Further information can be found, The Scoliosis Society Past Present and Future, Spinal Interface Training Group (STIG) Pilot. Muscular dystrophy refers to a group of genetic muscle-wasting disorders. The aim of this study was to document the rate of survival among 288 severely affected pediatric patients (154 females, 134 males) with spasticity and neuromuscular scoliosis who underwent spinal fusion (mean age at surgery 13 years 11 months, SD 3 years 4 months), and to identify exposure variables that could significantly predict survival times. Thoracic Insufficiency Syndrome (TIS) is commonly used to describe the potential combined spine and lung problems in EOS. Thus, patients with flaccid muscles (e.g., Duchenne’s muscular dystrophy, polio)4–6 (Fig. Figure 14–4 This older teenage girl with Rett syndrome has a severe kyphosis as her main deformity. 0 . Curves of large magnitude with pelvic obliquity require surgery for proper and permanent correction of trunk malalignment and leveling of the pelvis. Once it is felt that there is no longer much growth left in the spine, the commonest operation in the UK would be a posterior instrumented correction and fusion. It is the second most common form of scoliosis and is associated with disorders of the nerve or muscular systems such as cerebral palsy , spina bifida and spinal cord injury . The reference section of this chapter lists many book chapters from recently published texts that are quite complete and informative, written by experts in this area of spinal deformity. The neurologic condition may be the patient’s primary diagnosis or may be part of a larger syndrome. If the neuromuscular scoliosis is felt to be at high risk of worsening or worsens whilst being monitored, surgery will be advised. Spinal muscular atrophy (SMA) is a progressive neuromuscular condition characterized by hypotonia. After an Ober-Yount release, she was fitted for hip-knee-ankle-foot orthosis (HKAFO) and was able to ambulate with a walker. She developed a severe spinal deformity with 90 degrees of pelvic obliquity: (A) anterior/posterior (AP) and (B) bender thoracolumbar (TL) radio-graphs. Whether stapling will prove to be a permanent and final procedure or whether for a given patient treatment will need to be finished off by converting the stapled spine to a spinal fusion will need to be determined on an individual basis.16 Background: The primary goal in managing early-onset scoliosis (EOS) is delaying/preventing surgical intervention while allowing improved spinal growth and chest wall and lung development to improve life expectancy. This can dictate the timing and appropriateness of surgical treatment. Once a scoliosis is confirmed, you will need to see a Spine Surgeon at a recognised spine centre who will assess you fully and talk about what you may need. 14–4). They can be worn under your child’s clothes. The GP, Paediatrician or Orthopaedic Surgeon will refer you on to the Spine Surgeon. Surgical treatment of the initial injury was performed but failed: (A) AP and (B) lateral radiographs. “Successful bracing” is continued until age 10 or 11, at which time definitive fusion can be performed without as much trunk shortening as would have occurred with fusion at an earlier age. The literature on this topic is very extensive. The first thing the specialist will do is talk to you about your concerns. Scoliosis-related factors like accumulated stress could have an effect on life expectancy. Additionally, comorbidities associated with the underlying diagnosis (e.g., lung function, recurrent uri-nary tract infections, cardiomyopathy, etc. Also, the study that claims that Scoliosis decreases your life by 14.8 years says that the patients were untreated. The mean age was 12.3 years (± 5.9). Types of deformity Scoliosis Figure 14–2 This young girl has myelomeningocele with spotty lower extremity motor function and several neuraxis abnormalities as well as a complex spinal deformity but no sagittal plane malalignment: (A) pre-op AP radiograph. It can lead to the trunk and shoulders no longer being symmetrical. Even in those diseases with a fairly limited life expectancy, curve progression may occur early, and to improve the quality of life, early treatment may be necessary. They can develop subsequent spinal deformity from failure of the initial injury procedure as well as the superimposed paralytic factors11 (Fig. The Spine Surgeon will measure the angle of the scoliosis on the X - ray (Cobb angle). As people with neuromuscular conditions often have other illnesses as well as their scoliosis, your surgeon will have to make choices with you about the appropriateness of surgery balanced with all the potential risks. ASF with instrumentation alone provided nice correction: (B) AP and (C) lateral post-op radiographs. No neurosurgical intervention was involved, and pre-op neurological function was preserved. A bigger curve can cause more of the problems described above. Additionally, the curve needs to be flexible so that passive correction can occur and leveling of the pelvis can occur, otherwise the brace provides absolutely no benefit. Surgical treatment of the initial injury was performed but failed: (A) AP and (B) lateral radiographs. Traditionally definitive treatment means a long posterior spinal fusion to the pelvis (with possibly an anterior supplemental fusion) and posterior instrumentation. The scoliosis then progresses with an acute deterioration during the growth spurt somewhere between the ages of 13 and 15 years, such that it becomes difficult or impossible to sit unaided. 14–3). Neuromuscular scoliosis is a direct result of a disease process or injury that has caused the spinal muscles to be deficient in supporting and stabilizing the vertebral column. Share this: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) Developmental Medicine & Child Neurology, 45:677-682. Sometimes the head is not level or the pelvis is not level. The many causes of scoliosis include neuromuscular problems and inherited diseases or conditions caused by the environment. Neuromuscular scoliosis usually develops early in life and can progress rapidly through growth and in adulthood. Often the hospital will give you a gown, but wearing shorts and a T shirt may make things easier. There are many causative and contributory conditions to neuromuscular varieties of atypical spinal curvature and these conditions can strike at virtually any age. “Successful bracing” is continued until age 10 or 11, at which time definitive fusion can be performed without as much trunk shortening as would have occurred with fusion at an earlier age. Occasionally, especially if the scoliosis affects someone early in life, it can affect the development and function of the lungs. Neuromuscular Scoliosis For some of these conditions, treatment of the spinal deformity may in fact alter the natural history of the underlying condition because things such as decreased pulmonary function may improve with correction of a severe deformity. The severity of the abnormalities can vary between people and can change with time. Figure 14–3 This adolescent male had a spinal injury associated with an incomplete spinal cord injury. ALL Rights Reserved. Unfortunately there is no strong evidence that exercise alone stops a scoliosis progressing or improves it. Life expectancy in pediatric patients with cerebral palsy and neuromuscular scoliosis who underwent spinal fusion November 2003 Developmental Medicine & Child Neurology 45(10):677-82 (Curve progression and trunk imbalances are more severe in patients who are not able to walk). A big curve can result in the ribs touching the pelvis bone causing pain and skin problems. Braces can improve trunk alignment and make sitting better and more comfortable. The etiology of neuromuscular deformity is primarily that of muscular weakness and/or imbalance and control that affects trunk alignment. 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