Coxa Plana I.

Definition Legg-Calve-Perthes disease is when the head of the thighbone (femur) in the hip deteriorates due to insufficient blood supply to the area. Perthes disease is the name used for a condition which affects the ball-shaped end of the thigh bone (femoral head), where it enters into the formation of the hip joint. In this condition, the femoral head goes through a series of changes in which it softens, may become flattened and then gradually re-forms. It is a self limiting disease and the treatment is aimed at minimizing damage while the disease runs its course, not at 'curing' the disease. It is named after Legg(American) Calve(French) Perthes(German) physicians who simultaneously and separately reported it on 1910 distinguishing it from the TB of the hips. II. Other known name Legg-Calve-Perthes disease; Perthes disease; Idiopathic avascular necrosis of the proximal femoral epiphysis; Childhood Hip Disorder III. Incidences United States- One in 1200 children younger than 15 years is affected by LCPD. Race- Caucasians are affected more frequently than persons of other races. Sex- Males are affected 4-5 times more often than females. Age- LCPD most commonly is seen in persons aged 3-12 years, with median age of 7 years

IV. Risk factors positive family history low birth weight abnormal birth presentation children exposed to second hand smoke occurs most frequently in boys 4 to 10 years old

hormonal changes trauma infection metabolic abnormalities

V. Type/Stage/Classifications of the Disease Stages of Coxa Plana: Stage I (avascularity)The blood supply to the upper femoral epiphysis is halted spontaneously and bone growth is halted (lasts a few weeks) Stage II (revascularization) New blood vessels arise to supply the necrotic area, and bone resorption and deposition take place (lasts several months to 1 year). However the new bone lacks strength and pathologic fractures may occur; the weakened epiphysis may be progressively deformed Stage III (reossification)The head of the femur gradually reforms as dead bone is replaced with new bone, which gradually spreads to heal the lesion (lasts 2 toyears) Stage IV (postrecovery)The femoral head becomes permanently distorted, with resultant joint misalignment

Classifications: 1. Herring lateral pillar classification -Determined on AP x-ray at start of fragmentation stage, which usually occurs 6 months after the onset of symptoms Group A outcome - lateral pillar maintains full height, uniformly good

Group B - maintains 50 to 100% height, poor outcome in patients > 6 years

 Group C - maintains < 50% height, poor outcomes in all patients

2. Catterall classification Stages I and II - fragmentation involves < 1/2 of femoral head Stages III and IV - fragmentation involves > 1/2 of femoral head 3. Salter-Thompson classification Class A - cresent sign involve less than 1/2 of femoral head Class B - involves > 1/2 of femoral head 4. Stulberg classification gold standard for rating residual femoral head deformity and joint congruence recent studies show poor interobserver and intraobserver reliability

VI. Manifestations Knee pain (may be the only symptom, initially) Persistent thigh or groin pain Atrophy (wasting) of muscles in the upper thigh Slight shortening of the leg, or legs of unequal length Hip stiffness restricting movement in the hip Difficulty walking, walking with a limp (which is often painless) Limited range of motion Decreased range of motion (ROM), particularly with internal rotation and abduction Painful gait Muscle spasm Leg length inequality due to collapse Thigh atrophy: Thigh circumference on the involved side will be smaller than on the unaffected side secondary to disuse. Short stature: Children with LCPD often have delayed bone age.

VII. Pathophysiology
Predisposing factors: Age, sex, (+) family history

Precipitating factors: Metabolic abnormalities, infection, trauma, hormonal changes, children exposed to second hand smoke, abnormal birth presentation, low birth weight

Phase 1: Initial Absent blood supply in the femoral head

Hip joint becomes inflamed, stiff and painful Portions of the bone turned into dead tissues

Phase 2: Fragmentation The body cleans up dead cells and replaces them with new healthier bone cells

Femoral head begins to remodel

Joint is still irritated and painful

Phase 3: Reossification Irregular process starting in subchondral area

New formed areas of bone

Femoral epiphysis regains strength

Normal bone density returns

Phase 4: Healed Normal bone cells replace the new bone cell

Femoral head is healed Proximal VIII. Diagnostic studies femur is left with any deformity

Laboratory studies: CBC Erythrocyte sedimentation rate - May be elevated if infection present Imaging Studies: Plain x-rays of the hip are extremely useful in establishing the diagnosis. Frog leg views of the affected hip are very helpful. Multiple radiographic classification systems exist, based on the extent of abnormality of the capital femoral epiphysis. Waldenstrom, Catterall, Salter and Thompson, and Herring are the 4 most common classification systems. No agreement has been reached as to the best classification system. Five radiographic stages can be seen by plain x-ray. In sequence, they are as follows: 1. Cessation of growth at the capital femoral epiphysis; smaller femoral head epiphysis and widening of articular space on affected side 2. Subchondral fracture; linear radiolucency within the femoral head epiphysis 3. Resorption of bone 4. Re-ossification of new bone 5. Healed stage Technetium 99 bone scan - Helpful in delineating the extent of avascular changes before they are evident on plain radiographs Dynamic arthrography - Assesses sphericity of the head of the femur Treatment: 1. Nonoperative observation alone

o indications children < 6 years of age activity restriction, partial weight bearing, bracing, and physical therapy o indications children 6-8 years of age with early symptoms (controversial) 2. Operative femoral or pelvic osteotomy o indications children > 8 years of age, especially Lateral Pillar B and B/C children > 6 years of age with late symptoms (controversial) IX. Management 1. Medical Analgesics for pain. Monitoring: Monitor and assess pain level rising age-appropriate pain measurement tool Assess for gait, spasm, or presence of contractures Supportive care and education Instruct child and parents to maintain activities that promote range of motion, such as swimming and bicycling, but to avoid contact sports and high impact-running. Limitation of activities, bed rest with or without skin traction Provide equipment to assist with mobility (e.g wheelchair, walker) if needed Teach parents and siblings to assist only as needed. Allow child to care for self and participate as able. Reinforce to child that he or she is only temporarily restricted. Stress positive aspects of activity. Encourage follow up. Salicylates or anti-inflammatory agents are given to relieve synovitis, muscle spasm, and pain in the joint and help restore motion. 2. Surgical

Inominate osteotomy; varus osteotomy; osteotomy of the proximal femur, acetabulum (Salter innominate), or a combination of these maybe required Hip replacement

X. Nursing diagnosis Top 5 Priorities includes: 1. Acute Pain 2. Bathing or Self-Care Deficit 3. Impaired Physical Mobility 4. Disturbed Body Image 5. Ineffective Therapeutic Regimen Management

XI. Nursing responsibilities Preoperative Management: 1. Assess nutritional status; hydration, protein and caloric intake. Maximize healing and reduce risk of complications by providing I.V. fluids, vitamins and nutritional supplements as indicated. 2. Determine if the patient has had previous corticosteroid therapy could contribute to current orthopedic condition (aseptic necrosis of the femoral head; Osteoporosis), as well as affect his or her response to anesthesia and the stress of surgery. The patient may need corticotrophin postoperatively. 3. Determine if the patient has an infection (cold, dental, skin, UTI); it could contribute to development of osteomyelitis after surgery. Administer preoperative antibiotics as ordered. 4. Prepare patient for postoperative routines, w/c include coughing and deep breathing, frequent v/s and wound checks and repositioning. 5. Have the patient practice voiding on bed pan or urinal in recumbent position before surgery. This helps reduce the need for post operative catheterization. 6. Acquaint the patient with traction apparatus and the need for splint or cast, as indicated by the type of surgery. Post operative Management: 1. Monitor for hemorrhage and shock, which may result from significant bleeding and poor homeostasis of muscles that occur with orthopedic surgery.

a. Evaluate the blood pressure and pulse rates frequently report rising pulse rate or slowly decreasing blood pressure. b. Watch for increased oozing of wounds. c. Measure suction drainage if used. d. Report increased wound drainage or steady increase in pain of operativearea. 2. Administer I.V fluids or blood products as ordered. 3. Monitor neurovascular status. a. Watch circulation distal to the part where cast, bandage, or splint has been applied; check pulses, color, warmth, and capillary refill. b. Prevent constriction leading to interference with blood or nerve supply; check for swelling. c. Note movement, and ask about sensation of distal extremities. 4. Elevate affected extremity and apply ice packs as directed to reduce swelling and bleeding into tissues. 5. Monitor pain level and response to analgesia. 6. Immobilize the affected area and limit activity to protect the operative site and stabilize musculoskeletal structures. 7. Give analgesics that may cause respiratory depression cautiously. Monitor respiration depth and rate frequently. 8. Change position and encourage use of incentive spirometer and coughing and deep-breathing exercises every 2 hours to mobilize secretions and prevent atelectasis. Auscultate lungs frequently. 9. Monitor v/s for fever, tachycardia, or increased respiratory rate, which may indicate infection. 10. Maintain aseptic technique for dressing changes and wound care. 11. Encourage the patient to move joints that are not fixed by traction or appliance through their range of motion as fully as possible. Suggest muscle-setting exercises (quadriceps setting) if active motion is contraindicated. 12. Avoid giving calcium supplements patients on bed rest, and encourage other fluids to prevent other fluids to prevent urinary calculi.