Surgical Treatment of Scoliosis Due to Cerebral Palsy

  • Kirk DabneyEmail author
  • Wade Shrader
Living reference work entry


Scoliosis is the most common spinal deformity in cerebral palsy (CP) and is most common in children with greater motor involvement. Most patients present with an unbalanced thoracolumbar or lumbar curvature and pelvic obliquity making it very difficult for the non-ambulatory child to sit in a wheelchair and for the ambulatory child to maintain the head centered over the center of the sacrum for standing balance. Scoliosis may also cause pain, further motor dysfunction, pulmonary compromise, and overall decrease in quality of life. While nonoperative treatment may be temporarily helpful in some children, surgery is the only definitive treatment. The indications for spinal fusion must consider the child’s age, medical condition, scoliosis magnitude, scoliosis flexibility, and the desires of families and caretakers. Posterior spinal fusion with instrumentation is effective in most children with CP with scoliosis; however, very rigid curvatures may require anterior release and/or posterior osteotomy or complete vertebral resection. A multidisciplinary approach to the preoperative and postoperative assessment and medical management is critical to achieve optimum postoperative outcomes. The preoperative management should include preparation for intraoperative bleeding including the use of tranexamic acid, prophylaxis to prevent deep wound infection, maintaining spinal cord integrity, nutritional optimization, and when necessary the management of osteopenic bone. Surgical, functional, and more recent quality of life outcomes have been shown to be favorable in the child with CP undergoing scoliosis surgery.


Cerebral palsy Neuromuscular Scoliosis Spinal deformity Spinal fusion 


Scoliosis is the most common spinal deformity in cerebral palsy (CP). Scoliosis is most common in children with greater motor involvement. While the diagnosis of spinal deformity is relatively clear, there is still an important role for surveillance in order to ensure early diagnosis and treatment. Most children with CP initially develop a flexible curve pattern beginning in preadolescence and, if untreated, can develop into a more rigid and progressive curvature. While children with CP may present with a more balanced idiopathic scoliosis pattern, most present with an unbalanced thoracolumbar or lumbar curvature and pelvic obliquity making it very difficult for the non-ambulatory child to sit in a wheelchair and for the ambulatory child to maintain the head centered over the center of the sacrum for standing balance. Scoliosis may also cause pain, further motor dysfunction, pulmonary compromise, and overall decrease in quality of life. For the provider taking care of the CP child, it is therefore important to have a fundamental understanding of the etiology, prevalence, and natural history. Furthermore, while nonoperative treatment may be temporarily helpful in some children, surgery is the only definitive treatment. Surgical indications, preoperative and postoperative management, complications of treatment, as well as outcomes of treatment are also important to appreciate.


The etiology of scoliosis in CP is due to a combination of poor motor control, muscle imbalance, muscle weakness, and spasticity. Accordingly, it is more common in children with quadriplegic CP with greater motor involvement, GMFCS 4 and 5. Progression of an initially flexible curvature to a greater magnitude curvature with more rigidity is common, especially during rapid adolescent growth (Figure).

Incidence and Natural History

The overall incidence of scoliosis in CP is between 20% and 25%, with a higher risk in patients with quadriplegia ranging from 64% to 77% (Edebol and Tysk 1989; Madigan and Wallace 1981) (“Cerebral Palsy Spinal Deformity: Etiology, Natural History, and Nonoperative Management”). More recently, Hagglund et al. found the incidence of CP scoliosis in Sweden to be related to age and GMFCS level (Hagglund et al. 2018). In their study, individuals with CP at the age of 20 years old, the incidence of curvature with Cobb angle of ≥40° scoliosis for GMFCS V was 75%, GMFCS level VI 35%, GMFCS level III 8%, and GMFCS levels I and II 0%. Scoliosis was less common in younger children in their study. In comparison to those 20 years old, CP children at 10 years of age had only a 20%, 5%, 2%, and 0% incidence of curvatures ≥40° for GMFCS levels V, IV, III, and I and II, respectively (Hagglund et al. 2018).

It is important to understand and place the natural history of CP scoliosis along the perspective with the natural history of cerebral palsy. Scoliosis is typically not present in early childhood but when present is usually a result of a congenital component and/or syndrome. These curvatures can be rapidly progressive and may require early surgical intervention. Most curvatures are postural in early to middle childhood. Koop et al. found scoliosis in CP to be most progressive in quadriplegics, 88%. Diplegics and hemiplegics showed progression in 61% and 64% of curves, respectively, by skeletal maturity (Koop 2009). Yoshida followed the natural history of 113 children with CP and found that approximately 32% progressed by 10° over the age of 20 years old (Yoshido et al. 2018). In addition to quadriplegia, early-onset scoliosis at 6 years old and hip displacement were risk factors for scoliosis progression (Yoshido et al. 2018). Majd et al. found an average rate of progression of 2–4° per year in individuals with CP with stable function, 4.4% per year in those with loss of function, and 9.2% per year in those with kyphoscoliosis (Majd et al. 1997). Saito and colleagues found that if adults with CP had a curvature of greater than 40°, it progressed to a mean curvature of 80° (Saito et al. 1998). Thometz and Simon showed that curves progressed even after skeletal maturity with curves greater than 40° progress approximately 1.4° per year (Thometz and Simon 1988). Non-ambulators and curve patterns that were lumbar and thoracolumbar had greater progression. Recent survival studies show greater than 90% of children with CP survive their 18th birthday (Strauss et al. 2008). Given the long-term survival of children with CP and the progressive nature of CP scoliosis, especially in those with greater motor involvement, a surveillance program for CP scoliosis is warranted to maintain function and quality of life. The goal is to maintain sitting function, positioning, and quality of life initially through nonoperative means and to identify curve progression during rapid growth and intervene with surgical treatment during an appropriate time while the benefits outweigh the surgical risks.

Indications for Scoliosis Surgery

The indications for spinal fusion must consider the age of the child with CP, medical condition, scoliosis magnitude, scoliosis flexibility, and the desires of families and caretakers. Scoliosis curve magnitude, flexibility, and age should be considered together because they are very closely related. For young children, less than 8 years of age, the scoliosis is usually very flexible, and surgery can be delayed with seating adjustments. There are very rare, severe early curves that are discussed later in a special section (“Early Onset Scoliosis in Cerebral Palsy”). As children get to be 8 or 9 years of age, a standard instrumentation and fusion should be considered. For these young children, it is appropriate to allow the curve to go to a magnitude of 90–100° if it remains flexible. As individuals get older, 14–16 years of age, curves of over 60° should be considered for fusion because there is generally less remaining growth and minimal benefit in waiting. After the individual has completed growth and the curve is 30–40° or greater, spinal fusion is generally recommended because of the well-recognized risk of increased curve progression in adulthood.

In addition to age and curve magnitude, it is important to monitor the flexibility of the curve using the physical examination side bending test (Fig. 1). With this test, the curve is considered flexible if it can be completely reversed on side bending as demonstrated by palpating the spinous process and considered to be moderately stiff if it can be bent just to midline. If the curve definitely cannot be bent to midline, then it is considered very rigid, and correction with posterior spinal fusion alone is not likely to be successful. For young children, 8–14 years of age, the curve can generally be monitored until it is in the moderately stiff category and still be corrected with only a posterior spinal fusion. In an occasional very small or exceptionally young child, allowing the curve to progress to severe stiffness to allow for spinal growth may be worthwhile. However, families must be aware that waiting means an anterior release may be required with a posterior correction. An anterior release is needed at all ages for very stiff curves and is never needed for flexible curves regardless of curve magnitude. Occasionally, older individuals who are past skeletal maturity and have curve magnitudes approaching or greater than 90° with moderate curve stiffness may benefit from an anterior release. In general, however, most children who are in the moderately stiff category do not need anterior releases.
Fig. 1

Miller bend test (a). The patient is bent over the examiner’s thigh at the apex of the curvature. (b). If the curve reverses completely, it is flexible. (c). If the curve does not reverse so that the spinous processes come close to the midline with side bending, anterior release is required for adequate deformity correction

Another important factor to consider when considering whether or not a CP child with scoliosis should undergo a spinal fusion is their general health. General health is very subjective. In general, children who have had a combination of poor medical care, an extremely large and stiff curve greater than 120°, frequent respiratory infections, and extremely poor nutrition are considered at very high risk and are recommended against having spinal surgery. These children also have a limited life expectancy (Karatas et al. 2013). Another large factor in this decision-making process is the surgeons’ and medical team’s experience and comfort in dealing with severely involved individuals as to their sense of what is medically safe. There are no specific criteria that can be definitively made precluding an indication for spinal surgery. However, the child’s general medical condition and the physicians’ perceived risk, along with the families’ desires, should all be taken into consideration whether or not the child with CP with scoliosis should undergo spinal fusion. There are families who want all possible medical care for their children, and in the United States, it is the family who legally make the final decision. If physicians are not comfortable with the specific procedure or the families’ desires, they should suggest a second opinion from another physician with the required expertise. If two or three different medical opinions agree, families will usually come to understand the reality of the situation. However, physicians’ opinions may often be based more on philosophical opinions that these children should not have surgery than on experienced medical facts as to the safety of having the surgery. It is not the place of physicians to make these philosophical decisions.

Preoperative Orthopedic Evaluation

The preoperative orthopedic evaluation of the CP child with scoliosis includes an assessment of curve magnitude and flexibility. The former is done with a plain full spine AP radiograph in the sitting position for non-ambulatory children and in the standing position for ambulators. Sagittal spine deformity should also be assessed with a lateral full spine radiograph. Curve flexibility in the coronal plane can be assessed by bending or traction films, but is operator dependent and in our hands is best accessed by doing the previously described Miller bend test. If the former radiographic tests are chosen, it is wise for the surgeon to help perform the test in the x-ray department. It is also important to assess pelvic malalignment and flexibility in the sagittal and transverse planes. Sagittal plane flexibility can be checked in kyphosis by obtaining a lateral x-ray with a bolster positioned at the apex of the deformity. For lordosis, flexibility can be checked by obtaining a lateral x-ray of the spine and pelvis with one of the hips hyperflexed to minimize the lordosis (“Surgical Management of Kyphosis and Lordosis in Children with Cerebral Palsy”). Transverse plane pelvic deformity may require a CT scan for proper evaluation (Ko et al. 2011). All supra-pelvic malalignment (coronal, sagittal, and transverse) should be addressed during the spine fusion if it is contributing to poor sitting or standing balance. In addition, a separate AP radiograph of the pelvis should also be taken in order to recognize the presence of infra-pelvic pelvic obliquity due to windswept hip deformity as well as whether or not hip subluxation/dislocation is present (“Pelvic Alignment and Spondylolisthesis in Children with Cerebral Palsy”). Soft tissue contractures contributing to pelvic malalignment should also be addressed if they are contributing to poor seating. This can be done before or after spinal fusion (“Windblown Hip Deformity and Hip Contractures in Cerebral Palsy”).

Preoperative Management and Preparation

Preoperative medical management is critical to achieve optimum postoperative outcomes. In our institution, the combination of in-patient hospitalists and advanced providers serves to evaluate and coordinate care for patients both preoperatively and postoperatively, respectively (Rappaport et al. 2013a, b; Rappaport and Pressel 2008). Specialty evaluation is considered for children with a history of respiratory disease, seizure disorders, endocrine disease, and, in uncommon cases, cardiac disease. In general, a cardiac work-up with routine echocardiogram is not necessary in children with cerebral palsy unless the clinical history or physical examination findings are suggestive of cardiac disease (DiCindio et al. 2015). In addition, patients with severe nutritional deprivation may require preoperative nutritional measures prior to surgery to prevent wound complications.

Medical and surgical complications may occur during or after spinal fusion in the cerebral palsy child. Complications include excessive bleeding, neurologic compromise, intraoperative hardware failure, postoperative pulmonary compromise, pneumonia, and the need for mechanical ventilation, postoperative ileus, postoperative pancreatitis, and postoperative deep wound infection. The best way to prevent these complications is to prepare for them preoperatively. The management of acute and postoperative complications will be discussed in another section.

Preparing for Intraoperative Bleeding

Excessive intraoperative bleeding is common in the child with CP and may be due to nutritional deficiency, altered tissue integrity, hepatic dysfunction, and the use of anti-seizure medications (Brenn et al. 2004; Jain et al. 2012). Jain et al. reported the risk of increased blood loss, greater than one blood volume to be increased with greater coronal curve magnitude and with the unit rod construct (Jain et al. 2017a). Since the introduction of tranexamic acid (TXA), intraoperative bleeding has been markedly decreased in neuromuscular spinal surgery (Dhawale et al. 2012). In addition, the early infusion of fresh frozen plasma also decreases bleeding. Blood (packed RBCs) and blood products should be available in case of excessive bleeding. In our own center, a co-surgeon model has helped decrease both the length of surgery and therefore intraoperative bleeding.

Soft Bone

Osteopenic bone is common in children with cerebral palsy often due to one or a combination of non-weight-bearing status, seizure medication, and poor nutritional status. Instrumentation pullout (wires or pedicle screws) may occur as a result of osteopenia. In the child with severe low bone mineral density, a course of pamidronate may be helpful in improving low bone mineral density prior to surgery in order to decrease the risk of wire or screw pullout (Sees et al. 2016).

Maintaining Spinal Cord Integrity

During surgery, it is important to maintain spinal cord function through the use of both somatosensory and motor evoked potential monitoring. Monitoring should be performed in all patients who have sufficient motor function for functional ambulation, even if for only exercise ambulation (DiCindio et al. 2003) (“Surgical Spinal Cord Monitoring in Cerebral Palsy”). In addition, it is helpful to know if urological function is present or not prior to surgery. If not present, it is important to document and as well make certain that the patient does not have chronic urinary tract infection which may develop into postoperative sepsis if there is no treatment. If present, monitoring for continued urological function during surgery is important.

Prophylaxis to Prevent Deep Wound Infection

Deep wound infection has recently been reported as high as 10% in the cerebral palsy patient undergoing posterior spinal fusion (Sponseller et al. 2010) (“Infections and Late Complications of Spine Surgery in Cerebral Palsy”). The greatest opportunity to prevent surgical site infection after spinal fusion in the child with cerebral palsy is through prophylactic measures. Using consensus methodology, best practice guidelines have recently been recommended to prevent surgical site infections in high-risk pediatric spine surgery which include: (1) patients should have a chlorhexidine skin wash the night before surgery; (2) patients should have preoperative urine cultures obtained; (3) patients should receive a preoperative patient education sheet; (4) patients should have a preoperative nutritional assessment; (5) if removing hair, clipping is preferred to shaving; (6) patients should receive perioperative intravenous cefazolin; (7) patients should receive perioperative intravenous prophylaxis for gram-negative bacilli; (8) adherence to perioperative antimicrobial regimens should be monitored; (9) operating room access should be limited during scoliosis surgery (whenever practical); (10) UV lights need NOT be used in the operating room; (11) patients should have intraoperative wound irrigation; (12) vancomycin powder should be used in the bone graft and/or the surgical site; (13) impervious dressings are preferred postoperatively; (14) and postoperative dressing changes should be minimized before discharge to the extent possible (Vitale et al. 2013). Future research is needed to substantiate these guidelines.

Operative Treatment

Operative treatment, spinal fusion with instrumentation is the mainstay for the treatment of structural neuromuscular scoliosis in the cerebral palsy child (“Spinal Procedure Atlas for Cerebral Palsy Deformities”). Currently, it is the only treatment that has a permanent corrective effect on spinal deformity in addition to improving the overall function and quality of life of the CP child (Comstock et al. 1998; Miyanji et al. 2018). The goals of surgical treatment are to (1) correct frontal spine and pelvic malalignment, pelvic obliquity, and excessive pelvic sagittal and transverse malalignment when they are interfering with the child’s proper sitting posture, (2) center the head and thorax over the center of the pelvis in the coronal plane, (3) maintain/correct proper sagittal alignment, (4) correct rotational malalignment in the transverse plane when it is affecting sitting posture, and (5) minimize intraoperative time and bleeding. In most cases, the instrumentation includes the pelvis. The type of spinal instrumentation should be chosen which can achieve these goals. While accomplishing a solid spinal fusion usually from T1 or T2 down to the sacrum. Regardless of the type of instrumentation, a meticulous fusion should be done, especially at the thoracolumbar and lumbosacral junctions utilizing sufficient cortico-cancellous allograft bone with a final goal of obtaining a solid fusion.

Evolution of Instrumentation for Neuromuscular Scoliosis Correction

As posterior spinal fusion came to be the accepted treatment for idiopathic adolescent scoliosis, these same techniques were applied to selected children with CP. In the 1970s, Harrington rods and anterior Dwyer cable instrumentation were mainly used, with some reports of positive outcome (Bonnett et al. 1976). A major breakthrough occurred when Dr. Eduardo Luque introduced the concept of sublaminar wire fixation and the use of smooth stainless steel rods. This breakthrough led to an explosion of different facilities doing spinal fusions in children and young adults with CP. However, the early enthusiasm (Brown et al. 1982) was soon dampened by reports of high rates of postoperative curve progression in up to 30% of individuals (Sussman et al. 1996; Comstock et al. 1998), high rates of instrumentation complications ranging up to 21% of individuals (Gau et al. 1991), and high rates of pseudarthrosis up to 10% (Gau et al. 1991). One problem with the Luque construct that was identified early on was related to driving the rod through the lateral wall of the ilium, providing no control of anteroposterior pelvic tilt. This method of driving the rod through the pelvis also often caused pain at the ilium and large bone erosions. This problem was resolved very ingeniously by Ferguson and Allen with the Galveston technique of intermedullary placement of the rod into the ilium (Ferguson and Allen 1988). Independent movement of the unconnected smooth rods was soon recognized as another major problem in the Luque construct for failing to provide stable fixation (Fig. 2). One method for stabilizing the construct was an attempt to use postoperative immobilization; however, this was found to make no difference (Boachie-Adjei et al.).
Fig. 2

CASE: Leatrice, a 14-year-old girl with moderate spastic quadriplegia who had good speech and mild mental retardation, developed scoliosis requiring instrumentation using the Luque technique with unconnected rods (a). Initially, some correction was obtained; however, the intended lordosis all spun into the scoliosis (b). Over 3 years of follow-up with progressive growth, the rods shifted and rotated. After the fracture of one rod, 90° of rotation occurred with a rapid increase in symptoms of respiratory restriction (c, d). She was returned to the operating room where an anterior release was performed, followed by removal of posterior instrumentation, multiple-level osteotomies, and repeat instrumentation using a unit rod (e, f). This procedure provided excellent trunk alignment and correction of the trunk malrotation and completely relieved the restrictive respiratory symptoms

The next solution was to rigidly connect the rods, with the Texas Scottish Rite Hospital group developing mechanical rod connecting plates. At the same time, The Hospital for Sick Children in Toronto worked on developing a unified connected rod called a unit rod (Bell et al. 1989). The concept of requiring very rigidly connected rods has been widely accepted and is now the basis for almost all instrumentations in CP scoliosis. The simplicity of the unit rod from a hardware perspective allowed it to initially be one of the predominant instrumentation choices for surgeons who do high volumes of CP surgery.

Unit Rod

The unit rod (Fig. 3) has an advantage of being cheaper, is completely pre-bent, has no risk of failure at connecting sites, and generally is very easy to use. The use of two smooth rods that are rigidly connected after implantation into the pelvis is a second preferred technique and provides the same mechanical benefits as the unit rod with the exception that connectors may fail and the connection must be made with a correctly aligned rod before curve correction and rod wiring. Other hook and rod systems may also may be used, but these are at much greater cost, greater complexity, and with less reported correction. The unit rod combined with cantilever correction provides not only a very powerful method to correct coronal plane deformity but also supra-pelvic pelvic obliquity (Bell et al. 1989; Rinsky 1990). Both short-term and long-term reports have shown excellent short- and long-term correction of both scoliosis curve magnitude and pelvic obliquity with low loss of correction (Dias et al. 1996; Tsirikos et al. 2008). However, the learning curve with the unit rod may be high for some surgeons having difficulty with proper placing the pelvic limbs of the unit rod and increased blood loss due to its technical difficulty. Fortunately, the current use of TXA has decreased blood loss in all.
Fig. 3

The unit rod is commercially available from several vendors and comes in a range of sizes in 2-cm-length increments. It is designed for fusion from T1 to the sacrum so that the pelvic limbs, lumbar lordosis, and thoracic kyphosis are all sized to the changing length of the rod

Current Methods of Pelvic Fixation, Pre-contoured Rods with Pelvic Screw, and Segmental Spine Fixation

As mentioned, the unit rod has the advantage of being very rigid with already pre-contoured lumbar lordosis, thoracic kyphosis, and pelvic limbs, the latter to correct pelvic obliquity. The disadvantage of the unit rod has been its relatively high learning curve to position the pelvic limbs correctly into the pelvis so that they do not penetrate through the inner wall of the pelvis (Fig. 4). Patients may also be at risk for pelvic limb pullout. This is especially true in those patients with severe lumbar lordosis, anterior pelvic tilt, and severe pelvic obliquity. Also, those with thoracic hyper-kyphosis are at risk for proximal wire cutout and breakage, instrumentation prominence, and increased risk of proximal junctional kyphosis due to the dissection required for wire passage at the proximal end of the rod. In addition, it can also be difficult to judge the proper rod length with the unit rod. As a result of these complications, some authors have reported these patients requiring reoperation (Lonstein et al. 2012; Sponseller et al. 2009). Consequently, many surgeons treating CP scoliosis resorted to contouring straight rods to bend in lumbar lordosis, thoracic kyphosis, and the iliac limb for pelvic insertion. Sponseller and Shah et al. reported a large, multicenter retrospective study of 157 patients that compared the unit rod to “surgeon-bent” rods with iliac screw fixation. In that series, the unit rod group had slightly better pelvic obliquity correction, but the disadvantages seen in cases with the unit rod included increased mean surgical time, blood loss, hospital stay, infection rate, and proximal fixation problems (Sponseller et al. 2009). The increased blood loss may be a result of the multiple laminotomies, wire passage, and wire manipulation, although this is speculative. The authors further 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. Finally, the unit rod does not perform as well with atypical neuromuscular curves, such as those with an apex in the thoracic spine. 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. Using screws for fixation to the pelvis should potentially decrease the complications of the Galveston technique, which was up to 62% in Gau’s series and 47% in Lonstein’s series (Gau et al. 1991; Lonstein et al. 2012). A retrospective, single-center cohort study compared 2 groups of 20 patients each with Luque-Galveston constructs, 1 with a traditional Galveston rod limb and 1 with a Galveston iliac screw construct. The traditional Galveston group had four broken rods and two reoperations, while the iliac screw group had one broken screw and no reoperations (Peelle et al. 2006).
Fig. 4

A steep trajectory is needed for proper pelvic limb placement of the unit rod, especially with severe lumbar lordosis and pelvic obliquity with subsequent penetration of the pelvic limb through the inner pelvic wall

Also, in question is the best pelvic screw insertion technique. A recent series by Abousamra et al. demonstrated equal effectiveness in correcting pelvic obliquity with the unit rod, traditional iliac screws, or S2AI screws. There was similar asymptomatic loosening in all constructs (Abousamra et al. 2016). In addition, the additional required dissection of the paraspinous muscle to place the pelvic limbs of the unit rod at the posterior superior spine pelvic entrance site compromised the muscle flaps covering the rods. For insertion of a unit rod, these 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. Using the S2-Alar technique described by Sponseller allows for easier distal dissection in line with the distal aspects of the midline spinal approach, eliminating the additional dissection over the posterior iliac spine to place the Galveston limbs. This eliminates a significant amount of blood loss from occurring during the operation.

There is some biomechanical evidence to support the use of screws in this patient population. The use of iliac screws would seem to improve fixation due to better interdigitation of the threaded screw implant within the iliac cortical and cancellous bone (Kuklo et al. 2001). Iliac screws have been demonstrated to have improved pullout strength compared to smooth Galveston rods for pelvic fixation. In addition, 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 and may be supplemental to iliac fixation (Erickson et al. 2004).

The placement of pelvic screws as pelvic fixation connected to separate pre-contoured rods with rigid cross connectors is a step forward from “surgeon-bent” rods and allows for easier pelvic instrumentation and less risk of penetration through the pelvic wall (Fig. 5). It also allows for correction of sagittal plane realignment similar to the unit rod, but without the difficulty of pelvic limb placement. Previously, a comparison study comparing these spinopelvic fixation techniques connected to spinal rods with the unit rod showed similar comparative effectiveness for the correction of pelvic obliquity (Abousamra et al. 2016; Abousamra et al. 2018). Pelvic screw placement can be either at the posterior superior iliac spine, or the sacral-alar-iliac screw fixation technique can be utilized. Sacral-iliac screw placement allows the screw to be more in line with the rod and to be lower profile, negating the need for separate rod to pelvic screw connectors (Jain et al. 2016a), (2017b)..We feel that it is also important to place two rigid cross connectors to off-load the stress on the screw to rod connectors in order to prevent loosening (Fig. 6).
Fig. 5

This construct of two pelvic screws, each attached to a pre-contoured rod connected with two rigid connectors, allows the pelvic screws to be placed first, with less risk of pelvic wall penetration. Once the pelvic screws are placed, the remaining construct which appears very similar to the unit rod is then constructed “in situ” prior to deformity correction

Fig. 6

Cantilever correction shown here requires incrementally pushing the rod to the spine level by level first, usually with a rod pusher, prior to tightening the wire, not using the wires to pull the rod to the spine. Distal to proximal cantilever correction is performed in thoracolumbar and lumbar curvatures

Sublaminar Wires vs. Pedicle Screws

As discussed above, sublaminar wires with Galveston pelvic techniques have been demonstrated to be highly effective in treating scoliosis in children with CP. When using sublaminar wires to correct scoliosis and other spinal deformity, it is critical not to use the sublaminar wires to pull the spine to the rod, but to manually and sequentially push the rod to the spine first, one level at a time, and then use the sublaminar wire to secure the rod to the spine at each level. This so-called cantilever technique (Fig. 6) is important to prevent wire cutout and/or breakage by evenly dispersing the corrective forces over each vertebral level after a manual corrective force has been applied at the corresponding level. More commonly, this has been a distal to proximal cantilever maneuver for lumbar and thoracolumbar curvatures. Alternatively, for atypical thoracic neuromuscular curvatures, a proximal to distal cantilever technique can be used (Fig. 7). The use of sublaminar wires versus pedicle screws in the treatment of neuromuscular scoliosis has been debated in the literature. Most studies show similar complication rates but greater correction of the scoliosis with the more rigid pedicle screw constructs but less correction of the pelvic obliquity (Fuhrhop et al. 2013). One study showed greater blood loss with the unit rod construct vs. all-pedicle screw constructs thought to be due to greater epidural bleeding in the former. In addition, the pedicle screw construct cost was significantly more expensive (Fuhrhop et al. 2013). Whether or not the additional correction and cost of pedicle screws contributes significantly to improved quality of life in these patients has not been studied.
Fig. 7

Atypical thoracic curvatures can be corrected using a proximal to distal cantilever correction

All-pedicle screw fixation has become standard in the treatment of idiopathic spine deformities, especially idiopathic scoliosis. Pedicle screws have been shown to be safe and incredibly powerful in deformity correction. As such, surgeons have become increasingly more comfortable with placing pedicle screws, either by freehand techniques or with image-guided systems (fluoroscopy or navigation). With the combination of this increasing comfort level with the added benefit of possible improvement of sagittal plane deformity correction (especially hyperlordosis), the use of all-pedicle screw constructs has become increasingly common in the treatment of scoliosis in CP.

The goals of deformity correction in the setting of scoliosis and CP are, of course, the same whether the surgeon is using a wire- or screw-based construct: overall spinal balance, with improved deformity, correcting pelvic obliquity while achieving a solid fusion mass, to improve seating and prevent deformity progression. The cantilever deformity technique used with the unit rod can be easily adapted to the use of pedicle screws, using two pre-bent rods (including the extension of the rod onto the surface of the sacrum) and transverse rod connectors. Once the rod is secured to the pelvis and lower lumbar screws, the pelvic obliquity is corrected using the cantilever maneuver described previously. Furthermore, the biomechanical maneuver of using sequentially tightening sublaminar wires to “pull the spine to the rods” can be replicated using tabbed reduction screws. With these techniques, the overall technique of deformity correction in these patients is very similar, whether the surgeon uses wires or screws.

The use of segmental pedicle screw constructs have demonstrated at least equivalent deformity correction and high fusion rates, while accomplishing the goals of addressing pelvic obliquity to improve seating (Modi et al. 2009). Modular screw-based systems may offer improved performance in difficult patient scenarios, such as those with abnormal pelvic anatomy, significant pelvic asymmetry (windswept pelvis), osteoporotic bone, and hyperlordosis. Although all-screw constructs are certainly more expensive, these systems may avoid some of the risk of complications, such as early instrumentation failure, which of course is very costly.

Proximally, less dissection is required to place pedicle screws or hooks compared to a unit rod construct. The increased incidence of clinically significant proximal implant prominence may be a result of the shape of the unit rod, which has a dorsally directed bend at the top, as well as the superior dissection needed to pass the wires proximally (Sponseller et al. 2009). On the other hand, wires are lower profile than pedicle screws.

There are substantial data in the literature that pedicle screws in the lumbar and thoracic spine, by nature of their three-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 (Modi et al. 2009).

With the biomechanical advantage that screws offer, supplemental techniques, such as halo or femoral traction, intraoperatively may also afford improved deformity correction while avoiding the additional morbidity and risks of complication of anterior releases. Furhop et al. performed a matched cohort study comparing unit rods to all-screw constructs across 2 tertiary care pediatric centers, with 14 patients in each group. All-pedicle screw constructs had better correction of coronal Cobb angle, lower blood loss, and shorter hospital stays. There was improved pelvic obliquity correction with the unit; however it did not quite reach statically significant level, no difference in complications or reoperations (Fuhrhop et al. 2013). Tsirikos and Mains also looked at an all-screw construct in 45 consecutive patients with CP and scoliosis (Tsirikos and Mains 2012). They demonstrated pedicle screw instrumentation can achieve excellent correction of spinal deformity in quadriplegic cerebral palsy with low complication and reoperation rates and high parent satisfaction.

Overall, great difficulty exists in evaluating the published literature of spinal instrumentation because many articles mix together very different pathologies. The mixed diagnoses make it very difficult to separate out the problems related to CP scoliosis from the very different problems related to other neuromuscular diseases such as myelomeningocele scoliosis, muscular dystrophy, or spinal muscular atrophy. All of these conditions have somewhat different indications for surgery and tend to have different complications. More comparative studies comparing instrumentation are therefore necessary to compare outcomes in cerebral palsy patients only.

Rigid Scoliosis in the Cerebral Palsy Child

Untreated progressive scoliotic curvatures become rigid and more difficult to correct using posterior spinal fusion with instrumentation only. This typically occurs in curve magnitudes between 90 and 100°. In order to maximize correction of large and rigid spinal curvatures, we have usually employed anterior spinal release (Fig. 8) (multiple discectomies at the apex of the spinal curvature) as an initial staged procedure prior to posterior spinal fusion with instrumentation in order to regain curve flexibility prior to posterior fusion with instrumentation (Auerbach et al. 2009). This can be performed as a staged or a same-day procedure. Alternatively, some have used intraoperative traction during posterior fusion with instrumentation (Jackson et al. 2018; Takeshita et al. 2006). In extremely severe and stiff curvatures (>120°), vertebral column resection is also an option and gives excellent correction for severe pelvic obliquity and coronal and sagittal plane deformity (Fig. 9). Sponseller and colleagues reported on a series of 23 children undergoing vertebral resection for severe neuromuscular scoliosis with excellent correction but a risk of major complications (Sponseller et al. 2012).
Fig. 8

Anterior release: wedge resections of the discs are performed around the apical vertebrae if a stiff spinal deformity exists, usually greater than 100°

Fig. 9

This year-old female, GMFCS level 5, had a (a) 130° curvature and (b) underwent a vertebral resection of the L2 vertebrae with (c) contoured rods and pedicle screw instrumentation showing good coronal and (d) sagittal alignment

Surgical Outcomes

The goals of spinal surgery in patients with cerebral palsy are to enhance quality of life by improving balanced sitting posture, reducing pain, and deformity while minimizing both intraoperative and postoperative complications. However, neuromuscular surgery may be rather challenging and most reports show higher rates of complication compared to idiopathic scoliosis surgery. In a large multicenter study reviewing all cases of pediatric scoliosis, neuromuscular scoliosis had 17.9% complications (Reames et al. 2011). In our own series of 107 cerebral palsy patients undergoing scoliosis surgery utilizing the unit rod as fixation, complications included 14 deep infections, 3 deaths, 15 cases of prominent hardware, and 1 pseudarthrosis (Tsirikos et al. 2008). We concluded that unit rod instrumentation is simple to use and considerably less expensive than most instrumentation systems, associated with low reoperation rates, and achieves successful long-term correction of 70–80% of the preoperative curvature magnitude and 80–90% correction of pelvic obliquity. Caretakers reported a 96% satisfaction rate (Tsirikos et al. 2008). Three studies have looked at preoperative risk factors for postoperative complications (Lipton et al. 1999; Samdani et al. 2016; Nishnianidze et al. 2016). Non-ambulatory status preoperatively, dependence on G-tube feeding, and curve magnitude of greater than or equal to 60° were directly associated with increased risk of major complications. One study indirectly associated with increased length of stay (Samdani et al. 2016). A recent study compared neuromuscular scoliosis complication rates from 2004 to 2015 which included bleeding, infection neurologic deficit, respiratory complications, and mortality (Cognetti et al. 2017). Encouraging results showed a 3.5-fold decrease in the complication rate from 2004 to 2015, especially, infection rates, respiratory complications, and implant-related complications (Cognetti et al. 2017). Finally, Jain and colleagues subclassified children with Gross Motor Function Classification System 5 (GMFCS 5) undergoing spinal fusion according to their number of neuromotor impairments such as the presence of gastrostomy tube, tracheostomy, seizure disorder, and nonverbal status (Jain et al. 2016b). The rate of major complications was proportional to the number of neuromotor impairments (subclassification GMFCS levels 5.0 thru 5.3). Five out of seven of the patients who died within their follow-up period were level 5.3. The authors also utilized the CPCHILD to rate quality of life which decreased significantly from GMFCS levels 5.0 to 5.3, both preoperatively and postoperatively; however there was no difference between GMFCS levels in the improvement of CPCHILD scores from preoperative to follow-up evaluation (Jain et al. 2016b).

Historically, the possibility of ambulatory cerebral palsy patients losing their ambulatory status due to spinal fusion with instrumentation was a concern. Tsirikos et al. showed that 24 ambulatory patients with cerebral palsy who had posterior spinal fusion using unit rod instrumentation maintained their ambulatory status postoperatively (Tsirikos et al. 2003). We have also observed this preservation of ambulatory status in children undergoing posterior spinal fusion with modular instrumentation utilizing pre-contoured rods. This supports the principle that instrumentation that preserves normal spinal sagittal contour also preserves preoperative ambulatory function.

Functional Outcomes and Quality of Life

Important aspects of postoperative assessment also include overall function, appearance, ease of care, and improved quality of life. Outcome measures should differentiate treatment effects from underlying disease functional impairments (Bowen et al. 2012). Some might question whether spinal deformity surgery is truly beneficial for neuromuscular patients, particularly those most severely involved. In one survey of 190 parents and caretakers assessed for functional improvement of children with cerebral palsy after spinal fusion, 95.8% of parents and 84.3% of caretakers would recommend spinal surgery again (Tsirikos et al. 2004). Several other reviews report caretakers, families, and patients with severely involved neuromuscular diseases are most often satisfied with the surgical correction, improved appearance, and enriched quality of life (Dias et al. 1996; Larsson et al. 2005; Jones et al. 2003; Watanabe et al. 2009). A few studies have examined quality of life pre- and postoperatively following spinal fusion. An earlier literature review of patients undergoing spinal fusion with neuromuscular scoliosis concluded that quality of life improved in neuromuscular patients with cerebral palsy (Mercado et al. 2007). More recently, a prospective study was used to evaluate changes in quality of life and caregiver burden in CP children with GMFCS levels IV and V following spinal fusion (DiFazio et al. 2017). While quality of life appeared to improve at 1 year postoperatively, it regressed at 2 years, and caregiver burden did not change after spinal fusion.

In a retrospective study of a multicenter prospective registry, caregiver perceptions such as qualitative changes in global quality of life, comfort, and health, relative valuation of spine surgery versus other interventions in children with cerebral palsy, and quantitative changes in health-related quality of life scores were accessed (Jain et al. 2018). Spinal surgery was ranked as the most beneficial procedure in patients’ lives by 75% of 212 caretakers who were surveyed, second only to gastrostomy tube insertion. Health-related quality of life improved over a 2-year follow-up across several quality of life domains.

As surgeons recommending surgery for scoliosis, we must always evaluate the risks versus benefits of surgery, especially in the totally involved child with CP. In a final study, investigators looked at the risk-benefit ratio of undergoing scoliosis surgery in cerebral palsy by evaluating the benefits of health-related quality of life measures using the CPCHILD questionnaire as well as looking at improvements and the effects of complications on outcomes over 1-, 2-, and 5-year follow-up evaluations (Miyanji et al. 2018). The investigators found significant improvements in ease of personal care, positioning, and comfort domains at each follow-up time period. While the 1-year complication rate was 46.4%, it fell to 4.3% at 2–5 years postoperatively, and there was no apparent correlation between complications and the CPCHILD scores at each time period. Given that health-related quality of life scores improved significantly and were maintained over the 5 years, the authors concluded that the benefits of scoliosis surgery outweighed the risks despite the high rate of complications. Further studies are needed to solidify whether the risk-benefit ratio is worth scoliosis surgery in all cerebral palsy patients despite existing motor involvement, comorbidities, etc. Ultimately, determining the risk-benefit ratio according to increasing risk factors may further define who will benefit most from surgery.



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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Nemours/AI duPont Hospital For ChildrenWillmingtonUSA

Section editors and affiliations

  • Freeman Miller
    • 1
  1. 1.Nemours Alfred I. DuPont Hospital for ChildrenWilmingtonUSA

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