FIGURE 24–12 A and B, Fixation to pelvis in this patient with cerebral palsy was provided by iliac screws connected to 5.5-mm posterior rod system. If your child has this condition, your family doctor may recommend the … Because of the complexity of the medical and psychosocial issues associated with neuromuscular disorders and spinal deformity, care needs to be coordinated with a multidisciplinary team. In a direct comparison of 20 patients with Galveston rod fixation versus 20 patients with iliac screw fixation, Peelle and colleagues76 found that both techniques offer similar pelvic fixation with the iliac screw construct allowing additional screw fixation points to the sacrum and lower lumbar vertebrae. The complications associated with arrhythmias may be alleviated with glucocorticoid steroid treatment. The natural history for a given patient is largely determined by the specific underlying neuromuscular condition and the degree of involvement. A careful assessment and an understanding of the primary disease and its prognosis are essential for planning treatment which is aimed at maximising function. Although most of these patients have deteriorating courses, the correction of spinal deformity seems to improve their function and quality of life. The goal of treatment for this type of scoliosis is to maintain the spine in a balanced position and to control the curve of the spine during growth. Other techniques of rod contouring to fix to the pelvis include the Warner-Fackler and McCall techniques, both commonly used in the treatment of myelomeningocele-associated kyphosis in which posterior elements of the lumbar or sacral spine may be absent. With a molded wheelchair, the patient with neuromuscularscoli… Conversely, older patients may be obese, presenting further operative complications associated with their body habitus. The indications for anterior instrumentation have been a subject of investigation more recently. The rods also require three-dimensional bending that makes it difficult to contour the rod properly.68. Other systems of sacropelvic fixation use an “S” bend (Dunn-McCarthy), which hooks distally over the sacral alae, while the more proximal portion is secured to the lumbar spine at L4 or above with a pedicle screw or infralaminar hook. The pediatric orthopedic surgeon addresses the curve by realigning the bone using rods and bone grafts to fuse areas of the spine and maintain the correction. The addition of an anterior procedure can assist in the correction of neuromuscular spinal deformity and may be justified in several situations (Fig. There are non-surgical and surgical treatment options for neuromuscular scoliosis. ScienceDirect ® is a registered trademark of Elsevier B.V. ScienceDirect ® is a registered trademark of Elsevier B.V. The primary care physician should be well informed of all orthopaedic issues and play a central role in managing care. For assessment of skeletal maturity, a separate anteroposterior radiograph of the pelvis should be considered because scoliosis films often truncate the anatomy necessary to determine skeletal maturity. The benefit of scoliosis surgery in this population is a topic of much debate. The initial concern regarding the association between radiolucency around the screw tips (“windshield wiper” sign) and an increased incidence of complications is of little clinical significance (Fig. Copyright © 2021 Elsevier B.V. or its licensors or contributors. Although iliac screws provide a promising alternative to Galveston fixation, several studies have shown difficulty with implant prominence causing skin irriation. Early referral of patients who are candidates for surgery simplifies treatment and may improve its outcome. Unbalanced curves and significant pelvic obliquity make wheelchair positioning difficult and may cause uneven distribution of weight that may lead to pressure sores (Fig. Several medical therapies have been investigated for the treatment of spasticity in patients with cerebral palsy. 24–10).66,67 Although a biomechanical evaluation of the Galveston technique by Sink and colleagues28 showed that this construct creates a long lever arm that places considerable cantilever forces at the lumbrosacral junction, these forces lead to a high incidence of proximal fixation pullout and distal migration of Galveston rods. With the introduction of the Harrington rod in 1962, use of this instrumentation with fusion of the spine in patients with neuromuscular scoliosis became the standard. Depending on your child's age and the curve (C or S shaped) a nighttime bending brace (Picture 1) or a Boston-style brace will be prescribed. FIGURE 24–2 Severe spinal deformity can lead to skin maceration on concave side of curvature and pressure sores on convex side. Nonoperative treatmentshould be prescribed and managed by a multidisciplinary team to ensure that all clinical areas receiving the proper treatment: pulmonary, neurologic, genitourinary, orthopedic, nutritional and gastroenterologic. The authors’ current algorithm indicates an anterior procedure for “severe” curves (most often thoracolumbar). Bracing.Wearing a back brace can provide support to the spine and prevent the curve fromworsening to some extent. Eventually, many of these patients require surgical stabilization with a spinal instrumentation and fusion procedure. normal spine has several front-to-back curves between the neck and the pelvis These abnormalities in the spine, costal-vertebral joints, and the rib cage produce a ‘convex’ and ‘concave’ hemithorax. Thoracic cage deformity resulting from scoliosis can cause hypoventilation and subsequent increased pulmonary vascular resistance; this increased vascular resistance can cause right ventricular hypertrophy and eventually cor pulmonale. As a patient’s pelvis is brought back to a more normal, level position, it can then provide a stable platform for sitting. C and D, Posterior instrumentation T2 to pelvis was used in this spastic quadriplegic patient. Physicians may wish to provide information regarding clinical trials or refer families to clinical trial websites ( provides a current listing of open clinical trials). Neuromuscular scolioses are difficult deformities to treat. Posterior approach: The most frequently performed surgery for adolescent idiopathic scoliosis involves posterior spinal fusion with instrumentation and bone grafting. – The Functional Individual Therapy of Scoliosis approach (FITS) from Poland. Various systems have been proposed to provide fixation to the pelvis. A biomechanical evaluation of seating insert configurations by Holmes and colleagues. Many of these patients are poor operative candidates and risk much undergoing involved corrective surgery. Lonstein and Akbarnia29 reported that more than 50% of patients treated had functional improvement after surgery. Intrathecal baclofen is a well-established treatment that has been shown to provide significant benefits in controlling spasticity in patients with cerebral palsy. In general, surgical treatment is appropriate for children and adults whose curve is large and progressive, whose curve causes pain or difficulty with walking, sitting or breathing, and who have the nutritional and overall health status to tolerate surgery. Accurate measurements of the coronal Cobb angle, sagittal Cobb angle, and pelvic obliquity are crucial for complete preoperative planning and postoperative evaluations. Intrathecal baclofen has been shown to reduce the need for orthopaedic lower extremity procedures and the rate of postoperative complications associated with these procedures. Mild cases may not affect daily living. The evidence supporting STO use to prevent curve progression is limited, however, and skin intolerance found in 36 patients complicates its clinical practicality. Treatment for Neuromuscular Scoliosis. FIGURE 24–3 A and B, Bracing in neuromuscular scoliosis is often poorly tolerated. Fusion should be considered as coronal deformity approaches 40 to 60 degrees. Figure 24–14 A and B, This severe 140-degree lumbar curve was upsetting wheelchair sitting balance in this spastic quadriplegic patient. The side effects for tranexamic acid and aminocaproic acid are minor but should be discussed with the patient before using these agents. The classification of neuromuscular scoliosis can be based on the underlying disorder: neurologic (e.g., cerebral palsy) or muscular (e.g., muscular dystrophy). The effect of bracing on pulmonary dysfunction seems to depend on the level of muscle spasticity. DMD may be an important exception to this concept: Surgery has been advocated when the deformity reaches 20 degrees because of pulmonary considerations. Aprotinin, a serine protease inhibitor, was shown to reduce blood loss in adults, but its production was halted in 2007 by the U.S. Food and Drug Administration (FDA) because of concerns of higher mortality rate after its use in cardiac surgery.
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