
• Short Segment Osteotomy
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Spine Osteotomy
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4040
• Long Segment Osteotomy
– Unacceptable cosmetic
– Fixed deformity
– Unbalanced
• No other solution
• Salvage procedure
• Experienced spinal surgeon
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Principles of Spine Osteotomy
• Cutting through anterior fusion mass
• Resection of enough bone to allow correction
• Temporarily stabilize curve
• Compressive instrumentation allow closure of
osteotomy
• Bone graft ++++
Summary
• Challenges
• Decision making
• Treatment Options
• Safe
• Correct decision
• Long term Follow Up
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Orthopaedic Specialty Hospital - OSH
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Jeddah - Saudi Arabiawww.osh.med.sa