ScienceDirect ® is a registered trademark of Elsevier B.V. ScienceDirect ® is a registered trademark of Elsevier B.V. Intrathecal baclofen has been shown to reduce the need for orthopaedic lower extremity procedures and the rate of postoperative complications associated with these procedures. The objective of treatment in neuromuscular scoliosis is to maximise patient's function whilst preventing progression of the scoliosis. 53 Surgical spine fusion with instrumentation and early postoperative mobilization is the treatment of choice for individuals with progressive neuromuscular scoliosis. Some patients are capable of ambulation, although many lose their ability to walk early in life or never achieve ambulatory status at all. Large rigid curves restrict lung volume and impair respiration in patients who often already have limited pulmonary capacity. Left ventricular hypertrophy can be associated with Friedreich ataxia. The authors’ current algorithm indicates an anterior procedure for “severe” curves (most often thoracolumbar). For a posterior procedure, 4 U of packed red blood cells is generally sufficient; however, the addition of a kyphectomy or an anterior procedure may increase this requirement. These x-rays keep track of changes until your child has finished growing. Patients should have a preoperative anteroposterior and lateral film taken of the entire spine preferably in an upright (sitting or standing) position. More powerful instrumentation systems have led to less postoperative decompensation and pseudarthrosis; however, there remains a considerable risk of curve progression, sometimes necessitating revision surgery.26–28. Physicians may wish to provide information regarding clinical trials or refer families to clinical trial websites (. Bracing.Wearing a back brace can provide support to the spine and prevent the curve fromworsening to some extent. Scoliosis is a condition in which the spine curves sideways in a C- or S-shaped curve. In contrast, Comstock and colleagues,27 after review of 60 skeletally immature patients with cerebral palsy who underwent surgical scoliosis correction, concluded that skeletally immature patients have the best correction and long-term outcomes when treated with anterior and posterior procedures. In a more recent analysis of the interobserver and intraobserver variability of radiographic measurements of patients with neuromuscular scoliosis, Gupta and colleagues, History of Instrumentation in Neuromuscular Scoliosis, The introduction of segmental spinal instrumentation by Luque. Less rigid forms of bracing are better tolerated but do not seem to alter the natural history of curve progression. It is characterized by an abnormal lateral curvature of the spine and there are many different forms. Some patients are capable of ambulation, although many lose their ability to walk early in life or never achieve ambulatory status at all. The etiology of the patient’s scoliosis and the patient’s muscle tone have an impact on the practicality of brace treatment. Although there is limited research on the results of the STO brace, there are numerous studies investigating the TLSO brace. In a review article, Shapiro and Sethna, In a prospective, double-blinded, placebo control study of 40 pediatric patients, Neilipovitz and colleagues, Patients should have a preoperative anteroposterior and lateral film taken of the entire spine preferably in an upright (sitting or standing) position. Olafsson and colleagues18 followed 90 patients with various neuromuscular conditions treated with a soft Boston orthosis for an average of 3 years after brace treatment. These authors believed that this fusion allows greater mobility and improves the patients’ ability to carry out activities of daily living. Get the facts on scoliosis types and symptoms. Albumin should be greater than 3.5 g/L, and total lymphocyte count should be greater than 1.5 g/L40; in a study of 44 patients, Jevsevar and Karlin41 found that patients had a lower incidence of postoperative infections if they met these criteria. The treatment options available in neuromuscular scoliosis are limited. As a patient’s pelvis is brought back to a more normal, level position, it can then provide a stable platform for sitting. This procedure was followed by T2 to pelvis posterior instrumentation with completion of L1 and L2 corpectomy posteriorly. 24–8). If the curve progresses over 20 degrees, and your child is still growing, a brace may be recommended to keep the curve from worsening. About 3% of adolescents have scoliosis.Most cases of scoliosis are mild, but some spine deformities continue to get more severe as children grow. The sagittal profile is another important consideration because lordotic and kyphotic deformities can also impair sitting balance and pulmonary capacity (Fig. 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. However, there are treatments available for people who have neuromuscular scoliosis, including: Wheelchair modifications. Additionally, proximal fixation with sublaminar wires compromises the ligaments above, making junctional kyphosis more likely. Patients with neuromuscular scoliosis may lack sensate skin to feel pressure from the brace or the muscular control to pull away from the sides of the brace. Anterior release and fusion has generally been indicated in patients with rigid scoliosis, patients with rigid kyphosis, immature patients at risk for the development of crankshaft growth, and patients at risk for pseudarthrosis owing to incompetent posterior elements (myelomeningocele or severe osteopenia). In all other patients, brace wear was ineffectual in altering progression but did provide assistance in sitting. Although many patients already have neurologic compromise, they are still at risk for further compromise because of intraoperative spinal column manipulation. Because of these attributes, the Luque technique became the standard method for posterior spinal instrumentation in patients with neuromuscular spinal deformities. Advances in general care, glucocorticoid treatment, noninvasive ventilatory support, cardiomyopathy management, and scoliosis management have significantly changed the course of Duchenne muscular dystrophy (DMD). Psychosocial support for patients and parents is also vital. In the Warner-Fackler technique,70 Luque rods are bent to 90 degrees in two places at the distal end, allowing the rods to pass through the S1 foramina and lever against the front of the sacrum to provide sagittal correction (Fig. Thoracoscopic Approach for Spinal Conditions, Intraoperative Neurophysiologic Monitoring of the Spine. Progressive curves require surgical correction and stabilization. Although it provides modest correction as shown in these radiographs, rigid bracing may lead to excessive skin pressure in patients, who cannot actively pull away from brace. The primary care physician should be well informed of all orthopaedic issues and play a central role in managing care. The decision to operate on a patient with neuromuscular scoliosis is a highly individualized process that should involve a frank and open discussion with the family and patient about the risks and expectations of such a procedure. In a study of 20 nonambulatory patients with neuromuscular scoliosis with halo-femoral traction and 20 matched patients without halo-femoral traction, Takeshita and colleagues78 found that halo-femoral traction provided significantly improved lumbar curve and pelvic obliquity correction at 2-year follow-up. Whether your child has idiopathic, neuromuscular or congenital scoliosis, the primary goal of any treatment is to stop the curve from getting worse. Many patients with neuromuscular scoliosis are on long-term seizure therapy, which has some important operative ramifications. Treatment of neuromuscular scoliosis can also help the caretakers of these patients, improving the ease of transfers, positioning, feeding, and hygiene. Dietary supplements Series using only Harrington rods and posterior spinal fusion have been associated with high incidences of pseudarthrosis (19% to 40%), moderate initial correction (20% to 57%), and loss of correction ranging from 14% to 28%.29,48 After Harrington rod instrumentation, most patients required bed rest and bracing or casting for up to 1 year. Mercado and colleagues31 evaluated 198 publications and graded their results on the concept of Grades of Recommendation introduced in the Journal of Bone and Joint Surgery.32 These authors concluded that the current literature shows there is poor-quality evidence that spinal fusion improves the quality of life in patients with cerebral palsy or DMD.31 In a Cochrane Collaboration review, Cheuk and colleagues33 found that there were no randomized controlled clinical trials available to evaluate the effectiveness of scoliosis surgery in patients with DMD, and so no evidence-based recommendations could be made. Observation alone is employed until curves begin to cause functional impairment. We use cookies to help provide and enhance our service and tailor content and ads. Studies of patient outcomes with unit rod fixation have revealed excellent correction and maintenance of correction.54–56 Bulman and colleagues57 compared the unit rod with double Luque rods and reported superior correction of sagittal and coronal alignment and pelvic obliquity with the unit rod constructs. The surgeon and the anesthesiologist should familiarize themselves with these agents and make a collaborative decision on their use based on the needs and concerns of the individual patient. They concluded that brace wear was indicated only in a limited subset of patients—ambulatory patients with hypotonia and short thoracolumbar curves (<40 degrees). A solid spinal fusion to the pelvis aids in sitting comfort and balance1,65; however, achieving this goal can be troublesome (Fig. The increase in life span of patients with DMD has largely been attributed to the use of steroids and noninvasive ventilator support. Non-surgicaltreatment Theprimary goal of non-surgical treatment is to prevent the spinal curves from furtherworsening. The goals of achieving a level pelvis and balanced spine must be weighed against the added morbidity of an anterior release or instrumentation procedures or both. A biomechanical study conducted at the authors’ institution showed that the addition of bilateral L1 pedicle screws to a Luque-Galveston construct on a cadaveric axial skeleton increased construct stiffness by greater than 60%. – And others. If a patient cannot be assessed with formal pulmonary function tests, other signs of ventilatory capacity must be used, including crying, laughing, and other vocalizations.38–40. Advances in general care, glucocorticoid treatment, noninvasive ventilatory support, cardiomyopathy management, and scoliosis management have significantly changed the course of Duchenne muscular dystrophy (DMD). According to the American Association of Neurological Surgeons (AANS), scoliosis affects between 2% and 3% of the American population, or about six to nine million people. Caird and colleagues10 showed a significantly higher rate of complications associated with posterior spinal fusion and instrumentation in a group of 20 spastic cerebral palsy patients with intrathecal baclofen pumps compared with a matched control group. An anterior release of a large rigid curve increases the overall spine mobility and makes the posterior correction easier with a relatively high fusion rate. The timing for operative treatment is influenced by curve severity, underlying neuromuscular pathology, and other factors. Botulinum toxin has gained a growing acceptance as a treatment of upper and lower limb spasticity. 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. Early referral of patients who are candidates for surgery simplifies treatment and may improve its outcome. Treatment of neuromuscular scoliosis with posterior spinal fusion using the galveston procedure: retrospective of eight years of experience with unit rod instrumentation. This prospective observational study followed eight patients on noninvasive night ventilation for respiratory failure with 48 months after surgery and found that all patients recovered well with no major complications. The goal of treatment is preservation of function, which may entail maintaining ambulatory status, maintaining sitting without upper extremity support, or simply allowing assisted comfortable sitting. Surgery is frequently deemed to be the best treatment for scoliosis for adults, children with severe curves, and people of all ages with neuromuscular disorders. Botulinum toxin has gained a growing acceptance as a treatment of upper and lower limb spasticity. Studies have shown that patients with neuromuscular scoliosis have greater blood loss than patients with idiopathic scoliosis undergoing similar procedures. 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. Bracing.Wearing a back brace can provide support to the spine and prevent the curve fromworsening to some extent. The ultimate goal of treatment of patients with neuromuscular scoliosis is the maintenance of … Coordination of care is very important and will help ensure that every aspect of treatment is maximized. The selection of either of these two instrumentation systems is a choice that relies on surgeon experience and the needs of the individual patient. Nonsurgical options for neuromuscular scoliosis. Mild cases may not affect daily living. Preoperatively, patients may have compromised pulmonary function, limited cardiac capacity, poor bone stock, and high risk for aspiration, which put them in danger of intraoperative or postoperative complications. If possible, consideration should be given to weaning the patient off of this medication, or at least the surgeon should prepare for increased blood loss by having supplementary blood products available during surgery. Treatment for neuromuscular scoliosis. Neurologic deficiencies can be broken down further into upper motor neuron dysfunction, as seen in myelomeningocele, or lower motor neuron dysfunction, as seen in spinal muscular atrophy (SMA). Patients with spastic disorders generally do not tolerate rigid brace treatment, whereas patients with flaccid paresis are more apt to be compliant with brace treatment. In very small or thin patients, the authors continue to prefer a Galveston rod construct for pelvic fixation. Aprotinin, tranexamic acid, and aminocaproic acid have also been investigated to determine their effect on blood loss in spinal surgery. Randomized placebo-controlled trials have been conducted investigating the efficacy of several medical treatments for SMA, including creatine, phenylbutyrate, gabapentin, and thyrotropin-releasing hormone. Fusion should be considered as coronal deformity approaches 40 to 60 degrees. To assess spinal flexibility, supine bending films or traction films are used. Ultimately, the decision to include an anterior procedure (release or instrumentation) is multifactorial and depends on the experience of the surgeon, the overall health of the patient, and the characteristics of the deformity. If the curve remains rigid after an anterior release, the anterior instrumentation is skipped, and either an apical vertebrectomy is performed, or the correction is achieved after posterior osteotomies (Fig. The various types of scoliosis are classified by cause and age of onset; the speed and mechanism of progression also plays a role in deter… These patients rarely tolerate the rigid braces often used in idiopathic scoliosis. Correction of large deformities requires extensive exposures and long procedures that can lead to blood loss greater than one to two patient blood volumes. Several pharmacologic agents have been under investigation for their efficacy in reducing blood loss during surgery. It is recommended that the decision for surgical intervention be made based on the needs of the individual patient in consultation with the multidisciplinary neuromuscular care team. A solid spinal fusion to the pelvis aids in sitting comfort and balance. Reviewing the results of 67 patients, McCarthy and colleagues69 found that this technique had decreased operative time compared with Galveston fixation and excellent clinical results, although in 2 of the 67 constructs there was migration of the rods into the pelvis. About 3% of adolescents have scoliosis.Most cases of scoliosis are mild, but some spine deformities continue to get more severe as children grow. normal spine has several front-to-back curves between the neck and the pelvis Surgery is generally recommended if brace or cast treatment should fail to keep the scoliosis from progressing, or if the curve pattern does not appear amenable to brace or cast treatment. Copyright © 2003 Elsevier Ltd. All rights reserved. Posterior approach: The most frequently performed surgery for adolescent idiopathic scoliosis involves posterior spinal fusion with instrumentation and bone grafting. Log In or, Click to share on Twitter (Opens in new window), Click to share on Facebook (Opens in new window), Click to share on Google+ (Opens in new window), Natural History and Associated Complications, Neuromuscular scoliosis generally begins early in life, is rapidly progressive, and causes significant morbidity. This is not a problem as the spine has grown enough by that age. Curves of neuromuscu… These abnormalities in the spine, costal-vertebral joints, and the rib cage produce a ‘convex’ and ‘concave’ hemithorax. The rotation component starts when the scoliosis becomes more … Whether your child has idiopathic, neuromuscular or congenital scoliosis, the primary goal of any treatment is to stop the curve from getting worse. B, Owing to inflexibility, anterior disc excision and anterior instrumentation was used as the first stage of this procedure. Many patients are malnourished secondary to a combination of reflux, low calorie intake, and high metabolic demand from frequent illness. Hooks (transverse process) or pedicle screws or both may limit this complication in kyphotic patients at greatest risk. 24–14). The curve severity guidelines are loosely based on, but less aggressive than, the guidelines used in idiopathic scoliosis. Scoliosis causes abnormal curvature of the spine. Flaccid patients are more amenable to rigid bracing, although this bracing may significantly decrease chest expansion leading to compromised pulmonary function. 1. The complications associated with arrhythmias may be alleviated with glucocorticoid steroid treatment.14 Patients with myotonic dystrophy may also have cardiac arrhythmias. The introduction of segmental spinal instrumentation by Luque49 in 1976 led to major advances in the biomechanical stability and correction of these very deformed spines (Fig. In skeletally immature patients with idiopathic scoliosis, anterior release and fusion reduces anterior overgrowth that results in crankshaft deformity; however, whether this principle can be applied in neuromuscular scoliosis is controversial. Copyright © 2021 Elsevier B.V. or its licensors or contributors. Common conditions that can result in a neuromuscular scoliosis include: C, Age 23, curve measures 143 degrees. https://doi.org/10.1016/S0268-0890(03)00002-1. – The Functional Individual Therapy of Scoliosis approach (FITS) from Poland. The results of this study are in contrast to the work of Wimmer and colleagues,63 who found that there was no difference between Luque Galveston and Isola instrumentation in radiographic outcomes, patient satisfaction, or complication rate. Neuromuscular scoliosis can be treated both surgically and non-surgically. The authors noted decreased physical ability at the 6-month time point followed by a return to preoperative function by 12 months and concluded that scoliosis surgery in these patients can stabilize, but not improve, function; however, 75% of patients or caregivers were extremely pleased with the cosmetic results of the surgery. FIGURE 24–3 A and B, Bracing in neuromuscular scoliosis is often poorly tolerated. Patients may have cardiac problems secondary to their deformity and other cardiac issues that are comorbidities of the primary disorder. 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 natural history for a given patient is largely determined by the specific underlying neuromuscular condition and the degree of involvement. This is a tremendous advantage because postoperative casting carries the potential for skin and pulmonary complications. ... Neuromuscular scoliosis is caused by an abnormal development of the bones of the spine. Chiropractic manipulation 2. As with AIS, surgery carries heavy risks and side effects and should be considered as a last resort after other noninvasive treatment methods have been attempted. Bridwell and colleagues26 found similar trends in a study of 54 patients with neuromuscular disorders with all caretakers reporting benefit from the surgery, specifically in the areas of ease of patient care, skin breakdown, patient comfort, pulmonary complications, and quality of life. Baclofen can provide significant relief of spasticity, and this evidence must be considered in the context of any potential side effects. This may be achieved nonsurgically or through spinal fusion with instrumentation. If a near-complete correction of the major curve can be predicted with anterior instrumentation after an aggressive multilevel discectomy, a single rod anterior system is included. Patients and parents may need to be referred for genetic counseling to confirm the patient’s diagnosis and aid in family planning. 24–5). Correction of large deformities requires extensive exposures and long procedures that can lead to blood loss greater than one to two patient blood volumes. Peter O. Newton, MD, Eric S. Varley, DO, Burt Yaszay, MD, Dennis R. Wenger, MD, Scott J. Mubarak, MD. The rotation component start… Accurate measurements of the coronal Cobb angle, sagittal Cobb angle, and pelvic obliquity are crucial for complete preoperative planning and postoperative evaluations. Spinal surgery is considered the primary treatment option for correcting severe scoliosis in neuromuscular disorders. The effect of bracing on pulmonary dysfunction seems to depend on the level of muscle spasticity. Lonstein and Akbarnia, Although these positive results make a strong case for spine surgery in patients with neuromuscular scoliosis, several review studies have been unable to show a clear benefit of surgical intervention for the patient. Spine was dissected, subperiosteally, only pedicle screw instrumentation is a recent concept, up to the tip of the transverse processes at all levels. At treatment for neuromuscular scoliosis function Isola have also been shown to be a temporizing measure, used primarily to significant. Wires compromises the ligaments above, making junctional kyphosis more likely status should be when... Often poorly tolerated on long-term seizure therapy, which may remain asymptomatic and resolve spontaneously provide support to pelvis! To other studies reported in the outcome of the spine, costal-vertebral joints and... Progression but did provide assistance in sitting shown an improved fusion rate and high metabolic demand from frequent.. 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