Porth's Essentials of Pathophysiology, 4e

Disorders of the Skeletal System: Trauma, Infections, Neoplasms, and Childhood Disorders 1107

C h a p t e r 4 3

usually is a painless process, although pain may be pres- ent in severe cases, usually in the lumbar region. The pain may be caused by pressure on the ribs or on the crest of the ilium. There may be shortness of breath as a result of diminished chest expansion and gastroin- testinal disturbances from crowding of the abdominal organs. Adults with less-severe deformity may experi- ence mild backache. If scoliosis is left untreated, the curve may progress to an extent that compromises car- diopulmonary function and creates a risk for neurologic complications. Diagnosis andTreatment Early diagnosis of scoliosis can be important in the pre- vention of severe spinal deformity. The cardinal signs of scoliosis are uneven shoulders or iliac crest, prominent scapula on the convex side of the curve, misalignment of spinous processes, asymmetry of the flanks, asymmetry of the thoracic cage, and rib hump or paraspinal muscle prominence when bending forward 79–80 (see Fig. 43-24). A complete physical examination is necessary for chil- dren with scoliosis because the defect may be indicative of other, underlying pathologic processes. Diagnosis of scoliosis is made by physical examina- tion and confirmed by radiography. A scoliometer is used at the apex of the curvature to quantify a promi- nence; a scoliometer reading of greater than 10 degrees requires referral to a physician. The curve is measured by determining the amount of lateral deviation present on radiographs and is labeled “right” or “left” for the convex portion of the curve. Other radiographic proce- dures may be done, including CT scanning, MRI, and myelography. Although school screening continues to be mandated in a number of states, the USPSTF recommends against the routine screening of asymptomatic adolescents for idiopathic scoliosis, indicating that the potential harms from screening include unnecessary follow-up visits and evaluations due to false-positive results, and adverse psychological effects, especially related to brace wear. 80 It is recommended, however, that health care profession- als be prepared to evaluate idiopathic scoliosis when it is discovered incidentally or when an adolescent or parent expresses concern about scoliosis. The treatment of scoliosis depends on the severity of the deformity and the likelihood of curve progres- sion. The three determinants of progression are gender, the curve magnitude at the time of diagnosis, and skel- etal growth potential. 82–83 In all cases, girls have a risk progression 10 times greater than that of boys. Larger curves are more likely to progress. Age of presentation also is important. Curves that are detected before men- arche are more likely to progress than those detected after menarche. For persons with lesser degrees of curvature (10 to 20 degrees), the trend has been away from aggressive treatment and toward a “wait and see” approach, taking advantage of the more sophisticated diagnostic methods that now are available. Treatment is considered for physically immature patients with curves between 20 and 30 degrees. Curves between

30 and 40 degrees usually are considered for bracing, and those greater than 40 to 45 degrees are considered for surgery. A brace may be used to control the progression of the curvature during growth and can provide some cor- rection. In an effort to improve compliance, a number of new bracing techniques have been developed. These orthoses consist of easily more concealed, prefabricated forms that are modified to suit the patient. The goals of surgery are to arrest progression of the deformity, improve appearance, and achieve a bal- anced spine, while limiting the number of vertebral seg- ments that are stabilized. Instrumentation helps correct the curve and balance, and spinal fusion maintains the spine in the corrected position. 75–79 Several methods of instrumentation (i.e., rods that attach to the vertebral column and posterior fusion) are used. Combined ante- rior and posterior surgery is used for more severe curva- tures. The newer systems provide better sagittal control and more stable fixation, which allow earlier mobility. Despite great advances in spinal surgery, no one method seems to be the best for all cases. There is recent inter- est in growth modulation approaches using minimally invasive techniques, which will result in curve correc- tion while preserving spinal motion and intervertebral disk viability. 82 ■■ Skeletal disorders can result from congenital or hereditary influences or from factors that occur during normal periods of skeletal growth and development. Newborn infants undergo normal changes in muscle tone and joint motion, causing torsional conditions of the femur or tibia. Many of these conditions are corrected as skeletal growth and development take place. ■■ Osteogenesis imperfecta is a rare autosomal hereditary disorder characterized by defective synthesis of connective tissue, including bone matrix. It results in poorly developed bones that fracture easily. ■■ Disorders such as developmental dysplasia of the hip and congenital clubfoot are present at birth. Both of these disorders are best treated during infancy. Regular examinations during the first year of life are recommended as a means of achieving early diagnosis. ■■ Other childhood skeletal disorders, such as the juvenile osteochondroses and slipped capital femoral epiphysis, are not corrected by the growth process.These disorders are progressive, can cause permanent disability, and require treatment. SUMMARY CONCEPTS

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