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Neuromuscular Scoliosis Technical Monograph Intra-operative Techniques for Complex Deformities in a Diverse Patient Population Contents Author Title of Chapter 1. Betz, Randal Neuromuscular Scoliosis: Introduction 2. Shah, Suken Sacropelvic Fixation Techniques Page 1-4 5-16 3. Betz, RandalUnique Challenges with Scoliosis and Dislocated Hips 17-24 4. Dabney, Kirk The Correction of Pelvic Obliquity Using Cantilever Correction 25-32 5. Shah, Suken Correction of Pelvic Obliquity with an All Screw Construct 33-44 6. Dabney, Kirk Scoliosis in Cerebral Palsy 45-56 7.Gabos, Peter Spinal Fusion in Myelomeningocele 57-64 8. Samdani, Amer Spinal Cord Injury: Surgical Considerations Particularly with Respect to Sagittal Plane 65-72 9. Mackenzie, William Surgical Management of Neuromuscular Spinal Deformity – Muscular Dystrophy & Spinal Muscular Atrophy 73-80 10. Cahill, Patrick Complications in Neuromuscular Scoliosis Surgery 81-88 Neuromuscular Scoliosis: Introduction Randal R. Betz, MD Neuromuscular scoliosis is a very common condition, but it presents the orthopaedic surgeon or spine surgeon with many treatment challenges. The patient population is diverse, and the deformities can be complex. Most surgeons have developed their surgical treatment plans and intraoperative techniques through experience. While many different treatments work in a given surgeon’s hands, there is no one place that a younger surgeon or an experienced surgeon presented with a previously unseen deformity can go to read and gain knowledge from the vast experience of experts in the field. 1 Neuromuscular scoliosis is a very common condition, but it presents the orthopaedic surgeon or spine surgeon with many treatment challenges. The patient population is diverse, and the deformities can be complex. Most surgeons have developed their surgical treatment plans and intraoperative techniques through experience. While many different treatments work in a given surgeon’s hands, there is no one place that a younger surgeon or an experienced surgeon presented with a previously unseen deformity can go to read and gain knowledge from the vast experience of experts in the field. The purpose of this monograph is to present in a short, concise fashion some of the experiences of master surgeons, including key aspects of their surgical technique, ways to avoid problems, and how to deal with complications. The authors were instructed to assume that the reader has a significant knowledge base and some experience treating paralytic spine deformities using basic instrumentation. This monograph will highlight the key components of the surgeons’ techniques in treating neuromuscular scoliosis arising from different diagnoses. A key component of correction of neuromuscular scoliosis is that the instrumentation in the majority of cases extends down into the sacrum and the ileum. Multiple options for fixation will be presented in a chapter by Dr. Suken Shah, including the use of the S2 screw (S2AI screw fixation) developed by Drs. Sponseller and Kebaish. Additional chapters will expand on the use of the sacropelvic fixation to include correction of pelvic obliquity through the use of cantilever maneuvers via unit rods (by Dr. Kirk Dabney) and using an all screw construct (Dr. Shah). The new EXPEDIUM® Sacropelvic Collection provides a considerable assortment of fixation options into the sacrum and ileum, along with lateral connectors (closed, open, and polyaxial) to help facilitate fixation into this region. Drs. Dabney and Miller have extensive experience correcting pelvic obliquity purely by coronal cantilever maneuvers. In one chapter, Dr. Dabney will discuss the use of the unit rod as well as the precontoured rods and proximal connectors from the new EXPEDIUM Neuromuscular System. These pre-contoured rods and connectors mimic a u-rod to help correct the coronal deformity and pelvic obliquity. The use of distraction and compression alone with an all screw construct is not enough to correct pelvic obliquity; correction may require derotating the lumbar and thoracolumbar spine with the use of pedicle screws as described in the chapter by Dr. Amer Samdani. 2 While not discussed specifically in this monograph, many surgeons are now using halofemoral traction to correct some of the pelvic obliquity before a surgical incision is made. The surgeon should keep in mind that the use of halofemoral traction may be counterproductive in cases of hyperlordosis because the hips must be kept flexed to correct the hyperlordosis, and pulling with femoral traction is counterproductive. The presence of subluxed or dislocated hips in the patient with neuromuscular scoliosis presents unique challenges. The majority of neuromuscular disorders that include subluxed or dislocated hips usually fall into one of three diagnoses, which include 1) spinal cord injury; 2) myelomeningocele; and 3) cerebral palsy. However, the highest prevalence of dislocation is probably caused by sepsis, and it is essential that any relatively acute, radiographically identified dislocated hip have an aspiration and adequate work-up to rule out sepsis. Generally, the spine deformity is treated first followed by the hip, for reasons explained in Chapter 3 by Dr. Randal Betz which include the high incidence of deep vein thrombosis if the hip surgery were to be performed first. Perioperative medical management, for prevention both of deep vein thrombosis and heterotopic ossification about the hip following surgery, presents additional challenges for the spine surgeon. Spinal deformity secondary to cerebral palsy presents unique issues with regards to a hyperlordotic spine. Dr. Dabney will present some of his correction techniques and how he capitalizes on some of the technical advances such as reduction pedicle screws. Patients with myelodysplasia and spine deformity present their own challenges. Dr. Peter Gabos will provide suggestions on three major things that have changed his practice and resulted in significant improvements for these patients: 1) He includes PLIFs (posterior lumbar interbody fusions) where there are no lamina; 2) he uses S2 screws (S2AI screw fixation) to provide a low profile but good fixation in the pelvis; and 3) he enlists the help of plastic surgeons to complete the closure to help reduce the high infection rate. Dr. Samdani will describe treatment of patients with spinal cord injury and matters to consider during preoperative planning. Many of these patients use compensatory spinal motion to assist with getting their hand to their mouth/face for feeding/grooming. Placing them in a standard prefixed sagittal profile (unit rod) may eradicate this compensatory motion and render these patients completely dependent, whereas they were independent prior to the spinal fusion. These patients may need a different sagittal profile emulating their normal sitting posture in a wheelchair which requires a reduced lumbar lordosis and enhanced thoracolumbar kyphosis. Dr. Samdani will also describe how to assess the sagittal profile preoperatively and execute it intraoperatively. Dr. William MacKenzie will discuss the needs of patients with muscular dystrophy. Many of these patients are much smaller at the time of spinal correction and fusion and are less tolerant of the massive blood loss. The necessity of fusing or not fusing to the pelvis is discussed. Finally, a chapter by Dr. Patrick Cahill will discuss some of the unique complications that can occur in patients with neuromuscular scoliosis. The most common and more devastating for the patient is wound infection, and he discusses suggestions for prevention and treatment based on evidence from the literature. The authors are very appreciative of the opportunity to collectively incorporate our experience into this monograph. We thank Ines Troconis Merolli for her support and DePuy Synthes Spine for financially backing this important educational contribution. We also thank Carolyn Hendrix for her editorial assistance with this project. 3 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-60-000 9/2012 JC CA #8780 / DJ10051A Sacropelvic Fixation Techniques Suken A. Shah MD Indications for sacropelvic fixation include long spinal fusions for scoliosis, high grade spondylolisthesis, pelvic obliquity correction, sagittal plane deformity correction, sacral fractures and lumbosacral fusions in patients with poor bone quality and osteoporosis. Each of these indications requires strong distal fixation to resist significant flexion moments and cantilever forces in this transitional area. Despite many advances and developments in spinal instrumentation techniques, fixation failure at the lumbosacral junction continues to be a challenge. Due to the significant biomechanical forces across this junction and relatively poor sacral cancellous bone quality, fusion at the lumbosacral junction has been associated with rates of pseudarthrosis as high as 33-39%,1-2 loss of lordosis1-2 and instrumentation failure, especially with historical methods like body casting, Harrington and Luque instrumentation. Cotrel-Dubousset (CD) instrumentation and the Galveston technique were introduced in the 1980’s and although pseudarthrosis rates were lower, there were instrumentationrelated complications with the CD system and technical difficulties with rod contouring, insertion in the ilium and rod loosening with the Galveston technique. 5 Biomechanics Techniques of Sacropelvic Fixation McCord, et al pointed out the importance of the lumbosacral pivot point, which was defined as the point at the middle osteoligamentous column between the last lumbar vertebra and the sacrum. The further the implants extend anterior to this point, the greater the stiffness of the construct. Various instrumentation models (iliac fixation, S1 fixation, and S2 fixation) were tested. The two constructs that withstood the greatest load before failure had caudad iliac (rod or screw) fixation and they concluded that crossing the sacroiliac joint is warranted if instrumentation extends anterior to the pivot point.3 Various contemporary techniques of sacropelvic fixation have been described to achieve better fusion rates, improve bone purchase, neutralize forces, avoid pull-out, ease difficulty and reduce complications. In this chapter, I will cover the key components of surgical techniques for the majority of the sacral and iliac fixation options. This will include the following: 1) Galveston and unit rod technique, 2) Iliac bolts/screws with and without offset connections to the longitudinal rods, 3) Double iliac bolts and 4) S2AI screw fixation. O’Brien identified three distinct zones of the sacropelvic region. Zone 1 consists of the S1 vertebral body and the cephalad margins of the sacral alae; Zone 2, the inferior margins of the sacral alae, S2, and the area extending to the tip of the coccyx; and Zone 3, both ilia. Fixation strength improves progressively from Zone 1 to Zone 3. Zone 3 offers the greatest biomechanical fixation strength to counter the pull-out forces and bending moments at the lumbosacral junction. Also, in agreement with McCord, iliac fixation with pelvic screws or iliac rods allows placement of implants more anteriorly beyond the lumbosacral pivot point than any other implant type.4 Lebwohl and colleagues performed a biomechanical comparison of lumbosacral fixation in a calf spine model and noted that supplementary fixation distal to S1 pedicle screws provides a benefit over S1 fixation alone, and iliac fixation was superior to a second point of fixation in the sacrum.5 Cunningham, et al studied ex vivo porcine spines biomechanically to ascertain the value of anterior column support compared with that of iliac fixation in lumbosacral fusions. When tested to failure, the authors found that iliac screws significantly reduced lumbosacral motion, particularly with axial rotation, flexion-extension, and lateral bending. Iliac fixation was found to be more protective of S1 screws and more resistive of motion than anterior interbody cages.6 However, other investigators have argued in favor of anterior column support, particularly at L4-L5 and L5S1, in long fusions to the sacrum.7,8 Anterior lumbar interbody fusions place the bone graft ventral to the instrumentation and the lumbosacral pivot point, and the graft is placed in compression to optimize fusion and stability.8 This procedure improves the overall chance of fusion, decreases the strain on caudal pedicle screws and has a definite role in long fusions to the sacrum, especially in adults and other patients at risk for pseudarthrosis. 6 1. Galveston and Unit Rod Technique The Galveston technique allows for the incorporation of the ilium into the foundation of the construct via the insertion of rods between the inner and outer tables of the ilium, which provides a broader base and a more biomechanically advantageous position.1,3 The transverse portions of the rods are inserted under a large muscle flap and enter the ilium at the posterior superior iliac crest. The orientation is approximately 30° to 35° caudally and 20° to 25° laterally. The rods cross the SI joint, and contouring can be difficult.9 The technique lowers the pseudarthrosis rate of long fusions to the sacrum10 but it is also associated with a moderate incidence of loosening secondary to micromotion at the rod tips within the ilium, despite lumbosacral fusion.11 Radiographically, this is described as a windshield-wiper effect and may be associated with pain and the need for implant removal.11,12 However, in our long term experience of unit rod fixation in cerebral palsy patients, this has not been a common, symptomatic issue requiring re-operation. Lonstein and colleagues recently published their results and complications of 93 patients with cerebral palsy and scoliosis who underwent PSF with Luque-Galveston instrumentation with an average follow up of 3.8 years.12 Coronal curve correction was 50% and pelvic obliquity correction was 40% at latest follow-up. The late complication rate was 47% and included the windshield wiper sign, junctional kyphosis, pseudarthrosis (7.5%) and implant problems including breakage, dislodgement and prominence. Seven patients required reoperation, most commonly for pseudarthrosis and/or failed implants. The unit rod, by virtue of its pre-contoured unibody construction, provides rigid control of spinal deformities involving pelvic obliquity and allows for a cantilever mechanism to correct the pelvic obliquity and the scoliosis simultaneously.13 The rods are available in various lengths with corresponding thoracic, lumbar and pelvic contours. Our specific technique of unit rod insertion follows. At the inferior margin of the incision, the outer wing of the ilium is subperiosteally exposed down to the sciatic notch and sponges are packed out over the pelvis to maintain hemostasis. The right and left drill guides for the unit rod are placed in the respective sciatic notch; care should be taken to ensure that the drill guide is as inferior as possible along the posterior superior iliac spine (PSIS). The handles of the drill guide are the reference points for alignment: the lateral handle should be parallel with the pelvis and the axial handle parallel with the sacrum. Next, the drill hole is made utilizing the guide using a 3/8” drill to the predetermined depth directed towards the anterior inferior iliac spine (AIIS); the hole is then palpated with a ball-tipped feeler to confirm that there has been no breach of the cortical bone of the inner or outer pelvic table. Alternatively, after establishing the landmarks, the pedicle gearshift can be used to cannulate the cancellous bone of the iliac pathway, either freehand or with fluoroscopic guidance. Gelfoam should be inserted into the drill holes to control cancellous bone bleeding. After the proper length unit rod is selected, the pelvic limbs of the rod are crossed and inserted into their respective drill holes. Each limb should be advanced alternately in 1cm increments with an impactor. Care must be taken to maintain control of the rod and ensure that it does not penetrate either table of the pelvis. In the setting of hyperlordosis the marked anterior inclination of the pelvis increases the risk of the pelvic limb perforating the inner cortex during insertion. The pelvic ends of the rod need to be directed in a more posterior direction to accommodate this angulation; rod placement is facilitated by manual correction of the lordosis prior to rod insertion. In instances of marked lordosis, the pelvic limbs of the rod may be cut and inserted separately and then attached to the rod with rod-to-rod connectors. Figure 1A Figure 1C Figure 1B In our large series of surgical correction of scoliosis patients with cerebral palsy using unit rod instrumentation, we reported an average coronal curve correction of 68% and pelvic obliquity correction of 71% with a very cost-effective implant system (see Figure 1). Intraoperative complications with pelvic fixation occurred in some patients with sagittal plane deformities; lumbar hyperlordosis was a risk factor. Late postoperative complications occurred in 12 patients: 3 pseudarthroses, 3 deep infections and 6 prominent proximal implant issues. Although the unit rod provides excellent correction of pelvic obliquity and resistance to flexion moments, there is little resistance to axial pullout and torsion by virtue of the rods being smooth and immediate micromotion of the iliac construct after insertion. These concerns can be mitigated by adding a transverse connector distally to improve torsional rigidity and adding lumbar pedicle screws distally at L5 to significantly improve axial pullout, strength and stiffness.14 Figure 1D Figure 1. A,B) Preoperative sitting X-rays of a patient with severe thoracolumbar scoliosis and pelvic obliquity. C,D) Postoperative sitting X-rays of the patient after posterior spinal fusion with the unit rod. 7 2. Iliac Screws The difficult learning curve associated with rod contouring and insertion into the ilium using the Galveston technique has been resolved with the use of iliac screws, which permits screws of variable length and diameter to be inserted into each ilium separately. Simpler fixation to the pelvis with screws may also mitigate the potential complications of the Galveston technique, which was up to 62% in one series and 47% in Lonstein’s series.12 The screws can then be connected to the main construct by various connectors. This technique has simplified the process of obtaining iliac fixation, especially in patients with significant pelvic asymmetry or hyperlordotic lumbar deformities while improving pullout strength through better interdigitation of the threaded implant within the iliac cortical and cancellous bone.15 When placing iliac screws in lieu of smooth Galveston pelvic fixation, the starting point is similar, 1-2cm inferior to posterior superior iliac spine. To decrease implant prominence, a notch in the ilium can be used to bury the head of the screw below the contour of the cortical bone. A pedicle gearshift or drill is used to cannulate the cancellous bone between the inner and outer tables of the ilium, directed toward the anterior inferior iliac spine, 1cm above the sciatic notch. Careful attention should be made not to pass the probe down the Sacroiliac (SI) joint, taking the time to ensure that the pathway is between the cortical walls of the ilium. The pathway is then palpated with a ball tipped-probe and tapped to increase purchase and size the diameter and length of the screw. The exposure of the PSIS can be made through the same incision, elevating the paraspinal lumbosacral musculature and soft tissues, with a subperiosteal dissection of the PSIS and a portion of the outer table or through a separate, small oblique fascial incision by retracting the skin over the iliac crest. Alternatively, using a technique described by Wang and colleagues16 with the VIPER® Screw System, iliac screws can be inserted with a fluoroscopicallyguided percutaneous technique to avoid the morbidity and complications associated with such a large muscle dissection and soft tissue devitalization. Every effort should be made to insert the largest diameter, longest screws that can be safely accommodated to achieve favorable biomechanics. Screws should extend past McCord’s sacropelvic pivot point at the anterior edge of the L5/S1 disk at a minimum.3 The further anteriorly and laterally the screws extend, the better control of pelvic flexion, extension, obliquity and rotation. Even in smaller patients, we have been successful in implanting screws of 7.5-8.0mm in diameter and 65-80mm in length. Larger patients can accommodate screws of up to 10mm x 100mm. Large taps are available to enlarge the entry 8 site and size the screws by using insertional torque. These various options are all available in the EXPEDIUM® Neuromuscular System and the Sacropelvic Collection in closed and open screw (with the Top Notch® feature) head options along with offset connections that will allow modular, rigid connections to the longitudinal rods up to the spinal implants. Since the typical iliac pathway starting at the PSIS is 1-2cm lateral to the pedicular line, offset connectors are frequently needed. Options in the set are fixed or variable axis, open (with the Top Notch feature) or closed connectors (see Figure 2) that can be customized for length or short-throw rod contour and attached to the rod with slip strengths equal to EXPEDIUM pedicle screw connections (see Figure 3). Alternatively, the longitudinal rods can be contoured laterally in the coronal plane distal to the L5 or S1 screws to connect directly to the iliac screws either prior to rod insertion, or with in situ benders. A retrospective, single-center cohort study comparing two groups of 20 patients (flaccid and spastic paralytic scoliosis) each with Luque-Galveston constructs and iliac screws showed similar maintenance of pelvic obliquity and scoliosis correction, but the iliac screw technique avoids the complex lumbosacral 3-dimensional rod bends and had less haloing around the pelvic implants with minimal implant complications. The Galveston group had 4 broken rods and two reoperations and the iliac screw group had 1 broken screw and no reoperations. A larger, multicenter retrospective study of 157 patients with virtually equal distribution compared the unit rod to rods bent by surgeons intraoperatively with iliac screw fixation and found that the unit rod had better correction of pelvic obliquity. However, the drawbacks included longer mean surgical time, more blood loss, longer hospital stay, higher infection rate and more proximal fixation problems cited in the unit rod patients. Specific mention was also made of the challenges of unit rod implantation in a hyperlordotic patient. Figure 2. Various open and closed offset connections are useful to make the lateral translation from the longitudinal rods to the traditional iliac screw pathway. Figure 3A Figure 3B Figure 3C Figure 3D Figure 3. A,B) Preoperative sitting X-rays of a patient with severe thoracolumbar scoliosis and pelvic obliquity. C,D) Postoperative sitting X-rays of the patient after posterior spinal fusion with precontoured rods and proximal transverse connector from the Expedium Neuromuscular System and iliac screws with offset connectors from the EXPEDIUM Sacropelvic Collection. 9 3. Double Iliac Screws 4. S2 Alar Iliac (S2AI) Screws for Pelvic Fixation Occasionally, double iliac screw fixation is needed to overcome challenges of osteoporosis, fractures involving the sacropelvis or tumor resections/reconstructions to impart better biomechanical stability. An overview of the technique and experience in patients with neuromuscular scoliosis is available in the literature. Two screws may be placed on each side in the ilium (a total of four in the pelvis), connected by a rod and then subsequently to the longitudinal members with an offset connector. This technique seems to decrease complications such as rod disengagement, distal screw failure and implant failure cephalad to the pelvis. The technique involves a muscle splitting approach to the standard PSIS starting point, exposure of the outer table of the pelvis to the sciatic notch to identify the trajectory and cannulation of the cancellous bone between the inner and outer tables, aiming for the AIIS. The first screw is placed approximately 2cm superior to the PSIS with a second screw placed 2cm further superiorly. The S2AI technique, described by Kebaish and Sponseller,17-20 has become my preferred technique for various reasons. Since the starting point is 1.5cm deeper than the traditional iliac entry from the PSIS, decreased implant prominence is the main advantage. The starting point and consequently, the screw head, is in line with L5 and S1 pedicle screws, thus avoiding an offset connection from the longitudinal rod (see Figure 4); this point is easily found, requires little muscle dissection and can even be performed in a minimally invasive fashion16,18 (see Figure 5). The starting point for the screw is 2-4mm lateral and 4-8mm inferior to the S1 foramen; minimal muscle stripping and dissection is needed (see Figure 6). A sharp awl or burr is used to mark the starting point and penetrate the cortical bone. Then a drill or pedicle gearshift is used to enter the cancellous bone of the sacrum directed towards the dorsal aspect of the sacro-iliac joint, into the ilium. I find palpation of a point just above the ipsilateral greater trochanter of the proximal femur as a valuable virtual target (see Figure 7). The trajectory is lateral (approximately 40 degrees to the horizontal plane) and 20-30 degrees caudal (this depends on pelvic tilt) and fluoroscopy is helpful to guide this trajectory. The AP projection shows the pelvis and sciatic notch. The teardrop view helps ensure that the pathway is in the thickest part of the ilium without a cortical breach (see Figure 8). Once in the ilium, the pathway is 1-2cm above the sciatic notch directed towards the anterior-inferior iliac spine (see Figure 9). A polyaxial screw, typically 80-100mm long, is used and adults can accommodate diameters of 8-10mm; screw diameters of 7-8mm and lengths of 65-80mm can be used in smaller patients (see Figure 10). Figure 4A Figure 4B Figure 4. A) The S2AI pathway allows the iliac screw to line up with the lumbar and sacral pedicle screws so no offset connection is needed when securing the longitudinal rods. B) Intraoperative photo of the screw arrangement and alignment. The iliac screw is noted by the arrowhead. 10 Figure 5A Figure 5B Figure 5. A,B) Intraoperative photos illustrating use of a cannulated gearshift from the VIPER SAI Screw Set to cannulate the S2AI pathway under fluoroscopy and then use of a guidewire to tap and implant the SAI screw. Minimal muscle dissection is required and the technique is very efficient. C) illustration of guidewire placement in the S2AI pathway. Figure 5C Figure 6. The starting point of the SAI screw is a point between the S1 and S2 foramen, along the lateral border, inline with an S1 pedicle screw. Figure 7. From the starting point, one should aim for the AIIS, palpating the tip of the ipsilateral greater trochanter. 11 Figure 8A Figure 8B Figure 8C Figure 8. A-C) Fluoroscopy can be helpful in confirming the proper trajectory. The C-arm should be angled 20-30 degrees caudal (depending on the degree of pelvic tilt) and 40-50 degrees rotated in the horizontal plane to visualize the pelvic pathway, the iliac teardrop and direction of the implant. Figure 9. The trajectory should be 10-20mm superior to the sciatic notch aiming towards the AIIS. The iliac teardrop is formed by the medial iliac wall, lateral iliac wall and sciatic notch inferiorly. If the implant is contained in this teardrop view and confirmed, cortical breach is unlikely. Figure 10A Figure 10B Figure 10. A-C) Axial cross-section, posterior and lateral iliac views of the screw pathway in the ilium. 12 Figure 10C The new VIPER SAI screw has novel features that are desirable for use in this capacity: a favored angled polyaxial head for added tilt and ease of rod attachment, a smooth shank for enhanced outer diameter and strength at the proximal part of the screw and minimal disruption of the SI joint, large threadform for cancellous bone purchase and a diverse variety of sizes for all types of patients (see Figure 11). Kebaish and colleagues reported on a group of 52 patients (adult and pediatric) followed over two years using this technique and reported lower complication rates than other techniques with no adverse effect on the SI joint and only one patient who required implant removal.20 Excellent results were described in a cohort study of smaller patients (predominantly with scoliosis secondary to cerebral palsy) with the S2AI technique compared to screws inserted into the ilium from the traditional starting point of the PSIS. Patients with S2AI screws had better restoration of pelvic obliquity and fewer complications; no deep infections, prominent implants or anchor migration compared to 3 patients with infections and 3 instances of implant prominence, skin breakdown or anchor migration in the PSIS group (see Figure 12). The S2AI screw technique can also be performed minimally invasively using existing EXPEDIUM instruments and its cannulated components. This technique saves operative and fluoroscopy time after placement of the guidewire down the pathway with either a drill or cannulated gearshift. Figure 11. The VIPER SAI screw features a favored angle articulation and smooth, cannulated shank. Figure 12A Figure 12B Figure 12. A-B) Postoperative sitting X-rays of a patient with neuromuscular scoliosis after posterior fusion and instrumentation with precontoured rods and a proximal transverse connector from the Expedium Neuromuscular System and pelvic fixation using the VIPER SAI screw via the S2AI pathway. Complications and Avoidance Complications associated with sacropelvic fixation include injuries to the adjacent structures (bladder, colon, iliac or gluteal vessels) due to misplacement, cortical bone breach, implant prominence and loosening, wound problems, infections and implant failure and nonunion. S1 pedicle screws placed in a convergent manner may injure the middle sacral artery or veins. Diverging S1 screws or alar screws that are placed bicortically and are too long may cause injury to the common or internal iliac artery/vein or lumbosacral trunk (L5 nerve root). S2 screws that are too long and placed in a convergent trajectory may injure the inferior hypogastric plexus or colon. Iliac screws violating the sciatic notch may injure the superior gluteal artery and those that violate the medial ilium may injure the internal iliac artery/vein or lumbar plexus, causing an iliacus hematoma or violation of the hollow viscera of the pelvis. Iliac screws that are too long anteriorly may violate the acetabulum. Misplacement can be avoided by becoming familiar with the anatomy at risk, cadaver experience, navigation, the use of blunt probes, confirmation of bony end point at all steps of screw placement and knowledge about the fluoroscopic X-ray views to confirm proper screw placement.21-24 13 Implant (screw head) prominence is cited as a frequent cause of removal as in one series of 36 patients with iliac screw fixation of whom eight required subsequent removal,25 but this can be mitigated by resecting the top of the iliac crest, forming a notch for the screw head to lie deeper within the ilium or using the S2AI technique, which allows the screw head to be almost 15mm deeper than traditional iliac screws.17 Kebaish’s series of 52 adult patients with S2AI fixation listed only one patient that needed subsequent removal.20 The infection rate seems lower with this technique as well, perhaps since less dissection is needed and, combined with soft tissue preservation and lower profile, seems ideally suited even for our malnourished neuromuscular patients. In a report of 32 patients with cerebral palsy and scoliosis and two-year follow up from surgery, there were no infections, prominent implants or failures of the S2AI screws. The infection rate associated with iliac fixation alone is difficult to compare from the literature because iliac fixation is performed in conjunction with other procedures. However, in a thorough two-year follow-up study of patients treated with iliac fixation, a group reported an infection rate of 4% (3 of 81 patients). Those authors also noted iliac screw back-out in three patients, all of whom had spondylolisthesis; however, no pseudarthrosis occurred in these patients. Nonunion and implant failure typically occur together, and host biology as well as strength of fixation may play a role. Proper workup to rule out a pseudarthrosis must be undertaken prior to implant removal to allow for proper preparation in the operating room. Again, consideration of anterior interbody support is important, especially in the face of pseudarthrosis. At L5/S1, an anterior structural graft is biomechanically favorable since it is loaded in compression and anterior to the pivot point; this can optimize fusion and stability.8 14 Summary Many sacropelvic fixation techniques have been described historically to aid surgeons in long constructs to the sacrum, but only a few are still widely used, including sacral screws, the Galveston technique, iliac screws, and S2AI screws. S1 pedicle screws are most effective when placed with “tricortical” fixation, angled medially and upward towards the sacral promontory. Sacral ala and S2 pedicle screws have been used to improve the strength of the construct, but studies have shown no significant biomechanical alteration or improved clinical results. Iliac screws (bolts) have been shown to protect S1 screws and have superior pullout strength as well as high fusion rates. Offset connectors are used to connect the screws to the longitudinal rods with the traditional PSIS starting point. S2 alar iliac (S2AI) screws have the advantages of decreased implant prominence since the insertion point is up to 15mm lower than the prominent part of the posterior iliac crest; and, the use of an offset connector is avoided since the point of insertion is in line with the S1 screw and longitudinal rods. Some of these techniques are associated with complications, which can be minimized by paying close attention to the regional anatomy, minimizing soft tissue dissection, and choosing low-profile implants and techniques. To add anterior structural support, anterior interbody fusions or TLIFs should be considered in long fusions that extend to the thoracic spine, which will offload some of the stresses from the posterior implants and allow early bony union. References 1. Devlin VJ, Boachie-Adjei O, Bradford DS, et al. Treatment of adult spinal deformity with fusion to the sacrum using CD instrumentation. J Spinal Disord 1991;4:1–14. 14. Erickson MA, Oliver T, Baldini T, et al. Biomechanical assessment of conventional unit rod fixation versus a unit rod pedicle screw construct: a human cadaver study. Spine (Phila Pa 1976) 2004; 29: 1314-9. 2. Balderston RA, Winter RB, Moe JH, et al. Fusion to the sacrum for nonparalytic scoliosis in the adult. Spine (Phila Pa 1976) 1986; 11: 824-9. 15. Schwend RM, Sluyters R,Najdzionek J. The pylon concept of pelvic anchorage for spinal instrumentation in the human cadaver. Spine (Phila Pa 1976) 2003; 28: 542-7. 3. McCord DH, Cunningham BW, Shono Y, et al. Biomechanical analysis of lumbosacral fixation. Spine (Phila Pa 1976) 1992;17:S235-43. 16. Wang MY, Ludwig SC, Anderson DG, et al. Percutaneous iliac screw placement: description of a new minimally invasive technique. Neurosurg Focus 2008; 25: E17. 4. O’Brien MF. Sacropelvic fixation in spinal deformity. In: DeWald RL, editor. Spinal deformities: the comprehensive text. New York: Thieme; 2003. p 601-14. 17. Chang TL, Sponseller PD, Kebaish KM, et al. Low profile pelvic fixation: anatomic parameters for sacral alar-iliac fixation versus traditional iliac fixation. Spine (Phila Pa 1976) 2009; 34: 436-40. 5. Lebwohl NH, Cunningham BW, Dmitriev A, et al. Biomechanical comparison of lumbosacral fixation techniques in a calf spine model. Spine (Phila Pa 1976) 2002;27:2312-20. 18. Kebaish KM. Sacropelvic fixation: techniques and complications. Spine (Phila Pa 1976) 2010: 35: 2245. 6. Cunningham BW, Lewis SJ, Long J, et al. Bio-mechanical evaluation of lumbosacral reconstruction techniques for spondylolisthesis: an in vitro porcine model. Spine (Phila Pa 1976) 2002;27:2321-7. 7. Ogilvie JW, Schendel M. Comparison of lumbosacral fixation devices. Clin Orthop Relat Res 1986;203:120-5. 8. Crock HV. Anterior lumbar interbody fusion: indications for its use and notes on surgical technique. Clin Orthop Relat Res 1982;165:157-63. 9. Allen BL Jr, Ferguson RL. The Galveston technique of pelvic fixation with L-rod instrumentation of the spine. Spine (Phila Pa 1976) 1984; 9: 388-94. 10. Allen BL Jr, Ferguson RL. L rod instrumentation for scoliosis in cerebral palsy. J Pediatr Orthop 1982;2:87–96. 11. Broom MJ, Banta JV, Renshaw TS. Spinal fusion augmented by Luque-rod segmental instrumentation for neuromuscular scoliosis. J Bone Joint Surg Am 1989;71:32–44. 12. Lonstein JE, Koop SE, Novachek TF, et al. Results and complications after spinal fusion for neuromuscular scoliosis in cerebral palsy and static encephalopathy using Luque-Galveston instrumentation. Spine (Phila Pa 1976) 2012; 37: 583-91. 13. Bell DF, Moseley CF, Koreska J. Unit rod segmental spinal instrumentation in the management of patients with progressive neuromuscular spinal deformity. Spine (Phila Pa 1976) 1989;14:1301. 19. O’Brien JR, Matteini L, Yu WD, et al. Feasibility of minimally invasive sacropelvic fixation: percutaneous S2 alar iliac fixation. Spine (Phila Pa 1976) 2010; 35: 460-4. 20. Kebaish KM, Pull ter Gunne AF, Mohamed AS, et al. A new low profile sacropelvic fixation using S2 alar iliac (S2AI) screws in adult deformity fusion to the sacrum: a prospective study with minimum 2-year follow-up. Presented at: the North American Spine Society Annual Meeting; November 10–14, 2009; San Francisco, CA. 21. Lehman RA Jr, Kuklo TR, Belmont PJ, et al. Advantage of pedicle screw fixation directed into the apex of the sacral promontory over bicortical fixation: a biomechanical analysis. Spine (Phila Pa 1976) 2002; 27: 806-11. 22. Zindrick MR, Wiltse LL,Widell EH, et al. A biomechanical study of intrapeduncular screw fixation in the lumbosacral spine. Clin Orthop Relat Res 1986; 203: 99 –112. 23. Mirkovic S, Abitbol JJ, Steinman J, et al. Anatomic consideration for sacral screw placement. Spine (Phila Pa 1976) 1991; 16: S289-94. 24. Leong JC, Lu WW, Zheng Y, et al. Comparison of the strengths of lumbo-sacral fixation achieved with techniques using one and two triangulated sacral screws. Spine (Phila Pa 1976) 1998; 23: 2289 –94. 25. Emami A, Deviren V, Berven S, et al. Outcome and complications of long fusions to the sacrum in adult spine deformity: Luque-Galveston, combined iliac and sacral screws, and sacral fixation. Spine (Phila Pa 1976) 2002; 27:776-86. 15 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-68-000 9/2012 JC CA #8988A / DJ10057A Unique Challenges with Scoliosis and Dislocated Hips Randal R. Betz, MD The majority of neuromuscular disorders that include dislocated hips fall into one of three diagnoses, which include 1) spinal cord injury; 2) myelomeningocele; and 3) cerebral palsy. The highest prevalence of dislocation is probably caused by sepsis, and it is essential that any relatively acute, radiographically identified dislocated hip have an aspiration and adequate work-up to rule out sepsis. The prevalence for non-septic dislocated hips in these populations is as follows: In my experience with patients with SCI, there is about a 40% prevalence of hip instability. The prevalence increases with the length of time they are followed post injury. In patients with spina bifida, the prevalence varies according to the level of motor function. In cerebral palsy, hip instability almost always occurs in the severely involved patients (Gross Motor Function Classification System [GMFCS] V). 17 With the high prevalence of hip subluxation and dislocation in neuromuscular disorders, patients fall into one of three treatment groups with regards to the challenges of scoliosis and dislocated hips, to include: Scenario #1: Development of a scoliosis needing treatment with an early subluxed hip secondary to pelvic obliquity where the hip itself does not need treatment. This assumes that the scoliosis and pelvic obliquity are corrected prior to developing an actual unstable hip. Scenario #2: There is scoliosis needing treatment and the subluxed hip is now unstable and both the hip and spine need treatment. Scenario #3: There is severe scoliosis needing treatment and a longstanding dislocated hip or hips. 18 Treatment Scenario #1: Development of a scoliosis needing treatment with an early subluxed hip secondary to pelvic obliquity where the hip does not need treatment, provided the scoliosis and pelvic obliquity are corrected prior to developing an actual unstable hip. Most commonly, this occurs in a patient whose hips have a migration index < 40%. The hip at risk is always on the high side of the pelvic obliquity. Generally, complete correction via fusion and instrumentation into the pelvis to correct the pelvic obliquity helps to correct the early subluxation of the hip. Sometimes what were thought to be mild or moderate hip contractures preoperatively now cause problems with balance postoperatively and may require soft tissue releases (Figure 1A-G). Occasionally, one sees a more mild scoliosis that potentially could be braced, but the obliquity is being caused not by a severe scoliosis but by a pseudo hip flexion contracture (abduction contracture of the tensor fascia lata muscle and the gluteus medius) of the opposite hip from the one that is showing subluxation. Pseudo hip flexion contracture can be identified by taking a hip that clearly shows a hip flexion contracture > 45 degrees and then abducting the hip maximally such that the hip flexion contracture almost disappears. This demonstrates tightness of the abductors and the tensor fascia femoris muscles and causes a pelvic obliquity in both sitting and supine positions. It needs to be corrected so the scoliosis can be braced. The release of the hip muscle contractures will prevent further subluxation of the contralateral hip. The author of this chapter recommends a modified Soutter release.1 This is a standard anterolateral approach to the hip as one would do for osteotomies such as a Salter. The apophysis over the iliac wing is split anteriorly from approximately the mid axillary line down through the anterior inferior iliac spine. The apophysis and gluteus muscles are stripped from the outer table of the ilium posteriorly enough that the muscle alignment is straight off the midline of the torso (no future flexion vector pull of the muscles). The iliac crest then is trimmed such that reattachment to bone cannot cause recurrence of the flexion contracture. This muscle release corrects the pelvic obliquity. The mild scoliosis is now braceable, and the risk of the opposite subluxed hip progressing to full subluxation or dislocation is reduced. Figure 1A (AP) and 1B (lateral). This is an 18-year-old patient with a T1 paraplegia secondary to a motor vehicle accident. He has no trunk control and needs to utilize his upper extremities for balance. Therefore, the indications for surgery are prevention of further curve progression and restoring independent balance for bilateral upper extremity use. Figure 1C. Demonstrates pelvic obliquity with C7 falling far lateral to the center sacral line. Figure 1D. Shows an intraoperative radiograph with one rod in place, demonstrating incomplete correction of the pelvic obliquity. Further in situ rod bending and compression distraction forces are utilized, resulting in an aligned spine to the pelvis in Figure 1E. Figure 1E. Despite good correction of C7 to the center sacral line and balanced shoulders to the top of the pelvis, the patient is still sitting with a tilt to the left. Note the adducted position of the right hip, which is a fixed adduction contracture. Figure 1F (final AP) and 1G (final lateral). The patient underwent a right adductor tenotomy which resulted in correction of the sitting imbalance. 19 Scenario #2: There is scoliosis needing surgical treatment, and the subluxed hip is now unstable and also needs treatment. The overall principle here is to correct the spinal deformity and pelvic obliquity completely prior to the surgery on the hip. The first reason is that without correcting the pelvic obliquity first, the hip will not stay in the socket for long because of the persistent adducted position. In this author’s experience, if the spine is not corrected immediately after the hip is operated on, the hip usually resubluxes or dislocates. The second reason for doing the spine first is that there is very low prevalence of deep venous thrombosis (DVT) or pulmonary embolus after spine surgery in the neuromuscular population. In contrast, surgery on the hip has a high prevalence of DVTs, even with prophylaxis. If prophylaxis is not continued for three to six months after the hip surgery, there is risk of developing a late onset DVT. This makes it extremely difficult to operate on the spine because of the perioperative risk created by DVT prophylaxis and/or treatment, especially if a DVT does occur. Generally, the hip is treated a few weeks after the spine is corrected. Following spine surgery, the patient is put on Lovenox. Doppler studies to rule out DVTs are done at least twice. If the spinal wound is completely dry and without any evidence of draining hematoma or infection, then approximately 2-3 weeks later it is generally appropriate to operate on the subluxed hip that needs treatment. Stopping the Lovenox and restarting at the time of the hip surgery is common practice. 20 Treatment of the Hip Generally, the patients who are having their spine fused are older and are therefore not candidates for just an isolated varus osteotomy to treat the subluxed hip. If the hip is mildly subluxed, then in our experience a Dega osteotomy is the treatment of choice. Generally, Chiari osteotomies are not adequate with regards to posterior coverage, as most of these subluxed and dislocated hips in these paralytic populations are going out posteriorly. Procedures that do seem to be able to correct the posterior subluxation include a reverse triple innominate osteotomy or a Ganz osteotomy (Figure 2A-B), and a posterior shelf osteotomy. In patients who are flaccid, it appears that a muscle transfer is needed to help maintain reduction of the hip. The author currently recommends an external oblique transfer. Whereas it may not be an active transfer, it may act like a tenodesis. In patients who have spastic spinal cord injury or cerebral palsy, it is essential to control the spasticity first before attempting to relocate the hip. Oral medication is tried first; however, it may be necessary to insert a baclofen pump. This can be coordinated prior to or during the spinal fusion. If the baclofen pump is present before the spinal surgery, make sure to have a repair kit on hand (see Complications in NM Scoliosis Chapter for more details). Complications unique to this scenario include development of heterotopic ossification (HO) about the operated hip. Prophylaxis is essential, to include Indocin (25 mg three times a day). In addition, the use of Didronel is recommended. Both should be continued for 3-6 months. Whereas medication is counterproductive with regards to obtaining a fused spine, if the patient gets HO about the hip, this is disastrous in that he or she then cannot sit because of lack of hip mobility. If the patient does get HO and it needs to be resected, then the only treatment is radiation. The HO can be prevented most times with medication. In the author’s experience, the healing of the spine fusion in these patients is so robust that nonunions have not been seen despite prophylaxis for HO. Figure 2A. Preoperative AP of hips showing subluxation of the left hip in a 15 year-old skeletally mature patient with thoracic level SCI. Figure 2B. Postoperative film showing excellent posterior coverage with a Ganz osteotomy (Case courtesy of Dr. Harold Van Bosse). 21 Scenario #3: There is severe scoliosis needing treatment and a longstanding dislocated hip or hips. In this scenario, generally the dislocated hips are left alone…the risk of these patients ending up with stiff hips because of fibrous tissue or HO is high. Therefore, relocation is rarely recommended. The majority of these patients do not ambulate. Correction of the spine to include correction of the pelvic obliquity is important. Intraoperative techniques to provide them with good sitting balance include fixing the spine in slight thoracolumbar kyphosis and reduced lumbar lordosis based on emulating a sitting x-ray (see Chapter 8). In these patients with dislocated hips, it is essential in the immediate postoperative period prior to prolonged sitting in a wheelchair that they get seating pressures measured. 22 Summary In the presence of subluxed or dislocated hips, the patient with neuromuscular scoliosis presents unique challenges. A clear plan needs to be identified early on to address both orthopaedic problems in a sequence that is safe. As a general principle, if both the hip instability and the spine deformity require surgery, the author strongly recommends doing the spine first. References 1. Smith H. Ankylosis and deformity. In Edmonson AS, Crenshaw AH eds. Campbell’s Operative Orthopaedics. 6th ed. St. Louis: The CV Mosby Co., 1980:1153. 23 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-64-000 9/2012 JC CA #8782 / DJ10053A The Correction of Pelvic Obliquity Using Cantilever Correction Kirk Dabney, MD Spinal deformity in neuromuscular disorders is common and is frequently accompanied by pelvic obliquity. Severe pelvic obliquity affects sitting and standing balance, can lead to pressure sores, and can contribute to hip dislocation. In severe cases, the patient may lose the ability to sit upright. Surgical correction must bring the pelvis level to the sitting surface and perpendicular to the longitudinal axis of the spine, and restore pelvic sagittal alignment. 25 Patient Assessment In addition to the medical history, other important historical information should include asking the patient and/or caretaker questions regarding sitting and standing tolerance, history of pressure sores, and the presence or absence of pain. Physical examination should assess: 1) sitting and standing balance including whether the head and torso are centered over the pelvis in both the frontal and sagittal planes, 2) pelvic obliquity in both the frontal and sagittal planes, 3) rotational alignment of the spine and pelvis, and 4) stiffness of the spine and pelvic obliquity. The latter is best assessed by performing the bending test (Figure 1). As shown in the illustration, sagittal plane pelvic stiffness can be assessed by placing the patient supine and: 1) flexing the hips above 90 degrees to see if anterior Figure 1. Side-Bending Test. The pelvic tilt corrects, and 2) patient is bent over the examiner’s allowing the pelvis and thigh at the apex of the curve. If limbs to hang over the the patient’s curve reverses and the pelvis levels out becoming end of the exam table to perpendicular to the trunk, the see if posterior pelvic tilt curve remains flexible enough to corrects. An anterior release correct through posterior fusion alone. If not, an anterior release (anterior discectomy) in a is performed. stiff deformity should be considered if the pelvis does not correct to neutral in the frontal plane or to within the normal anatomic pelvic tilt in the sagittal plane. Anterior release is performed one to two levels above and below the apex of the stiff scoliotic, hyperlordotic,or hyperkyphotic curve to close down the convexity of the deformity. Radiographic assessment should include AP and lateral views, either sitting (if the patient can sit) or standing (if the patient can stand independently). The Cobb angle and pelvic obliquity (using the horizontal method) are measured in the coronal plane. Bending x-rays can also be helpful in assessing whether an anterior release should be performed. Pelvic tilt is assessed on the lateral radiograph. 26 The overall goal after surgical correction is to shift weight bearing during seating to the posterior thigh mass. Excessive anterior pelvic tilt and lumbar hyperlordosis causes the patient to sit with the abdomen resting on the anterior thighs and can often result in pain. On the other hand, excessive posterior pelvic tilt and lumbar hyperkyphosis causes the patient to sit with excessive pressure on the sacrum which can lead to pressure sores. Surgical Treatment and Instrumentation Cantilever correction is a powerful method to correct pelvic obliquity. It requires instrumentation that can firmly anchor into the pelvis and can then be used as a lever arm to swing the pelvis into a corrected position that is perpendicular to the longitudinal axis of the spine. Traditionally, the unit rod is ideal for this purpose; however, it can be difficult to place the pelvic limbs of the rod in cases of severe pelvic obliquity and/or lumbar hyperlordosis ( > 70 degrees), because it must be placed into the pelvis in one unit. The ideal system provides a lever arm that is as powerful as the unit rod and can allow for easier fixation to the pelvis through modularity. Cantilever correction using the EXPEDIUM® Neuromuscular System can accomplish this. The pelvis is exposed by dissecting up over the sacroiliac joint onto the lumbar muscle attachment on the inner table of the pelvis. It is important not to dissect into the sacroiliac joint subperiosteally, as significant bleeding can be a problem. By dissecting over the joint itself, little bleeding is encountered. The muscle is then sharply and bluntly dissected up over the iliac crest apophysis. The overlying fascia is then divided and the outer wing of the ilium is then subperiosteally exposed from the posterior superior iliac spine (PSIS) forward along the posterior one-third of the pelvis and down to the sciatic notch. A guide which hooks into the sciatic notch is then utilized to aim a drill hole (or alternatively a pedicle probe can be utilized) from the PSIS start point to just anterior and superior to the sciatic notch. If a guide is not available, the pedicle probe alone can be used and aimed just above the sciatic notch by direct palpation or by using intraoperative fluoroscopy. This is the region where the pelvis is most dense for pelvic rod or screw fixation1. An intraoperative AP and oblique fluoroscopic view (Figure 2) is taken to confirm the trajectory of the drill or probe to make sure there is no penetration of the inner or outer pelvic table. Pelvic screw fixation of largest diameter possible (7, 7.75, 8, 9 and 10mm diameter are available) is placed in this trajectory and should be of sufficient length to pass the sciatic notch by at least 1cm (Figure 2). The author prefers to use a closed polyaxial screw head to maximize the rigidity of the final rod-pelvic screw construct. Typically, pelvic screws alone are used; however, when additional fixation is needed to improve the rigidity of pelvic fixation, S1 screws are added. This is preferred over sacral screw fixation alone, because pelvic screw fixation provides a better lever arm to correct both pelvic obliquity and sagittal plane pelvic deformity. Alternatively, pelvic screws can be placed using the medial portal as described by Chang et al2. Advocates for this method state that there is less exposure time and less bleeding, and that the screw head is less prominent. While we have not found bleeding or exposure time to be less in our hands, the screw is less prominent using this approach. We have obviated the screw head being prominent at the PSIS start point by notching the ilium at the entrance point with a rongeur and countersinking the screw. The fixed lateral rodded connectors (usually the 10 or 20mm) are then provisionally connected to the pelvic screws. Figure 2. Intraoperative AP and oblique views showing proper placement of pelvic screw. A) Note the AP View shows the trajectory of the pedicle probe from the PSIS to just superior and adjacent to the sciatic notch and B) the final screw position at least 1cm lateral to the notch. The oblique view shows C) the probe and D) the final screw position between the inner and outer cortex just superior to the sciatic notch which appears as a “teardrop”. Figure 2A Figure 2B Figure 2C Figure 2D 27 After the iliac or sacral fixation is completed, sublaminar wires are passed at every level of the spine to be fused. If hyperlordosis ( > 60 degrees) in the lumbar spine exists, then pedicle screws with reduction posts are used in the lumbar spine (See Chapter 6 on Cerebral Palsy Scoliosis). The EXPEDIUM® pre-contoured (sagittal plane) stainless steel 6.35-inch rods are connected to the previously placed iliac screws via the lateral rodded connectors (Figure 3A). The 6.35-inch diameter rods are used to provide maximum rigidity. Critical to the correction is attaching and securing each of the pre-contoured rods to the iliac screws with the fixed lateral connectors so that each of the rods is perfectly perpendicular to the horizontal axis of the pelvis and that the sagittal contour of the rods is aligned with the sacrum (Figure 3A). This is done by adjusting the rod rotationally so that the line on the rod is directly posterior and in line with the sacrum (Figure 3B and 3D). The sagittal bend should be identical on each rod and should also be aligned so that the contour matches from proximal to distal. If these steps are not meticulously followed, the pelvic obliquity will not be fully corrected with the cantilever maneuver. Once this is done, the set screws on both the iliac screws are tightened and torqued down onto the rod. Next, a drop entry cross connector is attached so as to lie in the lumbar region of the spine to add further rigidity to the system and to prevent rotation and shift of the rods in any direction. Similarly, a proximal closed connector is also placed at the top of the construct and tightened which secures the rigidity of the rectangular construct (Figure 3A and 3C). The rods are not cut until it is secured proximally. After the rod construct is “built” from distal to proximal, cantilever correction can Figure 3A Figure 3. A-B) Shows AP profile of the prebent rods connected via the lateral connectors to the iliac screws. Note that the connection should make the rods perpendicular to the horizontal axis of the pelvis to maximize cantilever correction. A distal cross-connector and proximal closed connector make the construct rigid. The rods should match in both length and contour with rotation matched before final tightening of the connectors. C) Shows the lateral contour of the pre-bent rods in line with the sagittal profile of the sacrum. D) There is a line on the posterior contour of the rod to help line it up with the sacrum. Figure 3B Figure 3C Figure 3D Line on rod 28 be started. This begins with the surgeon pushing the rod down to the L5 lamina only and then tightening the wire to the rod (a jet wire twister is helpful). The wires must never be used to pull the rod to the spine. This is repeated in a step-by-step manner: the rod is pushed down to the L4 lamina and then the wire tightened. Next, the rod is pushed down to the L3 lamina and the wire tightened. The process is repeated one level at a time up to the T1 lamina (Figure 4). The wires are cut 1cm in length and then bent and impacted down to the midline so as not to remain prominent. Just prior to tightening the wires at the proximal levels, the rod can be cut at the T1 level and the closed connector brought up to the proximal end of the rod and retightened and torqued, and then the wires tightened. Figure 4A Alternatively, if one is using reduction pedicle screws in the lumbar spine then the same push the rod to the spine technique is used. This will help avoid screw pullout. If a screw does begin to pull out, then add a sublaminar wire at that same level and use in conjunction with the screw. Figure 4. A) The rod “construct” is manually pushed down at each level with a rod pusher at each level before tightening wires (if sublaminar wires are to be used). This is important to prevent wire breakage or cutout through the lamina. It is important to keep the spinous process centered between the rods. If pedicle screws are being used, it is still important to push the rod down at each level gradually so as to prevent plowing out of the screws. Reduction posts can be helpful, however, rod pushers help so that all of the reduction force is not placed on the screw alone. B) The rod is gradually pushed to the spine at each level. Figure 4B 29 Outcomes The outcomes of cantilever correction in neuromuscular scoliosis are documented in the literature primarily through the experience of using the unit rod. Our experience shows pelvic obliquity can be corrected by approximately 70-80%. Intraoperative complications with pelvic fixation are primarily due to the difficulty with pelvic rod placement in patients with preoperative lumbar hyperlordosis. This problem is obviated by placing pelvic screws separately and then connecting the pre-contoured rods, distal crosslink, and then proximal connector as described earlier in this chapter. It has been our experience that fusion to the pelvis does not cause a loss of ambulatory function. Restoration of sagittal alignment is key in preserving ambulatory function which can be easily done through the use of the precontoured rods from the EXPEDIUM® Neuromuscular System. Through the use of the pre-contoured rods, the lumbar spine is reduced to the proper sagittal alignment with either sublaminar wires or pedicle screws. 30 References 1. Miller F, Moseley C, Koreska J. Pelvic anatomy relative to lumbosacral instrumentation. J Spinal Disord 1990;3:169-73. 2. Chang TL, Sponseller PD, Kebaish KM, Fishman EK. Low profile pelvic fixation: anatomic parameters for sacral alar-iliac fixation versus traditional iliac fixation. Spine (Phila Pa 1976) 2009;34:436-440. 31 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-65-000 9/2012 JC CA #8785 / DJ10059A Correction of Pelvic Obliquity with an All-Screw Construct Suken A. Shah MD Neuromuscular scoliosis may be associated with significant pain due to sitting difficulties and truncal imbalance, especially with pelvic obliquity and pressure sores; however, many of the patients with static encephalopathy or TBI are unable to articulate their symptoms. Progressive deformity is believed to be the result of both muscle imbalance and anatomic deformity. Curve progression leads to subsequent deformity and trunk imbalance with associated loss of function. 33 Background Historically, there has been debate regarding when to extend the posterior spinal fusion to the pelvis. Pelvic obliquity has been noted to progress in neuromuscular scoliosis if the pelvis is not fused,1,2 and traditionally many authors have recommended fusion to the pelvis in nonambulatory patients. In the ambulatory patient with pelvic obliquity, fusion to the pelvis has been traditionally avoided due to the belief that it will adversely affect ambulatory function.3,4 Our experience demonstrated preserved ambulatory function in ambulatory patients with cerebral palsy who were fused with unit rod instrumentation, as determined by gait analysis. We hypothesized that the conventional assumption that ambulatory potential is limited by fusion to the pelvis arose from early attempts at pelvic fixation with Harrington rods that removed lumbar lordosis, and possibly confounded by variable of prolonged immobilization and bed rest in earlier segmental systems that did not provide sufficient rigidity for immediate postoperative mobilization and ambulation therapy. The Galveston technique to extend the fusion across the pelvis by placing each Luque rod between the pelvic tables4,7 has demonstrated acceptable fusion rates across the L5-S1 segment, and appears to provide good control of pelvic obliquity. While the impaction of two Luque rods into the pelvis with associated segmental fusion via sublaminar wires provides a reasonable construct in the sagittal plane with resistance of flexion/extension, there exists a moment arm of rotation about the two rods allowing for rod translation with respect to one another, loss of torsional control and subsequent progression of pelvic obliquity, pseudarthrosis, and implant failure.6 The use of Luque rods smaller than 6.35mm diameter may increase the incidence of implant failure2,5, but the intraoperative bending of 6.35mm diameter steel rods to the optimal 3-dimensional geometry for pelvic implantation in a patient with a severe lumbopelvic deformity presents a technical challenge. Immediate and durable rigid fixation is essential for surgical success. Sanders, et al2 found, in their retrospective study of Luque rod instrumentation, that a postoperative curve greater than 35 degrees, preoperative curves greater than 60 degrees, crankshaft deformity, and not fusing to the pelvis were factors associated with postoperative curve progression. 34 The unit rod developed by Bell, Mosley, et al5 addresses some of the potential limitations of dual Luque rod instrumentation. The implant design of a proximally connected, precontoured rod provides for better rotational control, as the degree of rotational freedom between two independent Luque rods is eliminated. Initial mean curve corrections with the unit rod were reported to be 54.6%, with a mean loss of correction of 6.5% at 2 year followup5; experience at our institution documented a mean scoliosis correction with the unit rod of 66% and 75% correction of pelvic obliquity. However, Luque-Galveston constructs and unit rods do have significant complications related to distal fixation, and treatment of significant sagittal plane deformities are a challenge. Patients with lumbar hyperlordosis are at risk for implant pullout, rod breakage, migration and pseudoarthrosis, and those with thoracic hyperkyphosis are at risk for proximal wire cutout and breakage, proximal junctional kyphosis and instrumentation prominence. Subsequently, many of these patients require reoperations, which are costly and not without further risk.8 Lonstein and his colleagues recently published their results and complications of 93 patients with cerebral palsy and scoliosis who underwent PSF with LuqueGalveston instrumentation with an average follow up of 3.8 years.7 Coronal curve correction was 50% and pelvic obliquity correction was 40% at latest follow up. The late complication rate was 47% and included the windshield washer sign, junctional kyphosis, pseudarthrosis (7.5%) and implant problems including breakage, dislodgement and prominence. Seven patients required reoperation, most commonly for pseudarthrosis and/or failed implants. Long iliac anchors provide superior control of pelvic obliquity, while necessitating more dissection of the outer table musculature for insertion. The standardized shape of the unit rod produces a relatively uniform coronal and sagittal profile. However, there are limitations to its use: a) it is particularly challenging to implant in a hyperlordotic patient, b) it does not lend itself to use of hooks or screws as originally designed, c) estimation of correct implant length is difficult in patients with large curves, d) dissection at the cranial end of the spine to pass wires may produce a tendency for junctional kyphosis and finally, e) the unit rod does not perform well in curves with an apex in the high thoracic spine. Advantages of Screw Fixation Iliac screws perform better in pull out strength than smooth Galveston rods for pelvic fixation, and the use of segmental pedicle screw constructs have shown substantial correction and high fusion rates, while accomplishing the goals of leveling pelvic obliquity and addressing seating problems. These newer, modular systems can navigate some of the substantial challenges in these patients, such as abnormal pelvic anatomy, osteoporotic bone and hyperlordosis and avoid some of the risk in early instrumentation failure, but come at a substantial monetary expense. Our experience shows that in cases of marked lordotic deformity, better fixation and correction was achieved with pedicle screws and frequently reduction-tab pedicle screws in the lordotic segment. A cadaveric biomechanical study has confirmed that the use of L5 pedicle screws significantly increases the lateral and oblique stiffness of the unit rod construct.9 McCall et al10 retrospectively examined a cohort of patients with neuromuscular scoliosis in whom those with a stable lumbosacral articulation were instrumented with a “U-rod” (unit-rod without the pelvic limbs) with L5 pedicle screw fixation. The L5-S1 interspace mobility was assessed on the basis of L5 tilt; patients with more than 15 degrees of L5 tilt were instrumented with a standard unit rod construct. McCall et al found that in follow-up the patients that were instrumented to L5 with the U-rod had similar results to those fused with the standard unit rod construct. However, it has been our institution’s practice to fuse all patients with spastic conditions to the pelvis to avoid late pelvic obliquity below an instrumented fusion. The difficult learning curve associated with rod contouring and insertion into the ilium using the Galveston technique has been resolved with the use of iliac screws, which permits screws of variable length and diameter to be inserted into each ilium separately. Simpler fixation to the pelvis with screws may also mitigate the potential complications of the Galveston technique, which was up to 62% in Gau’s series11 and 47% in Lonstein’s series.7 The screws can then be connected to the main construct by various connectors. This technique has simplified the process of obtaining iliac fixation, especially in patients with significant pelvic asymmetry or hyperlordotic lumbar deformities while improving pullout strength through better interdigitation of the threaded implant within the iliac cortical and cancellous bone.12,13 A retrospective, single-center cohort study comparing two groups of 20 patients (flaccid and spastic paralytic scoliosis) each with Luque-Galveston constructs and iliac screws showed similar maintenance of pelvic obliquity and scoliosis correction, but the iliac screw technique avoids the complex lumbosacral 3-dimensional rod bends and had less haloing around the pelvic implants with minimal implant complications. The Galveston group had 4 broken rods and two reoperations and the iliac screw group had 1 broken screw and no reoperations.14 We reported in a larger, multicenter retrospective study of 157 patients, with virtually equal distribution, that compared the unit rod to “surgeon-bent” rods with iliac screw fixation and found that the unit rod group had better pelvic obliquity correction. However, the disadvantages seen in cases with the unit rod include increased mean surgical time, blood loss, hospital stay, infection rate and proximal fixation problems.8 The increased blood loss may be a result of the multiple laminotomies, wire passage, and wire manipulation, although this is speculative. We hypothesized that the increased incidence of infection after unit rod surgery may be due to the dissection of the subcutaneous tissue and muscle needed to make the transition from the iliac wings to the spine. Other concerns with these constructs were the lucencies that surrounded the iliac rods and the rod bend, which could become prominent with any migration. In addition, dissection of the paraspinous muscle required for the unit rod transition from the spine to the pelvis compromised the muscle flaps covering the rods. For insertion of a unit rod, subcutaneous flaps must be elevated bilaterally to the iliac crests, and the paraspinous muscle over the midline is incised or stretched to where the rod joins the iliac posts at the entrance to the pelvis. These steps are not needed for insertion of sacral or iliac screws. For iliac screws, although the lateral dissection maybe the same for a traditional PSIS starting point, a connector may be used to join the screw to the rod with less tissue trauma and minimally invasive techniques are available. Iliac screws have decreased, but not eliminated, the rate of implant lucency and the need for revision seen with unit rods. The increased incidence of clinically significant proximal implant prominence may be a result of the shape of the rod, which has a dorsally-directed bend at the top, as well as the supralaminar dissection needed to pass the wires proximally.8,14,15 35 There are substantial data in the literature that pedicle screws in the lumbar and thoracic spine, by nature of their 3-dimensional correction control of the vertebral body, allow surgeons to obtain better correction of spinal deformities in all three planes and subsequently very little loss of correction over time and an insignificant pseudarthrosis rate. Pedicle screws, combined with posterior-based releases, have decreased the need and frequency of an anterior release for many of the severely involved neuromuscular patients and decreased the morbidity associated with combined anterior/posterior surgery. Combined with halo / femoral or cranial tong / boot traction, screws are a powerful correction tool that avoids the morbidity of an anterior procedure even for large, stiff curves (Figure 1). Traction helps tremendously in obtaining intraoperative balance of the patient’s trunk and pelvis. Sometimes the obstacles of severe hyperlordosis and/or hip and knee flexion contractures with or without hip dislocation will challenge the spinal deformity surgeon with positioning the patient in traction, but various strategies are available to ameliorate these problems. The patient’s hips can be flexed to assist with correction of lumbar hyperlordosis, unilateral leg traction may be used on the high side of the pelvis to assist with pelvic obliquity correction, and padding can be used to accommodate contractures. For stiff curves, or those over 90 degrees, anterior release of the apical levels of the curve may be indicated, since it is necessary to gain flexibility to obtain adequate curve correction, which then indirectly helps correction of some of the pelvic obliquity. Anterior surgery increases the complication rate and morbidity of spinal surgery in these patients, and it is unclear whether to stage the anterior and posterior procedures separately (one week apart) or to do both procedures on the same day. Evidence exists to support both strategies, and it is our practice to stage surgeries for patients with severe involvement and multiple medical co-morbidities and problems. For relatively healthy patients, we usually perform both stages on the same day, provided that the time under anesthesia or blood loss is not too substantial after the anterior release.7,8 Screw fixation with custom-bent rods or pre-contoured rods (available in the Expedium® Neuromuscular System) allow the surgeon to tailor fixation and correction options for a preferred technique and particular patient. Review of the literature available indicates that pedicle screw fixation offers better major curve correction, improved derotation, lower proximal implant complications and may avoid the need for an anterior release. Accuracy and neurological safety with freehand pedicle screw placement is similar to that of idiopathic scoliosis. Figure 1. Intraoperative photo of a patient in cranial tong and distal boot traction, frequently used to manage large curves with pelvic obliquity. Intraoperative traction is a powerful tool to obtain passive correction of large deformities, but high quality TcMEP monitoring is important during the procedure. 36 Surgical Technique After intubation, appropriate monitoring leads and establishment of large bore IV access and arterial and central venous catheterization, the patient should be placed prone on a radiolucent table or four post frame. Care should be taken to ensure that all bony prominences are well padded to avoid skin breakdown, especially in thin patients, and that the abdomen hangs free. The hips can be allowed to gently flex with knee and thigh support to passively correct lumbar hyperlordosis. Intraoperative traction has been described to correct pelvic obliquity. A standard posterior exposure of the spine from T1 to the sacrum is performed. An aggressive posterior release with radical facetectomy and ligamentum flavum resection is important in creating flexibility in the rigid apical portion of the curve and in all but the largest, stiff curves makes a posterior-only approach sufficient for correction of the scoliosis. Figure 2A Figure 2B Figure 2. A,B) Preoperative sitting X-rays of a patient with spastic quadriplegic cerebral palsy and severe thoracolumbar scoliosis and pelvic obliquity. The patient had significant difficulties sitting, pain and skin problems. At the inferior margin of the incision, the outer wing of the ilium can be subperiosteally exposed to the sciatic notch in the open version of the technique. When placing iliac screws in lieu of smooth Galveston pelvic fixation, the starting point is similar: 1-2cm inferior to the posterior superior iliac spine. To decrease implant prominence, a notch in the ilium can be used to bury the head of the screw below the contour of the cortical bone. A pedicle gearshift or drill is used to cannulate the cancellous bone between the inner and outer tables of the ilium, directed toward the anterior inferior ilica spine. I now prefer the S2AI screw technique for pelvic fixation and that is described in Chapter 2 on Sacropelvic Fixation Techniques. This technique allows a lower profile, favored angle implant that lines up with L5 and S1 screws, thus obviating the need for an offset connector. This preferred technique for pelvic fixation along with an Expedium Neuromuscular System modular rigid, closed transverse connector for at least the proximal end (distal end optional) results in a strong construct which allows control of pelvic obliquity, low profile and, to date after 3-4 year follow up, no implant failures (Figure 2). I have adopted a less invasive technique over a guide wire using the screw’s cannulated feature to preserve the soft tissues and limit fluoroscopy time. Figure 2C Figure 2D Figure 2. C,D) Postoperative sitting X-rays of the patient 2 years after posterior spinal fusion with the Expedium Spine System and components of the EXPEDIUM Sacropelvic Collection and Neuromuscular Systems. Note the profound correction of pelvic obliquity and restoration of coronal decompensation and sagittal sitting balance. 37 Due to the challenges of obtaining vertebral fixation in non-ambulatory patients with severe, rigid thoracolumbar curves, when using pedicle screw fixation in the thoracolumbar spine, use of polyaxial screws with reduction tabs will ease rod approximation and provide stable fixation. Alternatively, new Viper® Cortical Fix Screws are available, specifically designed to increase pull-out strength in bone with a cortical, short-pitch thread form for the pedicular bone and a long, dual lead thread form for cancellous purchase. Proper sizing of the screws should ensure good cortical fill and length in the vertebral body. In osteopenic bone, with careful technique, bicortical fixation through the anterior vertebral body (except L4), upsizing the diameter to achieve cortical pedicle fixation or other techniques may improve bony purchase. I prefer Expedium polyaxial screws with reduction tabs on the apical concavity to assist with translation and derotation of a severe curve; these screws are especially effective in pulling the spine dorsally to a prebent rod to correct lordosis (Figure 3). The reduction tabs allow a slow, controlled force to be applied to the spine and limits the risk of screw pullout; the polyaxial head allows accommodation of severe deformity and anatomic constraints of full correction. On the convex apex, Expedium uniplanar screws are helpful for derotation and cantilever forces to be applied for 3-dimensional correction. 38 Figure 3. Reduction tab polyaxial pedicle screws in the concave, lordotic portion of the curve facilitate correction of the spine to the rod in a slow, controlled manner without specialized instruments. The tabs are removed after final tightening of the set screws. The length of the rod is measured from T1/T2 to the pelvis. Note that the correction of a kyphotic deformity will shorten the spine and the correction of a lordotic deformity will lengthen the spine. Special attention should be paid to selection of the rod material (SS, Ti or CoCr), diameter and strength of the rod; the biomechanical properties of the rod and the force it can impart to the spine are important considerations when treating patients with osteopenic changes found in the vertebrae of neuromuscular scoliotic spines. The Expedium Neuromuscular System contains precontoured rods to save time in the operating room. The proper rod contour (hypokyphotic, normal or hyperkyphotic) and the length (short, standard or long) is selected for implantation and the rod lengths are tailored for the patient with a provisional look at how the rod will lay proximally and whether sufficient length is present for attachment to the iliac screws. The contours are checked and customized. Then, a specially designed modular rigid, closed transverse connector from the Expedium Neuromuscular System is selected for the proximal end and distal end (optional); they are available in 4mm increments and need to be slid over the top of the rods and secured prior to implantation. With this step, a modular, precontoured construct is quickly and easily made, and when connected to the iliac screws and distal lumbar pedicle screws is transformed into a powerful a cantilever correction tool to level the pelvis and correct severe thoracolumbar scoliosis like the unit rod (Figure 4 and Figure 5 - Clinical Example). Figure 4. Once the lumbo-pelvic foundation has been established by connecting the pre-bent rods to the iliac screws and lumbar pedicle screws, the two rods are secured proximally by a closed, transverse connector and the construct is now transformed into a powerful cantilever correction tool, like the unit rod, to level the pelvis and correct severe thoracolumbar scoliosis. 39 Facetectomies and decortication are performed and preliminary grafting of the area under the rod is performed. If the standard PSIS starting point for the iliac screws was used, then offset connectors are needed, but if the S2AI pathway is used, no offset connection is needed. The iliac screws have the Top Notch® feature to facilitate rod engagement with the Expedium Flex Clip Rod Reducer, or the set screws can be inserted freehand. S1 and L5 screws are captured and reduced; now the distal foundation is established and with the precontoured rods linked at the top with the closed transverse connector, superior control of the pelvis is obtained. The rods are cantilevered to simultaneously level the pelvis and correct the scoliosis and sagittal plane deformity. The screws are sequentially captured from distal to proximal and segmental fixation ensures that no single screw is prone to pull out. As previously stated, reduction tab polyaxial screws on the concave apex are important for a slow, guided correction of the spine, and the rod is directed to the spine and held in place while the screws are reduced to the rod. Sufficient time for viscoelastic creep and careful visualization of the bone-screw interface will avoid pull out, but occasionally in situ bending may be needed; coronal and sagittal benders are available in the set. 40 Pedicle screw fixation allows 3-dimensional control of the vertebrae and facilitates coronal, sagittal and axial plane correction of these complex deformities. The Expedium Spine System vertebral body derotators, Quick Sticks, and available racks to connect multiple derotators are important adjuncts in the correction of the axial rotation. Insertion of pedicle screws in this application is similar to the standard technique and can be used in conjunction with posterior-based osteotomies and vertebral column resection. Compression/distraction with simultaneous derotation optimizes correction before final set screw torque-limited tightening. Hemostasis is confirmed, decortication is performed and additional bone graft with antibiotics is placed over the posterior elements with special attention at the lumbosacral junction. A watertight closure with absorbable suture is performed (without a drain is our preference) and a sterile dressing with a water tight barrier to prevent soiling is applied. Correction of neuromuscular scoliosis with pedicle and iliac screw fixation is typically 63-70% with leveling of the pelvis and excellent sagittal alignment.8,15 With contemporary techniques and cantilever correction of the pelvis to neutral, it is unusual to have residual pelvic obliquity greater than 10 degrees. The patient’s personal wheelchair should be readjusted to accommodate his new trunk proportions and pelvic alignment (Figure 5). Figure 5A Figure 5B Figure 5. A,B) Preoperative sitting X-rays of a patient with spastic quadriplegic cerebral palsy and thoracolumbar scoliosis with pelvic obliquity. The patient had significant difficulties sitting, pain and hyperlordosis. Figure 5C Figure 5D Figure 5E Figure 5F Figure 5. E,F) Sitting X-rays of the patient demonstrating maintenance of erect alignment and sitting posture. Figure 5. C,D) Immediate postoperative X-rays showing restoration coronal, sagittal and pelvic alignment with an all-screw construct. Iliac screws were inserted via the S2AI pathway. 41 Summary Correction of neuromuscular scoliosis and pelvic obliquity with a screw construct offers many advantages including improved pelvic fixation with a low incidence of failure, infection, prominence or soft tissue complications, especially when using the S2AI technique. Furthermore, 3-dimensional correction of the severe apical deformity especially through derotation at the thoracolumbar junction results in better truncal balance and chest wall restoration important for pulmonary function and sitting. Finally, proximal screw constructs with custom bent rods result in less failure, pullout and prominence requiring revision than the unit rod. 42 References 1. Bradford D. Neuromuscular spinal deformity. In: Bradford D, Lonstein J, Ogilvie J et al (eds) Moe’s textbook of scoliosis and other spinal deformities. WB Saunders, Philadelphia: 1987; 271-305. 9. Erickson MA, Oliver T, Baldini T, et al. Biomechanical assessment of conventional unit rod fixation versus a unit rod pedicle screw construct: a human cadaver study. Spine (Phila Pa 1976) 2004; 29: 1314-9. 2. Sanders JO, Evert M, Stanley EA et al. Mechanisms of curve progression following sublaminar (Luque) spinal instrumentation. Spine (Phila Pa 1976) 1992;17:781789. 10. McCall RE, Hayes B (2005) Long-term outcome in neuromuscular scoliosis fused only to lumbar 5. Spine (Phila Pa 1976) 2005;30: 2056-2060. 3. Banta JV, Drummond DS, Ferguson RL. The treatment of neuromuscular scoliosis. Instr Course Lect 1999;48: 551-562. 4. Allen BL Jr, Ferguson RL. The Galveston technique for L rod instrumentation of the scoliotic spine. Spine (Phila Pa 1976) 1982;7:276-284. 5. Bell DF, Moseley CF, Koreska J. Unit rod segmental spinal instrumentation in the management of patients with progressive neuromuscular deformity. Spine (Phila Pa 1976) 1989;23:2308-2317. 6. Broom MJ, Banta JV, Renshaw TS. Spinal fusion augmented by Luque-rod segmental instrumentation for neuromuscular scoliosis. J Bone Joint Surg Am 1989;71:32–44. 7. Lonstein JE, Koop SE, Novachek TF, et al. Results and complications after spinal fusion for neuromuscular scoliosis in cerebral palsy and static encephalopathy using Luque-Galveston instrumentation. Spine (Phila Pa 1976) 2012; 37: 583-91. 8. Sponseller PD, Shah SA, Abel MF, et al. Scoliosis surgery in cerebral palsy: differences between unit rod and custom rods. Spine (Phila Pa 1976) 2009; 34: 840-4. 11. Gau YL, Lonstein JE, Winter RB, et al. LuqueGalveston procedure for correction and stabilization of neuromuscular scoliosis and pelvic obliquity: a review of 68 patients. J Spinal Disord 1991; 4: 399410. 12. Kuklo TR, Bridwell KH, Lewis SJ, et al. Minimum 2-year analysis of sacro-pelvic fixation and L5–S1 fusion using S1 and iliac screws. Spine (Phila Pa 1976) 2001; 26: 1976-83. 13. Schwend RM, Sluyters R,Najdzionek J. The pylon concept of pelvic anchorage for spinal instrumentation in the human cadaver. Spine (Phila Pa 1976) 2003; 28: 542-7. 14. Peelle MW, Lenke LG, Bridwell KH, et al. Comparison of pelvic fixation techniques in neuromuscular spinal deformity correction: Galveston rod versus iliac and lumbosacral screws. Spine (Phila Pa 1976) 2006; 31: 2392-2398. 15. Modi HN, Hong JY, Mehta SS, et al. Surgical correction and fusion using posterior-only pedicle screw construct for neuropathic scoliosis in patients with cerebral palsy: a three year follow up study. Spine (Phila Pa 1976) 2009; 34: 1167-75. 43 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-67-000 9/2012 JC CA #8980A / DJ10056A Scoliosis in Cerebral Palsy Kirk Dabney, MD Cerebral palsy (CP) is a heterogeneous disorder that is caused by a static lesion to the immature motor cortex. The prevalence of scoliosis in the cerebral palsy population is proportional to the severity of neurologic impairment. It ranges from 5% in spastic diplegia to 64-74% in spastic quadriplegia. Progression often also occurs during adulthood especially in lumbar and thoracolumbar curves > 40 degrees. Curvatures > 60 degrees begin to affect sitting and standing balance as well as head control. Pelvic obliquity is commonly associated with scoliosis. Surgical management is indicated in patients not tolerating seating (in non-ambulators) or standing (in ambulators). Surgery may also be indicated to alleviate pain or to correct pelvic obliquity prior to correcting a concomitant hip dislocation. Sagittal plane deformities can occur in combination with scoliosis or in isolation in the patient with CP. Hyperlordosis and hyperkyphosis can occur with or without scoliosis and in isolation are much less common than scoliosis in the patient with CP. In our experience, sagittal plane deformity alone can also cause seating problems and pain, especially when > 70 degrees. 45 Problems with Luque / Galveston Instrumentation The gold standard for surgical instrumentation in the treatment of neuromuscular scoliosis in the past has been Luque rod instrumentation (with Galveston extension to the pelvis and cross-linkage [to prevent rod rotation and shift] with sublaminar wiring). The unit rod incorporated these concepts into one unit and serves as a very powerful cantilever to correct scoliosis (especially lumbar and thoracolumbar curves) and pelvic obliquity. Technical difficulties with the unit rod include difficulty with pelvic limb placement (especially with hyperlordosis), problems judging final rod length proximally, inadequate correction of severe rotational deformity, and having sufficient lever to correct upper thoracic scoliotic and kyphotic curves. Due to the modularity of the EXPEDIUM® Neuromuscular System and the unique implant additions such as proximal rod connectors, reduction screws and an assortment of sacral iliac fixation options, these difficulties are significantly lessened. Patient Assessment Patients with cerebral palsy often have associated medical comorbidities which correlate strongly with postoperative complications, including gastroesophageal reflux, aspiration pneumonia, poor nutrition, the presence of a seizure disorder, and low bone mineral density. These should be identified preoperatively and co-managed medically. Physical examination should assess sitting and standing balance, pelvic obliquity, curve stiffness, and the curvature’s coronal, sagittal and rotational components. The orthopedic surgeon should also evaluate for the coexistence of hip subluxation or dislocation. The patient should also have a detailed neurological examination. Curves that are stiff in both the coronal (scoliosis) and sagittal planes (severe hyperlordosis and hyperkyphosis) may require anterior release or preoperative traction. The side-bending test is helpful for assessing coronal plane stiffness (Figure 1). Sagittal plane stiffness should also be assessed by seeing if kyphosis will reduce in the supine position over a bolster and assessing the flexibility of hyperlordosis by hyperflexing both the hips and pelvis in the supine position. If the deformity Figure 1. Side-Bending Test. The cannot be reduced in patient is bent over the examiner’s thigh at the apex of the curve. If either plane, anterior the patient’s curve reverses and discectomy should be the pelvis levels out becoming considered. It is important perpendicular to the trunk, the to assess and distinguish the curve remains flexible enough to correct through posterior fusion coexistence of a hip flexion alone. If not an anterior release is contracture and adduction performed. contracture. This can be done by stabilizing the pelvis in a neutral position (flexing the opposite hip to level the pelvis to detect hip flexion contracture and assessing hip abduction with the pelvis in neutral obliquity). If these contractures are present, the parents should be warned that a staged hip contracture release may be necessary 4 to 6 months after spinal surgery (see Figure 2 in Chapter 3, Unique Challenges with Scoliosis and Dislocated Hips). The surgeon must also assess the need for fusing to the pelvis. In the patient with CP, this is almost always necessary to prevent distal extension (late pelvic obliquity) of a curvature fused too short. Patients with a poor “righting reflex” should be fused to the pelvis and up to T1. The righting reflex is noted on physical exam in that the patient demonstrates a balancing reflex/ability to center the head over the body center or pelvis. 46 Standing or sitting AP and lateral radiographs are important not only to measure curve magnitude but to look at balance. Coronal and sagittal bending films are operator dependent and, if done correctly, can provide important information about curve flexibility. This author no longer obtains traction films, since the amount of correction that can be obtained using cantilever correction at the time of surgery is better than traction films would predict. The author does not routinely use bending films to determine anterior release; the clinical bending test is preferred because bending films are also very operator dependent and may be misleading. The author uses the amount of stiffness of the pelvic obliquity on physical exam to see whether or not the pelvis can correct back into a physiologic range by bending and adding traction to the patient and looking at visually. Others surgeons have stated that if the main curve correction is not better than 40 degrees on coronal bending, then anterior discectomy is warranted. It is reasonable to consider anterior release if residual kyphosis and lordosis is > 60 degrees on sagittal bending films. The anesthesiologist and surgeon should be prepared for the possibility of major intraoperative blood loss. Type and cross-matched blood (up to twice the patient’s blood volume) should be available as well as fresh frozen plasma and platelets. A cell saver machine is also helpful. Antifibrinolytics (transemic acid) should be considered to help reduce intraoperative bleeding. Spinal cord monitoring should be incorporated. Any loss of sensation, muscle spasticity, or loss of bladder or bowel control would be considered a poor outcome. Surgical Treatment and Instrumentation The principles of spinal deformity correction in cerebral palsy are to: 1) level the pelvis (by correcting pelvic obliquity) with sitting and/or standing; 2) center the head over the trunk and pelvis via C7 to CSL (center sacral line); 3) restore trunk balance (rib cage centered over the pelvis); 4) restore sagittal balance (lumbar lordosis and thoracic kyphosis, including the correction of anterior or posterior pelvic tilt, respectively); 5) maximize segmental fixation in the face of what is often osteoporotic bone; and 6) minimize operative time in these patients, who often have multiple comorbidities and who are at greater risk for wound infection. Rotational correction is less important unless the rotational deformity is affecting pelvic or trunk balance. Correcting the Pelvic Obliquity The correction of pelvic obliquity is detailed in Chapter 4 (The Correction of Pelvic Obliquity Using Cantilever Correction). Fixation and fusion to the pelvis should be considered in every patient with CP to prevent late pelvic obliquity. Only if the ambulatory patient has a level pelvis and adequate balance (“righting reflex”) should the surgeon consider ending fixation more proximal at L4 or L5. If fixation to the pelvis is not done, fixation in the lumbar spine at least 3 levels (6 screws) with pedicle screw fixation and complete derotation is essential. Cantilever correction and fixation to the remainder of the spine using sublaminar wires is then to be utilized as described. 47 Correction of Main Thoracic Curves Problems with the head being off center are most problematic in thoracic curvatures. Spinal curvatures in the thoracic region are more difficult to correct with cantilever correction. In such cases, starting distally at the pelvis or in the lumbar spine leaves a short lever arm at the proximal end of the rod by the time the surgeon reaches fixation in the thoracic spine. This makes it very difficult (if not impossible) to center the head over the remainder of the thorax and pelvis. This type of curvature is very difficult to correct with the traditional unit rod since it requires distal fixation into the pelvis first (Figure 2A). With this type of curvature, fixation using the Expedium Neuromuscular System can be started proximally first due to its modularity (Figure 2B). After exposing the spine and pelvis (in cases where pelvic fixation is required), pelvic screws and sublaminar wires are placed as previously described. The rod construct is then preassembled by first connecting the pre-contoured rods with the proximal closed rod connector at the top and placing a cross connector in the lumbar region. The rods should be parallel from proximal to distal with respect to their contour. Next, the top of the rod construct is secured using sublaminar wires, hooks, or screws from T1 down to the apex of the curvature. After the apical vertebra is secured to the rod, cantilever correction can be performed by gradually pushing the rod down to the next more distal vertebra, tightening the sublaminar wire or reducing into the implant, pushing the rod down to the next more distal vertebra, and then tightening the wires/implants, performing the same maneuver progressively down the spine until the pelvic screws are reached (Figure 2C). The lateral rod connectors are then utilized to connect the rod to the pelvic screws. Using this “proximal to distal” cantilever technique for cantilever correction allows for a better lever arm to correct thoracic scoliosis as well as thoracic kyphosis (Figure 2C). 48 Figure 2A. It is difficult to cantilever this type of thoracic curvature using the unit rod due to insufficient lever arm. Figure 2A Figure 2B. This diagram shows proximal to distal cantilever technique that can be used for thoracic scoliosis. Preoperative and postoperative radiographs are shown. Figure 2B Figure 2C. Similarly, proximal to distal cantilever technique can be used for thoracic kyphosis. Preoperative and postoperative radiographs are shown. Figure 2C 49 Correction of Lumbar and Thoracolumbar Curves Trunk imbalance is most problematic in lumbar and thoracolumbar curves. Trunk Balance is achieved with cantilever correction from distal to proximal as described for the correction of pelvic obliquity. In cases where severe rotation is present (in the trunk or pelvis) and it is significantly impacting sitting or standing balance, pedicle screws should be used around the apex of the curve instead of sublaminar wires. Depending on the severity of the deformity, an Expedium uniplanar screw provides easy sagittal accommodation of the rod to screw interface and gives maximum axial derotation transmission of forces. If the curve is too severe, then polyaxial screws are used to accommodate the coronal and sagittal plane alignments but sacrifice axial correction force. Reduction screws can make the rod insertion easier and allow even more slow, controlled reduction of the deformity over multiple levels, at once lessening screw pull out (especially in these patients who already have reduced bone mineral density) (Figure 3A and 3B). Cantilever correction is then performed first, securing the rod to the spine from distal to proximal with sublaminar wires or pedicle screws. A derotation maneuver at the apical level of the curve is also performed if pedicle screws were placed after the cantilever correction. The multiple reduction instruments in the Expedium Neuromuscular System allow slow, careful axial correction to minimize screw loosening in the bone. In addition, it is important to have screws placed in at least 4-5 vertebral levels around the apex in order to distribute the derotational force and prevent plowing of the screws within bone that is often osteoporotic. Larger diameter screws filling the pedicle to its cortical wall and/or the VIPER Cortical Fix Screw (Figure 4) can be helpful to reduce pullout problems of the screws when there is poor bone quality. 50 Figure 3A. Reduction of hyperlordosis can be achieved using pedicle screws with reduction posts. Figure 3B. Preoperative and postoperative photographs are shown. Correction of Hyperlordosis or Hyperkyphosis Restoring sagittal balance is critical. Excessive kyphosis or lordosis may occur with or without the presence of scoliosis. Reduction pedicle screws placed in the region of the hyperlordotic lumbar spine can significantly facilitate slow, controlled correction over multiple levels at the same time to minimize the risk of loss of fixation. After securing the rods to the pelvis with iliac screws as described in Chapter 4 (The Correction of Pelvic Obliquity Using Cantilever Correction), the pre-contoured rods are pushed down into the reduction post and secured with the set screw. Reduction of the hyperlordosis is achieved by gradually screwing down the set screws (Figure 3). Great care is taken to notice any evidence of posterior pullout of the screws. Cortical fixation screws (Figure 4) may be used to help prevent pullout. An additional suggestion is the use of Clements pedicle dilators which dial up the size of the pedicle to allow a bigger diameter screw and better pedicle cortex fixation. If pullout starts to occur despite all of the above-mentioned techniques, then a sublaminar wire can be placed at the same level as the screw for additional fixation, acting as belt and suspenders. Once the hyperlordosis is reduced, the remainder of the spinal instrumentation is completed. Figure 4. This diagram shows the screw design of VIPER Cortical Fix Pedicle Screw. Cortical Fix Thread Hyperkyphosis can also be corrected using the Expedium Neuromuscular System using either a “distal to proximal” (in cases of lumbar or thoracolumbar kyphosis) or “proximal to distal” cantilever technique (in the case of a thoracic kyphosis) (Figure 2). It is critical that fixation is completed up to at least T1 to prevent “drop-off” proximal junctional kyphosis at the cervicothoracic junction. Firm fixation at the proximal most end is recommended. The author usually does not use laminar hooks at T1, as they can result in unreliable fixation in soft bone as well as tending to pull out in patients with spasticity who do a lot of head posturing. Pedicle screws in T1 or T2 can be another alternative as can transverse process hooks on T1 if pedicle screws cannot be inserted. Maximize Fixation in Face of Osteoporotic Bone In patients with quadriplegic CP who have severe osteoporosis, a full evaluation of the patient’s bone density should be performed using DEXA scanning. In addition, evaluation of the patient’s vitamin D metabolism should be performed by obtaining a full vitamin D panel as well as calcium, phosphorous, and alkaline phosphotase levels. Preoperative treatment may be necessary with several courses of intravenous pamidronate (in cases of osteoporosis) or with vitamin D (in cases of osteomalacia, usually secondary to seizure medication). Sublaminar fixation is preferred since laminar fixation has been shown to be biomechanically stronger than pedicle screws in the presence of osteoporotic bone1. If screw fixation is necessary, such as in cases of hyperlordosis or severe rotation, sublaminar wire fixation should be added at each level of screw fixation. In the author’s experience, this is most commonly necessary at the L5 level. As described previously in this chapter, cortical fixation screws (Figure 4) and/or pedicle dilatation techniques can be effective adjuncts. Standard EXPEDIUM/VIPER Dual Lead Thread 51 Minimizing Operative Time Sublaminar Wire Passage Minimizing operative time is crucial, because the longer the spine is exposed, the higher the risk of bleeding and infection. Generally, in surgeons well trained in both techniques, passing sublaminar wires is faster than pedicle screw fixation. Most routine spinal deformity corrections can be performed with segmental sublaminar wire fixation alone. It is also more cost effective than screw fixation. When pedicle screw fixation is performed, it is limited only to the levels that are needed, usually around the apex of the scoliotic curve or in the region of hyperlordosis. In cases where epidural bleeding is problematic, pedicle screw fixation can also be considered to avoid further epidural bleeding. In the author’s experience, however, epidural bleeding can be minimized by limiting removal of the ligamentum flavum to the central portion and avoiding going too lateral where the veins are more prominent (Figure 5). When epidural bleeding does occur, the application of Surgifoam and thrombin usually stops epidural bleeding. Other considerations for minimizing the operative time include careful preoperative assessment of curve stiffness. A posterior only approach is usually all that is needed for curve correction when the curve is < 70 degrees and still flexible. In curves > 70 degrees, it may be safest to perform anterior release in a stiff curve to minimize the technical difficulty and risk of loss of fixation while attempting to correct a stiff deformity from a posterior approach alone. Although not used by this author, some surgeons prefer using anterior instrumentation to derotate the spine after anterior release which then makes posterior correction even easier. Some surgeons prefer a posterior only approach utilizing extensive posterior osteotomies and even vertebral body resections in the case of severely stiff curves. Other surgeons may prefer some form of preoperative or perioperative traction. After the spine is completely exposed, the spinous processes are completely removed and the ligamentum flavum is carefully removed to expose the sublaminar spaces. Double Luque wires are bent (prebent wires are also available) and passed under the lamina of L5 up to and including the T1 lamina. The radius of curvature for the wire bend must approximate the width of the lamina to allow safe passage of the wire. Two double wires are passed at the L5 and the T1 lamina only while single wires are passed at each of the other levels. The wires are pulled up to equal length and bent next, with the midline bent flat down onto the lamina and the beaded end flat down lateral onto the paraspinous muscles. This helps prevent the wires from getting inadvertently pushed into the spinal canal and allows for easier wire organization (Figure 5 A-G). 52 Figure 5. Luque Wire Passage-when passing wires, it is important to roll the wires under the lamina (A-D), being careful not to catch the tip under the lamina (E), which will lever the wire into the canal and place pressure on the spinal cord. Wires are bent down to the midline in the middle and the ends are bent down flat against the paraspinous muscles (F-G). Figure 5A Figure 5F Figure 5B Figure 5C Figure 5G Figure 5D Figure 5E 53 Outcomes The long-term results of cantilever correction of spinal deformity have been with the unit rod. In our experience, approximately 70% curve correction is achieved (in both posterior only or when anterior release is indicated). Improved appearance, quality of life, and ease of care is achieved in the majority of patients. Difficulties remain, however, using the unit rod when correcting hyperlordosis, judging rod length, correcting severe rotation, and having a sufficient lever arm to correct more proximal thoracic curves. The Expedium Neuromuscular System can produce similar results through cantilever correction of both scoliotic and sagittal plane deformities in patients with CP. In addition, pelvic fixation with iliac screws is technically easier than with the unit rod, especially in the face of severe lordosis. Problems with judging rod length is obviated with the Expedium Neuromuscular System as the rod is merely cut at either the top or bottom end of the fixation level. Severe rotational deformity can be addressed by adding pedicle screw fixation, especially Expedium uniplanar screws. Finally, thoracic curves can be more easily corrected using a ”proximal to distal” cantilever maneuver which cannot be performed with the unit rod, because the pelvic limbs must be placed first. 54 References : 1. Coe JD, Warden KE, Herzig MA, et al. Influence of bone mineral density on the fixation of thoracolumbar implants. A comparative study of transpedicular screws, laminar hooks, and spinous process wires. Spine (Phila Pa 1976) 1990;15:902-7. 55 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-63-000 9/2012 JC CA #8786 / DJ10058A Spinal Fusion in Myelomeningocele Peter G. Gabos, MD Surgical treatment of spinal deformity in patients with myelomeningocele presents a group of challenges unique to this patient population. The combined effects of severe multiplanar spinal deformity that can include scoliosis, kyphosis and lordosis with elements of congenital vertebral malformations, absence of posterior elements, markedly abnormal pedicle trajectory, compromised skin, subcutaneous dura, marked truncal obesity, and disuse osteopenia can challenge all aspects of surgical care. Confounding medical conditions that can have a major impact on postoperative morbidity include shunted hydrocephalus, Chiari malformation, tethered cord, bladder augmentation procedures, bowel incontinence, thoracic insufficiency syndrome, and marked lower extremity contractures. 57 Preoperative Evaluation In general, scoliotic curves < 40 to 50 degrees can be managed nonoperatively. Bracing can be utilized in select cases to assist in truncal stabilization and handsfree sitting but would not typically be used for curve management. Poor skin and anterior-placed genitourinary and bowel appliances can render bracing difficult at best. In addition to curve magnitude, these patients have other functional problems that may make correction and fusion of their spine desirable. The impact of curvature on seating and, where applicable, on the use of long-leg and pelvic-based lower extremity orthoses may be important to avoid pressure ulceration over insensate skin. Spinal stabilization may be advantageous if progressive curvature makes hands-free sitting difficult or impossible. Signs of impaired pulmonary function, including the presence of a “Marionette sign” or increasing loss of thoracic volume due to increasing spinal deformity or diaphragmatic intrusion into the thorax, should be recognized. In the presence of kyphosis, pressure ulceration over the apex of the kyphus can be a lifelong struggle, and chronic ulceration and vertebral osteomyelitis can occur (Figure 1). Figure 1. Preoperative photograph of a chronically infected myelomeningocele patient who sustained breakdown over an area of untreated severe lumbar kyphosis. There is a draining wound, loss of skin coverage and exposed, necrotic bone. 58 Preoperative evaluation should involve comprehensive characterization of all aspects of the spinal deformity. In addition, the exact location of bifid or open bony segments is critical to avoid dural tearing or further neurologic injury, since posterior deficiencies cephalad to the frankly obvious level of myelomeningocele can also exist. Pelvic and sacral anatomy must also be rigorously defined, as the majority of patients will require stabilization and fusion to the pelvis. Flexibility evaluation can include supine bending, fulcrum bending, and/or spinal traction films to assess the need for spinal osteotomies and/or vertebral column resection. Computed tomography (CT) scan and the use of 3D CT technology can facilitate preoperative planning of surgical approach, correction techniques, and spinal and pelvic anchor placements, especially in cases involving multiple congenital malformations where plain radiography alone may not be sufficient. Magnetic Resonance Scanning (MRI) scanning can evaluate for subcutaneous dura, spinal cord tethering, Chiari malformation, and syrinx. If the patient has a pre-existing ventriculoperitoneal (VP) shunt, a preoperative shunt evaluation should include radiographic confirmation of the structural integrity of any shunts from point of exit from the skull, with particular attention to the cervical region where shunt fracture is most common. Preoperative head CT or MRI can be invaluable as a comparison tool for assessing for postoperative hydrocephalus should shunt failure after deformity correction occur. This is of particular importance if a structural failure (separation) of a shunt is noted preoperatively, since arrested hydrocephalus should not be assumed. Postoperatively, radiographic confirmation of shunt integrity should be obtained. Symptoms of hydrocephalus due to shunt failure can include headache, nausea, emesis, lethargy, extra-ocular movement abnormalities, cognitive changes, neurologic deterioration, and even respiratory arrest and death. Whenever feasible, pulmonary function testing may be useful to characterize components of pulmonary insufficiency. A multidisciplinary approach, including consultation with colleagues from neurosurgery (for shunt management and consideration for detethering), plastic surgery (for assistance with closure and wound management), urologic surgery (for decreasing the risk of postoperative urinary tract infections, placement of preoperative urinary catheters if bladder reconstruction or diversion procedures have been performed), and rehabilitative medicine (for enhancing postoperative rehabilitation and functional outcome) can optimize decisions regarding timing of surgery and plans for postoperative recovery. The environment to which the patient will be returned after surgery can also greatly impact the overall success of the procedure, and evaluation of family structure and dynamics by a team from Social Services is essential. Functional assessments should include detailed evaluation of motor and sensory level, upper and lower extremity use, ambulatory function and adaptive equipment where applicable, method of catheterization, and overall level of desired postoperative independence. Preoperative nutritional parameters including serum albumin and white blood cell count, urinalysis, and urinary cultures should be obtained, and treatment of urinary tract infection should be completed prior to surgery. Aggressive postoperative nutrition via hyperalimentation may be required to support wound healing. Complete assessment of skin integrity, truncal obesity, dentition, and overall hygiene should be performed. Preoperative Considerations Surgical approach will vary based on curve stiffness and severity and may include single-stage or two-stage anterior and posterior surgery. Preoperative halo traction may be indicated for markedly stiff and/or severe scoliosis or kyphosis. If a halo is to be placed, the location of pre-existing VP shunts must be considered. Single-stage, posterior-only approaches can be performed in the majority of cases but may require the use of advanced osteotomy techniques, including vertebral column (apical) resection. Neuromonitoring is considered on a case-by-case basis depending on underlying neurologic deficit. In the non-ambulatory patient with little or no lower extremity function, upper extremity monitoring in the surgical position for a period of time may be useful to prevent brachioplexopathy or peripheral nerve compression, especially in cases where prolonged surgical time is anticipated. Consultation with a plastic surgeon for incision placement may be helpful in optimizing wound coverage, including the use of preoperatively placed soft tissue expanders. Surgical Techniques Skin incisions must take into account pre-existing skin scarring, and the skin quality must be assessed over the ilia bilaterally if pelvic fixation will incorporate fixation over the posterior superior iliac spines (PSIS). Careful attention to body positioning on the spinal OR table must incorporate adequate padding and positioning of all soft tissue and bony prominences, and lower extremity positioning may be difficult in the face of significant lower extremity deformity or contracture. Extending or flexing the hips can be helpful in achieving more or less lumbar lordosis, respectively. If fluoroscopic imaging will be utilized for optimizing pelvic fixation, taking the necessary images prior to prepping and draping will confirm visualization of the iliac wing teardrop for placement of PSIS or second-sacral (S2) to iliac screws to assure unobstructed radiographic access to the pelvis (Figure 2). Figure 2. An intra-operative FlouroScan image of the iliac teardrop is taken bilaterally to assure an unobstructed radiographic view during pelvic screw fixation. The images are obtained after initial positioning of the patient on the operating room table, just prior to prepping and draping. 59 As the skin and soft tissue dissection proceeds, definitive knowledge of the location of potentially exposed dural elements and posterior bony deficiencies must be taken into account to avoid dural tearing or spinal cord injury. In select cases, spinal cord transection and durotomy may be performed at this point. compromised and adherent areas. Iliac osteotomy should be performed with caution or not at all if PSIS screw placement is desired, as it may compromise fixation. Fixation options can vary based on surgeon experience and corrective goals. For the majority of patients, surgical goals will include restoration of spinopelvic balance for wheelchair use, so strategizing for reduction of pelvic obliquity and pelvic-to-shoulder (truncal) rotation is important. Restoring the center sacral line (CSL) to a more neutral position to bring the head back over the pelvis in both the sagittal and coronal planes also allows for better weight distribution from the ischial tuberosities to the posterior thighs to avoid skin breakdown and pressure ulceration. Coccygeal morphology should also be noted, as excessive surgical lordosis may impact ulceration over this prominence in thinner patients. Anchors based within the posterior elements, such as sublaminar wires or laminar hooks, cannot be utilized in regions of frank posterior element deficiency. Pedicle screws can offer improved versatility and strength of fixation in the spine and are essential at areas where vertebral column resection or osteotomies are planned. Temporary stabilization rods placed into pedicle screws around large osteotomies, and areas of vertebral column resection, can help avoid abrupt spinal translation, typically that which occurs from the distal spinal segment translating anteriorly. Reduction screws and polyaxial screws allow for easier and more gradual rod capture into the screw heads, especially in osteopenic bone. Uniplanar screws can enhance spinal derotation, typically at curve apices and at upper and lower construct foundations. In regions of posterior element absence, disc excision and placement of structural interbody supports from an anterior or posterior approach can add stability and surface area for fusion. When performed posteriorly, these supports are typically inserted prior to rod placement. The pedicles in the lower vertebral segments may be difficult to access secondary to marked lateral-to-medial trajectory from the embryologic failure of bony closure. Obese body habitus combined with increased lumbar lordosis can pose a particularly difficult problem to cannulating the pedicles. Osteotomy of the iliac wings can allow better access and trajectory for pedicle screw placement into the lower lumbar vertebral bodies and sacral promontory (Figure 3). In regions where dense scarring and compromised skin overlies the posterior iliac spines, osteotomizing the posterior iliac crest and leaving a thin rim of crest cartilage (if present) or bone attached to the overlying skin can also serve to prevent skin tearing in 60 Figure 3. Osteotomies of the iliac wings can allow better access and trajectory for pedicle screw placement into the lower lumbar vertebral bodies and sacral promontory (top Image). Second-sacral (S2) to iliac screw fixation allows deeper seating of the iliac screws to avoid skin breakdown over the screw heads and lower spinal rods (see Chapter 2: Sacropelvic Fixation Options), and is not compromised by iliac crest osteotomy. In many cases, the S2 to iliac screw allows rod seating directly from the spine to pelvis without the need for offsets or other lateral connectors to link up the rod, which may eliminate an area of potential fixation failure. Placement of caudal lumbar screws and/ or first sacral (S1) screws (if utilized) prior to selection of the start point for S2 to iliac screw insertion is helpful if direct rod seating from the spine is desired (Figure 4). If PSIS screw use is desired, recession of the screw head into a surgically-created iliac slot should be strongly considered to avoid implant prominence (Figure 5). Other screw options would include sacral fixation utilizing S1 and S2 sacral screws. Figure 5. A surgically created iliac slot will help avoid implant prominence when PSIS screw is used. Figure 4. Image shows the straight alignment of L5 pedicle screw, S1 promontory screw and S2 to iliac pelvic screws. The screws are placed in that sequence to allow rod seating directly from the pelvis to spine without the need for any type of lateral or offset connector from the pelvic fixation to the rod. Techniques of rod placement for deformity correction can vary depending on surgical goals and surgeon preference. Principles of load-sharing across multiple implants and gradual rod reduction should be adhered to in order to prevent pull-out of implants in osteopenic bone. Rod reduction techniques can vary from sequential versus simultaneous dual rod placement. When placing dual rods simultaneously, a surgeon-contoured or pre-contoured set of rods can be linked proximally with a cross-connector similar to unit rod instrumentation utilizing cantilever correction. In this case, the pelvic screws are captured first, followed by caudal to cephalad gradual rod capture. En bloc versus segmental rotational correction can be achieved with uniplanar screw fixation, typically required at curve apices. Other corrective techniques that can be utilized include translational correction, distraction (to restore kyphosis) and compression (to restore lordosis) and in situ rod bending. After initial correction is completed, intraoperative fluoroscopic images allow for assessment of any residual pelvic obliquity, which can be further improved utilizing compression or distraction across the lumbosacral and pelvic fixation. A full-length lateral spinal radiograph is also obtained to assess overall sagittal contour and screw length prior to wound closure (Figure 6 A-I). 61 Figure 6A. Preoperative sitting posteroanterior radiograph of a mid-lumbar level, non-ambulatory adolescent with progressive kyphoscoliosis and increasing difficulty with hands-free sitting in his wheelchair. Figure 6B. Preoperative sitting lateral radiograph. Figure 6D. Intraoperative photo after spinal exposure, detethering and dural patch reconstruction. A posterior lumbar interbody fusion is being performed to enhance curve flexibility, surface area for fusion and spinal stability. 62 Figure 6C. Preoperative skin over the region of previous myelomeningocele repair demonstrates markedly scarred, paper-thin skin that was adhered to the underlying posterior iliac crests. Figure 6E. Intraoperative photo taken after segmental pedicle screw placement. Sacral-2 (S2) to iliac screws have been placed to avoid screw prominence at the posterior superior iliac spines (PSIS), and the iliac crests were osteotomized to protect the overlying skin and to facilitate screw placement in the lower lumbar spine and sacral promontory. Wound Management Figure 6F. Intraoperative photo taken after dual rod placement using techniques of en bloc spinal translation and direct vertebral derotation. Figure 6G. Intraoperative appearance after skin closure utilizing wide mobilization of the subcutaneous tissue and drain placement to decrease skin tension from hematoma formation. Operative success is critically impacted by postoperative wound management. Skin closure in these patients can be extremely complex, and inability to close the skin in cases of maximal scoliosis correction can lead to catastrophic deep infection, especially over regions of exposed dura. The use of preoperative skin expanders may be considered in some cases. Careful skin and soft tissue handling during the entire procedure is critical to success. Mobilization of large soft-tissue flaps, muscle rotation flaps from the latissimus dorsi or abdominal obliques, and skin grafting may be necessary. Careful preoperative assessment of dependence on upper extremity and shoulder girdle function for mobility is important in counseling the patient or family regarding the potential effects of the use of muscle transfer flaps on crutch or walker use or wheelchair propulsion. Use of perioperative deep and superficial drains may help to alleviate swelling and its subsequent effect on skin tension. Specialized mattresses for use during postoperative recovery and frequent inspection of the wound for dehiscence, signs of infection, or soiling should be utilized, and the nursing staff should be carefully instructed in the proper replacement of occlusive dressings. Postoperative Care The majority of patients will recover in an ICU setting until they are medically stabilized. Upon successful extubation, sometimes up to several days postoperatively, patients are moved out of bed and into a chair. Their own wheelchair may need modifications and should be evaluated. Daily inspections of the incision are performed, and the lower portion is always kept resealed with a waterproof occlusive dressing to prevent soiling. Any surgical drains are kept in place until output totals less than approximately 50 to 100 cc of fluid. Weight bearing, if applicable, and transfer training are started immediately as tolerated. Figure 6H. Postoperative sitting posteroanterior radiograph one year after surgery. There were no wound complications, and the patient could enjoy handsfree sitting and unlimited sitting tolerance. Figure 6I. Postoperative sitting lateral radiograph one year after surgery. 63 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-64-000 9/2012 JC CA #8781 / DJ10052A The Patient with Spinal Cord Injury: Surgical Considerations Particularly with Respect to the Sagittal Plane Amer F. Samdani, MD Patients with spinal cord injury (SCI) fall under the spectrum of neuromuscular scoliosis, and many of the treatment principles outlined elsewhere in this monograph apply. However, distinct differences exist in patients with SCI, the knowledge of which may minimize the occurrence of complications. 65 Preoperative Considerations As for all patients with neuromuscular scoliosis, the preoperative evaluation includes a rigorous medical and anesthesia work-up. At our institution, we perform a 30 minute Hibiclens back scrub starting three days preoperatively. Many of these patients are on an every other day bowel program, and we ensure a bowel movement the day prior to surgery. Patients with SCI may develop deep venous thrombosis, and preoperative ultrasound of all four extremities is performed. These usually reveal clinically insignificant, chronic superficial vein thrombosis which then provide a baseline for comparison postoperatively. However, there have been reports of patients having clots in deep veins which have delayed surgery.1 We obtain a preoperative evaluation with physical and occupational therapy. The purpose of this evaluation is multifold: First, the therapist outlines to the patient and family the potential positive and negative functional consequences of a straighter, yet stiffer, spine. They emphasize the effect on activities of daily living that the patient currently performs. Part of this evaluation may include placing a rigid thoracolumbosacral orthosis (TLSO) on the patient to emulate spine fusion. This is often performed on marginally ambulatory patients to predict their ability to ambulate postoperatively. In addition, the TLSO trial on patients with high tetraplegia can predict what happens with loss of compensatory function patterns that may affect upper extremity function following a spinal fusion. The preoperative physical therapy evaluation also reinforces the postoperative restrictions. Generally, for a period of six months, the patients will not self-propel their wheelchair, assist in transfers, or flex their hips past 90 degrees. Factors which influence the duration of these restrictions include patient body habitus and bone quality. 66 The preoperative evaluation includes standard PA/lateral sitting full spine radiographs and supine full spine bend or traction films. In particular, a high quality sitting lateral radiograph is imperative, as these patients rely on an exaggerated kyphosis to permit the performance of activities of daily living, including self-catheterization and feeding. We analyzed the sagittal profile in 30 patients with SCI at our center and found the usually neutral T10-L2 region measured a kyphosis of 19.8 degrees. Similarly, the lumbar lordosis averaged 9.8 degrees. Thus, fusing these patients with a ‘normal’ sagittal thoracic kyphosis and lumbar lordosis may prevent them from performing at their preoperative level. This is particularly true of patients with limited upper extremity functioning. It is very instructive for the surgeon to observe these patients performing ADLs to truly appreciate the importance of maintaining their kyphosis. When a patient with tetraplegia cannot sit up, the authors will make a temporary TLSO and see how the patient can function. If the patient does well in the TLSO, then a lateral radiograph in the TLSO is obtained and the rods bent to match. Sometimes a malleable rod can be bent preoperatively against the film and then sterilized. Intraoperative Considerations Similar to other patients with neuromuscular scoliosis, these patients may benefit from the use of antifibrinolytics, although this should be weighed against the increased risk of deep venous thrombosis. The surgeon should also consider the use of a central line, as patients with cervical level injuries may demonstrate hemodynamic instability secondary to autonomic dysfunction.2 Furthermore, the anesthesia team should refrain from the use of succinylcholine, as it may cause release of potassium and sudden cardiac arrest.3 Intraoperative antibiotics includes strong coverage of gram positive and gram negative bacteria, as previously reported by others.4 We prefer cefazolin and gentamicin, with vancomycin substituting when there is a penicillin allergy. In addition, we mix vancomycin with the bone graft and also use vancomycin powder spread on the wound surfaces prior to closure, as demonstrated by others, to decrease the incidence of infection.5 Positioning Proper positioning is important in these patients. Although we prefer the Jackson table, often these patients have hip flexion contractures and are better suited to a regular OR table which allows greater customization by altering the size of gel rolls. Patients with severe flexion contractures (> 70 degrees) may require the four poster frame. On a rare occasion, these patients may need a hip contracture release a couple of weeks before the spine fusion. Hip contracture releases should be performed with a longitudinal incision, since horizontal incisions commonly dehisce, and this may delay the subsequent spine fusion. In addition to appropriate pressure point distribution, Figure 1. A 14 year old patient with a large, stiff deformity with marked pelvic obliquity. A) Pre-op sitting PA, and B) pre-op sitting lateral. C) She underwent a T2 to pelvis posterior spine fusion utilizing temporary internal distraction to maximize correction of the pelvic obliquity. Image shows Post-op PA x-ray. Figure 1A the surgeon should ensure the maintenance of optimal sagittal profile. In particular, the Jackson table will lordose the thoracic spine unless the chest roll is positioned caudal to the nipple line. Similarly, in some patients it is easy to overextend the hips, which imparts a nice lumbar lordosis well suited for an ambulatory patient but likely very different than the preoperative sitting sagittal profile of the patient. Overlordosing the lumbar spine in combination with decreasing thoracic kyphosis will result in significant negative sagittal balance with a decrease in the ability to perform daily tasks. In addition, a severe junctional proximal cervical thoracic kyphosis may result.6 Neuromonitoring We always perform upper extremity monitoring to prevent any loss of function from brachial plexus palsy secondary to prolonged positioning. Any patient with incomplete injury, including ASIA B (sensation only), should have neuromonitoring customized to their neurological status. Traction As a general rule of thumb, if the preoperative supine films demonstrate a residual pelvic obliquity of > 20 degrees, the authors consider utilizing intraoperative traction. This could be done with halo femoral traction (which provides excellent results with respect to correction of pelvic obliquity) or with a temporary device placed between the pelvis and two ribs above the end vertebra. The spine is distracted every 15-30 minutes, allowing for viscoelastic relaxation (Figure 1). In patients with an incomplete SCI and functional lower extremities, close attention to neuromonitoring is imperative with relaxing of the distraction for any significant change. Figure 1B Figure 1C 67 Construct At a minimum, the construct must include large iliac bolts with a strong foundation of screws at the base (iliac bolts and S1 screws are preferred by the authors), pedicle screws across the thoracolumbar junction, and pedicle screws at the top of the construct. The authors’ preference remains erring on the side of more screws, particularly in patients with poor bone quality (Figure 2). Strategically placed reduction screws at the apex of the concavity assist with rod reduction. Wherever possible, rod material should be a titanium or cobalt chrome alloy. The higher incidence of infections seen with stainless steel discourages its use in this high-risk population. Rod diameter is often 5.5mm, but in heavier patients (>70 kg), 6.35mm (¼ inch) rods should be considered, bone quality permitting. Metabolic syndrome may develop in these patients over the years, with a consequent substantial increase in BMI. Figure 2. The VIPER® Cortical Fix Screw maximizes pullout strength with its single to dual lead design. It is an excellent option in patients with poor bone quality, particularly in high stress areas. 68 Several features of the EXPEDIUM® Neuromuscular System facilitate the operation in these patients (Figure 3). In addition to the availability of a variety of screws and rod sizes/materials, the iliac bolts are particularly useful. The profile of the bolt is minimal, which is further marginalized with the use of a medial/inferior entry point for the iliac bolt (See Sacropelvic Fixation Techniques Chapter for more details on insertion techniques). This entry point will often obviate the need for a connector, but a variety of both open and closed multiaxial head connectors are available to ease connection to the rod (Figure 1). In addition, the iliac bolts are cannulated and thus can be placed with minimal dissection. Figure 3. (Left) EXPEDIUM iliac screw with a low profile head and a variable angle head allowing for easier rod connection. (Right) Cantilever correction of pelvic obliquity by introducing rods from distal to proximal unitized with a cross connector. This allows for powerful cantilever force to correct the deformity. Correction The rod is contoured to match the preoperative sitting sagittal profile in nonambulatory patients. In ambulatory patients, the EXPEDIUM Neuromuscular System’s prebent rods offer a good option. The authors will often add more thoracic or thoracolumbar kyphosis, anticipating loss of kyphosis from positioning prone. The rods are introduced simultaneously from the iliac bolts moving proximally. A cross connector linking the rods distally and proximally allows for strong cantilever forces to correct pelvic obliquity (Figure 4). In smaller, more flexible curves, a single rod introduced from the bottom will suffice. The majority of correction is obtained via cantilever forces, following which multiple rounds of compression/ distraction optimize correction. Although derotating the spine is considered a maneuver utilized in patients with adolescent idiopathic scoliosis, the authors have found it to be useful in attaining maximal coronal balance in patients with stiff, severely rotated lumbar curves. When looking at an intraoperative PA radiograph, sometimes the C7 to CSL is more than 2cm lateral. Instead of a coronal bend in the rods, one should assess the thoracolumbar rotation status. If still rotated, en bloc and segmental derotation which will secondarily correct the C7 to CSL line should be considered. Having uniplanar pedicle screws in the thoracolumbar spine allows this rotation correction. Sublaminar wires would require in situ bending which really is just compensating for not doing a good 3-D correction. Figure 4. EXPEDIUM® SFX® Cross Connector Options for spine constructs. Fixed (top two) and Variable (bottom) options. These allow for efficient attachment while maintaining a low profile. 69 Postoperative Care Summary In the postoperative period, we usually stop the gentamicin after 48 hours and continue IV gram positive coverage while the drains are in place. After the IV antibiotics are discontinued, the patient is switched to an antibiotic by mouth for six weeks after discharge to prevent seeded infection from infecting or contaminating urine. Since many of these patients are n ot continent of urine or stool, we strictly enforce covering the inferior one-third of the wound with a bioclusive dressing at all times for the first month after surgery. Postoperative ultrasounds of all four extremities are obtained to rule out DVT at one week. Lovenox is started after the drains are removed and used for six weeks to prevent DVT. Activity restrictions for six months include obtaining assistance with transfers, not flexing the hips past 90 degrees, and not self-propelling the wheelchair. Spine deformity secondary to spinal cord injury presents some unique challenges for the patient and surgeon. Careful attention to the preoperative evaluation and surgical planning is very important. We strongly recommend use of the advanced implant fixation options provided by the EXPEDIUM® Neuromuscular System to optimize the correction in all three planes. Utilizing 3-D correction techniques to restore the patient’s best sagittal balance (standing or sitting) is necessary to maximize the positive functional outcome and minimize the negative effects of a spine fusion. 70 References 1. Jones T, Ugalde V, Franks P, et al. Venous thromboembolism after spinal cord injury: incidence, time course, and associated risk factors in 16,240 adults and children. Arch Phys Med Rehabil 2005;86:2240-7. 2. Krassioukov A, Claydon VE. The clinical problems in cardiovascular control following spinal cord injury: an overview. Prog Brain Res 2006;152:223-9. 3. Nash CL Jr, Haller R, Brown RH. Succinylcholine, paraplegia, and intraoperative cardiac arrest. A case report. J Bone Joint Surg Am 1981;63:1010-2. 4. Vitale MG, Mackenzie WG, Matsumoto H, et al. Surgical site infection following spinal instrumentation for scoliosis: lessons learned from a multi-center analysis of 1,352 spinal instrumentation procedures for scoliosis. Scoliosis Research Society annual meeting, Louisville, KY, September 14-17, 2011. 5. Borkhuu B, Borowski A, Shah SA, et al. Antibioticloaded allograft decreases the rate of acute deep wound infection after spinal fusion in cerebral palsy. Spine (Phila Pa 1976) 2008;33:2300-4. 6. Sink EL, Newton PO, Mubarak SJ, et al. Maintenance of sagittal plane alignment after surgical correction of spinal deformity in patients with cerebral palsy. Spine (Phila Pa 1976) 2003;28:1396-403. 71 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-64-000 9/2012 JC CA #8784 / DJ9932A Surgical Management of Neuromuscular Spinal Deformity – Muscular Dystrophy & Spinal Muscular Atrophy William G. Mackenzie, M.D. Management of spinal deformity in patients with progressive neuromuscular disorders such as Duchenne’s muscular dystrophy (DMD) and spinal muscular atrophy (SMA) can be very challenging. Although neuromuscular disorders share many common features that result in the pathogenesis of neuromuscular spinal deformity, these disorders can vary greatly. Typically, the spinal deformity is a long, sweeping curve in the thoracolumbar region which is associated with pelvic obliquity and usually with kyphosis. These large spinal deformities progress through skeletal maturity and can cause back pain, sitting intolerance, loss of independent mobility, and loss of hand function, and can aggravate restrictive lung disease. Typically, bracing does not play a role in these deformities; it may slow progression but there is no evidence that it prevents it. Surgical management can be highly effective for these spinal deformities. The aim of surgery is to provide a well-balanced spine which will result in more comfortable seating and a better quality of life than that possible with the untreated scoliosis. 73 In the last 20 years, there has been a dramatic change in the management of patients with DMD. In the era before steroid therapy, progressive scoliosis occurred in more than 90 percent of these cases. Most were nonambulatory when the scoliosis was diagnosed. Although the curve often presents earlier, the peak progression occurred between 13 and 15 years of age and progressed at 1 to 3 degrees per month. Although these patients have a natural history of progressive, restrictive lung disease, the lung function is related to curve magnitude. Forced vital capacity (FVC) is usually > 35% of normal in more than 90% of patients with a scoliosis ≤ 30 degrees. However, with larger curves there is significant reduction of the FVC. There are increased postoperative respiratory complications when the FVC goes below 35%. My experience and that of others suggests that there is no difference in pulmonary deterioration and long-term survival following spine surgery. Non-surgical techniques such as bracing, lordotic positioning, and wheelchair modification may delay but do not prevent progression and are not indicated in these patients. Deflazacort, a steroid, has been shown to slow the progression of weakness and allow continued ambulation longer than possible prior to steroid use and to dramatically reduce the incidence of progressive scoliosis. Patients with SMA are classified by the International SMA Consortium according to age at presentation as follows: Type I: severe onset at birth to 6 months, never able to sit without support; Type II: intermediate onset before 18 months and able to sit but unable to stand or walk unaided; and Type III: mild onset after 18 months and able to stand and walk. Typically, Type I has the more severe muscle weakness and the more progressive deformity. Patients with SMA usually have thoracolumbar curves associated with kyphosis and pelvic obliquity. Despite the large magnitude, the curves are often flexible but can continue to progress. A soft or a polypropylene lined thoracolumbosacral orthosis (TLSO) can be used to improve sitting balance. Caution with braces should be exercised, as they can result in significant rib deformity and must be monitored closely. 74 Indications for Spine Surgery Classical indications for posterior spinal fusion in DMD include a progressive scoliosis > 30 degrees and preferably before the FVC declines below 35% of normal. With current medical management, many of these patients may not develop scoliosis or will develop scoliosis in late adolescence. If these patients have a scoliosis > 50 degrees in late adolescent (after skeletal maturity), they should be considered candidates for posterior spinal fusion and instrumentation. It is important to avoid anterior spinal fusion in these patients, as there is significant long-term reduction of pulmonary function with these techniques. Preoperative Evaluation Careful multidisciplinary preoperative evaluation is critical for these patients. Frequent upper respiratory tract infection and cough is very suggestive of underlying pulmonary disease. In these patients, it is important to determine lung volumes and do pulmonary function testing. A cardiology consultation is very important for patients with muscular dystrophy because of the associated cardiomyopathy. These patients can have problems with sudden hemodynamic changes intraoperatively, and it is important to have a good understanding of the cardiac function preoperatively. There can be significant intraoperative blood loss, and appropriate blood products should be available, as well as a cell saver. Levels of Fusion and Instrumentation Options There has been some controversy over the optimal levels of fusion for these patients. Conventional wisdom would indicate that in patients with neuromuscular disorders of this type the fusion should extend from T3 to the sacropelvis. In DMD, it is recommended that if the scoliosis is < 40 degrees and pelvic obliquity is < 10 degrees, fusion to L5 is adequate. Fusion to L5 or S1 has comparable results if the apex of the curve is above L1. There is a greater blood loss and longer hospital stay with pelvic fixation, but with recent modifications of pelvic fixation with iliac or S2 iliac screws, outcomes show no difference in hospital stay, and difference in blood loss is negligible. In SMA there are different challenges. In some patients, the pelvis can be very small, with abnormal anatomical features and poor bone quality complicating the pelvic fixation. The EXPEDIUM® 4.5mm Spine System can provide a lower profile system in thin patients. Neurophysiological monitoring, including motor evoked and sensory evoked potentials, should be used. In SMA the motor evoked potentials are not usually present in the more severely involved cases, but SSEPs are especially helpful for monitoring the upper extremities. Positioning can be difficult because of upper and lower extremity contractures. Severe hip flexion contractures can be present in patients with SMA and DMD. The author uses a Jackson table with either a sling to support the knees or a specially constructed trough. Rarely does the author need to release contractures prior to the spine surgery. There have been many posterior instrumentation techniques used in these patients, including a Luque system with Galveston pelvic fixation and sublaminar wires, two rods with cross links, or a unit rod, which is very stable and allows immediate immobilization without external support. Pelvic fixation with screws is ideal, as the screws can be placed in a perfect anatomic position and the rods connected to the pelvic fixation. Inserting the L rods using the Galveston pelvic fixation technique or the unit rod can be difficult, particularly in the patient with a small, deformed pelvis and/or excessive lordosis. The author favors pelvic screws inset into the iliac crest, but the S2 screw techniques (see Chapter 2, Sacropelvic Fixation Techniques) can be used as well, especially where skin and soft tissue coverage over the screw heads may be an issue. Other techniques involve the use of segmental fixation with all pedicle screws or a combination of pedicle screws and wire fixation. 75 Surgical Technique The surgical incision can be curved slightly into the concavity of the spinal deformity, as the correction will straighten out the incision. The posterior exposure should be thorough, out to the tips of the costotransverse processes and the tips of the lumbar transverse processes. When using screw fixation to the pelvis, the author elevates the erector spinae off the dorsal surface of the sacrum and exposes the posterior superior iliac spine under the muscle mass. It is relatively easy to divide the iliac apophysis and expose the outer table of the ilium with a Cobb elevator and sponge. An inset is created at Figure 1A. Lateral Pre-op 14 yrs Figure 1B. AP Pre-op 14 yrs Figure 1. A-B) 14 year-old patient with Duchenne’s muscular dystrophy. AP/lateral spine x-ray demonstrating thoracolumbar scoliosis. C-D) Technique of insertion of the pelvic screws. E-F) At 16 years old, two years after posterior spinal fusion and instrumentation, no complications. 76 the site of the screw insertion with a rongeur to reduce prominence. A blunt, straight pedicle finder is used to create the hole for the insertion of the pelvic screw. The image intensifier is used to monitor the course of the pedicle finder, which is ideally just above the convexity of the greater sciatic notch. To confirm the position in the pelvis, the image intensifier can be obliqued to make sure that the device is between the inner and outer cortices (Figure 1). Marrow can be aspirated for mixing with the allograft. The screw is generally 80mm in length in patients with DMD and much smaller for those with SMA (in the 45 to 60mm range). Figure 1C Figure 1D Figure 1E. AP Post-op 16 yrs Figure 1F. Lateral Post-op 16 yrs The author usually uses a unit rod and removes the pelvic extension with a rod cutter. The end is smoothed over with a metal cutting high speed burr. Alternately, two EXPEDIUM precontoured rods can be used with an EXPEDIUM modular connector proximally simulating the cut off unit rod. The author usually uses smaller rods than the 1/4” steel rods in patients with SMA because of the poor bone quality. Maintaining adequate thoracic kyphosis is important for function. The sublaminar wires are inserted with standard techniques and an extensive facetectomy is done. A thin layer of allograft is placed into the gutter bilaterally and the unit rod or EXPEDIUM modular construct is fixed to the pelvic screws. The position of the rod to the pelvis is checked using the image intensifier. The rod is then cantilevered to the spine and the sublaminar wires tightened from L5 to the top of the construct. Fixation with pedicle screws could be used rather than sublaminar wires (Figure 2). Figure 2A. Lateral and AP Pre-Op images of 15 year-old with Nemaline rod myopathy and neuromuscular scoliosis Figure 2B. Lateral and AP Post-Op images of 15 year-old with Nemaline rod myopathy and neuromuscular scoliosis 77 Postoperative Care Outcomes These patients are typically managed in the intensive care unit postoperatively and are intubated for at least 24 hours. The patients are mobilized into wheelchairs as quickly as possible by Nursing in conjunction with physical therapy. Often, the power chairs need to be modified. In addition, older patients with DMD have gained so much height after surgery that their van transportation may need to be modified. Long-term results are usually very good in these patients. The quality of life is improved in both populations, and the parents and caregivers are usually very satisfied. In the author’s experience, the combination of the rigid, fused spine and upper extremity weakness can make selffeeding and self-hygiene more difficult. In DMD, major complications have been seen and are usually related to significant blood loss and postoperative pulmonary problems. In SMA, the early postoperative problems are similar and are primarily respiratory with atelectasis and pneumonia. Pseudarthrosis and instrumentation failure are uncommon with modern instrumentation systems, especially with the use of iliac screw fixation. 78 79 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-69-000 9/2012 JC CA #8981A / DJ10054A Complications in Neuromuscular Scoliosis Surgery Patrick J. Cahill, MD Spinal deformity surgery for scoliosis related to neuromuscular conditions is a challenging area of clinical care due to the severity of deformity and influence of comorbidities and thus is fraught with complications. Patients with neuromuscular conditions often have larger magnitude deformities at the time of surgery due to the higher incidence of scoliosis in their population and the increased rate of progression as compared to idiopathic scoliosis.1 Also, the physiology of the underlying conditions contributes to the increased rate of complications. 81 The overall rate of complications associated with surgery for neuromuscular scoliosis has been variably reported but may be as high as 48%, and many are medical. Mortality is also higher in than in other populations that undergo spinal deformity surgery. Any discussion of the treatment of neuromuscular scoliosis complications begins with prevention. Careful preoperative evaluation and maximization of health status is imperative. Evaluation of nutritional parameters to ensure wound healing is important. Evaluation of pulmonary status often requires input from a pulmonary specialist to optimize the patient’s pulmonary function prior to surgery. Patients with myelomeningocele and spinal cord injury (SCI) have increased infection rates due to a variety of factors such as bladder incontinence, requiring frequent bladder catheterizations. Urinary tract infections are best avoided with routine preoperative screening and prevented with perioperative broad spectrum gram negative and positive prophylactic antibiotics. Patients with neuromuscular deformity are often malnourished. Infection after scoliosis surgery is a potentially serious complication that requires vigilance in prevention and diagnosis. The factors contributing to the rate of infection include the underlying diagnosis and age at surgery, which we have noted to have an inverse relationship with the risk of infection. In our experience, the rate of infection in patients with myelomeningocele is about 18% and is similar in patients with SCI, who share many of the same risk factors. There are a number of contributing factors to the high infection rate. Patients without sensation in their lower extremities, buttocks, and/or lower back are at risk for the development of decubiti which can lead to an infection either by direct contamination or hematologic spread. Those patients who lack bowel and bladder control risk seeding a wound with feces or urine. Furthermore, these patients develop frequent urinary tract infections which can spread to implanted instrumentation or a surgical wound through Batson’s venous plexus on the anterior spine. Previous surgery in the same area of the body (ex. closure of a meningocele) can put a patient at risk for further infection if the same area of the body undergoes reoperation. The skin can have deep ridges which cannot be cleaned with routine washing. For this reason, scrubbing with Hibiclens daily for three days preoperatively may be advantageous (Figure 1). Also, the patients with myelomeningocele do not have the normal paramedian spinal musculature at the level of their defect. The less vascular scar and adipose tissue and the relative proximity of the dural sac to the skin surface may be a predisposing factor to infection. Patients with myelomeningocele have a high rate of incidental intraoperative duratomy. If an anterior augmentation is included, additional risks are incurred including damage to abdominal structures, increased blood loss, prolonged anesthesia time, and perioperative malnutrition if staging occurs. A persistent cerebrospinal fluid leak postoperatively may interfere with wound healing and be a favorable medium for bacterial growth. Figure 1. Hibiclens scrub brush (Johnson & Johnson). The authors recommend providing the family with a Hibiclens scrub brush to scrub the patient the evening before surgery. 82 Emerging techniques seem to have benefits in reducing the burden in infection related to scoliosis in patients with neuromuscular scoliosis. We have noted a dramatic decrease in operative infections in neuromuscular scoliosis with the addition of intra-wound delivery of gentamycin within the bone graft. With this technique, there seems to be an incidence of infection of around 4% compared to approximately 15% in those treated without antibiotic laden bone graft. We have also noted a similarly dramatic reduction in infection rates for a series of spinal surgeries by placing vancomycin powder along the wound edges. No differences in other complications (including pseudarthrosis) and no episodes of red man syndrome (adverse reaction to vancomycin) have been noted. The treatment of an infection generally involves surgical intervention, including multiple debridements and, in about half of all deep infections, removal of instrumentation. Negative pressure wound dressings (VACs) have sharply decreased the need for instrumentation removal. A large proportion of implants with acute infection and/or wound dehiscence can be permanently retained with wound vacs. For late onset infections, we recommend a staged approach with removal of all metallic implants, inspection of the fusion mass, and a subsequent period of antibiotic treatment. If a pseudarthrosis is noted at the removal procedure or if the fusion mass is immature, or if curve progression is seen, then we recommend a reinstrumentation procedure with titanium instrumentation. Clark and Shufflebarger reported a similar algorithm in late onset infection in adolescent idiopathic scoliosis.2 Shufflebarger et al have also reported success with a single surgery instrumentation exchange procedure with titanium implants for the eradication of infection.3 Instrumentation related complications are also frequently encountered. Immobility in patients with neuromuscular disorders can lead to poor bone mineral density which contribute to loss of fixation. In addition, patients with myelomeningocele have missing or dysplastic posterior elements yielding a greatly decreased surface area of bone for fusion. In our experience, pseudarthrosis occurs in about 5% of patients undergoing posterior spinal fusion for neuromuscular scoliosis for most conditions but may be as high as 16% in patients with myelomeningocele undergoing posterior-only fusion. Treatment of pseudarthrosis generally requires revision instrumentation and fusion, although asymptomatic pseudarthrosis without deformity progression may only require observation The most common location of pseudarthrosis is at L5-S1. Thus, we feel that consideration should be given to anterior interbody support at the lumbosacral junction in patients with a high BMI or who will be otherwise likely to put high stress on that level – e.g. an ambulatory patient with neuromuscular disorder. Current techniques with secure segmental fixation and abundant bone graft should ensure a relatively low rate of pseudarthrosis. Tsuchiya et al demonstrated a higher rate of pseudarthrosis in adult patients with scoliosis fused to the pelvis who did not have interbody support than those who did.4 There are many factors predisposing to nonunion: age, diagnosis, BMI, etc. We suggest a low threshold for consideration of interbody support, especially at L5-S1 with the risk factors. 83 We do not routinely use bone morphogenetic protein (BMP) in primary surgeries mainly due to cost concerns and the lack of superiority data in this population. There are many bone graft substitutes available which may be utilized to enhance the fusion. These may include demineralized bone matrix and allograft crushed cortical or cancellous bone. Implant prominence is a particular problem in surgery for patients with neuromuscular scoliosis. Our rate of revision surgery for prominent iliac screws is around 10%. One promising technique that may avoid the prominence of iliac fixation is a transsacral iliac screw trajectory. Described by Paul Sponseller, this screw takes a trajectory starting on the dorsal sacrum at S2, traverses through the sacroiliac joint, and rests just superior to the sciatic notch within the ilium.5 This technique (described in “Sacropelvic Fixation Techniques” chapter) results in a much lower profile implant and also provides fixation in-line with the other points of fixation, obviating the need for off-set connection devices. Neurologic injury is a potentially devastating complication to an already impaired population. Loss of bladder control and protective sensation can compound the risk of decubiti in non-ambulatory patients. Fortunately, neurologic injury rarely occurs in this population. In our experience, incidence of neurologic injury in patients with neuromuscular scoliosis is lower than in those with idiopathic scoliosis. With the exception of patients with complete spinal cord injuries, intraoperative neurophysiologic monitoring is usually feasible. Ashkenaze et al reported that they were able to obtain somatosensory evoked potential signals reliably in 72% of their neuromuscular patients.6 They reported intraoperative signal loss in 40% of the cases that were able to be monitored. Only three neurologic injuries were seen in their series of 104 patients. Some surgeons consider motor evoked potential (MEP) monitoring to be contraindicated in patients with shunts and/or a history of seizures. However, other authors have reported no difficulties or complications in patients with neuromuscular scoliosis undergoing fusion who had a seizure history and 84 intraoperative MEP monitoring. An additional neurologic concern is ventricular shunt malfunction. Shunts are common in patients with CP and myelomeningocele. As these shunts are gravity dependent, prolonged periods of time not in an upright position (intra- and postoperatively) may lead to shunt malfunction. Preoperative evaluation by the patient’s neurosurgeon is advised. We also keep a slight tilt in the bed starting immediately postoperatively. Lastly, in rare cases it may be necessary to perform intraoperative intracranial pressure monitoring and periodic intraoperative decompression via removal of cerebrospinal fluid through the cranial shunt portal. An additional concern in patients with seizure disorder is the potential for excessive bleeding due to a coagulopathy related to antiseizure medication. Divalproex sodium (Depakote) is associated with increased intraoperative blood loss. We recommend attempting to bridge perioperative pharmacologic seizure management with other medications under the guidance of a neurologist. Many patients with CP have intrathecal catheters. The local delivery of intrathecal baclofen reduces the dose required to decrease spasticity compared to oral baclofen, thus minimizing unwanted side effects. Unfortunately, these catheters create difficulties at the time of spinal fusion surgery. The catheters traverse the surgical field and are at risk of being damaged or dislodged during surgery. Also, they are placed through laminotomies, the presence of which can increase the risk of incidental durotomy and cerebrospinal fluid leak at the time of surgery. The likelihood of inadvertently damaging the baclofen pump catheter is high. Thus, it is important that at the time of surgery, a baclofen catheter repair kit be made available for every patient with a baclofen pump. The authors feel strongly that spinal deformity surgery for neuromuscular scoliosis can have a positive impact on duration and quality of life. However, complications are frequent and require multidisciplinary attention to detail in perioperative planning, intraoperatively and postoperatively. A high level of suspicion must be maintained. 85 86 References 1. Kalen V, Conklin MM, Sherman FC. Untreated scoliosis in severe cerebral palsy. J Pediatr Orthop 1992;12:33740. 2. Clark CE, Shufflebarger HL. Late-developing infection in instrumented idiopathic scoliosis. Spine (Phila Pa 1976) 1999;24:1909-12. 3. Shufflebarger HL, Asghar J, Morales DC. Solution for late arising infection in stainless steel spinal implants placed for deformity: exchange for titanium implants. Scoliosis Research Society annual meeting, Louisville, KY, September 14-17, 2011. 4. Tsuchiya K, Bridwell KH, Kuklo TR, et al. Minimum 5-year analysis of L5-S1 fusion using sacropelvic fixation (bilateral S1 and iliac screws) for spinal deformity. Spine (Phila Pa 1976) 2006;31:303-8. 5. Chang TL, Sponseller PD, Kebaish KM, et al. Low profile pelvic fixation: anatomic parameters for sacral alar-iliac fixation versus traditional iliac fixation. Spine (Phila Pa 1976) 2009;34:436-40. 6. Ashkenaze D, Mudiyam R, Boachie-Adjei O, et al. Efficacy of spinal cord monitoring in neuromuscular scoliosis. Spine (Phila Pa 1976) 1993;18:1627-33. 87 DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-62-000 9/2012 JC CA #8783 / DJ10055A DePuy Spine, Inc. 325 Paramount Drive Raynham, MA 02767 USA Tel: +1 (800) 227-6633 www.depuysynthes.com ©DePuy Spine, Inc. 2012. All rights reserved. DF29-60-100 10/2012 JC CA #9207A / DJ10290A