Module 11: MSK Abnormalities In Children (c)

Question 1

Developmental Dysplasia Of the Hip DDH

-Info

Answer 1

Femoral head subluxation and/or acetabular dysplasia and/or complete dislocation of femoral head from acetabulum

1. Risk Factors - Fam hx, female, oligohydramnios, first pregnancy, breech presentation
2. Associated findings
   - Metatarsus adductus (CLUB FOOT) TEST
   - Congenital torticollis — infant head turned to the side
3. BEGIN Tx EARLY for better results
   - Use Pavlik harness in children younger than 4 months
   - Up to 12 months of age — closed reduction followed by spica or body cast x3 months
   - After 12 months — Surgical intervention — Cut and realign the femur

Question 2

Osteogenesis Imperfecta

-Info

Answer 2

1. “Brittle bone” disease
2. Collagen related bone dysplasia — Defect in either bone production or collagen production or both
3. There are more than 12 types of OI that are a result of a genetic anomaly
   —SERUM ALKALINE PHOSPHATASE level is elevated in ALL forms of disease

Question 3

Osteogenesis Imperfecta

-Clinical Manifestation

Answer 3

1. Osteoporosis & bone fractures more easily
2. Sclera will be BLUE at birth & whiten w/ age
3. Respiratory insufficiency from pulmonary hipoplasia

Question 4

Osteogenesis Imperfecta

-Triangular Facies **TEST

Answer 4

1. Infant will have a broad forehead & Deeply set eyes
2. Pointed chin
3. Straight nose with a bulbous tip

Question 5

Scoliosis

-Patho/Causes

Answer 5

Scoliosis —Rotational curvature of the spine

1. Non-structural — Curvature from a cause other than the spine
2. Structural — Curvature associated w/ vertebral rotation
3. Idiopathic — No known cause — 70% of cases

Question 6

Scoliosis

-Clinical Manifestations

Answer 6

1. Curve in spine
2. Asymmetry of hip height
3. Asymmetry of shoulder height
4. Scapular prominence or flaring
5. Rib prominence or rib flaring as well

Treatment
-Bracing will prevent progression but does not correct — Start brace when curvature reaches 20%.

Question 7

Juvenile Idiopathic Arthritis JIA

- Info/ Patho

Answer 7

1. Patho is the same as adult form of Rheumatoid Arthritis
2. Difference is mode of onset
   -Oligoarthritis — onset in fewer than 5 joints
   -Polyarthritis — onset in more than 5 joints
   -Systemic onset juvenile idiopathic arthritis — Formerly known as STILLS disease
   —Systemic onset includes high fevers, rheumatic rash, hepatosplenomegaly, & lymphadenopathy

Question 8

Juvenile Idiopathic Arthritis JIA

- JIA Vs RA clinical Manifestations

Answer 8

1. Large joints are affected INSTEAD of small joints in RA
2. Spinal changes include subluxation and ankylosis of cervical spine INSTEAD of synovial joints in adults
3. Joint pain is NOT severe
4. Antinuclear antibody (ANA) test is positive — Much more likely seen in JIA
5. Chronic Uveitis
6. RF is seldom detected
7. Subcutaneous rheumatoid nodules — common in heart, lungs, eyes, and other organs

Question 9

Osteochondrosis

-2 Types

Answer 9

1. Leg-Calve-Perthes & Osgood-Schlatter are types of Osteochondrosis
2. BOTH are avascular diseases of the bone

Question 10

Osteochondrosis

-Legg-Calve-Perthes Disease

Answer 10

1. Interrupted blood supply to the femoral head — self-limiting disease
2. Deformation d/t ischemia is PERMANENT
3. Usually in the young — 3-10 yrs of age — May or may not have pain
4. Treatment Goal
   - Keep ball in the socket
   - During active phase of Dz, avoid activities that apply stress to the hip
   - Don’t go to trampoline park during active phase

Question 11

Osteochondrosis

-Osgood-Schlatter Dz

Answer 11

1. Repeated trauma that causes tendinitis of the anterior patellar tendon and osteochondrosis of the tubercle of the tibia (Tubercle is located below the kneecap where patellar tendon inserts
2. Usually occurs in young athletes, MROE COMMON in boys

Manifestations
- Pain and swelling that are prominent and tender to direct pressure, especially s/p physical activity

Treatment
-Limit sports for 8 wks then reduce activity for another 8 wks

Question 12

Duchenne Muscular Dystrophy

-Info/Patho

Answer 12

1. X-linked recessive inheritance — MALES ONLY
2. Disease gene codes for protein dystrophin — in dz, dystrophin is absent
   - Dystrophin mediates the anchorage of the actin cytoskeleton of the skeletal muscle fiber to the basement membrane
3. If dystrophin is absent, the poorly anchored fibers will tear apart under repeated stress of contraction — When that occurs, free CALCIUM will enter the muscle cells and cause cell death and fiber necrosis

Question 13

Duchenne Muscular Dystrophy

-Clinical Manifestations

Answer 13

1. Clinical Manifestations appear at age 3
   - Slow motor development, progressive weakness, muscle wasting, delayed sitting and standing
   - WADDLING GAIT — muscular weakness begins in the pelvic girdle & it will cause the gait
   - GOWER SIGN — climbing up the legs when rising

Question 14

Ewing Sarcoma

-Info

Answer 14

1. Malignant round cell tumor of bone and soft tissue — Arises from bone marrow — can break through cortex to form soft tissue mass
2. Occurs in the DIAPHYSIS of the long bones or in flat bones —MOST COMMON sites:
   - Pelvis
   - Femur
   - Tibia
3. Most lethal malignant bone tumor that can occur during childhood **
   -Metastasis occurs early, and it’s usually apparent at diagnosis or w/in one year of diagnosis
   -Most common metastasis sites
   —Lungs, other bones, lymph nodes, bone marrow itself, liver & spleen — Can invade almost every organ

Question 15

Ewing Sarcoma

-Clinical Manifestations

Answer 15

1. Pain
2. Soft tissue mass — particularly at the original site at the DIAPHYSIS of the long bone or the flat bone
3. Fever
4. Malaise
5. Anorexia

Question 16

Cerebral Palsy

-Info

Answer 16

1. Non-progressive disorder of movement and posture caused by an ischemic insult to the brain
2. Pay attention to motor milestones that are not met
3. Treatment
   - NO CURE — multidisciplinary approach; surgery