Evidence-Based Treatment of Spina Bifida
Children with spina bifida manifest several congenital or developmental spine and limb deformities. Hip dislocation and scoliosis are among the most common musculoskeletal deformities in these patients. Surgical correction of these deformities has been ad
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Emmanouil Morakis and James Wright
Abstract
Children with spina bifida manifest several congenital or developmental spine and limb deformities. Hip dislocation and scoliosis are among the most common musculoskeletal deformities in these patients. Surgical correction of these deformities has been advocated to improve function and quality of life. Current evidence shows that surgical reduction of hip dislocation does not improve their function or ambulatory capacity. Correction of scoliosis may improve sitting balance, but does not change the function or quality of life for these patients. If spine deformity correction is performed, combined anterior-posterior spinal fusion with instrumentation provides the best correction with the lowest rates of pseudarthrosis. Current evidence should be used to counsel patients and their families. Any surgical intervention should aim to better physical function that may also translate into improved quality of life. Keywords
Spina bifida • Myelomeningocele • Hip dislocation • Scoliosis • Spine deformity • Evidencebased medicine • Surgical treatment • Complications • Quality of life • Treatment outcome
Introduction Spina bifida is a neural tube malformation resulting from abnormal closure of the caudal part of the neural tube during embryonic development. Spina bifida is of variable severity, but the most common presentation, myelomeningocele, is characterized by a failure of formation of the dorsal vertebral elements, a defect of the overlying skin with exposure of the meninges and spinal cord. Dysplasia of the spinal cord and nerve roots leads to partial or complete paralysis of bladder, bowel, motor, and sensory function below the level of the lesion. Other lesions may co-exist that affect the neurologic function such as a Chiari malformation, cerebellar hypoplasia, hydrocephalus, syringomyelia or diastematomyelia. E. Morakis (*) Royal Manchester Children’s Hospital, Manchester, UK e-mail: [email protected] J. Wright Oxford University Hospitals NHS Foundation Trust, Oxford, UK e-mail: [email protected]
Children with spina bifida present with variable musculoskeletal deformities that can affect their ability to ambulate and their general physical function. The musculoskeletal deformities can be congenital (e.g. kyphosis, congenital scoliosis, teratologic hip dislocation, clubfoot, vertical talus) or acquired during development of the child as a result of muscle abnormalities, paralysis, joint contracture, bone deformity and decreased sensation of the lower extremities. Orthopaedic management of these deformities must be part of a multidisciplinary approach. The presence of multiple medical co-morbidities makes orthopaedic treatment of these deformities challenging. The main goal of orthopaedic care is to maximize function and independence. While many factors affect physical function, the most important factor affecting the potential for ambulation is the level of neurologic involvement [1]. Correction of limb and spine deformities is frequently part
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