Management of Scoliosis in Cerebral Palsy

Is it worth it?

Prof. Dr. Frank S Plasschaert, MD PhD

Dr. Sophie Lauwagie, MD

Children’s Paedicatric Orthopaedic Unit

Objectives of this lecture

Epidemiology of scoliosis in cerebral palsy

Natural history

Principles of management for scoliosis in CP

Positioning, seating and bracing

The interrelationship with tone reduction

Growing rod

Spinal fusion

PROM patient related outcome measurement for CP scoliosis surgery

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Epidemiology

Progressive scoliosis has been reported to occur in 64% to 74% of severely impaired, nonambulatory patients with cerebralpalsy (CP) who are classified as functioning at level IV or V according to the Gross Motor Function Classification System (GMFCS)

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Balmer GA, MacEwen GD. The incidence and treatment of scoliosis in cerebral palsy. J Bone

Joint Surg Br. 1970 Feb;52(1):134-7.

Madigan RR, Wallace SL. Scoliosis in the institutionalized cerebral palsy population. Spine (Phila

Pa 1976). 1981 Nov-Dec;6(6):583-90.

Persson-Bunke M, Ha ̈gglund G, Lauge-Pedersen H, Wagner P, Westbom L. Scoliosis in a total

population of children with cerebral palsy. Spine (Phila Pa 1976). 2012 May 20;37(12):E708-13.

Natural History

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Natural History

Curve progression in 37 ‘untreated’ severe spastic CP patients

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Natural History

Curve progression as a function of spasticity

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Total Body Involved Non-Total Body Involved

Natural History

Curve progression as a function of initial physical capability

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Walkers Sitters Bed-Ridden

Natural History

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Natural History

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Windswept deformity of the hips develops in about 30% of CP childrenGMFCS III-V.

In most children, WS develops before 10 years of age, but the risk continues up to 20 years of age.

With early inclusion in a hip surveillance program, and early treatment of contractures, the frequency of WS starting in the lower extremities can bereduced.

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Early treatment of scoliosis might reduce the development of pelvic obliquity and WS.

With improved knowledge of the risk factors for progressionand new surgical techniques that allow for further growth, thismight be one way to reduce WS in the future.

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The Problem of the Growing Spine.

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Lung Function and Spinal Growth

Intrinsic alveolar hypoplasia

In the normal lung

• Alveolar hyperplasia (multiplication of alveoli) continues till the age of 8

• Hypertrophy of the existing alveoli till the end of growth (of the thorax)

lung “growth” (hyperplasia and airway expansion) is essentially complete by age 8 years, with a “golden period” of maximum growth occurring before age 5 years

Lung Function and Spinal Growth

Intrinsic alveolar hypoplasia

The “golden period” of rapid growth of the thoracic spine and rib cage thuscoincides with lung development.

• The circumference of the thorax, which is only 7% of adult size at birth, increases to 30% by age 5 years and 50% by 10 years.

• The length of the thoracic spine increases by 50% (from 12 to 18 cm) from birthto age 5 years, achieving some 60% of the adult length by that age

Fusion of the Spine before the Age of 8

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Karol LA, Johnston CE, Mladenov K, et al: The effect of early thoracic fusion on pulmonary function in

non-neuromuscular scoliosis. 40th Annual Meeting of the SRS, 2005, Miami, Fla.

Early fusion of the Spine

The principle that a short, straight spine produced by early fusionis better than a long, curved spine is no longer generallyaccepted!

The goal of management must be to control spinal deformitywithout impeding thoracic growth!

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Non Fusion Techniques

Seating

Bracing

Casting

Growing Rods

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Bracing

bracing remains ineffectivein preventing progression in case of scoliosis in Cerebral Palsy GMFCS III-V

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Miller A, Temple T, Miller F. Impact of

orthoses on the rate of scoliosis

progression in children with cerebral palsy.

J Pediatr Orthop. 1996 May-Jun;16(3):332-

5.

Terjesen T, Lange JE, Steen H. Treatment

of scoliosis with spinal bracing in

quadriplegic cerebral palsy. Dev Med Child

Neurol. 2000 Jul;42(7):448-54.

Growing Rods

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Growing Rods

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Growing Rods

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Growing Rods in CP

GRs are an effective treatment for scoliosis and pelvic obliquity in childrenwith CP and offer the benefit of delayingfusion until skeletal maturity.

Dual GR constructs that extend to the pelvis exhibit better pelvic obliquitycontrol but similar curve control compared with a single-rod or dual-rods ending in the lumbar region.

Deep wound infections are the most common surgical complications and maylead to instrumentation removal in thiscomplex patient population.

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(Posterior) Spinal Fusion in the

Treatment of Scoliosis in Cerebral

Palsy

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Objectives of Scoliosis Surgery in CP

To obtain a balanced spine

In order to facilitate equilibrium / sitting balance

To improve on pulmonary function

Lessen nursing demands

With the use of a safe technique!

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What makes people consider surgery?

Seating difficulties

Pain because of rib-pelvis impingement

Back Pain

Gastro-intestinal problems

Progression of deformity – sagittal plane problems with diffultsitting

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What makes surgeons consider surgery?

Aims

Stable hips

Level pelvis

Compensated/ balancedspine

Rewards

Comfortably seatedchild/patient

No pressure sores

Minimal/improved nursing care

Child can concentrate on his potential

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The Orthopaedic Solutions

Lucque technique

Segmental Instrumentation with the inclusion of pedicle screws

Pelvic fixation (Galvestone – Pedicle Screws)

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The problem of ‘Pelvic Obliquity’

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Is Scoliosis Surgery in Cerebral Palsy

Patients ‘truly’ Beneficial?

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Need for Pre-operative Assessment

Respiratory

Nutritional/Immunological

Intra-operatve blood-loss

Behvarioral/neurological

Metabolic

Risk of infection

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In Conclusion

The course of development of scoliosis in CP remains partiallyunclear… but:

Early management to keep midline arrangement ( seating –physiotherapy) is essential in order to protect the spine

This does involve multidisciplinary approach (tone – seating-…)

Growing rods might address the problem of early spine deformationand alter the development op windswept deformity

In Conclusion

On the basis of the natural history and risk factors for progression of scoliosis in spastic cerebral palsy:

A patient with severe spastic cerebral palsy should be examined fromas young an age as possible to determine onset of scoliosis.

Surgical treatment should be considered an option if the spinal curve exceeds 40° before age 15 years.

When the patients have total body involvement, are bedridden, or have a thoracolumbar curve, early surgical intervention, we believe, would be desirable.

In Conclusion

Scoliosis surgery leads to a significant improvement in HRQoL for patients with CP who have GMFCS level-IV or V function.

The effects of surgery are maintained 5 years after surgery.

The overall complication rate for scoliosis surgery in CP is around 45 % at 1 year, with an additional 5 % up to 5 years ….

These complications did not correlate with HRQoL gains and perceived satisfaction with surgery as reported by the caregivers

In Conclusion

Continued discussion is needed regarding the most technicallyand ethically suitable treatments for these patients.

We believe that the natural course of scoliosis in patients withsevere spastic cerebral palsy represents important background data for guiding these difficult therapeutic choices.

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