Treatment of scoliosis with spinal bracing in quadriplegic cerebral palsy
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1 Treatment of scoliosis with spinal bracing in quadriplegic cerebral palsy Terje Terjesen* MD PhD; Johan E Lange MD; Harald Steen MD PhD, Department of Orthopaedic Surgery, The National Hospital, Oslo, Norway. *Correspondence to first author at Department of Orthopaedic Surgery, The National Hospital, Trondheimsv. 132, N-0570 Oslo, Norway. To evaluate the clinical results of the treatment and to assess the factors that influenced the rate of scoliosis progression, a retrospective study of spinal orthosis in 86 patients with spastic quadriplegic cerebral palsy was performed. The mean age of the patients was 13.8 years (range 5 to 33 years). Their scoliotic deformities were treated with custom-moulded, polypropylene thoraco-lumbar-sacral orthoses. Cobb angles were measured on radiographs taken in a sitting position before treatment, in orthosis, and during follow-up. The mean initial Cobb angle was 68.4 (range 25 to 131 ). The mean correction in orthosis was 25 (range 3 to 60 ). Seventytwo patients had a follow-up period of more than 2 years. At the latest follow-up, average 6.3 years (range 2 to 14 years) after the start of treatment, the mean Cobb angle without orthosis was 93.1 (range 40 to 145 ). The mean progression per year was 4.2 (range 3 to 21 ). Linear multiple regression revealed that age and initial correction in orthosis were the only variables that significantly influenced the rate of progression. Twenty-two patients had no progression or progression <1.0 per year. Correction in orthosis was the only variable that predicted progression <1.0 per year in both age groups (<15 years and 15 years). Of the 57 patients who were still alive and had not undergone surgical fusion, 72% used their orthoses at a mean age of 22 years. Parents and caregivers expressed satisfaction with the use of orthosis, mainly because of improved sitting stability which gave better overall function. Scoliosis occurs frequently in patients with cerebral palsy (CP), especially in those with spastic quadriplegia (Madigan and Wallace 1981). Although surgical treatment is usually indicated when scoliosis exceeds 45 to 50, there are certain risk factors that must be taken into consideration before operation is recommended. Many patients with quadriplegia with large curvatures of the spine have impaired general health, epilepsy, and reduced respiratory capacity, making them poor candidates for major surgery like spine fusion. Moreover, the complication rate after such surgery is substantial (Lonstein and Akbarnia 1983, Boachie-Adjei et al. 1989, Cassidy et al. 1994). Therefore, other treatment alternatives should be available. The role of spinal orthosis in those with quadriplegia with scoliosis has not been frequently studied and reports have rather short follow-up periods (McMaster and Clayton 1980, Letts et al. 1992). We have used this type of treatment for several years, based on clinical evidence that it has a beneficial effect in many patients. This study aimed to evaluate the clinical effects of spinal bracing in quadriplegic patients with scoliosis and to assess the progression of the scoliosis and factors that might influence the rate of progression. Method During the period 1982 to 1996, treatment with spinal orthosis was initiated in 99 patients with CP and scoliosis. To obtain more homogenous material, seven patients with diplegia or hemiplegia were excluded from the study, as were two patients with insufficient radiographs. Four patients with severe kyphosis, which was the main indication for treatment, were also excluded. The remaining 86 patients were individuals with severe spastic quadriplegia with learning disabilities* and many had additional problems like epilepsia, visual disturbances, frequent respiratory infections, and problems with nutrition. None of them had any independent walking capacity and most were dependent sitters using adaptive wheelchairs. There were 55 female and 31 male patients with a mean age of 13.8 years (range 5 to 33 years). To evaluate the effect of age on the progression of scoliosis and other parameters, patients were divided into two age groups: <15 years (55 patients) and 15 years (31 patients). Anteroposterior (AP) and lateral radiographs with the patients in the sitting position were taken. Those who could not sit independently were manually supported by an assistant, but no efforts to correct the scoliosis were made. The degree of scoliosis was measured by the Cobb method. One of the authors experienced in the management of scoliosis (JEL) performed radiographic measurements in all patients and also conducted clinical follow-up examinations, usually 2 to 3 times during the first year, then yearly or more seldom after that, depending on the age of the patient and severity of the curve. On these occasions, the patients, parents, and caregivers were asked about the fit of the brace and the patient s tolerance and compliance towards it. The scoliosis was clinically examined and sitting balance, fit of the brace, and radiographs were assessed. From this evaluation, the brace was adjusted or renewed when needed. *UK usage. US usage mental retardation. 448 Developmental Medicine & Child Neurology 2000, 42:
2 The radiographs before treatment and at the most recent follow up were reviewed to assess pelvic obliquity (PO). The AP radiograph taken in the sitting position was used and PO was measured as the angle between the horizontal line and the line connecting the most proximal point of the iliac crest on both sides. Only angles 10 were registered as PO. The elevated side of the pelvis was noted. Characteristics of the primary curve, such as side of convexity, type of curve (C- or S-shaped), and apex vertebra were noted. We divided the curve patterns into two major groups according to Lonstein and Akbarnia (1983): curves with no or small PO (<10 ) and those with PO 10. Although several patients also had increased thoracic kyphosis, the degree of kyphosis was not routinely measured; therefore, these data are not included in the present study. Seventy-nine patients had radiographs of their hip joints taken either shortly before or at the start of treatment for scoliosis. Hip joints were assessed regarding frequency of hip pathology. Hips were classified as normal, subluxed (<67% coverage of the femoral head), or dislocated (whole femoral head outside the acetabulum). Follow-up radiographs of the hip joints were not routinely performed. Treatment consisted of a total-contact thoraco-lumbarsacral orthosis (TLSO, body jacket ) made of polypropylene or polyethylene (Fig. 1). Indications for spinal bracing were scoliosis with Cobb angle >25 (all patients) and problems with sitting balance (all except seven patients). Orthoses were custom made using a plaster-of-paris mould of patients in the supine position without using traction, but in the correction that could be obtained without causing discomfort for the patients. The body jackets were made by orthotists who had long experience with spinal bracing in neuromuscular scoliosis. Radiographs in the sitting position with the patient in orthosis were obtained to assess the degree of correction. Parents and nurses were instructed that the orthosis should be used as long as tolerated during the day, but not during the night. When the orthosis had become too small or was worn out, it was replaced by a new one. Follow-up radiographs were taken when this happened and at clinical follow-up examinations. The most recent radiographs of the patients without orthosis were assessed to evaluate the rate of progression of the curves. If additional radiographs with the patients in orthoses at the most recent follow-up examination were available, the first and last radiographs were compared with regard to correction caused by the brace. Parents and caregivers of patients who were still alive and had not undergone surgical spine fusion were contacted by telephone by TT at the end of 1998 to obtain more details on the use of orthosis than were available from case records. They were asked a standard set of questions about where they lived, effect of treatment, complaints caused by the brace, grade of satisfaction with the orthosis, duration of brace-wear per day, whether or not the orthosis was still used, and reasons why the treatment had been discontinued. Statistical evaluation was performed using t tests, the oneway analysis of variance (ANOVA) with the Scheffe post hoc test and the Pearson correlation test. The rate of curve progression was also analysed by multiple regression.the significance level was set at A B Figure 1: (A) An 11-year-old boy with quadriplegia and severe scoliosis. (B) There is a marked improvement in sitting balance and posture when he wears his spinal orthosis. Treatment of scoliosis with spinal bracing in quadriplegic cerebral palsy Terje Terjesen et al. 449
3 Results Most of the patients had long C-shaped thoraco-lumbar or lumbar curvatures (Fig. 2). The primary curve was convex to the right side in 44 patients and to the left in 42. There were 62 C-shaped and 24 S-shaped curves. The apex vertebra was usually in the lower-thoracic (Th) and upper-lumbar (L) segments. The most frequent levels were L2 (23 patients), L3 (16 patients), L1 (15 patients), and Th12 (13 patients). Only eight curves had the apex above Th10 (Th8 in four patients and Th 9 in the other four). The mean Cobb angle at initiation of brace treatment was 68.4 (range 20 to 131 ) (Table I). C-shaped curves were larger in patients with PO >10 than in those with no PO or PO <10 (Table II). The severity of the curve correlated with the degree of pelvic obliquity (r=0.59) and with age (r=0.29). There were no significant correlations between Cobb angle and the following parameters: type of scoliosis (C-shaped or S-shaped), convexity, and apex. The mean Cobb angle in orthosis was 43.9 (range 8 to 100 ) and the correction caused by the brace was 25.1 (range 3 to 60 ). The correction in degrees (absolute correction) was similar in both age groups (see Table I). Absolute correction increased with initial Cobb angle (r=0.42) and PO (r=0.51). In patients with C-shaped curves, correction was larger in patients with PO>10 than in those with PO<10 (Table II). The mean percentage correction in orthosis was 38.1% (range 3 to 76%). The percentage correction decreased with age (r= 0.35). There was no significant relation between percentage correction and type of scoliosis or whether there was PO greater or less than 10 (see Table II). At the latest follow-up the correction of the Cobb angle in orthosis could be assessed in 36 patients with radiographs both in and out of the brace. The mean correction was 20.7, which was not significantly different from the absolute correction at initial examination. The mean percentage correction at follow-up was 22.6%; this was significantly lower than the initial percentage correction (p=0.001). Fifty-nine patients had an initial PO of >10. The mean PO in these patients was 22.0 (range 11 to 75 ). The scoliosis was convex to the lower side of the pelvis in all patients with C-shaped curves. In those with S-curves, the distal curve was convex to the lower side. At the latest follow-up (n=51), the mean PO was 29.5 (range 10 to 75 ), a statistically significant A B Figure 2: (A) Spinal radiograph of a 10-year-old girl with spastic quadriplegia, showing C-shaped scoliosis of 69 with apex at L1 and pronounced pelvic obliquity. (B) In orthosis, there was a pronounced reduction of the scoliosis to Developmental Medicine & Child Neurology 2000, 42:
4 increase compared with the initial PO (p=0.001). Of 79 patients with available radiographs of the hip joints, both hips were normal in 36, both hips were subluxed or dislocated in 26, and 17 patients had unilateral hip subluxation or dislocation. There was no significant correlation between hip pathology and initial scoliosis angle, as mean angles varied between 67 and 72 in the three groups. Neither were there any significant differences between the status of the hips and other parameters: curve correction in orthosis, progression per year, and pelvic obliquity. Eleven of 17 patients with unilateral dislocation had PO with the dislocated side higher and scoliosis with the convexity to the low side, whereas the dislocated side was lower in six patients. During the follow-up period, 13 patients died at a mean age of 17.4 years (range 12 to 30 years) and 17 patients had undergone surgical spine fusion at a mean age of 18.3 years (range 13 to 31 years). One of the patients who had been operated on died 11 years postoperatively. There were no significant differences between either of these subgroups and the rest of the material with regard to initial Cobb angle, correction in orthosis, or curve progression per year. Eight of the patients who had been operated on had suffered skin problems caused by the brace and one had gastrointestinal problems. Treatment was discontinued in the latter and in one patient with skin irritation; the other patients used their braces until surgical treatment. Of the patients who died, two had to discontinue bracing because of respiratory problems while others continued the treatment until death. PROGRESSION OF SCOLIOSIS Seventy-two patients had radiographs after a follow-up period of 2 years or more. The mean duration of follow-up in these patients was 6.3 years (range 2 to 14 years) and age of the patients at the latest follow-up was 18.2 years (range 10 to 31 years). At the latest follow-up, the mean Cobb angle was 93.1 (range 40 to 145 ) and the mean progression per year was 4.2 (range 3.0 to 21.3 ). Progression per year was significantly higher at <15 years of age compared with older patients (see Table I). Mean yearly progression was somewhat larger <10 years of age (6.6 ) than in those aged 10 to 14 years (4.7 ), but the difference was not significant. Mean progression was 2.1 per year at age 15 to 19 years and 0.8 in those aged 20 years; the difference was not significant. In the latter group only one of nine patients had progression >2.0 per year. When comparing the five types of scoliosis (see Table II), progression per year was significantly greater in S-shaped curves with the largest Cobb angle proximally than in the two other types of S-shaped scoliosis (p<0.05). There were no significant differences between C-shaped and S-shaped curves or between those with and without PO >10. Owing to the broad age range, initial Cobb angle, and other variables, linear multiple regression analysis was performed to evaluate the variables that had the greatest influence on the Table I: Radiographic measurements (mean, SD) at the start of treatment in all 86 patients and divided into two age groups All patients Age (y) <15 15 p (n = 86) (n = 55) (n = 31) Age (y) 13.8 (5.8) 10.4 (2.4) 19.8 (4.8) Initial scoliosis angle( ) 68.4 (24.0) 63.7 (25.3) 76.7 (19.3) Cobb angle in orthosis ( ) 43.9 (21.9) 38.6 (20.9) 52.8 (20.8) Correction in orthosis ( ) 25.1 (12.6) 25.8 (13.0) 23.9 (11.9) Percentage correction (%) 38.1 (16.8) 41.4 (16.5) 32.3 (15.8) Pelvic obliquity ( ) 22.0 (10.9) 21.8 (12.0) 22.3 (8.5) Progression per year of scoliosis ( ) 4.2 (4.5) 5.4 (4.4) 1.6 (3.4) p,difference between patients <15 years and 15 years of age. Table II: Radiographic measurements (mean) according to type of scoliosis C-shaped scoliosis S-shaped scoliosis Pelvic obliquity Pelvic obliquity Yes No p Yes No Proximal a p (n=45) (n=17) (n=13) (n=6) (n=5) Age (y) ns ns Initial scoliosis angle ( ) ns Correction in orthosis ( ) ns Percentage correction (%) ns ns Pelvic obliquity ( ) Progression per year of scoliosis ( ) ns b p, differences between the two C-groups and between the three S-groups. a in this group of five S-shaped scolioses, the largest curve was proximally, and pelvic obliquity was >10 in only one case; b progression per year was larger in the group proximal (ANOVA, p<0.05). Treatment of scoliosis with spinal bracing in quadriplegic cerebral palsy Terje Terjesen et al. 451
5 rate of progression. Age and initial correction in orthosis were the only variables with significant influence. Age explained 18% of the progression per year. When correction in orthosis was included, 30% of the variation was explained and this was the most effective two-factor combination. The multiple regression equation was: progression per year = 9.84 (0.34 age) (0.10 correction in orthosis). Initial Cobb angle, type of scoliosis, PO, and other variables exerted no significant influence on the rate of progression. Progression of scoliosis <1.0 per year occurred in 8 of 50 patients <15 years of age and in 14 of 22 patients 15 years with follow-up of 2 years or more (Table III). The only variable that was significantly related to this benign development in both age groups was large initial correction in orthosis. CLINICAL RESULTS IN 57 PATIENTS NOT OPERATED ON As sufficiently detailed clinical information about brace treatment was not available from patients case records, supplementary information was obtained from the 57 patients who were still alive and who had not been operated on. Twentyfive patients (mean age 19 years) were living with their parents and 32 patients (mean age 26 years) were living in separate flats in group-home settings for people with disabilities or in institutions. Most of the patients tolerated their spinal orthoses well. The most obvious functional benefit was improvement of sitting balance and thereby better head/neck control. Sitting function was improved in all except one patient. Several parents and caregivers spontaneously said that the patient was totally dependent on the brace because they had no useful sitting balance without it and that they otherwise would be bedridden. Forty-one patients were still using their orthoses at a mean age of 22 years (range 14 to 42 years) while the treatment had been discontinued in 16 patients. According to the parents and caregivers, 35 patients had no specific problems caused by the brace. Twenty-two patients had experienced various complaints, of which the most frequent was skin pressure and irritation (Table IV). After adjustments of orthoses, eight of the 12 patients with skin problems continued the brace treatment, whereas the treatment had been stopped in most of the patients with gastrointestinal, respiratory, and other problems. The reasons for ending treatment in six patients with no specific complaints caused by the brace were increased respiratory problems during follow-up in two cases, change to custom-moulded wheelchair inserts in three, and satisfactory sitting function without bracing in one patient. Most of the parents and caregivers said that the orthosis was worn all day with one or a few interruptions only for rest or physical therapy, while others said that the bracing time varied quite a lot. Average brace-wearing time per day was >10 hours in 34 patients, 6 to 10 hours in 15, and<6 hours in eight patients. The mean initial Cobb angle (78.0 ) was larger in those who used the brace <10 hours per day than in those with longer brace time (63.9 ). There were no significant relations between time in brace and either curve correction in orthosis or rate of progression. Parents and caregivers were asked whether or not they and the patients were satisfied with the spinal bracing when benefits and problems were valued together. Answers showed that they were satisfied in 46 cases whereas 11 were indifferent or dissatisfied. Of those who were satisfied, 40 of 46 patients still used their orthoses while this was the case in only one of the patients who were dissatisfied. We found no significant relations between grade of satisfaction and the following variables: initial Cobb angle, correction in orthosis, and rate of progression of the curve. Discussion The aims of non-operative treatment of scoliosis in CP are improved seating support and curve control, i.e. slowing the progression of the scoliosis (Renshaw 1996). In accordance with other studies (McMaster and Clayton 1980, Letts et al. 1992), we found that spinal bracing had a beneficial clinical effect, as it improved sitting balance, which is of major importance to these patients who are bound to their wheel-chairs for most of the day. Improved trunk support provides better head/neck control and allows better use of upper extremities. The initial Cobb angle of 68 is larger than that reported by others in non-operative treatment of CP scoliosis (McMaster and Clayton 1980, Letts et al. 1992). Scoliosis had been detected earlier in several patients but the brace treatment was usually not started until the curvature had become quite large or severe sitting problems had occurred. The mean percentage correction of 38% of the initial Cobb angle caused by the TLSO was quite satisfactory and corresponds with the 36% correction in patients with CP Table III: Radiographic measurements (mean, SD) according to rate of curve progression (<1 per year or >1 per year) in 72 patients with more than 2 years follow up Age (y) <15 Age (y) 15 Progression per year Progression per year <1 >1 <1 >1 n=8 n=42 p n=14 n=8 p Age (y) 10.8 (3.1) 10.2 (2.4) (3.7) 19.9 (5.7) 0.99 Initial scoliosis angle ( o ) 77.6 (27.5) 61.7 (24.7) (19.8) 72.4 (24.4) 0.41 Correction in orthosis ( o ) 5.5 (13.5) 23.2 (12.0) (11.6) 14.6 (9.3) 0.02 Percentage correction (%) 46.1 (11.9) 38.8 (12.0) (13.1) 22.6 (16.2) 0.07 Pelvic obliquity ( o ) 35.8 (22.6) 19.1 (6.7) (10.7) 20.0 (4.7) 0.32 Follow-up period (years) 6.3 (2.6) 6.8 (2.8) (3.4) 4.5 (2.8) 0.31 Progression per year of scoliosis ( o ) 0.3 (1.0) 6.5 (4.1) 0.4 (1.3) 5.0 (3.3) p, differences between patients with progression <1 per year and those with progression >1 in each of the two age groups. 452 Developmental Medicine & Child Neurology 2000, 42:
6 treated with a soft Boston brace (Letts et al. 1992). However, the correction was below the 50 to 60% correction in brace reported in idiopathic scoliosis (Willner 1984, Olafsson et al. 1995). The percentage correction was reduced in patients 15 years old, in keeping with the common clinical impression that the curves are flexible until the end of growth and thereafter are quite structural (Fisk and Bunch 1979). There was no significant difference in absolute correction between the initial and the last follow-up examinations, but the percentage correction was considerably less at the last examination. This confirms that curves become more rigid with age. In spite of the brace treatment, there was a pronounced progression of 4.2 per year. Our results confirmed the findings of Miller et al. (1996) that orthotic treatment rarely succeeds in controlling a curve. One reason for the large progression could be that patients did not use the orthosis long enough each day and were allowed to be without the brace during the night. This is, however, unlikely because progression per year was not reduced in our patients who used the brace >10 hours per day as compared to those with less brace-wearing time. As expected, the progression per year was reduced in patients 15 years, whereas there was no significant difference between those <10 years and those aged 10 to <15 years. This disagrees with previous authors who state that progression is more rapid during the adolescent growth spurt (Fisk and Bunch 1979). To evaluate the effect of treatment, the rate of curve progression should be compared with untreated scoliosis. Sparse data exist, however, on the development of scoliosis in CP. Saito et al. (1998) studied the natural history of scoliosis in institutionalized patients with CP <15 years of age at the time of the initial radiographs and found a mean rate of progression of 4.5 per year between age 10 and 15 years. This is in agreement with the rate of 4.7 in our patients in the same age group. After 15 years of age, Saito et al. (1998) reported decreased rates of progression: 3.5 at age 15 to 20 years and 2.5 at >20 years of age. In the same age groups, we found somewhat lower rates of progression, 2.1 and 0.8, respectively. Majd et al. (1997) studied untreated scoliosis in patients above 15 years of age and found somewhat higher progression per year in C-shaped curves (3.5 ) than in S-shaped curves (2.3 ). No such difference occurred in our patients. In untreated CP scoliosis Thometz and Simon (1988) found that the rate of progression after skeletal maturity varied according to Cobb angle, being 0.8 per year when the initial curve was <50 and 1.4 per year when the curve was >50. Only two patients 15 years in the present study had initial Cobb angle <50, so the number of patients Table IV: Complaints caused by spinal orthoses in 57 patients who had not been operated on Problem Nr of patients Total Spinal orthosis Continued Discontinued (n = 57) (n = 41) (n =16) No problems Skin irritation Gastrointestinal problems Respiratory problems Other problems was too small for meaningful statistical analysis. In the 20 patients 15 years of age with Cobb angle >50, the mean progression was 1.4 per year, in keeping with the results of Thometz and Simon (1988). They emphasized that curves could deteriorate markedly between 20 to 30 years of age, which is not quite in agreement with the present results, where progression of >2.0 per year occurred in only one of nine patients 20 years of age. When comparing these natural history studies with the present results, it seems that spinal bracing generally does not delay the rate of curve progression. This is supported by the fact that progression was not reduced in our patients who used the brace >10 hours per day compared with those with less brace-wearing time. However, although the mean rate of progression was large in the present study, it showed great individual variation. Progression <1.0 per year was seen in 16% of patients <15 years of age and in 64% of older patients with a follow-up period of more than 2 years. This indicates a slightly better prognosis than that reported by Renshaw (1996), who stated that no more than 15% of bracetreated curves stop progressing. We found that large initial correction in orthosis was associated with progression of <1 per year in both younger (<15 years) and older patients. Moreover, multiple regression showed that high age and large initial correction in orthosis were the most important single factors in preventing progression. Thus, greater correction in orthosis seems to be the only changeable factor associated with the rate of curve progression. This indicates that high expertise among the orthotists is needed and considerable effort should be executed during preparation and adjustment of the orthosis to increase the degree of correction. There are few previous studies on the rate of progression in brace-treated patients. McMaster and Clayton (1980) reported on 20 patients with neuromuscular scoliosis and a mean age of 12 years. They had a very strict regime of wearing the brace 23 hours a day. The mean Cobb angle decreased from 42 before treatment to 31 in orthosis after 2 years of treatment and most curves improved in the brace. Unfortunately, the Cobb angle without orthosis at follow-up was not given. Pelvic obliquity correlated with the degree of scoliosis. Convexity of the scoliosis was to the lower side of the pelvis in all patients with PO >10, in accordance with other reports, and confirms the strong association between PO and scoliosis in patients with CP (Madigan and Wallace 1981, Letts et al. 1984). However, whether the pelvic obliquity occurred first and was the cause of the scoliosis or whether the opposite was true could not be determined because the data regarding time sequence were insufficient. Muscle imbalance with increased spasticity and contractures of the hip adductors and flexors often causes hip subluxation and dislocation in CP. If dislocation is unilateral, it often leads to PO with the pelvis higher on the dislocated side, which predisposes to scoliosis with convexity to the lower side of the pelvis (Letts et al. 1984). In the present series, 11 of 17 patients with unilateral dislocation had PO with the dislocated side higher, which seems to be in accordance with the experience of Letts et al. (1984). However, this relation was not constant, indicating that the etiology and pathogenesis in CP scoliosis does not necessarily have anything to do with the status of the hip joints, as reported by Treatment of scoliosis with spinal bracing in quadriplegic cerebral palsy Terje Terjesen et al. 453
7 Lonstein and Beck (1986). A total-contact orthosis is the most effective means of providing improved trunk support (Renshaw 1996). An important requirement, especially in individuals with severe disabilities, is good tolerance by the patient and acceptance by parents and caregivers. To meet these demands, the body jacket must be properly made and fitted. As most patients with neuromuscular scoliosis in our country were treated at our institution, orthotists have through the years gained great experience and skill in spinal bracing. This was probably an important reason why >80% of the parents and caregivers were satisfied with the orthoses and why most patients who have not been operated on have continued bracing even after 20 years of age. The strength of the acceptance of the orthoses was reflected by more than half the patients using the brace >10 hours per day; practically from the time they got up in the morning until bedtime, only interrupted by one or two breaks for rest and physical therapy. Special measures for additional adaptive seating were rarely necessary when the patients used their orthoses. Most patients only needed simple side support to be properly seated both in and out of the wheelchair. Although a cotton stockinette was used under the orthosis, the tight fitting plastic body jacket caused complaints in nearly one-third of the patients. The most frequent complaint, skin irritation, was usually solved by proper brace adjustments and was the cause of brace discontinuation in only four patients. When other complaints like respiratory or gastrointestinal problems arose, the orthoses had to be abandoned in most of the cases. To improve the management of scoliosis in individuals with quadriplegia, the scoliosis should be detected earlier by clinical examination and radiography from the age of 4 to 5 years. Surgical treatment should be considered when the curve exceeds 45 to 50. We do not know all the specific reasons why relatively few patients in the present study were operated on, but reasons noted in the case records were: risk factors (poor general health, respiratory impairment, severe epilepsy), relatively low Cobb angle or lack of curve progression, and parents being reluctant to have major surgery on children with severe disabilities. However, in individuals who for medical or social reasons are not candidates for surgical stabilization, bracing is a useful treatment option although the curve will continue to progress in most patients. Our results indicate that spinal orthosis should be tried in those with spastic quadriplegia with scoliosis, either permanently or as temporary treatment in patients scheduled for later surgical stabilization. If patient tolerance is good, bracing should continue as long as the parents and caregivers consider it useful for sitting, even in adult individuals. The only reason for discontinuing bracing is severe complaint that has not been satisfactorily managed by brace adjustments. In such cases custom-made wheelchair inserts, although difficult to fit properly (Renshaw 1996), might be better tolerated by the patient. References Boachie-Adjei O, Lonstein JE, Winter RB, Koop S, Brink KV, Denis F. (1989) Management of neuromuscular spinal deformities with Luque segmental instrumentation. Journal of Bone and Joint Surgery 71A: Cassidy C, Craig CL, Perry A, Karlin LI, Goldberg MJ. (1994) A reassessment of spinal stabilization in severe cerebral palsy. Journal of Pediatric Orthopaedics 14: Fisk JR, Bunch WH. (1979) Scoliosis in neuromuscular disease. Orthopedic Clinics of North America 10: Letts M, Shapiro L, Mulder K, Klassen O. (1984) The windblown hip syndrome in total body cerebral palsy. Journal of Pediatric Orthopaedics 4: Rathbone D, Yamashita T, Nichol B, Keeler A. (1992) Soft Boston orthosis in management of neuromuscular scoliosis: a preliminary report. Journal of Pediatric Orthopaedics 12: Lonstein JE, Akbarnia BA. (1983) Operative treatment of spinal deformities in patients with cerebral palsy or mental retardation. Journal of Bone and Joint Surgery 65A: Beck K. (1986) Hip dislocation and subluxation in cerebral palsy. Journal of Pediatric Orthopaedics 6: Madigan RR, Wallace SL. (1981) Scoliosis in the institutionalized cerebral palsy population. Spine 6: Majd ME, Muldowny DS, Holt RT. (1997) Natural history of scoliosis in the institutionalized adult cerebral palsy population. Spine 22: McMaster WC, Clayton K. (1980) Spinal bracing in the institutionalized person with scoliosis. Spine 5: Miller A, Temple T, Miller F. (1996) Impact of orthoses on the rate of scoliosis progression in children with cerebral palsy. Journal of Pediatric Orthopaedics 16: Olafsson Y, Saraste H, Söderlund V, Hoffsten M. (1995) Boston brace in the treatment of idiopathic scoliosis. Journal of Pediatric Orthopaedics 15: Renshaw TS. (1996) Cerebral palsy. In: Morissy RT, Weinstein SL, editors. Lovell and Winter s Pediatric Orthopaedics. New York: Lippincott-Raven. p Saito N, Ebara S, Ohotsuka K, Kumeta H, Takaoka K. (1998) Natural history of scoliosis in spastic cerebral palsy. Lancet 351: Thometz JG, Simon SR. (1988) Progression of scoliosis after skeletal maturity in institutionalized adults who have cerebral palsy. Journal of Bone and Joint Surgery 70A: Willner S. (1984) Effect of the Boston thoracic brace on the frontal and sagittal curves of the spine. Acta Orthopaedica Scandinavica 55: Accepted for publication 6th December Developmental Medicine & Child Neurology 2000, 42:
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