Figure 4. Luque-Galveston fixation a Entry point of iliac fixation at the posterior superior iliac spine (PSIS). b Bending rod for Galveston fixation comprises two bends, one 90° and one 45° in two different planes. c Contouring of the rod to adapt to the sagittal profile. d Luque- Galveston fixation for neuromuscular scoliosis. are crucial for the unit rod and Galveston techniques as this will determine if the pelvis will be leveled after the reduction maneuver. For severe pelvic obliquity a maximal width (MW) segmental pelvic fixation has also been described and shown to be effective [2]. MW pelvic fixation comprises a pedicle screw inserted in a Galveston fashion down the iliac wing 1 cm above the sciatic notch. As an added lever arm to correct the pelvis, a sublaminar hook pushes or pulls on an ili- osacral screw, as described by Dubousset [31]. The construct has a maximal The MW fixation allows for a very stable sacropelvic fixation width fixation across the lumbosacral junction and on the AP and axial imaging has an “M&W” configuration; hence the eponym MW fixation ( Fig. 5). The hook placement obviously is dependent on the obliquity of the pelvis; hence the hook facing down is on the iliosacral screw of the elevated hemipelvis side while the hook going up is on the iliosacral screw on the lower hemipelvis. Great forces can be exerted across these iliosacral screws, thus allowing significant correction Neuromuscular Scoliosis Chapter 24 683 Figure 5. MW fixation The drawings illustrate the placement and appearance of MW fixation. The pelvis anchorage points comprise an iliac screw (1) and iliosacral screws (2) which have downgoing (3) and upgoing hooks (4) to provide leverage in opposite directions to level the pelvis. Inset view of pelvis illustrates placement of screws. Note the iliosacral screws end in the promontory of S1. Note the location of the hooks harnessing the added lever arm of the iliosacral screws. Figure 6. Cantilever correction with MW sacropelvic fixation a The initial step of reduction is to achieve solid distal pelvic fixation. In this illustration MW pelvic fixation is achieved. Rods must be as perpendicular as possible to the pelvis. b The second step is to cantilever the proximal rod to the spine, thus achieving initial correction of the pelvic obliquity. c The third step consists of correcting if needed the residual pelvis obliquity by distracting down via the hook resting on the iliosacral screw on the higher hemipelvis. In contrast, on the lower hemipelvis, the hook will pull up (compressing) the iliosacral screw proximally to level the pelvis. 684 Section Spinal Deformities and Malformations of oblique pelvis (Fig. 6). From a technical point of view, to improve our accuracy of the insertion of the iliosacral screws we identify and delineate the medial wall of the pedicle of S1 via a small laminotomy. We then identify our entry point on theoutertableoftheiliacbone,aimingjustabovethesacralalaanddowntheS1 pedicle, entering the vertebral body of S1. As one establishes their entry point on the iliac bone one must ensure that the screw will be superficial to the sacral ala, thus allowing some room for the laminar hook to pass underneath it and catch the iliosacral screw ( Case Study 1). Bone Grafting Allograft fusion is well accepted for fusion of neuromuscular scoliosis The general consensus is that an allograft is a well-accepted bone grafting substi- tute for spinal fusion in neuromuscular scoliosis [52]. Many factors have led to this consensus. In part the pelvises of neuromuscular patients tend to be small, never providing enough bone. Furthermore they are often used as a fixation point. It is therefore standard treatment to supplement a local bone graft (spi- nous process, facets and lamina) with an allograft. Anterior vs Posterior Surgery vs Combined Surgery The classic surgical management of neuromuscular scoliosis comprises a single posterior spinal fusion. Undertaking anterior spinal surgery has been associated with an increased morbidity especially in NMD patients [12]. Indications for anterior spinal surgery are threefold: skeletal immaturity rigidity of the deformity risk of non-union The literature remains unclear on the absolute indications because of the added morbidity. Patients at risk of crank- shafting should undergo additional anterior fusion The general principle is that patients who are at risk of a crankshaft phenome- non (i.e., progressive rotation of the anterior column around the fused posterior elements) after posterior fusion should undergo an anterior growth arrest and fusion. Keeping in mind that patients with neuromuscular disorders have altered growth patterns [16, 25], patients younger than 10 years of age, Risser 0, with open triradiate cartilage, and who have not yet reached their peak growth veloc- ity are at risk of crankshaft. It is recommended for these patients to proceed with an anterior spinal fusion if they can tolerate the surgical insult. Anterior release may be necessary for the correction of rigid deformity The second indication for anterior surgery is the need for an anterior release to allow the pelvis to be leveled. If one is unable to correct the pelvis manually by bringing it within 10° of the perpendicular of the trunk by applying external forces over the iliac crests and the trunk with the patient in a prone position with the legs hanging free in flexion, then it is recommended that an anterior release should be done or even an apical vertebrectomy considered. Curve flexibility can be assessed with traction films and supine bending films. However, in some cases of severe spasticity, only intraoperative examination and imaging with the patient under general anesthetic will provide curve flexibility ( Case Study 4). Patients at risk of non-union (e.g. myelodysplasia) should undergo interbody fusion Thirdly, anterior spinal fusion should be also considered when the risk of non- union is elevated. The typical example is that patients with myelomeningocele with deficient posterior spinal elements should systematically have an anterior interbody fusion [45]. The biology of posterior grafting remains in tension mode, while anterior grafting is in compression mode, which favors a solid fusion. Achieving solid anterior fusion can be crucial, as about half of myelome- ningocele patients with posterior spinal fusion [20] will develop a deep posterior Neuromuscular Scoliosis Chapter 24 685 ab c d e f Case Study 4 This is a 16-year-old boy with a T10 myelomeningocele with a progressive severe coronal and sagittal spinal deformity ( a–d). His deformity led him to have recurrent pressure sores over the gibbus, constant GI problems secondary to the increased abdominal pressure, as well as severe pulmonary restrictive disease. Surgical management required preopera- tive gravity halo traction and aggressive chest physiotherapy to minimize perioperative respiratory collapse. The patient then underwent a kyphectomy with a retroperitoneal extraperiosteal resection of the proximal kyphotic segment ( e) allowing a maximal distal fixation point. To minimize distal instrumentation, pull-out Dunn-McCarthy presacral rods were used supplemented with far lateral pedicle screws almost behaving as anterior vertebral screws. Once the proximal bone was excised (yellow shadow), the deformity was corrected in a cantilever maneuver closing the gap ( f) and correct- ing the deformity. 686 Section Spinal Deformities and Malformations g h Case Study 4 (Cont.) The patient then had an anterior structural tibial graft inserted via a thoraco-abdominal approach to ensure solid ante- rior spinal fusion across the residual kyphosis ( g, h). spinal infection with the possible necessity of hardware removal [27, 30, 38]. Finally, patients with severe kyphotic deformities requiring significant correc- tions should also have anterior structural bone grafting (tibia or ribs) to prevent the deformity from recurring. Itis preferable to achieve sagittal balance with nor- malization of the sagittal alignment but moderating the urge to overcorrect the kyphosis. Single anterior only surgery is indicated only in minor curves without the need for sacropelvic fixation Single anterior only spine surgery canbedoneforspecificcurvepatternsand patients with specific contraindications to posterior surgery, i.e., chronic infected wounds. The surgical indications that Sponseller recommends for ante- rior spinal fusion in myelomeningocele are: a relatively small supple curve of less than 70 degrees with no need to extend the fusions down to the pelvis [44]. If combined anterior and posterior surgery is required, the ideal timing of the anterior surgery is still controversial [10]. Anterior surgery can be done on the same day or staged with a period of halo traction, achieving some gradual cor- rectionovertime.Gravity halo traction [5] and intraoperative halo femoral traction [17] are options. Irrespective of the type of traction, close neurological examination including cranial nerve testing, muscle strength in the upper and lower extremities, sensory examination and long tract signs is mandatory to avoid injury to the spinal cord. Complications in staged surgery have been found tobehigherandsomeadvocatesamedayfrontandbacksurgery[10]. Severe Rigid Spinal Deformities Some of the neuromuscular spinal deformities can be severe, and particularly rigid spinal osteotomies, vertebrectomies, or even kyphectomies may be required to rebalance patients. When one needs to proceed to a kyphectomy, the neuromuscular kyphoscoliosis has reached its end stage disease and is an exam- Neuromuscular Scoliosis Chapter 24 687 ple of what can happen with neuromuscular curves. The severe spinal deformity canleadhasledtosignificantlossofspinalcolumnheight,resultinginsignifi- cant disability and morbidity. This child’s kyphotic deformity was not addressed at an early age, as there was a false perception that delaying surgical manage- ment would allow for better pulmonary function. The problem is that kyphosis will always progress in this population and that the complexity of the case will only increase. There are two types of kyphosis in myelomeningocele. The more classic collapsing C shape type kyphosis that can be addressed by pedicle sub- traction type osteotomies [27] is classically performed in the newborn and young infant by removing the ossific nuclei. The second type is described as Rigid S shape kyphosis requires spinal column resection a so-called rigid S shape kyphosis [21] due to the associated thoracic lordosis above the lumbar kyphosis. To address such a deformity a spinal column resec- tion is required. When planning a spinal resection, one must achieve solid fixation above and below the resection. Distal fixation can be problematic if distal vertebrae have been resected, thus keeping as many distal spinal vertebrae as possible, to maxi- mize distal spinal anchorage points. Pelvic/sacral fixation is best achieved with a modified Dunn-McCarthy presacral rod [28] augmented with pedicle screws in the most distal vertebral bodies. The entry points for these screws tend to be much more lateral (in the remnant pedicle) and must converge much more than theusualpediclescrews.AstheDunn-McCarthyrodsareanteriortothesacrum and sacral alae, one is able to exert a significant corrective force across an osteo- porotic pelvis and sacrum. With such a construction one is able to flex in a canti- lever fashion the distal spine and pelvis, thus correcting the deformity. Proximal fixation can be performed with sublaminar wire, hooks or pedicle screws. Shar- rard first described this as apical vertebral resection [43]. We tend to identify and isolate the dural sac [40]. If it poses a physical barrier to our dissection, we ligate the sac and transect the cord; however, we prefer to spare it by mobilizing it and then transecting the roots. We then proceed in an extraperiosteal dissection just as one would do a classic anterior approach. We identify the disc levels, then, by Apical vertebral resection is technically very demanding and associated with significant blood loss using a blunt dissection we reflect the great vessel and the peritoneum off of the spine from either side. We then ligate the segmental vessels, and reflect anteriorly the peritoneum and the abdominal contents. This is facilitated by the prone posi- tion as the abdominal contents fall forward. Once the vertebrae identified have been circumferentially dissected, we place blunt retractors around the spine and proceed to cut the vertebra at a bony surface with an oscillating saw above and below the planned resected spine, thus providing bony apposition. As one does this, significant blood loss is encountered, and it persists until the two ends of the vertebrectomy are reapproximated. Therefore the spinal anchorage points must already be in place and the actual kyphectomy is done last ( Case Study 4). Spinal Cord Monitoring Spinal cord monitoring remains mandatory though not always feasible In patients with neuromuscular scoliosis, one sometimes cannot have any form of intraoperative spinal monitoring due to inadequate somatosensory evoked potential (SSEP) or even motor evoked potential (MEP) and one must rely on the Stagnara wake-up [14, 50]. Sometimes, the wake-up test is also not feasible if patients are uncooperative. The wake-up test is often unreliable In such situations we tend to keep all our instruments sterile on the back table untilwellafterthesurgeryhasendedanduntilthepatienthasmovedalllimbs. If there is a problem then we do not need to wait for the resterilization of the instruments and proceed to immediate hardware removal or decrease the amount of correction. 688 Section Spinal Deformities and Malformations Recapitulation Epidemiology. Scoliosis, in the presence of a neuro- muscular disorder (NMD), behaves entirely differ- ently than the more predictable idiopathic scolio- sis. The overall incidence of spinal deformity varies between underlying NMDs but it also varies accord- ing to the severity of the underlying NMD. In gener- al, the greater the neuromuscular involvement, the greater the likelihood of having a spinal defor- mity and the greater the deformity will be. Pathogenesis. The pathophysiology of neurogenic spinal deformities remains unclear. Clinical presentation. The classical spinal deformi- ties encountered in NMD consist of kyphoscoliosis, scoliosis, kyphosis, lumbar hyperlordosis and pelvic obliquity. On taking the history one needs to find clues, which may confirm the presence of neuromus- cular scoliosis. Clues suggestive of neuromuscular scoliosis are birth anoxia, delayed developmental milestone, acquired or familial neuropathies and/or myopathies, spinal deformity before the age of 7 years, or a painful scoliosis. A systemic examination is mandatory of head to toes and further clues can be found confirming the presence of neuromuscular spi- nal deformity. Neurocutaneous skin markings such as hairy patches or midline nevi (or vascular lesion) can be superficial clues to intradural pathologies. If pelvic obliquity is present, one should assess whether its or- igin is: suprapelvic, intrapelvic, or infrapelvic. Dubo- usset saw the pelvis as the 6th lumbar vertebra and the pelvis being a simple extension of the scoliotic deformity resulting in pelvic obliquity. In contrast, in- frapelvic obliquity is secondary to hip contractures, whichresultinpelvicobliquity.Thecontractures, which drive the pelvic obliquity, tend to be abduction or adduction hip contractures. It is crucial to know if the patient is a walker, sitter (wheelchair bound) or non-sitter. In the walker, one must determine gait pattern and mode of ambulation, as it determines the extent of instrumented fusion (whether or not to in- clude the pelvis). The neurological examination needs to be thorough: Flaccid faces can be sugges- tive of subtle myopathies while asymmetrical shoe size can be a subtle sign of syringomyelia. Diagnostic work-up. Confirming the diagnosis of neuromuscular scoliosis is best done in multidisci- plinary fashion by including the neurologist and geneticist. Patients with neuromuscular scoliosis tend to have severe deformities with associated pa- thologies that are directly or indirectly related to their spinal deformity that puts them at higher risk of morbidity and mortality. The onus is on the treating surgeon to exclude hidden pathologies that can worsen the deformities as well as harm the general health of the patient. Pulmonary function less than 35% of predicted is associated with a pro- tracted postoperative course with an increased risk of ventilation dependency. Cardiac dysfunctions can be seen in the muscular dystrophic patients. A large proportion of patients with neuromuscular scoliosis have concomitant dietary problems lead- ing to malnutrition which may require supplemen- tation. Part of the preoperative imaging, supine bending films and/or traction films should be ob- tained to guide surgical planning. Any scoliotic pa- tients with a hint of neurological signs or symptoms or with neuroectodermal skin lesions must have an MRI scan of the entire spine taken (occiput to sa- crum) to assess any presence of intradural lesions: syringomyelia, tethered cord, or spinal tumor. Non-operative treatment. The natural history of neuromuscular spinal deformity is one of curve pro- gression irrespective of etiology. Factors influencing curve progression are as follows: age of onset of NMD, severity and rapidity of weakness, evolving or static neuromuscular disease, skeletal maturity, am- bulation status, and severity of curves. Their curve progression has been reported to be from 7° to 40° per year. In patients with cerebral palsy, because their onset of puberty is highly variable (8 –20 years), it is difficult to quantify the risk of curve progression and it has been shown that their scoliosis does progress into adulthood. Bracing for neuromuscular scoliosis is “functional bracing”. It provides an external sup- port to the spine, allowing some patients to be more functional. Bracing has not been shown to prevent curve progression in the neuromuscular scoliosis. Operative treatment. In contrast to idiopathic sco- liosis, neuromuscular deformities tend to alter the patient’s functional status by interfering with their ability to sit, stand, and walk. This loss of function is the more common indication to proceed with sur- gical management as all of these curves progress. One must prepare for and expect longer surgical times with greater blood loss. Surgical planning is crucial not to miss the associated sagittal deformi- ties. The majority of these patients will need the postoperative intensive care unit mainly to monitor Neuromuscular Scoliosis Chapter 24 689 for fluid shift and respiratory status. The cornerstone of the surgical management of these types of curve is to achieve perfect spinal balance both in the coronal and sagittal planes. Classically these patients do not have compensatory mechanisms (muscle tone, intact proprioception) to rebalance themselves. Their curves tend to be long and they often have associated pelvic obliquity necessitating long fusions to the pelvis. Treating neuromuscular- spinal deformity requires a vast knowledge of pelvic and spinal fixation techniques such as the Luque- Galveston techniques, unit rods, and MW pelvic fix- ation. One should apply all the modern principles of spinal deformity correction to these cases in order to minimize the extent of the approach, to maximize their postoperative function (walking capacity or sit- ting balance) and to achieve a successful outcome with no postoperative immobilization. Key Articles Mazur J, Melelaus MB, Dicksen DR, et al. (1986) Efficacy of surgical management for scoliosis in myelomeningocele: correction of deformity and alteration of functional sta- tus. J Pediatr Orthop 6:568 Paper summarizing the impact of spinal surgery on the myelomeningocele patient. Askin G, Hallett R, Hare N, Webb JK (1997)Theoutcomeofscoliosissurgeryinthe severely physically handicapped child: An objective and subjective assessment. Spine 22(1):44 – 50 A broad summary of the subjective impact of spinal surgery on patients with neuromus- cular scoliosis. Lonstein J, Akbarnia B (1983) Operative treatment of spinal deformities in patients with cerebral palsy or mental retardation. J B one Joint Surg Am 65:43 – 55 Landmark paper providing insight into management of neuromuscular scoliosis. Winter S (1994) Preoperative assessment of the child with neuromuscular scoliosis. Orthop Clin North Am 25:239 – 2 45 Thorough review and clear recommendations for preoperative work-up of patients with neuromuscular scoliosis going for surgery. The following papers describe surgical techniques for pelvic fixation, which are required for management of spinal surgery in this patient population: Allen BL, Ferguson RL (1984) The Galveston technique of pelvic fixation with L-rod instrumentation of the spine. Spine 9(4):388 –94 This article describes the classic sacral fixation technique in neuromuscular scoliosis. Bulman W, Dormans J, Ecker M, et al. (1996) Posterior spinal fusion for sc oliosis in patients with cerebral palsy: a comparison of Luque rod and unit rod instrumentation. J Pediatr Orthop 16:314 – 323 In this study the results of 15 patients who underwent arthrodesis with dual Luque rod instrumentation are compared with the results of 15 patients in whom unit rod instru- mentation was used. The unit rod instrumentation allowed a significantly greater correc- tion of both the major curve and pelvic obliquity. McCarthy RE, Bruffett WL, McCullough FL (1999) S rod fixation to the sacrum in patients with neurom uscular spinal deformities. Clin Orthop Relat Res 364:26 – 31 This article describes anewform ofpelvic fixationforusein patientswith neuromuscularspi- nal deformities to overcome the problems imposed by the Galveston technique. One end of a Luque rod is prebent into an S-shaped configuration and placed over the sacral ala, supply- ing firm fixation acrossthe lumbosacral junction withoutcrossing thesacroiliac joint.It fixes firmly against the sacral ala by distracting against a hook or screw in the lumbar spine Arlet V, Marchesi D, Papin P, Aebi M (1999) The ‘MW’ sacropelvic construct: an enhanced fixation of the lumbosacral junction in neuromuscular pelvic obliquity. Eur Spine J 8(3):229 – 31 The authors introduce a new fixation system, in which iliosacral screws are combined withiliacscrews.ThisismadepossiblebyusingtheAOUniversalSpineSystemwithside opening hooks above and below the iliosacral screws and iliac screws below it. The whole sacropelvisisthusencompassedbyamaximumwidth(MW)fixation,whichgivesan’M’ appearance on the pelvic radiographs and a ‘W’ appearance in the axial plane. 690 Section Spinal Deformities and Malformations References 1. Allen BL, Ferguson RL (1984) The Galveston technique of pelvic fixation with L-Rod instru- mentation of the spine. Spine 9(4):388–94 2. Arlet V, Marchesi D, Papin P, Aebi M (1999) The ’MW’ sacropelvic construct: an enhanced fixation of the lumbosacral junction in neuromuscular pelvic obliquity. Eur Spine J 8(3):229–31 3. Askin G, Hallett R, Hare N, Webb JK (1997) The outcome of scoliosis surgery in the severely physically handicapped child: An objective and subjective assessment. Spine 22(1):44–50 4. 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Lonstein J, Akbarnia B (1983) Operative treatment of spinal deformities in patients with cerebral palsy or mental retardation. J Bone Joint Surg Am 65:43–55 23. Luhmann SJ, Lenke LG, Kim YJ, Bridwell KH, Schootman M (2005) Thoracic adolescent idi- opathic scoliosis curves between 70 degrees and 100 degrees: is anterior release necessary? Spine 30(18):2061–7 24. Luque E (1982) Segmental spinal instrumentation in correction of scoliosis. Clin Orthop 163:192–198 25. Majd ME, Muldowny DS, Holt RT (1997) Natural history of scoliosis in the institutionalized adult cerebral palsy population. Spine 22:1416–1466 26. Marchesi D, Arlet V, Stricker, Aebi M (1997) Modification of the original Luque technique in the treatment of Duchenne’s neuromuscular scoliosis. J Pediatr Orthop 17(6):743–9 27. Mazur J, Melelaus MB, Dicksen DR, et al. (1986) Efficacy of surgical management for scolio- sis in myelomeningocele: correction of deformity and alteration of functional status. 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Yazici M, Asher M (1997) Freeze-dried allograft for posterior spinal fusion in patients with neuromuscular spinal deformities. Spine 22:1467–1471 692 Section Spinal Deformities and Malformations . Section Spinal Deformities and Malformations of oblique pelvis (Fig. 6). From a technical point of view, to improve our accuracy of the insertion of the iliosacral screws we identify and delineate. found tobehigherandsomeadvocatesamedayfrontandbacksurgery[10]. Severe Rigid Spinal Deformities Some of the neuromuscular spinal deformities can be severe, and particularly rigid spinal osteotomies, vertebrectomies,. of neuromuscular spinal deformity is one of curve pro- gression irrespective of etiology. Factors influencing curve progression are as follows: age of onset of NMD, severity and rapidity of weakness,