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Osteogenesis Imperfecta, Slipped Capital Femoral Epiphysis,…
Osteogenesis Imperfecta
What is it
Brittle bone disease
11+ different types
May be mistaken for child abuse
Connective tissue disorder
Can be autosomal dominant or recessive
Decreases in severity at puberty
S/S
Multiple fractures
Blue sclera
Thin, soft skin
Enlarged anterior fontanel
Short stature
Increased joint flexibility
Weak muscles
Conductive hearing loss (adolescents)
DX
May be detected prior to delivery
Often detected after first fracture or with delayed walking
X-ray
DEXA scan
Tx
nutrition
Hematologic stem cell transplant
Supportive
Nursing
Normal intelligence
Involve parents
Manual BP
Support
Handel carefully
Educate on injury prevention and mobility optimization
Slipped Capital Femoral Epiphysis
Risks
Obesity
Medications (steroids)
Hypothyroidism
Pituitary Tumors
Down Syndrome
Symptoms
Pain/stiffness
Decreased ROM
Worse with activity
Acute v. Chronic
Dx
Mild
Moderate
Severe
Imaging (XR, US &/or MRI)
Treatment
Further slipping (hip deformity)
Avoid AVN of femoral head
Goal: dx early to prevent
Maintain adequate function of hip joint
Nursing
Monitor post op site
Observe for numbness
Enforce bedrest
Traction
Pain control
Prognosis
Degree of joint slippage
Duration of hip slip
Stability of joint
If symptoms continue
Osteoarthritis
Avascular necrosis of femoral head
Osteonecrosis of femoral head
Prevention
Treat/prevent obesity
Legg–Calvé–Perthes Disease
4 stages
Necrosis (asymptomatic)
Revascularization (pain, limited movement)
Bone healing re-ossification (decreasing pain)
Remodeling (no pain, regain joint movement)
What is it
Blood supply to head and neck of femur
Dx
Bone scan and MRI
Look for coagulation issue (cause of decreased blood supply to femoral head)
Therapy
Toronto or Scottish Rite brace, PT, traction
Anti-inflammatory meds (NSAIDS)
Outcome
Good outcome if caught early
Otherwise may develop osteoarthritis
Muscular dystrophy / Duchenne
Group of inherited diseases characterized by muscle fiber degeneration and muscle wasting
4 types including duchenne
What is it
Increased disability and deformity
Loss of strength
Progressive weakness (notice around 3 years old, gradual degeneration) (can't get up anymore)
Muscle enlargement due to fatty infiltration
X linked recessive trait
Weak lower muscles, use upper extremities
Dx
Elevated serum enzymes (CK, aldolase, AST) before clinicals signs appear
EMG (decreased amplitude)
DNA analysis of blood or muscle biopsy (fatty infiltration))
Treatment
Maintain functioning muscles as long as possible
Respiratory and cardiac problems eventually are focus of treatment
None
Nutritional support
Spinal muscle atrophy
What is it
Degenerative disorder of anterior horn cells of spinal cord
Most common recessive genetic disorder that is lethal in children
Results in...
Weakness
wasting of voluntary muscles
3 types
Treatment: supportive
Scoliosis
Dx
Scoliometer
X ray
Observation
Treatment
Surgery
For 40 degree or greater deviation
post op child is logrolled
Up with brace
Post Op complications
Urinary retention
Paralytic ileus
Pain
Braces
Two types
Kyphosis: curvature of the thoracic spine. No pain.
Lordosis: accentuated cervical or lumbar curvature. Pain.
What is it
Lateral S or C shaped curve of spine with a rotational deformity of the spine and ribs
10-13 years old
Curvatures >10 degrees is considered aabnormal
usually girls
Osteomyelitis
Dx
Increased WBC, ESR
May have positive bone and blood cultures
X-rays may be normal
most connon in
Boys
in femur or tibia
What is it
Infection of the bone - usually bacterial
Treatment
Antibiotics for 4 weeks +
Talipes equinovarus
Teaching cast care
Bathing, clothing
car seat use
What is it
Clubfoot
Treatment
Serial casting
After casting is done -> denis browne splint and reverse corrective shoes
Surgery?
Changed every 1-2 weeks
Developmental dysplasia of the hip
S/S
buttcheeks/ knees not even
Dx
Barlow and ortolani maneuvers
X ray or US
Alli's signs = one knee lower than the other knee with flexion
Treatment
Pavlik harness
Fractures
S/S
Swelling
Pain
Deformity (may or may not be visible)
Not using the extremity
Bruising
Crepitus
Kids
Rare in infancy
Xray showing different healing stages shows abuse
Heal faster
Fracture on epiphyseal places may affect bone growth
Treatment
Immobilization
Casting
Kids in casts
Joints above and below are immobilized
Swelling post application
Edges of cast need to be smooth
Avoid using finger tips when moving
Hot spots – make window for observation
Good hiding spot for food and toys
Cast care
24-48 hours to fully harden
Elevate above heart
Circle any areas of drainage
Keep dry
Distal pulse check/ capillary refill
Don’t place an object into the cast to scratch!
Compartment syndrome
Pain
Paresthesia
Pressure
Pallor
Paralysis
Pulselessness
Open reduction
Traction
Skin
Skeletal (into bones)
Nursing
Routine skin observation and care
Weights must be free hanging at all times
Ensure position
Color
Movement
Sensation
Splinting
External pins/fixators
Marfan syndrome
Associated abnormalities
Cardiac
Long arms and fingers
Scoliosis
Long face
High arched palate
What is it
Autosomal dominant condition of the connective tissue
Avg age of dx is 3 years old
Soft tissue injury
What is it
crush injuries
Sprains
Ligaments torn or stretched
Strain
Tear to tendon
Dislocations
Nursemaids elbow
Shoulder dislocations
Intervention
Ice (immediately, towel between skin, on and off for 24-36 hours)
Compression
Rest
Elevation