pediatric and neuromuscular orthopedics
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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
• 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
cubitusvarus
coxavalgagenu
varum
heelvarus metatarsus
varus
cubitusvalgus
genuvalgum
halluxvalgus
heelvalgus
coxavara
Va us
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
• 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
• Nursing
• Pain management
• Pin care
• Compartment syndrome
• Emotional support
Osgood-Schlatter’s Disease
• F 8-13 yrs M 11-15 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
• 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 non-ambulating 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 aspirationB. Mongolian spots about the lower backC. Metaphyseal fractures of the distal femurD. 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 cribB. Change his diapers lifting him by the feetC. 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
• Psychological & functional levels• Support systems• Bowel/bladder function• Skin, Nutrition, Immobility
Neuromuscular Disorders
• 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
• 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 neurofibromatosisB. 2 of myasthenia gravisC. 2 of Von Recklinghausen’s diseaseD. 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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