mohammed attiah, mb,ch.b. frcsc assistant professor,orthopaedic surgery uqu

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