Pediatric_and_Neuromuscular

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Pediatric and
Neuromuscular
Orthopedics
Objectives
• 11%-self-care-comfort -avoid
complications
• Identify signs and symptoms in selected
pediatric and neuromuscular disorders.
• Outline nursing interventions for medical
and surgical protocols.
• List strategies to maximize function in
patients and families with neuromuscular
disorders
Development
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•
•
•
•
Infancy 0-18 months
Toddler 18 mos – 3 yrs
Pre-school 3-5 yrs
School age 6-12 yrs
Adolescence 13 –19
Pediatric Differences
• Visualization
– ossification
– ultrasound
– X-ray
•
•
•
•
Bone
Thick periosteum
Metaphyseal growth
Re-modeling
Nursing Intervention
• Nursing see Table 11-1 from Core
Curriculum 4th ed.
• Child’s developmental level
• Parent and child’s ability to learn
• Amount disorder interferes with:
– ADLs
– Growth
– Learning Ability
– Social Adjustment
Nursing Intervention
Parents
• Realistic expectations
• Understanding of the disease/disorder
• Follow-up with treatment
• Response to public inquiry
Nursing Intervention
• Team approach
– Include child in decisions
• Play
– Independence, socialization,
– Creativity, problem solving
Upper Extremity Disorders
• Congenital anomalies
• Syndactyly webbing 2+
digits
• Polydactyly extra digits
• Congenital amputations
/deficiencies
• Brachial plexus injuries
Lower Extremity Disorders
• DDH
• Legg-Calvé-Perthes
• Intoeing, torsional
problems
• SCFE (presentations)
• Limb length inequality
• Osteomyelitis, septic
joint
• Trauma
Developmental Dislocation of the Hip
•
•
•
•
DDH
F>M 1:500-1000 births
Breech, 1st born
Family history
Developmental Dislocation of the Hip
• Physical Exam - hip clicks
and clunks
• Barlow’s – push hip out back
• Ortolani’s abduct click in
• Decreased abduct
• Unequal skin folds
• Ultrasound - X-ray
DDH Rx
• Pavlik, abd. splint
• Traction Bryant’s/
mod Bryant's
• Surgical reduction
closed vs open
• Spica cast
DDH Nursing Care
• Traction
• Neurovascular Exam
– Amputation 2° necrosis
• Cast syndrome
• Can be fatal
Talipes Equinovarus
• “Clubbed Foot” 1.24:1000
births
• M 2x > F
• Inversion adduction and
equinus of the forefoot
• Fixed rigid deformity
• Etiology: controversial
• Uterine position
• Idiopathic
Talipes Equinovarus
• Dx: clinical
apparent
• Calf smaller,
Achilles short
• Rx:Stretching
• Casting
• Surgical releases
Talipes Equinovarus
• Nursing, watch toes in
cast
• Foot will be smaller!
• Rocker bottom from
push up on metatarsals
• Can play sports
Intoeing
• Most common complaint!
• Metatarsus adductus
(foot deformity)
• Tibial torsion
• Femoral anteversion
Metatarsus Adductus
• Foot deformity
– 1:1000 births
– Dx: exam
– Rx: stretching
• long leg molded
cast
• surgery
Tibial Torsion
• Physiologic bowing of tibia
•
2º intrauterine position
• NORMAL for toddler
• No treatment unless present
at 8 years of age
• Nursing - bring
grandparent to
appointment
Femoral Anteversion
• Curvature of the
femur
• After 3 yrs resolves
by 8yrs
• Brace not effective
• Osteotomy if
functional problems
after 8 yrs
Va
us
cubitus
valgus
cubitus
varus
coxa
vara
heel
varus
genu
varum
metatarsus
varus
coxa
valga
heel
valgus
genu
valgum
hallux
valgus
Genu Valgus
• Knock knees
• Joint laxity -medial collateral ligs.
• Common: 3 - 5 years
• improves by 7 years
• normal angulation
• girls: 7 - 9° valgus
• boys: 4 - 6° valgus
Genu Varus
• ^ dist between knees >2.5
cm
• W/ ankles together
• external torsion of femur
• improves spontaneously
by 18 - 24 mos
 in early amb & heavy
children
Blount’s Disease
• X-ray changes medial
prox tibia
• 50% unilat. tibial bowing
• ^Black, ^obese,
• Rx: no Δ 6 mos bracing
• Surgical >30 mos old w/o
Rx or structural /
functional deform
Blount’s Disease
• Rx:
• Corrective prox.
osteotomy.
• Hemiepiphysiodesis
• External fixator
– Some with gradual
correction
Osteogenesis Imperfecta “OI”
• Brittle bone disease
fracture with minimal
stress
• Multiple types some fatal
• Etiology: autosomal
dominant vs. recessive
• Defect collagen synthesis
• 1:20,000 births
Osteogenesis Imperfecta “OI”
• Dx: Clinical deformities
• Blue sclera, shepherds crook
deformity
• Dentinogenesis, deafness
• Radiographs
• Biopsy
• Bone density
Osteogenesis Imperfecta “OI”
• Rx brief Light wt. immob
• I-M rods “Bailey” grows
with child
• Pamidronate treatment
• Pain meds.
• “Brittle baby” NO BP’s
signs Don’t pull limbs,
• Physical NOT mental
handicap
• Encourage independence
Legg Calvé Perthes
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•
•
•
Aseptic necrosis femoral head
Idiopathic vs heredity
M 4x >F 1:2000 births
Age 3-12 years
Legg Calvé Perthes
• Sx: Knee, hip,
thigh pain
• Limp
• Decreased ROM
• Dx: exam / X-ray
Slipped Capital Femoral Epiphysis
•
•
•
•
•
•
Endocrine/ obesity/ growth /
trauma
F 8-15yrs. M 10-17yrs
1 : 10,000 adolescents
Sx: Hip, knee, pain (long duration)
Limp, decreased ROM
Dx: Hallmark sign- lack of internal
rotation
X-ray -“Ice cream falling off the
cone
Slipped Capital Femoral Epiphysis
•
•
•
•
Rx: bedrest,
N W B, crutches
Surgical pinning
Teach S&S for other
hip - >50%
Limb Length Inequality
• Congenital vs acquired
• Sx: short limb, limp,
back pain
• Dx: X-ray, CT, bone
age
Limb Length Inequality Rx:
• < 2cm no treatment
• 2-6 cm lifts
epiphysiodesis
• >6 - 15cm shorten
opposite limb
• Limb lengthening,
external fixation
Limb Length Inequality
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•
•
•
Nursing
Pain management
Pin care
Compartment
syndrome
• Emotional support
Osgood-Schlatter’s Disease
• F 8-13 yrs M 1115 yrs
• Sx: painful
swelling tibial
tubercle
• cosmetic
deformity
Osgood-Schlatter’s Disease
• Dx: exam
• X-ray traction
apophysitis
• Rx: rest, ice,
stretch
hamstrings
• Casting
Trauma
• Leading cause of death in children
• Common injuries (see table 11-3
)
• Child abuse – Battered Child Syndrome
Trauma: Child Abuse
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•
•
•
3.14 million children reported abused
Physical abuse
Greatest < 3 years (66-78%)
30% under 6 mos
Salter-Harris Classification
1
2
3
4
5
Child Abuse - Fractures
• Most common
presentation!
• >30% under 3 mos.
• 70% under 6 mos.(1yr)
are inflicted
Common Fractures Inflicted
• Metaphyseal
• Rib Fxs seen in 5-20%
of abused
• Scapular/distal clavicle/
night stick
• Vertebral fx. or
subluxation
Common Fractures Inflicted
• Finger Fx. In nonambulating child
• Humerus Fx. (X
supracondylar) < 3 Yrs.
• Bilateral, multiple, or Fxs
in different stages of
healing
• Complex skull Fx
Fracture treatment
Questions
Linsey, 5 months old presents with knee
swelling which of the following would
prompt you to file for suspected child
abuse:
A. Septic joint with staph on aspiration
B. Mongolian spots about the lower back
C. Metaphyseal fractures of the distal femur
D. Parent are obviously worried and seem
really nice.
Questions
Three year old Stephanie has a history on limping
and refusing to put weight on her left leg. She is
lying with her leg externally rotated. You are
admitting her for:
A. Traction to treat her Perthes disease and high
fever.
B. Observation for bracing of her septic hip.
C. fever, high white count Perthes treatment.
D. Severe malaise, fever, pre-op for septic hip
aspiration.
Questions
An 18 month old infant is admitted to your
unit with a fractured femur. You notice
blue sclera and shepherd crook
deformities of his arms. You would:
A. Hang “Brittle Baby” sign on his crib
B. Change his diapers lifting him by the feet
C. Keep his medication strictly by weight
even if ineffective to prevent overdosing.
D. Use real plasters for casting.
Achondroplasia
• Most common dwarfing syndrome
• 1 of every 26,000 live births M>F
• Etiology: abnormal endochondral
ossification
• genetic defect autosomal dominant
– 90% spontaneous mutations
Achondroplasia
• Hypotonia resulting in slow motor development
• C-spine injuries secondary to poor head control
• Low back pain 2° to spinal stenosis
• Thoracolumbar kyphosis hyperlordosis
• longitudinal growth most affected
• difficulty performing ADL’s
Scoliosis
• Lateral curvature of the
spine
• Congenital
• Paralytic
• Neuromuscular
• Idiopathic = most common
Idiopathic Scoliosis
•
•
•
•
M=F, Rx 10x more
3% teens screened
Rx - <20° none
20-40° brace - to
prevent further
curvature
• >40 PSF sequential
fixation w/graft
Questions
Your neighbor says her 13 year old daughter has
scoliosis and the brace is not making it better
she might need surgery. You know:
A. She would improve the curve if she wore the
brace.
B. Her curve must be close to 40°,and bracing
dose not make it better.
C. Surgery should be performed to make her back
completely straight.
D. She will have osteoporosis if it is not fixed
immediately.
Neuromuscular Disorders
• Nursing see Table 2 Core Curriculum 3rd ed.
• Level of knowledge of the disease
– Realistic expectations
– Activities with-in patient limits
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•
•
•
Psychological & functional levels
Support systems
Bowel/bladder function
Skin, Nutrition, Immobility
Neuromuscular Disorders
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•
•
•
•
Fatigue
Communication
Intellectual stimulation
Dressing
Emotional needs
Neuromuscular Disorders
• Non Progressive
– Cerebral Palsy
– Myelodysplasia- Spina Bifida
– Arthrogryposis
Cerebral Palsy - CP
• Motor disorder following anoxia to
cerebral cortex
• Single largest disability in
children
• 1-5:1,000
• Time of occurrence
– Prenatal 80%
– Perinatal 5%
– Postnatal 15%
Cerebral Palsy- CP
• Dx:
• Spastic quadriplegia –
cortical motor tract
• Athetotic –
extrapyramidal tract
• Ataxic – cerebellum
/balance disturb
• Rigid - mixed
Cerebral Palsy- CP
• Nursing
• Parental education non
progressive
• Mainstream as much as
possible
• Grieve loss of typical
child
Myelodysplasia - Spina Bifida
• Group of malformations of spine/post. laminae of
vertebra fail to close
• Occulta 20-25% all births
– some with no symptoms
• Cystica - Sac
– Meningocele – meningeal sac
– Myelomeningocele – spinal cord too!
• Has neuro deficits distal to lesion
• Hydrocephalus 90%
Myelodysplasia - Spina Bifida
• F > M 1: 1,000 births
• Etiology: genetic,  folic
acid, valproic acid
• Dx: clinical exam, X-ray,
MRI
• Rx: neuro, ortho, urologic
• Wide range of symptoms
Myelodysplasia- Spina Bifida
• Nursing – multi-disciplinary
team
• Promote mobility, ADLs, diet,
• Bowel bladder program
• Latex precautions
• Neuro status – shunt problems
• Insensate skin, water temp,
braces
• Multiple ortho corrections
Arthrogryposis
• Non-progressive
disorder with multiple
congenitally rigid joints
• Etiology: unknown
0.03% general
population
• Dx: clinical exam X-ray,
muscle bx.
Arthrogryposis
• Rx: Aggressive PT
at birth
• Casting, bracing,
surgical releases
• Normal intelligence
Progressive
– Muscular Dystrophy
– Neurofibromatosis
– Peroneal Muscular Atrophy
– Friedrich’s Ataxia
– Polio
Muscular Dystrophy - Myopathy
• Progressive hereditary degenerative
weakness of skeletal muscles
• 2-3 of 10,000 males
• DX: Muscle Bx. ^ CPK abnormal EMG
• Gower’s sign –climb up legs w/ hands
• Many types Duchenne’s most common &
severe < 20 yr life span
• Becker’s seen at 7yrs < 40 yr life span
Muscular Dystrophy
• Nursing
• Depression – frustration guilt
• Respiratory - cardiac
complications
• Malignant hyperthermia
Neurofibromatosis
• Von Recklinghausen’s disease
• Progressive
• Tumors in central and
peripheral nervous system
• 1:400,000 births M=F , 4
Stages
• Dx: café au lait spots >6
• Lisch nodules in iris
Neurofibromatosis
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•
•
•
Instigated by puberty
Spinal deformities 10-15%
Scoliosis, pseudoarthrosis
Most common predisposition to
cancer for children
• Elephantitis
• Nursing - genetic counseling
• Multi system effected
(See table 13 Core Curriculum)
Questions
The single largest cause of disability in children is
A. Neurofibromatosis
B. Myelodysplasia
C. Arthrogryposis
D. Cerebral Palsy
Questions
John is 11 year old pubertal male. He is being
seen today for scoliosis screening you notice
has 6 large light brown spots on his trunk. You
worry that he is in stage
A. 4 of neurofibromatosis
B. 2 of myasthenia gravis
C. 2 of Von Recklinghausen’s disease
D. 5 of osteogenesis imperfecta
Peroneal Muscular Atrophy
• Charcot-Marie-Tooth – hereditary atrophy
of peripheral and motor nerve roots & freq
spinal cord
• Etiol: unknown 2-5 100,000 people M>F
• Dx: decrease sensation / function
• Rx: orthotics – surgical releases
• Genetic counseling
Friedrich’s Ataxia
• Hereditary degeneration of the spinal cord
and cerebellum
• M=F presents 5-20 yrs.
• Dx: unsteady gait (ataxia) 1st symptom
• Aggravated by puberty <40 yr life span
• Fibromyopathic changes heart muscle
Polio
• Acute infection of nervous system by polio
virus
• Incidence rising due to immunization
refusal by parents
• Sx: fever malaise, muscle pain, paralysis,
some recover 4 mos-2yrs
• Rx: symptomatic post polio 20yrs
• Immunizations
Thank-you!
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