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Factors Influencing the Evaluation and Management of Neuromuscular Scoliosis

Title Page

Title: Factors Influencing the Evaluation and Management of Neuromuscular

Scoliosis: A Review of the Literature

Type of Article: Review article

Key words: Neuromuscular diseases, cerebral palsy, Duchenne muscular dystrophy,

scoliosis, surgery, bracing, orthoses.

Word Count: 3556 words

Ethical approval: None required.

Number of Figures: 10 (Fig. 1, Fig. 2a-d, Fig. 3a-d, Fig. 4)

Number of Tables: 2

Number of References: 83

Authors: 1. Simon B Roberts ECAT Fellow/Specialist Registrar Trauma and Orthopaedics, 2. Athanasios I Tsirikos Consultant Orthopaedic and Spinal Surgeon

Authors’ Institutional Address/Affiliations: University of Edinburgh/Scottish National Spinal Deformity Centre, Royal Hospital for Sick Children, Edinburgh, EH9 1LF, UK

First Author: S B Roberts MBChB, BSc, MSc, MRCS(Ed); postal address - 12 Littlejohn Avenue, Edinburgh, EH10 5TG, UK; email - [email protected]; tel – 07832084911.

Corresponding author: Mr Athanasios I Tsirikos; postal address - Scottish National Spinal Deformity Centre, Royal Hospital for Sick Children, Edinburgh, EH9 1LF, UK; email – [email protected]; tel – 0131 536 0784; fax – 0131 536 0924.

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mailto:[email protected]


Abstract

Neuromuscular scoliosis (NMS) is the second most prevalent spinal deformity (after

idiopathic scoliosis) and is usually first identified during early childhood. Cerebral

palsy (CP) is the most common cause of NMS, followed by Duchenne muscular

dystrophy (DMD). Progressive spinal deformity causes difficulty with daily care,

walking and sitting, and can lead to back and rib pain, cardiac and pulmonary

complications, altered seizure thresholds, and skin compromise. Early referral to

specialist spinal services and early diagnosis of NMS is essential to ensure appropriate

multidisciplinary patient management. The most important goals for patients are

preservation of function, facilitation of daily care, and alleviation of pain. Non-

operative management includes observation or bracing for less severe and flexible

deformity in young patients as a temporising measure to provide postural support.

Surgical correction and stabilisation of NMS is considered for patients with a

deformity >40-50o, but may be performed for less severe deformity in patients with

DMD. Post-operative intensive care, and early mobilisation and nutritional

supplementation aim to minimise the rate of post-surgical complications, which are

relatively common in this patient group. However, surgical management of NMS is

associated with good long-term outcomes and high satisfaction rates for patients, their

relatives and carers.

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1. Introduction

Scoliosis is defined as a coronal plane spinal curvature of at least 10 degrees. A

scoliotic deformity arising in the clinical setting of muscle imbalance due to an

underlying neuropathic or myopathic disease can be diagnosed as neuromuscular

scoliosis. Neuromuscular scoliosis is the second most prevalent spinal deformity, after

idiopathic scoliosis. Patients with neuromuscular scoliosis frequently also have

cardiac, gastrointestinal, and respiratory compromise in addition to other

musculoskeletal and neurological disorders. Management of the scoliosis is therefore

complex and requires careful multidisciplinary care. This review will focus on the

diagnosis, management, and outcomes following the treatment of scoliosis in patients

with neuromuscular conditions.

2. Epidemiology and Aetiology

The incidence of scoliosis in the most common neuromuscular conditions is shown in

Table 1. Cerebral palsy is the most prevalent cause of neuromuscular scoliosis,

followed by Duchenne muscular dystrophy (DMD). Children with neuromuscular

conditions develop spinal deformities due to a combination of poor muscular control

across the trunk and pelvis, inherent muscle weakness, and occasionally spasticity.

The abnormal curvature develops as a consequence of the effect of gravity on the

vertebral column, which is inadequately supported due to generalised weakness of the

posterior spinal and abdominal muscles during a period of accelerated skeletal

growth. The degree of spinal deformity in these patients is therefore directly related to

the degree of neuromuscular involvement and the child’s ambulatory ability.

Wheelchair-bound growing patients with any underlying aetiology are much more

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likely to develop scoliosis that demonstrates a high risk of rapid progression, and

which often also affects the pelvis, than children who still retain walking capacity [1].

3. Clinical Manifestations and Presentation

3.1. Types of Deformity

Neuromuscular scoliotic deformities are generally first identified during early

childhood. Initially, a flexible postural curve develops which is correctable with the

patient lying flat or on suspension. This often progresses into a torsional rigid

deformity during late child life and the pubertal growth and finally into a stiff curve of

larger magnitude before growth is complete [1]. The typical curvature of

neuromuscular scoliosis is a long, collapsing C-shaped curve affecting the thoracic

and lumbar spine (Figure 1) [2]. The deformity often extends to include the pelvis,

producing pelvic obliquity with the elevated one-half of the pelvis corresponding to

the concave side (inside) of the curve. Asymmetric hip positioning due to hip

contractures, subluxation or dislocation, as well as unbalanced function of the trunk

and pelvic muscles may also cause pelvic deformity and accentuate the underlying

scoliosis. If a severe scoliosis and pelvic obliquity co-exist with associated hip

subluxation/dislocation, it is generally preferable to treat the spino-pelvic deformity

first in order to produce a horizontal pelvis before surgical treatment to relocate the

displaced femoral head within the dysplastic hip joint is planned. Finally, a kyphotic

deformity with collapse of the trunk often in conjunction to the scoliosis is also

common in neuromuscular conditions [3]. In contrast, isolated lumbar hyperlordosis

or a severe lordoscoliosis is less frequent but produces major trunk imbalance, sitting

intolerance and persistent pain, which necessitates surgical treatment [4].

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3.2. Clinical signs and symptoms

Deformity progression leads to significant trunk and spino-pelvic imbalance with

consequent loss of function and increasing difficulty with daily care. Walking and

sitting ability may become difficult, leading to pain or the patient becoming confined

to bed. Rotation and shortening of the trunk occurs and can lead to compression of the

heart and lungs, reduced mobility of the ribs and restriction of breathing capacity. If

tilting of the pelvis develops, mechanical back or rib impingement pain can result, as

well as cardiac or pulmonary complications. Feeding can also be affected and

exacerbation of pre-existing swallowing disorders or gastro-oesophageal reflux can

occur. Seizure activity may become worse due to uncontrolled pain. Asymmetrical

loading in the area of the buttocks due to pelvic obliquity can cause pressure sores,

especially in patients with impaired skin sensitivity (myelomeningocele, occasionally

cerebral palsy) [1]. Many patients are also unable to articulate their symptoms, which

can lead to a delay in presentation and diagnosis. Due to the universal progressive

nature of neuromuscular scoliosis, early diagnosis of deformity is essential to optimise

the multidisciplinary management. Early referral to specialist spinal centres of any

patient with a neuromuscular condition and suspected spinal deformity is

recommended.

4. Clinical Evaluation

Patients with a neuromuscular scoliosis are initially assessed by clinical review in

order to identify the severity of the spinal deformity followed by a systemic

examination to identify associated co-morbidities or medical problems related to the

development of the curve. Upper and lower limb function is assessed and evaluation

of lower limb deformities is performed as these may contribute to the development of

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spino-pelvic imbalance. Whole-spine postero-anterior and lateral radiographs are then

performed. Sitting radiographs may be obtained if the patient is unable to stand.

Supine x-rays in maximum traction can be useful to assess curve flexibility. Curve

magnitude (as measured by the Cobb method) [5], spinal balance, pelvic obliquity,

curve flexibility and curve progression are all closely monitored. An MRI scan of the

whole spine is required if there is suspicion of intraspinal pathology. A patient with

established neuromuscular scoliosis requires at least annual follow-up examination to

assess curve progression, but more frequent reviews, usually every 6 months, are

required during periods of rapid skeletal growth and for more severe or progressive

curves.

5. Risk Factors for Disease Progression

In general, there is a proportional relationship between the severity of neurological or

myopathic involvement and curve severity, as well as the risk for deformity

progression. For example, quadriplegic cerebral palsy typically causes a more severe

and progressive scoliosis compared to hemiplegic or diplegic cerebral palsy. The

progression of scoliosis in patients with cerebral palsy treated with intrathecal

baclofen is not significantly different from cerebral palsy patients not receiving

baclofen therapy [6]. For patients with DMD, later age at loss of walking ability and

greater duration of corticosteroid therapy are related to later onset of scoliosis,

whereas asymmetry of the hips and forced vital capacity at age 11-12 years are

directly correlated with subsequent severity of scoliosis [7]. While the rate of curve

progression is highly variable, the average progression for children with cerebral

palsy has been reported as 0.8 degrees each year for curves that are less than 50

degrees and 1.4 degrees each year for curves that are more than 50 degrees [8].

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During periods of rapid growth such as the pubertal growth spurt, much more rapid

progression can occur, with curves increasing as much as 2-4 degrees each month,

resulting in rapid deterioration of spinal deformity and the patient’s function [9, 10].

6. Nonoperative Management

6.1. Aims of Treatment

Several factors need to be considered during the global planning of disease

management in patients with neuromuscular scoliosis. The most important goals for

patients are preservation of function, facilitation of daily care, and alleviation of pain.

Non-operative management of patients with neuromuscular spinal deformities should

be directed at maximising sitting ability and postural control in order to facilitate

motor function and interaction to the environment. Due to the lack of randomised

controlled trials investigating treatment modalities for spinal deformity in

neuromuscular patients, much of the management of neuromuscular scoliosis derives

from results of cohort studies, comparative cohort studies, anecdotal evidence and

expert opinion. Growing patients with mild, flexible curves can be managed by

observation only. For patients with DMD, corticosteroids may delay the development

and progression of scoliosis and may be considered for this purpose, though the

optimal dosing regimen remains to be determined [11-14].

6.2. Bracing

If progression of neuromuscular scoliosis occurs, bracing may be considered. It is

generally accepted that spinal bracing is ineffective in significantly altering

progression of deformity in patients with neuromuscular disease. Although bracing

may not ultimately prevent the need for surgical intervention, bi-valved or soft full-

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body contact thoraco-lumbo-sacral braces can be used in patients in whom surgery is

contra-indicated or declined, and in young children with small, flexible curves. In

these circumstances, bracing can help to manage the spinal deformity by providing

postural support, maintaining sitting position, improving muscle balance, and

augmenting function, while permitting further growth to occur prior to surgical

intervention [15-17]. Furthermore, bracing may limit complications such as impaired

pulmonary development, and therefore reduce the risks associated with any future

surgery [3]. However, bracing is often ineffective due to shortening duration of brace

wear, skin irritation, and respiratory problems, as well as the aggressive nature of the

deformity. Cognitive impairment associated with cerebral palsy may also reduce a

patient’s tolerance of bracing. It is important that the potential advantages,

disadvantages, and expected outcomes of bracing are clearly understood by the

patients, their family and carers if it is used [9, 18].

6.3. Orthotics

Wheelchair adaptations can be used in addition to, or as an alternative to, bracing.

These adaptations include offset lateral chest supports, shoulder/waist harnesses,

modular seating systems, and moulded shells. The latter permit the patient to lie and

be tilted, and may be better tolerated than braces, are simple to use for carers, permit

free access to the abdomen if gastrostomy feeding is required, and do not restrict

respiratory movements, which is particularly important for children at high risk for

chest infections [15]. There is conflicting evidence regarding the role of knee-ankle-

foot orthoses in prolonging functional walking, and any association with subsequent

progression of scoliosis. Therefore knee-ankle-foot orthoses cannot be recommended

for the purpose of preventing development or progression of scoliosis in

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neuromuscular patients [19, 20]. Braces and orthoses that protect all functions are not

available, and therefore their selection and use must be tailored to the needs of each

individual patient.

6.4. Physiotherapy

Physiotherapy, including regular stretching, is widely performed and important in the

general care of neuromuscular patients, but does not improve any aetiological factors

for neuromuscular scoliosis or prevent progression of an established scoliosis.

Physiotherapy can be helpful to prevent any adverse effects from prolonged brace use,

prevent the onset of joint contractures, and maintain both chest mobility and

respiratory excursion [21].

6.5. Other Non-operative Treatments

There is no evidence to support electrical stimulation or botulinum toxin injections to

manage spinal deformity in patients with neuromuscular conditions. The use of lycra

suits may be beneficial in patients who retain some degree of muscle function and

who develop small and flexible postural curves as an alternative to a conventional

brace due to the fact that they are usually better tolerated and in order to stimulate

upright posture and preserve function [22].

7. Operative Management

7.1. Aims of Surgical Intervention

The decision to proceed with operative correction and stabilisation of neuromuscular

scoliosis is dependent on individual patient-specific factors, in particular the nature of

the underlying neuromuscular condition and the patient’s current level of function.

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The patient’s individual needs, overall medical state and long-term prognosis of the

underlying neuromuscular condition have to be taken into account and the decision

for scoliosis surgery has to be individualised and discussed thoroughly with the

patients and their families. For higher-functioning patients, operative intervention

aims to provide a more normal spinal balance and alter the progression of disease with

the goal of preserving function with respect to ambulatory capacity. In wheelchair-

dependent patients, the aim is to maintain sitting independence, promote more

physiological respiratory and gastrointestinal functioning, and facilitate overall care

[23].

7.2. Indications for Surgery and Surgical Techniques

In general, surgical intervention is considered for patients with a curve greater than 40

or 50 degrees and significant deterioration in function (Figure 2). Such curves will

deteriorate even after the patients have completed their skeletal growth. An exception

to this is DMD scoliosis in which, due to the natural history of the disease, surgery is

considered for progressive spinal curvatures greater than 25 degrees in order to

prevent respiratory compromise produced by the deformity. Surgical treatment will

aim to correct the spinal deformity and pelvic obliquity, restore coronal and sagittal

spinal balance, and achieve a solid spinal fusion. This is usually performed using a

posterior surgical approach to the spine, with instrumentation to correct the

curvatures, and autologous and allograft bone to achieve a bony fusion [24-26]. This

typically results in up to 75-80% overall correction of deformity [27-29]. For patients

with severe curves, which may be permitted to develop in patients with cerebral palsy,

the challenge is to manage spinal growth while controlling the deformity. For curves

of 60-90 degrees magnitude, surgery is considered when the deformity becomes stiff

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on clinical and radiographic examination. If the spine remains flexible, surgery may

be delayed until the curve reaches approximately 90 degrees of magnitude, and this

can usually be corrected by posterior spinal fusion in the advent of modern segmental

pedicle screw instrumentation techniques (Figure 3). For stiff curves or those greater

than 90 degrees with fixed pelvic obliquity, surgical release and fusion of the anterior

column of the spine may be required to increase flexibility of the deformity and allow

for better correction in addition to posterior spinal fusion; this combined procedure

increases the potential morbidity and complications associated with surgery.

The instrumentation and fusion of the deformity in neuromuscular scoliosis usually

extends from the upper thoracic spine (T2 vertebra) proximally to the sacrum/pelvis

distally as this prevents the development of recurrent deformities above and below the

instrumentation that can occur with further skeletal growth if a shorter fusion is

performed [26, 30-33]. In the absence of significant pelvic deformity the lower

lumbar spine may be selected as the lower extent of the spinal fusion to preserve the

patient’s lumbosacral flexibility [34, 35].

Growth preservation techniques (growing rods) may be considered in young patients

with severe but flexible curves as a temporising measure spanning the levels of the

deformity across the spine and often extending down to the pelvis. The benefits of

preserving spinal growth and delaying the need of the definitive spinal fusion for a

later age have to be balanced over the considerable risk of major complications in the

need of serial operations which is increased due to the underlying medical co-

morbidities and inherent bone weakness. Control of scoliosis and spinal growth with

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growing rod surgery has been reported as an effective strategy in patients with spinal

muscular atrophy [36].

8. Perioperative Care

8.1. Perioperative Risks and Preoperative Planning

The risks associated with surgery for neuromuscular scoliosis are correlated with the

severity of neurological impairment and associated co-morbidities. Factors that are

strongly associated with increased risk of postoperative complications include non-

oral feeding, severe cognitive impairment, inability to communicate vocally, presence

of seizures, and non-independent sitting ability. As patients with neuromuscular

disease often have varied and complex medical conditions, pre-operative assessment

requires a multidisciplinary involvement. In particular, it is important to recognise and

optimise any seizure activity, cardio-respiratory problems, gastro-intestinal reflux or

dysmotility, feeding and nutritional difficulties. As the risk of surgery is greatly

increased by cardiac dysfunction or arrhythmias associated with DMD, pre-operative

assessment by a paediatric cardiologist and anaesthesiologist is mandatory [37]. Pre-

operative pulmonary function tests positively correlate with the incidence of

postoperative pulmonary complications, with values below 30-40% of predicted vital

capacity providing the best indication of a high risk of major postoperative respiratory

complications [38]. Spinal stabilisation can, however, be performed safely in patients

with low vital capacity and those with pre-existing respiratory failure requiring

nocturnal non-invasive ventilation [39, 40]. The use of preoperative non-invasive

ventilation may be helpful for patients with forced vital capacity less than 40% of

predicted values or dependent on arterial blood gas results.

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Further specific anaesthetic measures beneficial in the peri-operative management of

patients with neuromuscular scoliosis include hypotensive anaesthesia, use of cell

salvage, tranexamic acid, haemodilution, haemodynamic monitoring, and careful

maintenance of normothermia [41-44]. A summary of preoperative investigations for

neuromuscular patients undergoing spinal stabilisation is provided in Table 2. To

protect function, spinal cord monitoring is performed intra-operatively with

transcranial motor and somatosensory evoked potentials for patients who are

ambulatory, can stand, or have purposeful lower extremity movement or for those

patients with normal bladder and bowel function. Spinal cord monitoring is therefore

utilised intra-operatively for patients with muscular causes and mild cerebral palsy,

but may not be appropriate, for example, in patients with severe quadriplegic cerebral

palsy or severe spinal cord dysfunction. When performed together, transcranial motor

and somatosensory evoked potentials have 95-100% reliability and permit motor

function testing intra-operatively and adaptation of recording sites to the relevant

motor function for each patient [45-47]. Patients, family and caregivers should be

fully informed regarding the potential for prolonged and complex intensive care and

in-patient stay postoperatively (additional education resources are shown in Figure

4). Furthermore, resuscitation decision-making should also be discussed

preoperatively with the patient and their family as appropriate.

8.2. Postoperative Care

Patients may remain in an intensive care unit, often still intubated, for several days

postoperatively, for close monitoring of cardio-respiratory and haemodynamic status,

before returning to standard ward care. The required duration of postoperative

ventilation is determined individually and is usually no more than 36 hours. Early re-

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instigation of nutritional intake, often with the use of nasogastric, nasojejunal or

gastrostomy tubes and nutritional supplementation, is important in the postoperative

period. This is particularly important perioperatively for patients with cerebral palsy

who are often malnourished prior to surgery [48]. Patients are encouraged to mobilise

out of bed or in a wheelchair as soon as medically safe following surgery. Patients are

usually able to mobilise using a wheelchair by day 2-5 postoperatively [42, 49].

Adjustments to patients’ wheelchairs can also be made during this period as necessary

and by assessment of an orthotist. Patients are usually advised to avoid rotation of the

spine or flexion of the hips beyond 90 degrees for 4-6 months postoperatively, and

appropriate techniques are learnt with the assistance of a physiotherapist.

Consideration should be given to the need of a hoist for patient transfers during

subsequent rehabilitation. Postoperative trunk support in the form of a brace is usually

not required following the use of modern spinal stabilisation techniques. Following

discharge, children can usually return to school within 3-4 weeks.

9. Outcomes of Treatment

9.1. Patient Satisfaction and Quality of Life

With modern medical care, the life expectancy of patients with complex

neuromuscular conditions has been considerably prolonged. Therefore, treatment of

their spinal deformities is now becoming a greater necessity in order to maintain

functional abilities and preserve quality of life. For patients with severe

neuromuscular disease, careful consideration must be given on whether or not the

risks of an extensive surgical procedure in the presence of complex medical problems

outweigh any anticipated benefits. Surveys following surgical correction for patients

with total-body-involvement spastic cerebral palsy and scoliosis demonstrated that

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85-96% of parents and caregivers are satisfied with the outcomes for the patient after

surgery and would choose the procedure again [29, 50, 51]. Surgery resulted in

improved sitting ability, function, activities of daily living, physical appearance,

comfort, ease of care, and self-image [52-55]. Such improvements may positively

affect quality of life for these patients, which is important as the mean patient survival

following surgical correction for scoliosis secondary to severe cerebral palsy has been

reported as 11.2 years [56]. Similar satisfaction rates have been demonstrated in

surveys of patients treated for DMD scoliosis, who also report improvements in

breathing, digestion and posture postoperatively [57]. In DMD scoliosis, early

surgical correction improves the deformity, and may have a positive impact on

survival [58, 59].

9.2. Effect of Surgical Intervention on Respiratory Function

Prior to surgery, patients with neuromuscular disease and scoliosis have lower

pulmonary function parameters than those without scoliosis and healthy controls [60].

In a prospective study including neuromuscular patients, pulmonary function declined

up to 60% immediately following surgery, reaching a nadir at 3 days postoperatively,

but returning to preoperative values between 1 and 2 months after surgery [61]. One

year following spinal stabilisation, peak cough flow and end tidal CO2 measurements

are reportedly well maintained in neuromuscular patients [62]. However, the long-

term effect of surgical stabilisation of scoliosis on respiratory function is incompletely

understood and existing evidence is conflicting, with studies reporting both no

difference in subsequent rate of decline and other studies indicating a reduced rate of

decline in respiratory function [63-69]. The effect of spinal stabilisation on gastric

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emptying, upper gastrointestinal symptoms and clinical nutritional state has been

reported as minimal [70].

9.3. Complications of Treatment

The incidence of complications following surgical correction of neuromuscular

scoliosis has been estimated as ranging from 18% to 68%. Complications should

therefore be actively anticipated, especially in patients with DMD who have higher

overall rates [71, 72]. Non-ambulatory status and severe preoperative curve

magnitude greater than 60o are associated with increased risk for major postoperative

complications [73]. The most common complications include superficial and deep

wound infections (13.1%), non-surgical site infections (10%), pseudoarthrosis,

(1.9%), problematic instrumentation (3.4%), and pulmonary/respiratory complications

(19.4%) [42, 74-76].

The most frequent postoperative complication in patients with cerebral palsy is

pulmonary infection, with a particular risk of aspiration pneumonia. Pulmonary

complications are also common following surgical correction in patients with DMD;

postoperative atelectasis is relatively frequent in DMD patients, and the incidence of

pulmonary infection has been estimated at 7-8% [62, 72]. Wound infections also

occur at a similar frequency in patients with DMD, but are less common in patients

with cerebral palsy [72, 77]. In patients with spinal cord dysfunction, particular care

must be given to surgical site and general skin pressure management due to insensate

skin, and also to care of the urinary system to avoid urosepsis.

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Other recognised complications include postoperative ileus, pancreatitis, and superior

mesenteric artery syndrome, while neurological complications are rare [78]. However,

surgical complications rarely cause any permanent problem for the patient or

caregivers, but rather extend the need for inpatient care. Indeed, the mean hospital

stay for surgical correction of neuromuscular scoliosis can be prolonged and this has

been previously reported as between 14 and 19.6 days [42, 56].

10. Conclusion

Scoliosis is common in patients with neuromuscular conditions. The spinal deformity

is progressive and can lead to loss of ambulation, difficulty sitting, feeding disorders,

pain, poor self-image, and increasing difficulty with daily care. Non-operative

treatment cannot control the deformity but may be used while significant spinal

growth remains. Early referral of patients with neuromuscular conditions and

suspected spinal deformity to specialist spinal centres is essential to optimise the

multidisciplinary management and long-term outcomes. Surgical correction and

stabilisation of the spinal deformity is effective, but it is associated with significant

risks that are usually manageable. Surgical management of neuromuscular scoliosis is

associated with good long-term outcomes and high satisfaction rates among patients,

their families and caregivers.

Acknowledgements

Both authors contributed to the design and writing of the article. The authors have no

relevant interests to declare. This study has no conflict of interest. No funding was

received for this work.

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28


Table 1. Scoliosis Research Society classification and incidence of spinal deformity

in neuromuscular conditions.

Classification Diagnosis Co-incidence

of Scoliosis

Central neurological

cause

Cerebral palsy 25-74%

Central motor neuron

involvement

Friedreich’s ataxia

Syringomyelia

Other (encephalopathy, Rett’s

syndrome)

80%

25-85% [79]

Peripheral neurological

causes

Acute anterior poliomyelitis 17-65% [80]

Peripheral motor

neuron involvement

Spinal muscular atrophy

Hereditary motor and sensory

neuropathy

Hereditary sensory and vegetative

neuropathy (familial dysautonomia)

67%

26% [81]

86% [82]

Mixed central and

peripheral neurological

causes

Traumatic paralysis (before 10

years of age)

Myelodysplasia

Myelomeningocele

100%

60%

52-89% [83]

Neuromuscular junction Myasthenia Unknown

Muscular causes Duchenne Muscular Dystrophy

Arthrogryposis

Other muscular dystrophies

90%

30-67% [80]

29


Table 2. Preoperative clinical and radiological investigations for patients undergoing

surgical correction of neuromuscular scoliosis

Role of Investigation Investigation for Neuromuscular Scoliosis

Assessment of Spinal

Pathology

Whole spine radiographs (PA, lateral & supine

maximum traction)

Whole spine MRI (if any suspicion of intraspinal

pathology)

Hips & pelvic radiographs

Anaesthetic &

Medical Assessment

FBCs, U&Es, LFTs

Blood cross-match

Albumin/calcium/vitamin D

Coagulation screen

Chest radiograph

ECG, echocardiogram

PFTs, ABGs, sleep studies, spirometry

Nutritional assessment

Urinalysis, urine culture & sensitivities

Neurological assessment

Keys: FBC (full blood count), MRI (magnetic resonance imaging), U&Es (urea &

electrolytes), PFTs (pulmonary function tests), ABG (arterial blood gases), ECG

(electrocardiogram), LFTs (liver function tests), PA (posteroanterior).

30


Figure Captions

Figure 1. Spinal deformity patterns in cerebral palsy associated neuromuscular

scoliosis. In types A and B (double curves of the thoracic and lumbar spine), the spine

remains well balanced with little or no pelvic obliquity. In types C and D (large

thoracolumbar or lumbar curves), the deformity extends into the sacrum and often

causes significant pelvic obliquity.

Figure 2. Radiographs of a patient with Friedrich’s ataxia showing pre-operative a)

posteroanterior (PA) and b) lateral radiographs of the spine at age 14 years and 6

months. A primary thoracolumbar scoliosis measuring 50o with a compensatory

thoracic curve measuring 35o and no pelvic deformity can be seen. Post-operative c)

PA and d) lateral radiographs at age 17 years and 2 months following posterior spinal

fusion demonstrate excellent correction of the thoracolumbar and thoracic curves to 8o

and 5o, respectively, with restoration of global spinal balance.

Figure 3. Radiographs of a patient with quadriplegic cerebral palsy showing pre-

operative a) posteroanterior (PA) and b) lateral radiographs of the spine at age 14

years and 8 months. A collapsing thoracolumbar scoliosis measuring 88o with

associated thoracic hyperkyphosis measuring 98o and severe pelvic obliquity can be

seen. The pain had severe back and left costo-pelvic impingement pain (bony pain

produced due to the lower ribs sitting against the elevated left side of the pelvis)

which did not allow him to sit on his wheelchair. Post-operative c) PA and d) lateral

radiographs at age 17 years and 1 month following posterior spinal fusion

demonstrate correction of the thoracolumbar curve to 25o, levelling of the pelvis and

normalisation of thoracic kyphosis to 40o. An intrathecal Baclofen pump may also be

seen in the radiographs and this was preserved in place during scoliosis surgery.

Figure 4. Additional education resources for patients and healthcare professionals.

31


Figure 1

32


Figure 2a

33


Figure 2b

34


Figure 2c

35


Figure 2d

36


Figure 3a

37


Figure 3b

38


Figure 3c

39


Figure 3d

40


Figure 4

Scoliosis Association (UK): charity supporting patients with scoliosis;

www.sauk.org.uk

Scottish National Spine Deformity Service: information regarding the

service, spinal deformities, and their treatment;

http://www.nhslothian.scot.nhs.uk/Services/A-Z/ScottishNationalSpineDef

ormityService/Pages/default.aspx

British Scoliosis Society: society supporting surgeons, healthcare workers

and researchers regarding scoliosis; http://www.britscoliosissoc.org.uk

British Scoliosis Research Foundation: charity promoting research into

scoliosis and allied health conditions in the UK; http://www.bsrf.co.uk

Scoliosis Research Society: society supporting education for healthcare

workers and research in spinal deformities; http://www.srs.org

41

http://www.srs.org/

http://www.bsrf.co.uk/

http://www.britscoliosissoc.org.uk/

http://www.nhslothian.scot.nhs.uk/Services/A-Z/ScottishNationalSpineDeformityService/Pages/default.aspx

http://www.nhslothian.scot.nhs.uk/Services/A-Z/ScottishNationalSpineDeformityService/Pages/default.aspx

http://www.sauk.org.uk/

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