Download Spinal Cord Injury - The Royal Children`s Hospital

Spinal Cord Injury
Dr. Jayasri Srinivasan
Paediatric Neurologist
VPRS, The Royal Children’s Hospital, Melbourne
Spinal Cord Injury (SCI)
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Also referred to as Spinal Cord Injury &
Disease (SCID)
SCI is rare in children
However, huge implications
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Permanent loss of motor & sensory function
Dysfunction of bowel & bladder
Social & psychological consequences for child and
family
Often comorbid injuries such as TBI
SCI rates
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Australian SCI stats
• Population 22.5 million
• 350-370 new SCI per year
• 80% due to trauma
• Overall incidence 15 SCI/million
population/yr
SCI rates
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Australian SCI stats
• Population 22.5 million
• 350-370 new SCI per year
• 80% due to trauma
• Overall incidence 15 SCI/million
population/yr
Unpublished VPRS data courtesy of Dr Adam Scheinberg
Victorian SCI stats
• Paediatric SCI rates reported as
1.99/100,000 in the US, Europe and
South America
• Based on this, could expect 20-25 per
year in Victoria
• Anecdotal evidence suggests numbers
much lower than this
Causes: SCI & SCD
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Transection
Distraction
Compression
Bruising
Haemorrhage
Ischaemia
Inflammation
Aims of this lecture
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What you should know for the exams
What you should know when dealing with SCIrelated emergencies
What you should about disability care in
children with SCI
Spinal cord anatomy
The spinal column
consists of 33 vertebrae:
– 7 cervical vertebrae
– 12 thoracic vertebrae
– 5 lumbar vertebrae
– 5 sacral vertebrae
– 4 coccygeal vertebrae
Spinal cord anatomy
– Located in upper 2/3 of the vertebral
column
– The terminal portion of the cord is the
conus medullaris, which becomes
cauda equina (horse’s tail) at
approximately the L1–L2 vertebrae
– The spinal cord has white matter
surrounding an inner core of gray
matter
Neuroanatomy for the neuroscientist, 2008
Physical Medicine and Rehabilitation Board Review 2nd ed, 2010
Spinal cord anatomy
https://courses.stu.qmul.ac.uk/smd/kb/microanatomy/brain/CAL1/BaBy1CALwk1.htm
Spinal cord anatomy
https://courses.stu.qmul.ac.uk/smd/kb/microanatomy/brain/CAL1/BaBy1CALwk1.htm
Definitions
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Quadriplegia
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Cervical SCI
causing
dysfunction of
arms, legs, bowel
& bladder
Paraplegia
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Thoracic, lumbar
or sacral SCI
dysfunction of
legs, bowel &
bladder
http://www.bio.davidson.edu/courses/anphys/2000/Rigel/Anatomy.htm
Classification
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Complete
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Entire cross section of SC affected, with loss of all
motor and sensory function below level of injury
Incomplete
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Spinal cord has been partially injured, with
preservation of either motor or sensory function
ASIA
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American Spinal Injury
Association (ASIA)
Created the AIS (ASIA
Impairment Scale)
Constructed to provide a
standardized method for
communication between
professionals
American Spinal Injury Association: http://www.asia-spinalinjury.org/
Acute spinal cord trauma
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General signs of acute SCI
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Flaccid paralysis below level of injury
Loss of spinal reflexes below level of injury
Loss of sensation (pain, touch, P&V, temp) below
level of injury
Loss of sweating below level of injury
Loss of sphincter tone and bowel & bladder
dysfunction
Acute management
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ABC
Regular monitoring of vitals
Spinal immobilisation
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Collar, sandbags +/- forehead strap
Early surgery for reduction&/or fixation
Halo & orthotic devices to maintain correct spinal
alignment
Use patient slide to move patient, log roll to turn
patient
No pharmacological agent has been proven to limit
damage and optimize function in acute SCI
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Including steroids
Scenarios of incomplete
syndromes
Scenarios
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15yo boy
MVA – hyperflexion injury
No movement below the level
of injury to the spinal cord
Reduced pain & temperature
below level of injury
Normal light touch and
position sense distal to the
injury
Scenario 1: Anterior cord syndrome
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Anterior cord syndrome
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Damage to ant 2/3 spinal
cord, usually due to
vascular occlusion
Features
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motor paralysis
(corticospinal tract) (Variable)
Loss of pain &
temperature(spinothalamic
tract) – (Variable)
sparing of the dorsal
column
Physical Medicine and Rehabilitation Board Review 2nd ed, 2010
Scenarios
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15yo boy
MVA – hyperextension injury
Weaker in upper limbs vs
lower limbs
Variable sensory loss below
the level of injury
Scenario 2: Central cord syndrome
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Central cord syndrome
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Typically caused by
hyperextension causing
central cord injury
Features
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Greater motor weakness in the
upper limbs than the lower limbs
(damage to central corticospinal
tract)
With variable loss of sensation,
bowel and bladder function (fibres
affecting voluntary bowel &
bladder function are also centrally
located)
Physical Medicine and Rehabilitation Board Review 2
nd
ed, 2010
Scenarios
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15yo boy
Chronic malabsorption
Difficulty walking due to
proprioceptive deficits
Abnormal joint position sense
Normal muscle strength
Scenarios 3: Posterior cord
syndrome
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Posterior cord
syndrome
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Least frequent syndrome
Causes include B12
deficiency, syphilis, HIV
Features:
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Injury to the posterior
columns results in
proprioceptive loss (dorsal
columns)
Spared muscle strength, pain
and temperature spared
Physical Medicine and Rehabilitation Board Review 2nd ed, 2010
Scenarios
15yo boy
Stabbed in the back
motor loss on the same side
as stabbing
sensory loss on both sides
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V & P loss on same side of the
injury
P & T loss opposite side of the
injury
Scenario 4: Brown-Sequard
syndrome
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Hemisection of spinal cord
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• Neurological deficits distal to
the level of the lesion vary from
the different nerve tracts crossing
at different locations
Features:
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Ipsilateral flaccid paralysis at the level of
the lesion (LMN) & ipsilateral spastic
paralysis (UMN) below level of lesion
Ipsilateral loss of position sense and
vibration below the lesion
Contralateral loss of pain and temperature
below the lesion(spinothalamic)
Physical Medicine and Rehabilitation Board Review 2
nd
ed, 2010
Scenario 5
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Patient seen by
you has a lesion
at A/B
Another has a
lesion at C
Syndrome &
features of A/B &
C?
Scenario 5: Conus & Cauda Equina
Syndromes
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Conus Medullaris Syndrome
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• The conus medullaris = terminal segment of the adult
spinal cord (L1–L2)
• Features:
• areflexic bladder and bowel, and LL weakness (type
can vary depending on high (A) or low (B) lesions)
Cauda Equina Syndrome
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Injuries below the L1–L2 affect the cauda equina
Results in lower motor neuron injury (C)
Features:
• Produces motor weakness and LL atrophy
• Bowel and bladder involvement
• Impotence
• Absent bulbocavernous reflex
Has a better prognosis relative to UMN injuries
Physical Medicine and Rehabilitation Board Review 2nd ed, 2010
Clinical implications of SCID
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Motor – Muscular & Bony
Sensory
Bladder
Bowel
Autonomic nervous system
Respiratory
Other medical issues
Psychosocial & educational
Motor
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Many musculoskeletal implications
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Paralysis
Spasticity
Joint contractures
Osteoporosis
Hip subluxation
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Scoliosis
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Standing is essential
Sitting symmetry is important
Shoulder problems
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Rotator cuff problems, premature degenerative changes
Highest level of SCI that someone can live
independently without an attendant is C6
Physical Medicine and Rehabilitation Board Review 2nd ed, 2010
Spasticity - ask does it need to be
treated?
FOCAL
GENERALIZED
REVERSIBLE
• BOTULINUM TOXIN
A
• PHENOLIZATION
• CASTING/SPLINTIN
G
• ORAL MEDICATIONS
(SEMI)PERMANENT
• ORTHOPAEDIC
SURGERY
• RHIZOTOMY
• INTRA-THECAL
BACLOFEN
Hip subluxation - importance of
standing and hip surveillance
early subluxation of the right hip
The Journal of Bone & Joint Surgery. 1998; 80:1068-82
Why Stand?
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Promotes bone density
Promote appropriate development of
the shape of the hip joint
Provides a prolonged stretch to
muscles
Regular standing also provides:
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Opportunity to stand at peer level
Optimum positioning of gastrointestinal system
(can improve digestion and bowel troubles)
Optimum positioning for breathing as
respiratory muscles at best advantage to work
efficiently and lungs are able to expand
Slide courtesy of Dr Adam Scheinberg, VPRS
Different standing frames
Upright
Prone
Supine
Dynamic
Slide courtesy of Dr Adam Scheinberg, VPRS
General principle for Standing
position
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Bottom back (strap around hips not waist)
Head, neck and spine in line
Thighs supported
Feet flat and supported
Arms in the middle and supported
Symmetrical body if possible
Slide courtesy of Dr Adam Scheinberg, VPRS
Scoliosis - importance of
surveillance and good seating
http://milliemaesworld.co.uk/millie/tag/scoliosis/
Sitting & Supportive seating
•Good
seating in wheelchair
to promote symmetrical hip
and trunk posture
Slide courtesy of Dr Adam Scheinberg, VPRS
Head rest
Thoracic supports
Harness
Seat belt
Pelvic supports
Pommel
Footplates
Tilt, recline
Good sitting posture
Seat cushion
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Centre pelvis
Keep hips abducted
Aim for symmetry of the pelvis and good hip
position
Lap belt important to keep pelvis at rear of cushion
– needs to be firm
Pressure support for decreased sensation
Teach kids to re-adjust self
Slide courtesy of Dr Adam Scheinberg, VPRS
Good sitting posture
Backrest
• Chest support to keep trunk/spine symmetrical
• Harness or thoracic supports to provide postural
support
• Harness for safety in travel
Slide courtesy of Dr Adam Scheinberg, VPRS
New Equipment
Functional Electrical Stimulation
(FES) cycle
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Self adhesive electrodes are
attached to the child’s leg muscles
and attached to a stimulator which
activates the muscles
Can also be used for arms
Reverse muscle atrophy
Improve local circulation
Increase range of motion
Reduce muscle spasms
Slide courtesy of Dr Adam Scheinberg, VPRS
FES-Ergometer Cycling in Recently
Injured SCI
Effect of electrical stimulation-induced cycling on bone mineral density in spinal cord-injured patients. Eser P,
de Bruin ED et al. Eur J Clin Invest. 2003 May;33(5):412-9.
Slide courtesy of Dr Adam Scheinberg, VPRS
New Equipment
• Body weight supported
Treadmill training
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may enhance output of
a‘central pattern
generator' of stepping
movement from circuitry
intrinsic to the patient’s
spinal cord.
Cardiopulmonary fitness
Slide courtesy of Dr Adam Scheinberg, VPRS
Skin & sensation
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Lack of sensation = high risk or
pressure areas & burns
Hazards of hot & cold
temperatures
Pressure mattress & regular
repositioning
Hygiene & regular skin checks
Adequate nutrition
Rx of symptomatic orthostatic
hypotension
No robust paediatric specific
evidence regarding safety and
efficacy of thromboprophylaxis in
paediatric SCI
http://en.mebo.com/Clinical/ShowInfo.asp?InfoID=49
Normal bladder:
Sympathetics “Store” and Parasympathetics “Pee”
Physical Medicine and Rehabilitation Board Review 2nd ed, 2010
Neurogenic bladder
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Level of injury
predicts function
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B/w pons & conus –
detrusor sphincter
dyssynergia (DSD)
• VUR,
hydronephrosis,
renal failure
Below conus –
affects PNS =>
areflexic bladder
Neurogenic bladder
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Aim
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Assessment
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prevent UTI, manage incontinence, renoprotection
Bladder chart (including CIC times & volumes)
Normal bladder capacity in mL = 30 + (30 X age in yrs)
EUCs, urinalysis, Renal US +/- KUB yearly
Pre & post CIC bladder scans
Management
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Principles
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Sustained contraction: bladder relaxant drugs, bladder augmentation
Floppy bladder: Clean Intermittent Catheterisation (CIC)
Sphincter too tight: CIC, botulinum toxin
Sphincter too weak: surgery to tighten sphincter
Education
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Especially CIC
Autonomic dysreflexia
Avoid caffeine
Neurogenic bowel
Physical Medicine and Rehabilitation Board Review 2nd ed, 2010
Neurogenic bowel
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Injuries
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SCI above conus: UMN – increased
anal tone
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Prone to constipation
Below conus: LMN – lesions causing
colonic & pelvic floor dysfunction =>
areflexic bowel
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Flaccid, poor sphincter tone – prone to
soiling
Neurogenic bowel
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Aims
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Assessment
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Regular emptying, no incontinence
Bowel chart – review evacuation times, amount, type (Bristol stool
chart)
Management
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Diet (fibre, fluid intake)
Make use of gastrocolic reflex
Positioning (commode, importance of regular standing)
Digital rectal stimulation, local chemical triggers
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Medications:
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Produces reflex peristaltic wave
Stimulants, softeners, osmotic, bulking
Best for UMN & LMN: bulking agents in am +/- stimulants at night
Other:
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Tight underwear (support pelvic floor) – NB pressure areas
Autonomic dysreflexia
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Characterized by HBP, sweating,
headache, flushing above level,
bradycardia
SCI at T6 & above
MEDICAL EMERGENCY!
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ICH, seizures
Management
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Sit up/elevate head of bed
Loosen clothing, stockings, abdo binders
Check BP regularly
Remove noxious stimulus
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Urinary retention, faecal impaction, ingrown
toenail, abdo pathology, pressure sores,
spasticity, fractures, (labour)
Consider medications
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Nifedipine
CMAJ October 28, 2003 vol. 169 no. 9
Respiratory
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Respiratory difficulty depends on injury level
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Assessment of respiratory function (acute)
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Pattern, effort, cough ability, auscultation, SpO2, ETCO2,
ABG
Physiotherapy
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C1-4: diaphragmatic paralysis - needs mechanical
ventilation
C5-T6: intercostal paralysis, diaphragm ok – may need
respiratory support
T6-12: abdominal muscles paralysed – may have reduced
respiratory function
Chest physio, cough assist, BiPAP
Immunisations
Sexual function
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Depends on motor and sensory level
Definition of sexuality?
In general
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Sexual education
Contraceptive devices
Disability related management
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Erection aids
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Continence, positioning, skin care
Meds (oral & intracavernosal), retention bands, vacuum
devices, penile prostheses
Autonomic dysreflexia risk!
Fertility issues
Psychosocial impact
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HUGE!
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Guilt
Loss of potential
Financial implications for family
• Loss of earnings?
• Equipment?
Carer fatigue
Psychiatric disorders
Impact on peer relationships
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A rehab approach:
Interdisciplinary Goals
• Return to school
• Education Consultant – School
• DR’s – rv continence, bone health, chest, autonomic dysreflexia
• Nurses – carer training, CIC, wound care
• Speech Pathologist – trache, PEG/oral intake, communication
• Clinical Psychologist – behaviour, adjustment
• Neuropsychologist – cognition
• Physiotherapist – mobility and transport
• Occupational Therapist – access to school, equipment
• Social Worker –family support
• AHA – school support
• Stream Leader – external providers, transport, funding, internal
communication
Slide courtesy of Dr Adam Scheinberg, VPRS
Summary
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SCID has significant implications
• Medically
• Psychologically
• Socially
Important to recognize acute presentations
and chronic complications
Best dealt with via a multidisciplinary team
Acknowledgements
• Thanks
to the
VPRS team at
RCH for all your
input!