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Pediatric-Onset Rehabilitation

Orthopedic Complications

Orthopedic complications are common in children with neuromuscular dysfunction of any etiology, and pediatric SCI is no exception. Orthopedic complications of SCI may include scoliosis, hip dysplasia, contractures, and fractures.

Scoliosis refers to an abnormal spinal curvature involving both vertebral rotation about the long axis of the spine as well as curvature in the coronal plane. Scoliosis is diagnosed once the coronal curvature is at least ten degrees (Murphy et al. 2015). The implications of scoliosis vary dramatically based on the degree of abnormality, with some patients being completely asymptomatic, and others being significantly affected by abnormal positioning, unequal weight-bearing in seated, and even restrictive lung disease related to rotation of the thoracic cage.

Hip dysplasia refers to a condition in which the components of the hip joint (one or both of the proximal femur and/or acetabulum) become abnormally shaped such that the joint can more easily slide apart. The degree of joint displacement, as measured by the amount of the femoral head not covered by the acetabulum (migration percentage), determines whether the joint is described as subluxed (partially displaced) or dislocated (completely displaced) (Miller et al. 2017). Neuromuscular hip dysplasia is distinct from congenital hip dysplasia (also called developmental dysplasia of the hip) in that the hips of those with neuromuscular hip dysplasia were normal at birth but become dysplastic over time. The pathophysiology underlying this is not well understood, though abnormal muscle tone and strength and a lack of typical weight-bearing through the joint likely all contribute to the development of hip dysplasia across pediatric neuromuscular disorders, including SCI.

Muscle and joint contractures (causing fixed limitations in range of motion) can occur for a number of different reasons.  In pediatric SCI, contractures are most likely related to immobility and increased muscle tone (spasticity), causing shortening of muscles, tendons, and peri-articular connective tissue.  Fractures may be more likely to occur in this population due to reduced BMD below the level of the injury (discussed elsewhere in this review).

Author, Year

Country

Study Design

Sample Size

Population

Intervention

Outcome Measure

Results

(Kulshrestha et al., 2020)

United Kingdom

Observational

N=62

Population: Age at injury: 17 (13-17) yr; Gender: males=44, females=18; Injury etiology: traumatic=51, non-traumatic=11; Time since injury: 28 (22-33) yr.

Intervention: None. Chart review.

Outcome Measures: Incidence of scoliosis.

Scoliosis

1.         At the time of discharge, 4/62 patients (6%) had developed scoliosis, increasing to 19/62 (30%) 10 yr post injury and 21/62 (34%) at the latest clinical assessment.

2.        The Cobb angle could be determined for seven patients, giving a median value of 70° (21–72).

3.        The overall incidence of scoliosis was smaller in the traumatic group (13/52; 25%) than in the neurological group (8/11; 72%).

4.        Patients older at injury were less likely to have developed scoliosis at 10 yr (p<0.001).

5.        Younger age did (p=0.001) but being non-traumatic did not (p=0.29) predict development of scoliosis.

(Mulcahey, Gaughan et al. 2013)

USA

Observational

N=217

Population: Age at interview: 13.2 yr; Age at injury: 9.0 yr; Gender: males=127, females=90; Time since injury: 4.2 yr; Level of injury: tetraplegia=112, paraplegia=95; Severity of injury: complete=105, incomplete=96.

Intervention: None. Chart review.

Outcome Measures: International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI), Cobb angles as a measure of the prevalence of scoliosis, prevalence of spinal fusion.

Scoliosis

1.         78% (145/186) of subjects had >10° scoliosis, 47% (87/186) had >20° scoliosis, and 20% (37/186) had >45° scoliosis.

2.        When grouped as an entire sample, age at injury (p=0.0001) and AIS classification (p=0.0095) were the only significant predictors of worse curve.

3.        With the exclusion of subjects with AIS D, age at injury (p=0.0140) was the only significant predictor of worse curve.

4.        Age at injury (p=0.007) was the only significant predictor of spinal fusion in the entire sample and remained as such with the exclusion of subjects with AIS D (p=0.009).

5.        The risk of spinal fusion increased 11% for each year decrease in age at injury; this finding is consistent with or without inclusion of subjects with AIS D.

6.        Sex, motor score, and neurological level were not predictors of worse curve or spinal fusion.

7.        Of the subjects >14 yr old at the time of their evaluation and injured at <12 yr old (16/43), 13% required a spine fusion compared to 4% injured at >12 yr old (27/43).

8.        Subjects injured at <12 yr old were 3.7 times more likely to require a spinal fusion than those injured at >12 yr old.

(Johnston, Betz, et al., 2009)

USA

RCT

N=30

PEDro=2

Population: Age: 9.7±2.5 yr; Gender: females=13, males=17; Level of injury: C=11, T=19; Severity of injury: ASIA A=22, AIS B=5, AIS C=3.

Intervention: Subjects were randomized to one of three groups: 1) Functional Electrical Stimulation (FES) cycling (50 rpm while seated in wheelchair, pulse duration=150 ls, frequency=33 Hz, amplitude max 140 mA, increased automatically to generate sufficient

force to maintain the cadence); 2) passive leg cycling (50 rpm), or 3) non-cycling with 20 min daily surface stimulation to lower extremity muscles. Sessions were conducted for 1 hr/day, 3 days/wk for 6 mo.

Outcome Measures: Hip subluxation as measured by migration index of the femoral head.

Hip Subluxation

1.         No differences in migration indices were found between baseline and 6 months (p=0.667), indicating that the intervention had no effect on these values.

2.        No differences were found between groups over time (p=0.891); however, differences were found between groups (p<0.001), with the passive cycling group having greater migration indices than the FES cycling group at any time (p<0.001).

(Sison-Williamson et al., 2007)

USA

Observational

N=20

Population: Age: 10.9±3.0 yr; Gender: males=10, females=10; Level of injury: cervical=1, thoracic=17, lumbar=2; Severity of injury: AIS A=18, AIS C=2.

Intervention: Subjects were positioned on a standard chair to decrease sitting and posture differences, introduced by variations in wheelchair designs. Subjects’ hips, knees, and ankles were positioned at 90°. Reflective markers were placed on the C7, sternal notch, acromion joints, olecranon, ulnar and radial styloid, and hands. Three-dimensional upper extremity motion analysis using an 8-camera Motion Analysis System was used to capture the subjects’ reaches.

Outcome Measures: Workspace volume and reach with and without a thoracic lumbar sacral orthosis (TLSO).

Scoliosis

1.         Without the TLSO, the average reaching volume was 112,836 cm3.

2.        With the TLSO, the average reaching volume was 80,711 cm3, which represents a 28% decrease in volume of reach (p=0.0002).

3.        The largest increase in volume of reach without the TLSO was 77.3%, while the smallest increase in volume was 4.9%.

4.        10 of 39 cases had less than a 10% change between the TLSO and non-TLSO conditions, while 6 cases had increases in volume greater than 50%.

5.        There were 6 cases in which volumes were larger in the TLSO condition compared with the non-TLSO condition; the percent differences ranged from 5-28%.

6.        The non-TLSO average ranges of reach in the anterior-posterior (AP), medial-lateral (ML), and vertical (V) directions were 80±26 cm, 118±24 cm, and 97±21 cm, respectively.

7.        The TLSO average ranges of reach in AP, ML, and V directions were 72±19 cm, 113±24 cm, and 94±21 cm, respectively.

8.        The AP and ML average ranges of reach were statistically greater in the non-TLSO condition than the TLSO condition (p=0.002, p=0.01), whereas the V reach showed no significant difference.

9.        When comparing non-TLSO condition to TLSO condition, the nondominant ML reach was significantly greater in the non-TLSO condition than the TLSO condition (p=0.003), while AP and V reaches showed no significant difference.

10.      For the non-TLSO condition, AP reach was significantly greater than the TLSO condition (p=0.009), while ML and V reaches showed no significant difference.

11.       There were no significant differences between AP, ML, and V ranges of reach when comparing nondominant and dominant arms in the non-TLSO condition.

12.      In the TLSO condition, ML reach was significantly greater for the dominant arm than the nondominant arm (p<0.05).

(Chafetz et al., 2007)

USA

Pre-Post

N=14

Population: Age: 10.8±2.4 yr; Gender: males=7, females=7; Level of injury: cervical=1, thoracic 1-5=3, T6-11=10.

Intervention: Subjects completed the activities of the functional activities scale (FAS) and the timed motor test (TMT) which included 6 activities involving dressing, transfers, and wheelchair propulsion, with a thoracic lumbar sacral orthosis (TLSO) and without a TLSO. Subjects were asked their preference for wearing or not wearing the TLSO during each of the activities.

Outcome Measures: Functional Independence Measure (FIM), 6 wheelchair/transfer skills, Timed Motor Test (TMT), preference.

Scoliosis

1.         For donning a shirt, there was a 26% increase in time with a TLSO compared to without a TLSO (p<0.001).

2.        For donning pants, there was a 21% increase in time with a TLSO compared to without (p<0.001).

3.        The time to complete increased by 42% for even transfers and 28% for uneven transfers with TLSO compared to without (p<0.001).

4.        For wheelchair propulsion down a hallway, there was a 6% increase in time with a TLSO than without (p<0.001).

5.        Wheelchair propulsion ascending a ramp was not significantly impacted by wearing a TLSO (p=0.11).

6.        Wearing a TLSO did not impact the activities of eating, grooming, wheelchair propulsion, curb management, ‘‘popping wheelies,’’ or transitioning from sitting at the edge of a bed to a supine position.

7.        A reduction of scores was evident for dressing (upper and lower body), bladder management, bed transfers, reaching for the floor, and transitioning from a supine position to sitting at the edge of the bed, but only upper-body dressing was statistically significantly different (p<0.01).

8.        For eating, grooming, wheelchair propulsion, and popping a wheelie, subjects did not have a preference to wear or not wear a TLSO.

9.        Preference for not wearing a TLSO was significantly different (p<0.05) for lower-body dressing, reaching for the floor, and transitioning from a supine position to sitting at the edge of the bed.

(Mehta et al., 2004)

USA

Observational

N=123

Population: Age at interview: 7.4 yr; Age at injury: 5.3 yr; Gender: males=69, females=54; Injury etiology: traumatic=115, non-traumatic=8; Level of injury: cervical=69, thoracic=54; Severity of injury: AIS A=71, AIS B=49, AIS C=1, AIS D=2.

Intervention: None. Chart review.

Outcome Measures: Radiographic curve severity of the spine, prevalence of bracing, surgery and cessation of growth

Scoliosis

At follow-up (range 2-13 yr), 95% of patients had developed scoliosis; surgical stabilization was required in 65% of the total sample.

 

Group I (initial curve <10°; n=42)

1.         29 of the patients in this group were braced, and 13 who were not.

2.        Of the braced group, 13 (45%) went on to surgery, whereas 10 (77%) of the non-braced group had surgical correction (p=0.03).

3.        Of the patients who were initially braced, the average time to surgery was 8.5 yr, whereas that for the non-braced group was 4.2 yr (p=0.002).

4.        There was no significant difference between time to surgery for the braced and non-braced patient groups at higher (>20°) initial curve presentations.

 

Group II (Initial curve 11 ° to 20°; n=25)

1.         Eighteen (72%) patients in this group were braced and 72 8%) were not braced.

2.        Nine of the 18 children in the braced group (50%) required surgery at 6.8 years after initial presentation, whereas 6 of 7 of the nonbraced group (86%) required surgery at 3.7 years after presentation.

3.        The difference between the rate of surgery (p=0.04) and the length of time to surgery (p=0.008) in the braced vs nonbraced group was statistically significant, whereas the curve at the time of surgery was not (p=0.52).

 

Group III (Initial curve 21 ° to 40°; n=28)

1.         Of the 20 (61%) children initially braced in this group, 1 2 (60%) went on to have surgery at 4.2 years after presentation, whereas 8 (40’7’o) did not require surgery.

2.        Of the 8 children (3 9%) who were not braced, 6 (7 5%) went on to surgical correction at 3.2 years after presentation.

3.        While there was no statistical difference for time to surgery between the braced and nonbraced patients in group III (p=0.3 6), there was a trend toward less surgical intervention in the braced patients (p=0.08).

 

Group IV (Initial curve > 41 ° but < 50°; n=16) & Group V (curves > 51 ° at presentation; n=12)

1.         In Group IV, one patient (6%) was not braced and proceeded to surgery, whereas 15 (94%) were braced, of which 12 (80%) went on to have surgical correction of their deformity.

2.        In Group V, ten patients (83%) were braced and 2 (17%) were not braced; surgical correction of the spine was performed on 8 children (80%) in the braced group and both children (100%) in the nonbraced group.

3.        In group IV and V, There was no significant difference between time to surgery for the braced and non-braced patient groups.

(Vogel et al., 2002c)

Part II

USA

Observational

N=216

Population: Age at injury: 14.1±4.0 yr; Age at interview: 28.6±3.4 yr; Gender: males=150, females=66; Time since injury: 14.2±4.6 yr; Level of injury: tetraplegia=123, paraplegia=93. Severity of injury: C1-4 ABC=41, C5-8 ABC=67, T1-S5 ABC=82, tetra/para D=26.

Intervention: None. Survey.

Outcome Measures: Prevalence of scoliosis.

Scoliosis

1.         Scoliosis affected 40% of participants and was significantly associated with younger age at injury (p<0.001); prevalence of scoliosis was 86% in children injured at ≤12 yr, and 31% in those injured at older ages.

2.        Subjects with scoliosis had a longer duration of injury (p<0.001) and were more likely to have had a violent etiology (p=0.003) compared to those without scoliosis.

3.        Scoliosis was not associated with gender or level of injury.

4.        Scoliosis more commonly affected individuals with hip subluxation or contractures compared to those without these hip complications (p=0.003).

5.        Scoliosis was not statistically associated with back pain, pressure ulcers, or respiratory complications.

Hip Subluxation

1.         Hip subluxation or contractures affected 68 participants, with 18 experiencing hip subluxation alone, 33 contractures alone, and 17 with both hip contractures and subluxation.

2.        Hip subluxation was significantly associated with younger age at injury and longer duration of injury (p<0.001); rates of hip subluxation were 43% (6/14) for those injured at ≤5 yr, 52% (11/21) injured at ≤8 yr, and 41% (11I27) of those injured at ≤10 yr.

3.        Hip subluxation was not significantly associated with gender, neurological level, ASIA motor score, or FIM scores.

4.        Hip contractures were not associated with age at injury or duration of injury or spasticity.

5.        Hip contractures were significantly more prevalent in those with injuries due to violence than non-violent injuries (59% versus 20%; p<0.001) and those with paraplegia compared to those with tetraplegia (p<0.001).

6.        Those with hip contractures demonstrated significantly higher total FIM (p=0.001) and motor FIM scores (p=0.002).

Fractures

1.         A total of 45 pathological fractures were experienced by 32 subjects.

2.        Individuals who developed pathological fractures were significantly older at the time of interview, (p=0.038) had a longer duration of their SCI (p=0.011), and more likely to have lower cervical level injuries (C5-C8 A-B-C) (p=0.010).

3.        There were no other significant associations between pathological fractures and the remaining study variables.

Ankle Pain and Contractures

1.         Ankle pain or contractures affected 53 subjects, with 29 individuals having contractures alone, 18 reported pain only, and 6 had complaints of both contractures and pain.

2.        Ankle pain was significantly associated with older age at injury (p=0.018) and tetraplegia (p=0.005).

3.        Ankle contractures were not significantly associated with any of the study variables.

Elbow Pain and Contractures

1.         Elbow pain or contractures affected 43 subjects with 27 experiencing elbow pain alone, 10 had elbow contractures alone, and 6 had both.

2.        Those with elbow pain were significantly older at follow-up (p=p=0.026) and had a longer duration of their SCI (p=0.041).

6.        As expected, elbow contractures were significantly more common in those with tetraplegia (p=0.040) and were significantly associated with lower ASIA motor scores (p=0.016) and lower total FIM (p=0.010) and motor FIM scores (p=0.009).

(Moynahan, Betz, et al., 1996)

USA

Observational

N=51

Population: Age: 14.5±4.2 (3-20) yr; Gender: males=30, females=21; Level of injury: cervical=19, thoracic/lumbar=32.

Intervention: Patients underwent bone density measurements using dual photon absorptiometry.

Outcome Measures: Bone Mineral Density of femoral neck, Ward’s triangle and intertrochanteric region of the hip, presence of spasticity, number of pathological fractures.

Fractures

1.         Subjects with SCI had lower bone densities compared to their non-disabled peers, ranging from 56-65 % of normal across the three anatomic regions.

2.        On average, subjects who had a previous history of fractures had significantly lower bone density measurements than those without fractures (p<0.05).

3.        At the intertrochanteric region, a 10.6% difference was noted between subjects with tetraplegia versus those with paraplegia.

4.        At the femoral neck and Ward’s Triangle, an 8.5% difference was noted between subjects with and without spasticity.

(Dearolf et al., 1990)

USA

Observational

N=155

Population: Preadolescent Group (N=61): Age at injury: 8.5 yr (males, N=43), 7.5 yr (females, N=18); Injury etiology: traumatic=57, non-traumatic=4; Level of injury: cervical=13, thoracic=45, lumbar=3; Severity of injury: complete=50, incomplete=11.

Post-adolescent Group (N=94): Age at injury: 17.0 yr (males, N=68), 16.0 yr (females, N=26); Injury etiology: traumatic=94, non-traumatic=0; Level of injury: cervical=67, thoracic=23, lumbar=4.

Intervention: None. Chart review.

Outcome Measures: Prevalence of scoliosis.

Scoliosis

Preadolescent Group

1.         55 (96.5%) of the patients that did not receive treatment developed a paralytic scoliosis.

2.        Curve progression was rapid in this group; 8 (14%) of patients who were <1 year postinjury had developed curves of >20 deg; 17(31%) of patients who were <2 years post injury had developed curves of >20 deg.

3.        Curve progression was 10.6 deg/year on average

4.        The degree of curve that developed was not related to the level or completeness of the spinal cord injury.

5.        Of the 12 patients braced and followed for an average of 48 mo, 5 progressed and either have had surgery already or planned, while 7 have progressed <5°. 2 of the braced patients developed pressure sores.

6.        19 (33%) patients required surgical intervention for a progressive paralytic scoliosis.4 of them initially underwent Luque rodding with wires without fusion; their curves progressed without gains in spinal height, without gains in spinal height necessitating further spinal stabilization with fusion.

7.        The average prefusion curve was 52° with post-fusion correction to 25°, for an average correction of 50%.

8.        Pseudarthrosis developed in 5 (26%) of the patients and 11 (58%) of the patients required reoperation.

9.        Loss of correction after surgery >5° occurred in 10 patients.

Post-adolescent Group

10.      41 (48%) of the patients that did not receive treatment developed a progressive paralytic scoliosis.

11.       Curves slowly progressed to a significant degree in 15 (36%) of the patients in whom curves >20° developed >2 yr post injury.

12.      Curve progression was 5.4 degrees/year, on average.

13.      13. 5 patients (5.3%) eventually required a surgery for a progressive paralytic scoliosis; the average prefusion curve was 50 degrees and the average postfusion curve was 12 degrees.

Discussion

Scoliosis is a major potential complication of pediatric SCI. Estimates of the incidence of progressive scoliosis related to paralysis range from 46 to 98% (Mehta et al. 2004). A review of the studies presented here suggests that more precise estimates of the frequency of scoliosis in pediatric SCI can be made if one considers the age of the individual at the time of injury. This is perhaps best demonstrated in the observational study by Dearolf et al. (1990), where children were divided into two groups based on their age when they were injured: the preadolescent group and the skeletally mature group. In the preadolescent group, without treatment, 96.5% developed scoliosis. Spinal curves progressed rapidly (at a rate of 10.6 degrees per year on average), and degree of curvature was not related to the level or completeness of injury. Of the patients who underwent bracing, about half stabilized, and half still went on to need surgery (and it was noted that 2 of the 12 braced patients developed significant pressure sores with bracing). Overall, 33% of this group required spinal surgery to manage the degree of scoliosis, with the authors carefully noting that if individuals in this group who were within one year of reaching skeletal maturity at the time of injury were excluded from analysis, that number rose to approximately 60% requiring surgical management. In contrast, only 48% of individuals who were skeletally mature at the time of injury had scoliosis without treatment. Curve progression was much slower (5.4 degrees per year) and only 18% went on to require surgery. In any instance of very rapid progression (>15 degrees in 6 months), it is suggested that evaluation for syringomyelia be considered (Dearolf et al. 1990). Kulshrestha et al. (2020), Mulcahey et al. (2013) and Vogel et al. (2002c) also reported that age at time of injury appeared to be the most important predictor of progressive paralytic scoliosis amongst children with SCI.

Bracing for scoliosis in the setting of SCI was discussed in a number of studies. In their chart review of 123 patients, Mehta et al. (2004) found that those braced when curves were smaller (less than 20 degrees) were less likely to go on to require surgery, or at least were able to prolong the time to surgery. This raises an interesting question around early bracing, in contrast to conventional treatment protocols, which dictate that bracing should be considered for scoliotic curves between 20-40 degrees (Mehta et al. 2004). Dearolf et al. (1990) found bracing to be effective for a small number of preadolescent individuals with scoliosis, though the rationale for who was and who was not braced was not well described. In younger patients, delaying the need for surgery may be desirable, so as to avoid spinal instrumentation before skeletal maturity is reached. There is a limited role for bracing larger curves (>40 degrees) (Dearolf et al. 1990; Mehta et al. 2004) and older (skeletally mature) patients (Dearolf et al. 1990), as successful management of scoliosis is less likely in these cases. Two studies examined the impact of bracing with a thoracolumbosacral orthosis on function amongst children with SCI. Sison-Williamson et al. (2007) demonstrated that use of a thoracolumbosacral orthosis resulted in reduced anterior/posterior and medial/lateral reach. Chafetz et al. (2007) demonstrated that, while some functions were not significantly impacted by thoracolumbosacral orthosis use, there was a significant impact on dressing and transfer time, and that patients preferred not to have the thoracolumbosacral orthosis on for certain tasks (such as reaching to the floor, dressing the lower body, and certain transfers).  These functional implications, coupled with the risk of pressure ulceration with thoracolumbosacral orthosis use (Dearolf et al. 1990) serve as a reminder that bracing should be considered as any other intervention, with considerations given to potential risks and benefits of treatment.

In comparison to scoliosis, information about other orthopedic complications of pediatric SCI is relatively limited. In one observational study, hip subluxation was documented in 40-50% of individuals who were injured before the age of 10 (Vogel et al. 2002c), with younger age at time of injury and longer duration of SCI found to significantly impact hip position. No information about rate of subluxation in older age categories was provided. A single interventional study assessed the impact of FES cycling, passive leg cycling, and surface stimulation without cycling on hip position (as measured by migration percentage) (Johnston, Betz, et al. 2009). None of the interventions were found to have an impact on hip subluxation at six months, though it could certainly be argued that the follow-up interval would be too short to observe a significant effect. Information regarding contracture and fractures in SCI was similarly limited.  Vogel et al. (2002c) observed ankle contractures in 16% of their sample of 216 individuals with chronic pediatric SCI, while elbow contractures were documented in 7.5% of the sample.  There were no associations between ankle fractures and any of the study variables, though elbow fractures were significantly associated with tetraplegia and lower American Spinal Injury Association motor scores, and Functional Independence Measure total and motor scores. Vogel et al. (2002c) also documented 45 pathologic fractures in 35 individuals with pediatric SCI (representing fracture(s) in 16% of the overall sample). Fractures were significantly associated with older age at the time of the study, longer duration of SCI, and cervical level injury. BMD amongst individuals with pediatric SCI was found by Moynahan et al. (1996) to be reduced by 56-65% as compared to peer controls, with greater reductions noted in those with a documented history of fracture.

Conclusion

Orthopedic complications of pediatric SCI are common. The risk of scoliosis requiring intervention is particularly high amongst those who experienced their injury at least one year prior to reaching skeletal maturity. While bracing with thoracolumbosacral orthosis does have some negative implications for function and skin health, bracing may delay or eliminate the need for invasive spine surgery, particularly if it is used before curves are overly large (certainly before 40 degrees, with some literature suggesting it could be instituted at less than 20 degrees) and while individuals are still skeletally immature. Hip subluxation is also relatively frequent, again with rates appearing to be higher amongst those who are injured at a younger age. Very little is known about effective rehabilitation-based intervention to slow the progression of hip subluxation. Contractures and fractures also occur at significant rates in this population, but there is no available literature regarding interventional strategies to reduce their incidence.

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