1229 Exam 8: Osteogenesis

Question 1

What is Osteogenesis Imperfecta (OI)?

Answer 1

most common osteoporosis syndrome in children, characterized by excessive fractures and bone deformity
Deficit of type 1 collagen due to faulty bone mineralization, abnormal bone architecture, and increased susceptibility to fracture
Also referred to as Perthes Disease, Legg-Perthes Disease, Osteochondritis Deformans Juveniles, and Coax Plana

Question 2

What are the signs and symptoms of osteogenesis imperfecta?

Answer 2

vary with the types
varying degrees of bone fragility, deformity, fracture
Blue sclera, hearing loss, and dentogenesis imperfect

Question 3

What is the cause of osteogenesis imperfecta?

Answer 3

severe form autosomal recessive inheritance

most common form autosomal dominant

Question 4

How do you treat osteogenesis imperfect?

Answer 4

supportive and palliative
Biophosphonate therapy with Pamidronate, Olpadronate, Neridronate, or Alendronate to promote increase bone density and prevent fracture
Prevent contractures and deformities
Prevent muscle weakness and osteoporosis
Prevent misalignment of lower extremities joints, prohibiting weight bearing
Lightweight braces and splint help support limbs, prevent fractures, promote ambulation
PT prevents disuse osteoporosis and strengthens muscles
May need surgery to correct deformities that interfere with bracing, standing, or walking
Recurrent fractures inserting intramedullary rod to provide bone stability

Question 5

Nursing Management of osteogenesis imperfect

Answer 5

Careful handling to avoid fractures
Supported when being turned, positioned, moved and helped
Hand under neck, shoulder, arms, and other hand under buttocks and legs
Log roll when moving
Avoid lifting under armpits
Avoid lifting by legs during diaper changes

Question 6

How can I teach my patient or patient parents about osteogenesis imperfect?

Answer 6

Teach parents about the child’s limitations

Teach parents about suitable activities that promote development and that will protect child from harm

Question 7

What is legg-calve-perthes-disease?

Answer 7

Self limiting disorder in a child, aseptic necrosis of the femoral head

Question 8

What are the risk factors of legg-calve-perthes-disease?

Answer 8

Affects most children between 4-8 yrs old
Mostly affects boys
Caucasian child more affected than African American

Question 9

What is the patho of legg-calve-perthes-disease?

Answer 9

cause unknown but there is a disturbance of circulation to the femoral head that produced ischemic aseptic necrosis of the femoral head that occurs in 4 stages

Question 10

Stage 1 of legg-calve-perthes-disease

Answer 10

Initial or avascular, aseptic necrosis or infarction of the head with degenerative changed producing flattening of the upper surface

Question 11

Stage 2 of legg-calve-perthes-disease

Answer 11

Fragmentation or Revascularization, capital bone reabsorption w/ revascularization gives it a mottled appearance

Question 12

Stage 3 of legg-calve-perthes-disease

Answer 12

New bone formation seen on x-ray as calcification or increased density

Question 13

Stage 4 of legg-calve-perthes-disease

Answer 13

Residual or regenerative, gradual reformation of the head of the femoral hopefully into a sphere

Question 14

What are the s/s of legg-calve-perthes-disease?

Answer 14

onset is usually insidious

hip soreness, stiffness that is constant or intermittent, joint pain, limp

Question 15

How is legg-calve-perthes-disease diagnosed?

Answer 15

x-rays, definitive dx is with MRI= osteonecrosis

Question 16

How is legg-calve-perthes-disease treated?

Answer 16

Is to eliminate hip irritability restore and maintain adequate hip ROM, prevent collapse, extrusion, and subluxation, ensure well rounded femoral head at the end of healing
Varies according to child age and appearance of the femoral head vasculature and position in the acetabulum
Containment in acetabulum can be achieved by abduction casts
Pelvic or femoral osteotomy-used to contain femoral head
Rest and non weight bearing- prevent synovitis, stiffness, adductor contracture
Abduction brace- Atlanta Scottish Rite Orthosis- leg cast
Leather harness sling- non weight bearing on affected limb
Must be continued for 2-4 years
Surgical correction returns child to normal activity in 3-4 months

Question 17

What is the prognosis of legg-calve-perthes-disease?

Answer 17

later the dx more damage to the femur before tx is implemented
Child under 5 yrs old has best outlook
Children over have significant r/o degenerative arthritis

Question 18

What can I teach my patient and parents about legg-calve-perthes-disease?

Answer 18

Teach purpose, function, application, and care of corrective devices for the child
Teach suitable activities to keep to the child and meet developmental needs-sense of initiative or industry
Teach activities that meet the creative urges