Surgical Treatment of Scoliosis Due to Cerebral Palsy
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.
KeywordsCerebral 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.
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.
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, 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
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.
Current Methods of Pelvic Fixation, Pre-contoured Rods with Pelvic Screw, and Segmental Spine Fixation
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).
Sublaminar Wires vs. Pedicle Screws
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
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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