MUSCULOSKELETAL CONGENITAL DISORDERS ATI CHAPTER 28 Musculoskeletal Congenital Disorders ▪ Musculoskeletal congenital disorders might be identified at birth or might not be present until later in infancy, childhood, or adolescence. ▪ These disorders can involve a specific area of the child’s body or affect the child’s entire musculoskeletal system. ▪ Careful assessment and interprofessional management assist in promoting the child’s growth, development, and mobility. CLUBFOOT ▪ A complex deformity of the ankle and foot ▪ Can affect one or both feet, occur as an isolated defect, or in association with other disorders such as cerebral palsy and spinal bifida ▪ Categorized as positional clubfoot (occurs from intrauterine crowding), syndromic (occurs in association with other syndromes), and congenital (idiopathic) CLUBFOOT ▪ RISK FACTORS: ▪ presence of other syndromes, hereditary factors ▪ EXPECTED FINDINGS ▪ ▪ ▪ ▪ ▪ Talipes varus: inversion (bending inward) Talipes valgus: eversion (bending outward) Talipes calcaneus: dorsiflexion (toes are higher than the heels) Talipes equinus: plantar flexion (toes are lower than the heels) Talipes equinovarus: toes are facing inward and lower than the heel ▪ DIAGNOSTIC PROCEDURES: ▪ Prenatal ultrasound provides data for identification of the deformity. CLUBFOOT ▪ NURSING CARE ▪ Encourage parents to hold and cuddle the child. ▪ Encourage parents to meet the developmental needs of the child. ▪ Perform neurovascular and skin integrity checks. ▪ THERAPEUTIC PROCEDURES: ▪ Castings ▪ Series of castings starting shortly after birth and continuing until maximum correction is accomplished. CLUBFOOT ▪ Weekly manipulation of the foot to stretch the muscles with subsequent placement of a new cast. ▪ Following casting, a heel cord tenotomy is usually performed followed by a long leg cast for 3 weeks. ▪ A Denis Browne bar that connects specialized shoes can be applied to maintain the correction and prevent recurrence. ▪ NURSING CARE ▪ Assess neurovascular status. Perform cast care. ▪ CLIENT EDUCATION ▪ Teach cast care. ▪ Teach the family follow-up care for cast changes. CLUBFOOT ▪ COMPLICATIONS Growth and development delays ▪ NURSING CARE: Monitor growth and development. ▪ CLIENT EDUCATION: Teach the family strategies to enhance normal growth and development. ▪ Effects of casting ▪ Skin breakdown ▪ Neurovascular alterations CLUBFOOT LEGG-CALVE-PERTHES DISEASE Aseptic necrosis of the femoral head can be unilateral or bilateral ASSESSMENT RISK FACTORS ▪ Age: Affects children 2 to 12 years but more common between ages 4 to 8 years ▪ Gender: More common in boys ▪ Trauma, inflammation to the femoral head EXPECTED FINDINGS ▪ ▪ ▪ ▪ ▪ ▪ Intermittent painless limp Hip stiffness Limited ROM Hip, thigh, knee pain Shortening of the affected leg Muscle wasting ▪ DIAGNOSTIC PROCEDURES: Radiograph of the hip and pelvis LEGG-CALVE-PERTHES DISEASE NURSING CARE ▪ Treatment varies with the child’s age and the appearance of the femoral head. ▪ Administer NSAIDs as prescribed. ▪ Maintain rest and limited weight bearing. ▪ Abduction brace ▪ Casts LEGG-CALVE-PERTHES DISEASE ▪ Physical therapy ▪ Traction ▪ Advance to active motion as prescribed. SURGICAL INTERVENTIONS: Osteotomy of the hip or femur CLIENT EDUCATION ▪ Teach about the limited weight bearing treatment prescribed. ▪ Offer developmental appropriate strategies for learning and activities during the limited weight bearing periods. DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) A variety of disorders resulting in abnormal development of the hip structures that can affect infants or children ▪ Acetabular dysplasia: delay in acetabular development ▪ (acetabular roof is shallow and oblique) ▪ Subluxation: incomplete dislocation of the hip ▪ Dislocation: femoral head does not have contact with ▪ the acetabulum DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Risk Factors ▪ ▪ ▪ ▪ ▪ ▪ Birth order (firstborn) Female gender Family history Breech intrauterine position Delivery type Joint stability DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ EXPECTED FINDINGS INFANT ▪ Asymmetry of gluteal and thigh folds ▪ Limited hip abduction ▪ Shortening of the femur ▪ Widened perineum DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Positive Ortolani test (hip is reduced by abduction) ▪ Positive Barlow test (hip is dislocated by adduction) CHILD ▪ One leg shorter than the other ▪ Positive Trendelenburg sign (while bearing weight on the affected side, the pelvis tilts downward) ▪ Walking on toes on one foot ▪ Walk with a limp DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ DIAGNOSTIC PROCEDURES Ultrasound: performed at 2 weeks of age to determine the cartilaginous head of the femur X-ray: can diagnose DDH in infants older than 4 months of age PATIENT-CENTERED CARE nursing care ▪ Treatment starts as soon as DDH is diagnosed and depends on the child’s age and the extent of the dysplasia. ▪ Encourage parents to hold and cuddle the infant/child. ▪ Encourage parents to meet the developmental needs of the infant/child. DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Newborn to 6 months Pavlik harness ▪ Maintain harness placement for to 12 weeks. ▪ Check straps every 1 to 2 weeks for adjustment. ▪ Perform neurovascular and skin integrity checks. DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Removing the harness is dependent on the client. CLIENT TEACHING ▪ Teach the family not to adjust the straps. ▪ Teach the family how to place the harness if removal is prescribed. ▪ Teach the family skin care (use an undershirt, wear knee socks, assess skin, gently massage skin under straps, avoid lotions and powders, place diaper under the straps). DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) When adduction contracture is present Bryant traction ▪ Skin traction ▪ Hips flexed at a 90° angle with the buttock raised off of the bed NURSING ACTIONS ▪ ▪ ▪ ▪ Neurovascular checks Maintain traction (ropes, boots, pulleys, and weights) Ensure the client maintains alignment Skin care DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Hip spica cast ▪ Needs to be changed to accommodate growth NURSING ACTIONS ▪ Assess and maintain the hip spica cast. ▪ Perform frequent neurovascular checks. ▪ Perform range of motion with the ▪ unaffected extremities. DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Perform frequent assessment of skin integrity, especially in the diaper area. ▪ Assess for pain control using an age-appropriate pain tool. Intervene as indicated. ▪ Evaluate hydration status frequently. ▪ Assess elimination status daily. DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) CLIENT EDUCATION ▪ Reinforce teaching regarding positioning, turning, neurovascular assessments, and care of the cast. ▪ Position casts on pillows. ▪ Keep the casts elevated until dry. ▪ Encourage frequent position changes to allow for drying. ▪ Handle the casts with the palm of the hand until dry. DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Note color and temperature of toes on casted extremity. ▪ Give sponge baths to avoid wetting the cast. ▪ Use a waterproof barrier around the genital opening of spica cast to prevent soiling with urine or feces. ▪ Educate regarding care after discharge with emphasis on using appropriate equipment (stroller, wagon, car seat) for maintaining mobility. DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) 6 months to 2 years ▪ Surgical closed reduction with placement of hip spica cast NURSING ACTIONS ▪ Prepare family and client for surgery ▪ Perform neurovascular checks ▪ Manage postoperative pain DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Skin care ▪ Cast care CLIENT EDUCATION: Teach about spica cast and home care management Older children ▪ Surgical reduction with presurgical traction ▪ Femoral osteotomy, reconstruction, and tenotomy are often needed DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) COMPLICATIONS ▪ Postoperative complications: Atelectasis, ileus, infection ▪ Effects of immobilization: Decreased muscle strength, bone demineralization, altered bowel motility ▪ Effects of casting: Skin breakdown, neurovascular alterations Infection DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) Infection can be caused by bacteria, such as Staphylococcus aureus. NURSING ACTIONS ▪ Monitor vital signs. Observe changes in temperature that could be associated with complications of infection. ▪ Keep the cast dry and intact. ▪ Monitor for changes in neurovascular status (numbness; tingling; decreased mobility, sensation, or capillary refill). DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) COMPLICATION Reposition the child frequently. ▪ Maintain a high-fiber diet and promote adequate hydration. ▪ Monitor bowel and bladder elimination. Report any changes, especially the decrease or absence of bowel sounds or distention. ▪ Report any foul odor from cast or urine. DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH) ▪ Observe changes in behavior, especially increasing irritability in infants. CLIENT EDUCATION ▪ Discuss possible complications of specific treatment or procedure with the child and/or family. ▪ Reinforce the need to notify the provider with any concerns or indications of complications. ▪ Educate the child and family about follow-up. OSTEOGENESIS IMPERFECTA ▪ An inherited condition that results in bone fractures and deformity along with restricted growth. ▪ ASSESSMENT RISK FACTORS: Parent who has osteogenesis imperfecta EXPECTED FINDINGS: Classic manifestations ▪ Multiple bone fractures ▪ Blue sclera OSTEOGENESIS IMPERFECTA ▪ Early hearing loss ▪ Small, discolored teeth DIAGNOSTIC PROCEDURES: Bone biopsy NURSING ACTIONS ▪ Prepare the client for the procedure. ▪ Assist with positioning the client. OSTEOGENESIS IMPERFECTA ▪ NURSING CARE: Treatment is supportive. ▪ MEDICATION: Pamidronate ▪ Increase bone density ▪ NURSING ACTIONS ▪ Administer IV. ▪ Monitor for adverse effects (hypokalemia, hypomagnesemia, hypocalcemia, hypophosphatemia, thrombocytopenia, neutropenia, dysrhythmias, kidney failure, general malaise). ▪ CLIENT EDUCATION ▪ Teach adverse effects and when to call the provider. ▪ Instruct the family and client that antibiotics will be needed prior to some dental work OSTEOGENESIS IMPERFECTA ▪ ▪ ▪ ▪ ▪ ▪ ▪ Teach the family and client low-impact exercises. Teach the family about medication regime as prescribed. Consult with physical therapy. Assist with braces and splints as prescribed. Assist client with meeting developmental milestones. Educate the parents on the client’s limitations. Encourage support groups. ▪ SURGICAL INTERVENTIONS ▪ For severe cases ▪ Correct bone deformities, placement of rods. OSTEOGENESIS IMPERFECTA ▪ COMPLICATIONS ▪ Disuse osteoporosis ▪ Limit time in casts. ▪ Encourage activity. ▪ Hearing loss Permanent deformities SCOLIOSIS ▪ Scoliosis is a complex deformity of the spine that also affects the ribs. ▪ Characterized by a lateral curvature of the spine and spinal rotation that causes rib asymmetry. ▪ Idiopathic or structural scoliosis is the most common form of scoliosis and can be seen in isolation or associated with other conditions. SCOLIOSIS RISK FACTORS ▪ Genetic tendency ▪ Gender: more common in girls ▪ Age: highest incidence between 8 to 15 years of age EXPECTED FINDINGS ▪ Asymmetry in scapula, ribs, flanks, shoulders, and hips ▪ Improperly fitting clothing (one leg shorter than the other) DIAGNOSTIC PROCEDURES SCOLIOSIS ▪ Screen during preadolescence for boys and girls. ▪ Observe the child, who should be wearing only underwear, from the back. ▪ Have the child bend over at the waist with arms hanging down and observe for asymmetry of ribs and flank. ▪ Measure truncal rotation with a scoliometer. ▪ Perform radiography. ▪ Use the Cobb technique to determine the degree of curvature. ▪ Use the Risser scale to determine the skeletal maturity. SCOLIOSIS ▪ NURSING CARE ▪ Treatment depends on the degree, location, and type of curvature. ▪ THERAPEUTIC PROCEDURES ▪ Bracing: Customized braces slow the progression of the curve. NURSING ACTIONS ▪ Assist with fitting the client with a brace. ▪ Assess skin. ▪ Promote the client’s positive self-image. CLIENT EDUCATION: Teach the client how to apply the brace. SCOLIOSIS SURGICAL INTERVENTIONS ▪ Spinal fusion with rod placement: Used for curvatures greater than 45° PREOPERATIVE NURSING ACTIONS ▪ Inform and assist the adolescent to obtain autologous (self-donated) blood donations. ▪ Obtain routine laboratory studies, including a type and cross match for blood as prescribed. ▪ Orient the adolescent and family to the ICU. ▪ Inform the adolescent and family about what can be expected during the postoperative period, such as monitoring equipment, NG tube, chest tubes, indwelling urinary catheters, and self-administering analgesic pumps. SCOLIOSIS POSTOPERATIVE NURSING ACTIONS ▪ Monitor the adolescent initially in the intensive care unit. ▪ Perform standard postoperative care to prevent complications ▪ Monitor pain using an age-appropriate pain tool. ▪ Administer analgesia using a patient-controlled analgesic (PCA) pump as prescribed. ▪ Perform frequent neurovascular checks. ▪ Turn the adolescent frequently by log rolling to prevent damage to the spinal fusion. SCOLIOSIS POSTOPERATIVE NURSING ACTIONS ▪ Assess skin for pressure areas, especially if a brace has been prescribed. ▪ Provide skin care by keeping skin clean and dry. ▪ Monitor surgical and drain sites for indications of infection. Provide wound care as prescribed. ▪ Assess bowel sounds and monitor, observing for paralytic ileus. ▪ Monitor for decreases in Hgb and Hct. Observe for indications of bleeding. SCOLIOSIS POSTOPERATIVE NURSING ACTIONS ▪ Administer blood transfusion as prescribed. The adolescent can have self-donated blood available for transfusion. ▪ Encourage mobility as soon as tolerated. ▪ Assess for infection. ▪ Perform range of motion on unaffected extremities. ▪ Provide age-appropriate activities and opportunities to visit with friends and family during the hospital stay SCOLIOSIS- CLIENT EDUCATION PREOPERATIVE ▪ Reinforce teaching (use of incentive spirometer, turning, coughing, deep breathing) to prevent complications. ▪ Perform extensive preoperative teaching to educate the adolescent and family and to promote cooperation and participation in recovery. SCOLIOSIS- CLIENT EDUCATION ▪ Demonstrate the use of a PCA pump if age-appropriate. ▪ Demonstrate log rolling that will be used after surgery. ▪ Demonstrate the respiratory therapy techniques that will be used postoperatively to reduce complications of anesthesia. ▪ Discuss medical terms that are unfamiliar to the adolescent and/or family. SCOLIOSIS - POSTOPERATIVE ▪ Emphasize the importance of physical therapy and proper positioning of the spine. ▪ Encourage independence following surgery for the adolescent who has a brace. ▪ Encourage the adolescent to contact friends when able. ▪ Emphasize the necessity of follow-up care. SCOLIOSIS - POSTOPERATIVE CARE AFTER DISCHARGE ▪ Reinforce the expected course of treatment and recovery. ▪ Suggest that the family arrange the environment to facilitate the adolescent’s ability to be as independent as possible (keep favorite items within reach). ▪ Emphasize the necessity of follow-up care. SCOLIOSIS --COMPLICATIONS ▪ Breathing difficulties (with severe curvatures) Spine or nerve damage Lowered self-esteem: Assist the client with ageappropriate actions to promote positive self-esteem. Infection following surgery ▪ Infection can be caused by bacteria, such as Staphylococcus aureus. NURSING ACTIONS ▪ Monitor vital signs. Observe changes in temperature that could be associated with complications of infection. ▪ Monitor for changes in neurovascular status (numbness; tingling; decreased mobility, sensation, or capillary refill). SCOLIOSIS --COMPLICATIONS ▪ Reposition the child frequently. ▪ Maintain a high-fiber diet and promote adequate hydration. ▪ CLIENT EDUCATION ▪ Discuss possible complications of specific treatment or procedure with the child and family. ▪ Reinforce the need to notify the provider with any concerns or indications of complications. ▪ Educate the child and family about follow-up.