This document summarizes orthopedic considerations for various neuromuscular disorders. It discusses evaluations of gait, deformity, muscle weakness and spasticity. Specific conditions covered include cerebral palsy, muscular dystrophies, spinal cord injuries, peripheral neuropathies, poliomyelitis and Charcot-Marie-Tooth disease. Management may involve physical therapy, bracing, medications, or surgery to address deformities, contractures and other complications in order to maximize function and mobility. Surgical options vary depending on the condition and stage of disease.
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Orthopedic considerations in neuromuscular disorder
1. Orthopedic considerations in neuromuscular disorders
Both neurological deficits and bone disorders lead to locomotor
system abnormalities, joint complications and limb problems.
A functional assessment requires the examiner to look, feel and
move the patients and their limbs.
Other members of the multidisciplinary team are essential in this
assessment to identify the patients' needs and plan treatment and
several areas in the assessment should be
Gait and posture:
Typical recognised patterns of neuromuscular disorders include a
spastic gait, drop foot gait, high steppage gait, waddling and
ataxic gait.
Deformity :
When muscles are weak, the joint is unstable and the limb floppy
and flail; this is balanced paralysis. Unbalanced paralysis occurs
when one group of muscles is relatively weak compared with its
antagonist pull; the deformity may be initially correctable, but may
become fixed with time.
Muscle weakness :This may be due to upper or lower motor
neurone lesions or muscle disorders.
Spasticity and contracture.
2. Cerebral palsy :
The term cerebral palsy includes a group of disorders that result
from non‐progressive brain damage
Children usually have delayed developmental milestones and
later present with a number of problems, including ataxia,
dystonia, athetosis, weakness and spasticity.
With better management, patients with cerebral palsy are living
longer
orthopaedic manifestations characteristic of CP
primary
• abnormal tone
• loss of motor control
• impaired balance
• spasticity
• (hypotonia)
• (dyskenesias such as chorea and athetosis)
secondary (growth and spasticity related)
• contractures: starts as dynamic contractures, become static
with time (continuous muscle contraction results in
shortening) and growth (growth of bones occurs at a faster
longitudinal rate than muscles in spastic cerebral palsy)
• upper extremity deformities
• hip subluxation and dislocation
3. • spinal deformity
• foot deformities
• gait disorders
• fractures often associated with non-ambulatory secondary to
low bone mineral density.
GMFCS
Management :
physical therapy, bracing/orthotics, medications for
spasticity
• spasticity control :
• botulinum - A usually lasts 2-3 months
• used to maintain joint motion during rapid growth when a
child is too young for surgery
• often injected into gastrocnemius
• helpful treatment in dynamic contractures; little benefit with
static contractures
• baclofen
• intra-thecal administration is preferred route to avoid
cognitive impairment seen with oral administration.
• Intra-thecal baclofen tends to be used for non-ambulatory
CP.
Spastic hip disorder vs DDH
There is adductor contracture ,extreme anteversion and increased
neck shaft angle.
Level I Near normal gross motor function, independent ambulator
Level II
Walks independently, but difficulty with uneven surfaces,
minimal ability to jump
Level III Walks with assistive devices
Level IV Severely limited walking ability, primary mobility is wheelchair
Level V
Nonambulator with global involvment, dependent in all aspects
of care
4. Acetabular deficiency posterior superior as apposed to ddh which
is anterior.
Huge relation to GMFCS.
GMFCS 1 LESS THAN 10%
GMFCS 5 MORE THAN 80%
Hip at risk managed surgically if MI >25 ,increased
valgus ,excessive anteversion and shallow acetabulum.
Adductor tightness carefully looked for as it is progressive.
If MI MORE THAN 50 HIGH IN GMFCS 5 this requires bone and
soft tissue surgery.
Often younger child tend to have recurrence even if they have
proper care after surgery.
Pelvic osteotomy: sometime only VDRO is needed and
sometimes dega osteotomy
Dega leads to decrease in acetabular volume.
Knees: exam is critical
Popliteal angle is very important
Hip/knee flexion contractures:
5. Big knee flexion contracture DFEO w/pt
advancement as only hamstring
lengthening will not be helpful.
Intra op coorection to max 30 degree
pop angle
Becareful from over lengthening .
Stiff rectus : some of patient can’t
clear foot can’t bend knees from sit to
stand
Rectus transfer and release .
Ankle/feet: important to know what to
lengthen with the aid of silverskiiold test
Equinus : strayer vs TAL
KEEP IN MIND RECURRENCE IS VERY HIGH IN CHILD LESS
THAN 8 YEARS 70%
Equinovarus: most coomon in hemiplegic
Also seen in high GMFCS QUADS most often children remain
quite flexible and typically treated with orthotics
Valgus hindfoot: if no pain can brace stretch and adaptive shoe
wear.
If they need surgery:
soft tissue proceeders will not benefit
Lateral column lengthening (evans/mosca)
Address any malrotation and bunion they tend to be dorsal and
painful.
Gait:
True vs apperant equinus :
▪ SEMLS surgery (Single-Event, Multi-Level Surgery)
▪ concept arose to limit multiple surgeries, anesthetics,
and rehabilitation time for children
6. ▪ most successful when combined with a thorough gait
lab assessment that predicts improvemenet in function
with multiple level surgical interventions
▪ simple lengthenings can cause deterioration in gait
when other contractures are "uncovered"; SEMLS
management seeks to avoid these iatrogenic
complications
▪ can be done on bilateral lower extremities in efforts to
improve gait
Upper extremity issues: typically internal roation of the
shoulder,forearm pronation ,wrist flex/ulnar deviation thumb in
palm.
Best candidates for ue surgery:
• Spastic
• Reasonable intelligence/motivation
• Stable trunk
• Good hand propirioception
• No fixed contravtures
• Resonable hand function(placement and control).
Casting , passive night stretch ,ot for mild contractures
Transfere release depending on specific patteren.
7. Spine issue
occurs mostly in quads :
- Often very large hyperlordosis
2nd to hip flexion contractures
Compensatory below rigid thoracic hyperkyphosis
- Hyperkyphosis
Young child with poor spinal extension
Over several levels
almost always flexible
Any degree of pelvic obliquity should be addressed as this may
be unresponsive to bracing and worsen with age.
- Scoliosis 75% of quads
Young age poor sitting balance pelvic obliquity increase risk
Curve more than 40
Unresponsive to bracing
Surgery if curve becomes inflexible or sitting balance tolerance
decrease.
Stroke :
patients who have had a stroke may cont to improve upto 6
months after their vascular event;
- patients with traumatic brain injury may improve upto 18 mo
after injury
to walk independently, the hemiplegic requires:
- adequate balance to stand independently
- hip flexion to advance the limb
- normal strength of uninvolved side
- normal proprioception
- if the patient does not have these requirements, walking
may not be a realistic goals
Orthotic aims in stroke differ dramatically according to the time
from onset.
8. In early stroke, orthoses may be used to challenge stability,
optimise alignment and be used alongside physiotherapy to
promote best recovery; in the chronic stages after stroke, they are
more likely to be used to provide stability to maximise function.
Intensive home stretching and physiotherapy are required, often
with muscle relaxants and phenol treatment in some patients.
Surgical treatment is frequently needed. The indications for
surgery are:
1. Deterioration or uncontrollable spastic posture
2. Fixed deformity that interferes with function
3. Secondary complications such as bony deformities,
dislocation of the hip and joint instability.
Surgical options are limited and mainly consist of releasing tight
muscles or lengthening tendons, augmenting weak muscles by
tendon transfers and correcting of fixed deformities by osteotomy,
arthrodesis.
Treatment of principal limb deformities in stroke
Deformi
ty
Orthotic
intervention Surgery
Foot Flaccid
equinus
Spring‐loaded
dorsiflexion
Lengthen tendo Achillis
Foot Spastic
equinova
rus
Limiting orthosis to
eversion and
dorsiflexion
Lengthen tendo Achillis and transfer
lateral half of tibialis anterior to
cuboid
Knee Flexion Knee–ankle–foot
orthosis
Hamstring release
Hip Adducti
on
Seating adaptation Obturator neurectomy or adductor
muscle release
Shoul
der
Adducti
on
Positioning pillow Subscapularis release
9. The muscular dystrophies :
These are a group of hereditary disorders of the skeletal muscle
that produce progressive degeneration of the skeletal muscle and
associated weakness.
The X linked dystrophies such as Duchenne's( caused by absent
dystrophin protein )and Becker's muscular dystrophy(dystrophin
protein is decreased instead of absent).
(DMD and BMD) are the most common dystrophies encountered
in clinical.
The major goal of early treatment is to maintain functional
ambulation as long as possible.
Orthopedic treatment :
Patients with DMD are susceptible to the development of
contractures and scoliosis.
Stretching exercises and nightly bracing can help to prevent the
contractures from becoming severe.
At the age of 8–15 years, children with DMD have a sensation of
locking of the knees.
It is easier to keep children with DMD walking than to induce
walking once they have stopped, therefore, contractures should
be treated early to maintain ambulation, because, if it is
Elbow Flexion Night orthosis Release elbow flexors
Wrist Flexion Wrist orthosis Lengthen or release wrist flexors
Finger
s
Flexion Night orthosis Lengthen or release flexors
10. maintained even for 1–2 years, it considerably benefits these
children and delays the inevitable development of scoliosis and
kyphosis.
Three approaches are usually used to correct lower extremity
contractures:
1. Ambulatory approach (early lower limb surgery): The goal
here is to correct any contractures while the patient is still
ambulatory. This is often needed early, between 4 and 6
years at the initial appearance of contractures, at the plateau
of muscle strength or when there is difficulty maintaining an
upright posture with the feet together. a three‐step approach to
correct early contractures, consisting of bilateral tenotomy of
the superficial hip flexors, aponeurectomy of the iliotibial band,
and subcutaneous tenotomies of the knee and foot.
2. Rehabilitative approach: Surgery is performed at or just
after the patient has lost the ability to walk, but with the
intention that walking will resume. Walking and standing can
be extended for a few years by the use of long leg orthoses in
patients with DMD who reach the critical phase of loss of
independent walking ability. Before the use of most orthoses,
surgery is required to release the Achilles tendon alone or with
superficial hip and medial knee flexors.
3. Palliative approach: This is used for treating contractures that
interfere with shoe wear and comfortable wheelchair
positioning. Even when a patient is too weak to walk, he
should continue to stand using a standing frame or a stand‐up
wheelchair.
• physical therapy for range of motion exercises
• adaptive equipment
• power wheelchairs
11. • KAFO bracing.
Scoliosis :
neurogenic curve
Usually by the age of 12–13 years, most children with DMD can
no longer walk, and the progressive spinal deformity becomes the
major problem, affecting sitting and causing breathing problems.
12. Three patterns of spinal deformity :
begins with mild hyperlordosis
progresses with general kyphosis and scoliosis with varying
degrees of pelvic obliquity
Conservative treatment is not effective. Bracing and wheelchair
support may slow the progression but spinal fusion is ultimately
required.
progresses 1° to 2° per month starting at age 8 to 10 years.
curve progresses rapidly from age 13 to 14 years.
Treatment
nonoperative
• bracing is contraindicated may interfere with respiration
•
operative :
early PSF with instrumentation
• indications : curve 20-30° in nonambulatory patient
treat early before pulmonary function declines
can wait longer ~ 40° if responding well to
corticosteroids
• FVC drops ≤ 35%
• rapidly progressive curve
PSF with instrumentation to pelvis
• indications
curves ≥ 40°
pelvic obliquity ≥ 10°
lumbar curve where apex is lower than L1
anterior and posterior spinal fusion
• rarely for stiff curves
13. Peripheral neuropathy:
Disorders of the peripheral nerves may affect motor, sensory and
autonomic nerves.
Diabetic neuropathy is the most common cause of neuropathy
The most common management problem is the diabetic foot,
where treatment is aimed at skin care, and maintaining tissue
integrity.
Orthopaedic management of this group includes the treatment
and prevention of ulcers and/or infection and the management of
neuropathic joints.
Ulcers in neuropathic feet develop because of associated
ischaemia, abnormal loading/shear (due to foot deformity or tight
Achilles tendons), inappropriate shoe wear or trauma.
Surgery may include lengthening of the Achilles tendon to reduce
forefoot loading, debridement of ulcers, total contact casting of the
foot to equalize pressure distribution, removal of necrotic bone
and revascularisation procedures.
Nonoperative :
total contact casting,
shoewear modifications,
first line of treatment
• contact casting
•
• casts changed every 2-4
weeks for 2-4 months
orthotics
• Charcot restraint orthotic
walker (CROW) boot can
be used after contact
casting
shoe modifications
14. • double rocker shoe modifications will best reduce risk for
ulceration at the plantar apex of the
deformity
medications
• bisphosphonates
• neuropathic pain medications
• antidepressants
• topical anesthetics
outcomes 75% success rate
Operative :
resection of bony prominences
(exostectomy) and TAL
• indications "braceable" foot with equinus
deformity and focal bony prominences
causing skin breakdown
• goal is to achieve plantigrade foot that allows ambulation
without skin compromise
deformity correction, arthrodesis +/- osteotomies
• indications
• severe deformity that is not "braceable"
• very high complication rate (up to 70%)
amputations
• indications
• failed previous surgery (unstable arthrodesis)
• recurrent infection
• goal is for a partial or limited amputation if vascularity allows.
◦ partial calcanectomy +/- Achillis tendon lenghtening
▪ indications
▪ large heel ulcers with associated
calcaneal osteomyelitis
▪ outcomes
▪ preserves limb length and decreases morbidity
compared to higher level amputations
◦ Syme amputation
15. ▪ indications
▪ forefoot gangrene and a palpable posterior tibial
artery pulse
◦ Keller resection arthroplasty
▪ indications
▪ IPJ plantar neuropathic ulcer with hypomobile/stiff
MTPJ that has failed total contact casting
Poliomyelitis:
Poliomyelitis is caused by a viral infection of the anterior horn
cells of the spinal cord, which may lead to permanent paralysis of
isolated groups of muscles and death.
Postpolio syndrome
• an aging phenomenon where more nerve cells become
inactive with time it does not represent reactivation of the
virus
• occurs after middle age occurs in up to 50% of polio cases
• patients should exercise at sub-exhaustion levels to tone
affected muscle groups without causing muscle breakdown
hallmark is motor weakness with normal sensation.
Treatment :-
Post-polio Syndrome
• nonoperative
• limited exercise with periods of rest, +/- lightweight orthosis
indications
first line of treatment to maintain but not
overuse muscles
• operative
• tendon transfers, contracture releases, and arthrodesis
• indications used to optimize functional capacity
Polio foot deformities
• nonoperative lightweight orthoses
16. first line of treatment
help patient remain functionally independent
• operative
• contracture release, tendon transfer, and arthrodesis
• indication
• if orthoses do not achieve satisfactory standard of living
Charcot-Marie-Tooth Disease :
Description
◦ Charcot-Marie-Tooth Disease, also known as peroneal
muscular atrophy, is a hereditary motor sensory neuropathy
(HMSN) that results in muscles weakness and sensory
changes
▪ muscle weakness leads to cavovarus foot, scoliosis,
and other orthopaedic conditions
most common inherited progressive peripheral neuropathy
• Pathophysiology
◦ Type I
▪ abnormal myelin sheath protein is the basis of this
degenerative neuropathy.
▪ results in a combination of motor and sensory
disturbances.
◦ Type 2
▪ intact myelin sheath with wallerian axonal
degeneration that results in mild sensory and
motor conduction velocities.
◦ pathoanatomy
▪ affected muscles become weak
▪ peroneus brevis
▪ peroneal involvement is typically first
and most profound
▪ results in muscle imbalance and varus
deformity
17. ▪ tibialis anterior
▪ weakness results in dropfoot
▪ intrinsic muscles of hand and foot
▪ wasting of 1st dorsal interosseous
autosomal dominant
PMP 22 may also be
▪ autosomal recessive
▪ X-linked
• Orthopedic manifestations
◦ pes cavovarus
◦ hammer toes
◦ hip dysplasia
◦ scoliosis
◦ hand muscle atrophy and weakness
it’s always important to check hands
you want to get an AP pelvis x-ray very high risk dysplasia
especially the type ones
and also to pay attention to their spines as they do have a high
rate of scoliosis
Coleman block test this is important to assess hindfoot flexibility it
is critical if you’re planning a surgical procedure they tend to get a
plantar flexed first ray
18. trying to assess is whether or not the deformity is forefoot driven
coming from the first ray or whether it’s really hindfoot driven
coming from the calcaneous and these are that tend to be fixed in
Varus with coleman block test
many of these patients treated early with a full light semi rigid
insole it’s important to depress the first right and to add a lateral
wedge
Surgery : soft tissue vs bony !
tenden transfers alone which can leads to high failure rate
Plantar fascial release is critical for all of these procedures you
might consider peroneous longus to brevis transfere as well as
transferring PTT to the dorsum of the foot if there is sever tip
weakness
Jones procedure comes is for clients and involves transferring
EHL to 1st MT and lesser extensors to MT neck
first metatarsal dorsiflexion osteotomy is incredibly powerful tools
at a flexible Varus And fixes calcaneus if there is fixed varus in the
heel