Musculoskeletal Congenital Disorders - PEDS

Define Clubfoot (Congenital Talipes Equinovarus) (2)

• Complex deformity of ankle & foot that can affect one or both feet

• Occurs as an isolated defect or in association with other disorders (CP & spina bifida)

Risk Factors - Clubfoot (2)

• Hereditary factors

• More common in boys

Expected Findings - Clubfoot (5)

• Talipes Varus - Inversion (foot bending inward).

• Talipes Valgus - Eversion (foot bending outward).

• Talipes Calcaneus - Dorsiflexion (toes higher than heels).

• Talipes Equinus (“horse foot”) - plantar flexion (toes lower than heels).

• Talipes Equinovarus - Toes facing inward & lower than heel.

Ponseti Method - Clubfoot (5)

• Serious of casting applied during 1st month f life & continuing until max correction is accomplished (5-8 weeks)

• Weekly manipulation of foot to stretch muscles with subsequent placement of a long-leg cast

• Following casting, a heel cord tenotomy is performed followed by application of long leg cast for 3 weeks.

• After casting a Denis Browne Bar & specialized sandals (abduction brace) are applied to maintain alignment & prevent recurrence

• Abduction brace worn at bedtime for 3-5 years

Nursing Care & Client Education - Clubfoot (4)

• Assess skin integrity & neurovascular status (5 Ps)

• Cast care & regular cast changes (educate)

• Change diapers frequently

• Check for decreased circulation (pain, pallor, coldness) & notify HCP

Interprofessional Collaboration - Clubfoot (2)

• Orthopedic provider

• Physical therapist

Define Legg-Calve-Perthes Disease (2)

• Impaired circulation to femoral head that results in aseptic necrosis. 

• Condition can be unilateral or bilateral with insidious onset.

Risk Factors - Legg-Calve-Perthes Disease (4)

• Age - 2-12 y/ more common in 4-8 y/o

• More common in boys

• Trauma & decreased circulation

• Inflammation to femoral head

Expected Findings - Legg-Calve-Perthes Disease (5)

• Intermittent painless limp

• Hip stiffness - Due to misplaced ball joint of hip

• Limited ROM

• Hip, thigh, knee pain

• Shortening of affected leg

Diagnostics - Legg-Calve-Perthes Disease (2)

Radiograph - Hip & pelvis

MRI

Nursing Care - Legg-Calve-Perthes Disease (6)

1. Treatment varies with age & condition of femoral head

2. Maintain rest & initally limited weight bearing (ensure family understands & adheres)

3. NSAIDs as prescribed

4. Abduction brace or casts

5. PT

6. Traction

Complications - Legg-Calve-Perthes Disease (3)

• Joint degeneration

• Permanent disability

• Chronic pain

Define Developmental Dysplasia of the Hip (DDH)

Variety of disorders resulting in abnormal development of hip structures that can affect infants or children

Infant Expected Findings - DDH (6)

1. Asymmetry & unequal number of skin folds on posterior glutel/thigh

2. Limited hip abduction

3. Shortening of femur on affected side

4. Audible clunking sound

5. Positive Ortolani Test - Hip reduced by abduction

6. Positive Barlow Test - Hip is dislocated by adduction

Child Expected Findings - DDH (3)

• One leg shorter than the other

• Walking on toes on one foot

• Walk with a limp & waddle gait

Diagnostics - DDH (2)

• Screening - Ortolani & Barlow at each wellness visits to check for presence of DDH (up to 12 months of age)

• Ultrasound & X-ray

Therapeutic Procedures - Newborn to 6 Months: DDH (6)

1. Abduction Splint - Pavlik harness, prevents hip flexion & adduction

2. Maintain harness placement for 12 weeks.

3. Harness typically worn 23 hours per day until hip is stabilized then during sleep for next 6 weeks

4. Straps may need readjustment by HCP at least every week.  

5. Check straps frequently at least 2 times/day.

6. Perform neurovascular & skin integrity checks.

Newborn to 6 Months Therapeutic Procedures Education - DDH (5)

• Skin care

• Use an undershirt, wear knee socks

• Assess skin, gently massage skin under straps

• Avoid lotions & powders

• Place diapers under straps & assess skin under straps

Therapeutic Procedures - 6 Months to 2 Years: DDH (4)

1. Surgical closed reduction with placement of hip spica cast

2. Indicated for when abduction splinting (Pavlik harness therapy) is unsuccessful

3. Surgeon positions femoral head back into acetabulum

4. Skin & cast care needed

Client Education - DDH (4)

• Understand proper positioning, turning, neurovascular assessments, & care of the cast.

• Note color & temperature of toes on casted extremity.

• Give sponge baths to avoid wetting the cast.

• Use a waterproof barrier around genital opening of spica cast to prevent soiling with urine or feces.

Define Osteogenesis Imperfecta

An inherited connective tissue condition that results in bone fractures & deformity along with restricted growth

Expected Findings - Osteogenesis Imperfecta (4)

• Multiple bone fracture (fragile bones & deformities)

• Blue sclera

• Early hearing loss

• Small discolored teeth

Nursing Actions - Osteogenesis Imperfecta (2)

• Frequent oral care

• Manual BP

Medication - Osteogenesis Imperfecta (3)

• Bisphosphonate Therapy (pamidronate) - Increases bone density & prevents fractures, IV

• Monitor for adverse effects & respiratory infections

• Vaccines with live viruses not recommended

Bisphosphonate Therapy Adverse Effects - Osteogenesis Imperfecta (5)

• Hypokalemia, Hypomagnesemia, Hypocalcemia & Hypophosphatemia

• Thrombocytopenia & Neutropenia

• Dysrhythmias

• Kidney failure

• General malaise

Define Scoliosis (3)

• Deformity of the spine that also affects the ribs

• Characterized by a lateral curvature of the spine & spinal rotation that causes rib asymmetry.

• Can affect lung capacity & bad cases can cause lung collapse (monitor SpO2)

Expected Findings - Scoliosis (2)

• Asymmetry in scapula, ribs, flanks, shoulders, & hips

• Improperly fitting clothing (one leg shorter than the other)

Diagnostic Procedures - Scoliosis

• Lung capacity - pulmonary function studies, chest x-ray

Surgical Interventions - Scoliosis

Spinal fusion with rod placement - Curvatures > 45 degrees