Non-ambulatory Children Research Articles

Health-related quality of life and caregiver burden after hip reconstruction and spinal fusion in children with spastic cerebral palsy.

To evaluate the effect of hip reconstruction or spinal fusion on health-related quality of life (HRQoL) in non-ambulatory children with spastic cerebral palsy (CP) and caregiver burden. This was a prospective, longitudinal study of changes in HRQoL and caregiver burden over the 5 years after surgical correction of hip instability or scoliosis in children with bilateral spastic CP classified in Gross Motor Function Classification levels IV or V. Serial parent proxy measures of HRQoL and caregiver burden were obtained before and 6 weeks, and 3, 6, 9, 12, 24, and 60 months after surgery using the Caregiver Priorities and Child Health Index of Life with Disabilities and the Assessment of Caregiver Experience with Neuromuscular Disease. Scores 5 years or more after surgery were compared to pre-surgery scores using paired Student's t-tests. Serial outcome trajectories were estimated by linear mixed modeling. Of 69 participants (40 males, 29 females; mean age 11y 6mo, SD 4y 1mo, range 3y 10mo-20y 7mo), 43 had hip reconstruction and 26 had spinal fusion. Clinically significant improvements in HRQoL were detected (average increase 7.6 points) 5 years or more postoperatively, with hip reconstruction providing greater benefit. Domains improved by surgery included positioning, transferring/mobility, comfort/emotions, and health. Caregiver burden did not change as these children remain maximally dependent. Surgical treatment of hip and spine deformity improves HRQoL, especially for painful hip instability, but does not change caregiver burden.

Incidence, pattern and mechanisms of injuries and fractures in children under two years of age

BackgroundFractures in children under 2 years of age are rare, and little has been published on their mechanisms. We aimed at examining the incidence, mechanisms, pattern and fracture characteristics in a large, population-based cohort of otherwise healthy children.MethodsThis retrospective, cross-sectional study includes all children aged 0–2 years, attending the Accident and Emergency department in Bergen between 2010 and 2015, due to an injury warranting radiography. Clinical data was categorized from the medical notes, and all radiographs were reviewed by an experienced paediatric radiologist.ResultsIn total 408 children (212 male), 3–23 months of age (mean 17.7 months), were included. 149 (77 male) children had a total of 162 fractures, yielding an annual incidence of 5.4 per 1000, varying from 0.7 per 1000 for those under 12 months of age, increasing tenfold to 7.3 per 1000 for children aged 12–24 months of age. More than half of the fractures (53.1%) were seen in children aged 18–23 months, while none was found in those under 7 months of age. The youngest age group had mostly femur and tibia fractures, the oldest mostly forearm fractures (n = 55, 33.9%), followed by tibia fractures (21.6%) and fractures to the clavicle (14.8%). The reported mechanisms for the 162 fractures were fall from a chair/bed/table (41.4%), fall from own height (18.5%) or crush injury (15.4%). In 8 of 162 (4.9%) fractures, the history was clearly inconsistent and suspicious of non-accidental injury (NAI).ConclusionInjuries and fractures in young children in general, and non-ambulant children in particular, are rare and should be thoroughly assessed for NAI. Level of evidence: Retrospective, population based cross-sectional study. Level 3.

The Femoral Head-Shaft Angle Is Not a Predictor of Hip Displacement in Children Under 5 Years With Cerebral Palsy: A Population-based Study of Children at GMFCS Levels III-V.

Background:The aim of this study was to evaluate whether the femoral head-shaft angle (HSA) is a predictor of hip displacement in children with cerebral palsy (CP).Methods:The patients were recruited from a population-based hip surveillance program. Inclusion criteria were age under 5 years, bilateral CP, Gross Motor Function Classification System (GMFCS) levels III-V, and migration percentage (MP) of both hips <40% at the primary radiograph. With these criteria, 101 children (61 boys) were included. GMFCS was level III in 26 patients, level IV in 23, and level V in 52. An anteroposterior radiograph of the pelvis was taken at diagnosis and at the last follow-up. Only the worst hip of each patient (the hip with the largest MP) was used for the analyses.Results:The mean age at the primary radiograph was 2.4 years (range, 0.8 to 4.9 y). The mean primary HSA was 171.0 degrees (range, 152 to 190 degrees). The mean follow-up time was 4.3 years (range, 0.9 to 11.8 y). The mean MP at the primary radiograph was 17.5% (range, 0% to 39%) and at the last follow-up 41.9% (range, 0% to 100%). At that point, MP was <40% in 54 hips and ≥40% in 47 hips. There was no significant difference in primary HSA between patients with final MP<40% and those with final MP≥40% (170.8 and 171.3 degrees, respectively; P=0.761). At the last follow-up, the mean HSA was significantly larger in hips with final MP≥40% than in hips with final MP<40% (171.1 vs. 167.4 degrees; P=0.029).Conclusions:There was a markedly increased valgus position of the proximal femur in nonambulatory children with CP. However, the primary HSA in children below 5 years of age was not a predictor of later hip displacement, defined as MP≥40%.Clinical Relevance:Measurement of HSA is not necessary in routine hip surveillance in children below 5 years.Level of Evidence:Level I—investigating a diagnostic test.

Variability in Postoperative Immobilization and Rehabilitation Following Reconstructive Hip Surgery in Nonambulatory Children With Cerebral Palsy.

Background:Despite being a common procedure, there are no standard protocols for postoperative immobilization and rehabilitation following reconstructive hip surgery in children with cerebral palsy (CP). The purpose of this study was to investigate variability in postoperative management and physical therapy (PT) recommendations among orthopaedic surgeons treating hip displacement in children with CP.Methods:An invitation to participate in an anonymous, online survey was sent to 44 pediatric orthopaedic surgeons. The case of a child undergoing bilateral femoral varus derotation osteotomies and adductor tenotomies was presented. Surgeons were asked to consider their typical practice and the case scenario when answering questions related to immobilization, weight-bearing, and rehabilitation. Recommendations with increasingly complex surgical interventions and different age or level of motor function were also assessed.Results:Twenty-eight orthopaedic surgeons from 9 countries with a mean 21.3 years (range: 5 to 40 y) of experience completed the survey. Postoperative immobilization was recommended by 86% (24/28) of respondents with 7 different methods of immobilization identified. All but 1 (23/24) reported immobilizing full time. Most (20/23) reported using immobilization for 4 to 6 weeks. Return to weight-bearing varied from 0 to 6 weeks for partial weight-bearing and 0 to 12 weeks for full weight-bearing. PT in the first 1 to 2 weeks postoperatively was reported as unnecessary by 29% (8/28) of surgeons. PT for range of motion, strengthening, and return to function was recommended by 96% (27/28) of surgeons, starting at a mean of 2.6 weeks postoperatively (range: 0 to 16 wk). Only 48% (13/27) reported all of their patients would receive PT for these goals in their practice setting. Inpatient rehabilitation was available for 75% (21/28) but most surgeons (17/21) reported this was accessed by 20% or fewer of their patients.Conclusions:Postoperative immobilization and PT recommendations were highly variable among surgeons. This variability may influence surgical outcomes and complication rates and should be considered when evaluating procedures. Further study into the impact of postoperative immobilization and rehabilitation is warranted.

From the Luttrell Psalter to the Lobster Boy: Split hand and foot awaken many facets of human nature

A marginal illustration of a non-ambulatory child in the 14th Century Luttrell Psalter is the earliest currently identifiable image of an individual with Split Hand Split Foot with Long Bone Deficiency (SHFLD). Changes in portrayal of SHFLD over the centuries reflect changes in social perception of disabilities from pious sympathy to scientific curiosity and unfortunately also morbid fascination. Hopefully understanding of the past attitudes toward split hand and foot as reflected in art can help in moving toward acceptance of individuals with this highly visible malformation.

Multisite Veno-Venous Cannulation for Neonates and Nonambulatory Children.

Neonates with respiratory failure are ideally supported with veno-venous rather than veno-arterial extracorporeal membrane oxygenation due to the reduced rate of neurologic complications. However, the proportion of neonates supported with veno-venous extracorporeal membrane oxygenation is declining. We report multisite veno-venous extracorporeal membrane oxygenation, accessing the neck, returning to the inferior vena cava via the common femoral vein in neonates and children less than 10 kg. Retrospective case series with 1 year minimum follow-up. Patients less than 10 kg supported with veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein. A 30-bed pediatric intensive care delivering extracorporeal membrane oxygenation to approximately 20 children annually. Veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein was delivered using two single lumen cannulae. January 2015 to August 2019, 11 patients underwent veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein with median weight of 3.6 kg (interquartile range 2.8-6.1 kg), and median corrected gestational age of 13 days (interquartile range, 2-175 d). The smallest patient weighed 2.1 kg. Seven patients had comorbidities. Extracorporeal membrane oxygenation was technically successful in all patients with median flows of 126 mL/kg/min (interquartile range, 120-138 mL/kg/min) and median arterial oxygenation saturation of 94% (interquartile range, 91-98%) at 24 hours. Nine survived to home discharge, and two were palliated. Common femoral vein occlusion was observed in all patients on ultrasound post decannulation. There was no clinical or functional deficit in the cannulated limb at follow-up, a minimum of 1 year post extracorporeal membrane oxygenation. Veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein was performed safely in patients under 10 kg with the smallest patient weighing 2.1 kg. Although occlusion of the common femoral vein was observed in patients post decannulation, subsequent follow-up demonstrated no clinical implications. We challenge current practice that veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein cannot be performed in nonambulatory patients and suggest that this strategy is preferred over veno-arterial extracorporeal membrane oxygenation in infants requiring extracorporeal membrane oxygenation for respiratory failure.

Vitamin D, Bone Mineral Density and Serum IGF-1 Level in Non-ambulatory Children With Cerebral Palsy

To compare serum 25-hydroxy vitamin D (25-OHD) status, bone mineral density and Insulin-like growth factor (IGF-1) level among children with cerebral palsy (CP) aged 1 to 8 years with age- and gender-matched controls. A cross-sectional study enrolled 30 children in each group: CP with epilepsy, CP without epilepsy, and healthy controls. Bone mineral density (BMD), serum 25-OHD levels, and serum insulin like growth factor (IGF)-1 levels were measured. z-scores of BMD [-1.80 (1.03), -2.12 (0.85) vs -1.40 (0.90); P<0.01], 25-OHD levels [19.26 (8.28), 20.59 (8.92) Vs 26.79 (12.76) ng/mL; P<0.01] and IGF-1 levels [20.90 (6.42), 23.37 (8.11) vs 31.77 (11.21) ng/mL; P<0.01] were significantly low among children with CP with epilepsy, CP without epilepsy when compared to controls. Children with CP with or without comorbid epilepsy were prone to vitamin D deficiency, low bone mineral density and growth hormone axis suppression with low IGF-1 levels.

Residual lumbar hyperlordosis is associated with worsened hip status 5 years after scoliosis correction in non-ambulant patients with cerebral palsy.

Cerebral palsy (CP) is a static encephalopathy with progressive musculoskeletal pathology. Non-ambulant children (GMFCS IV and V) with CP have high rates of spastic hip disease and neuromuscular scoliosis. The effect of spinal fusion and spinal deformity on hip dislocation following total hip arthroplasty has been well studied, however in CP this remains largely unknown. This study aimed to identify factors associated with worsening postoperative hip status (WHS) following corrective spinal fusion in children with GMFCS IV and V CP. Retrospective review of GMFSC IV and V CP patients in a prospective multicenter database undergoing spinal fusion, with 5years follow-up. WHS was determined by permutations of baseline (BL), 1year, 2years, and 5years hip status and defined by a change from an enlocated hip at BL that became subluxated, dislocated or resected post-op, or a subluxated hip that became dislocated or resected. Hip status was analyzed against patient demographics, hip position, surgical variables, and coronal and sagittal spinal alignment parameters. Cutoff values for parameters at which the relationship with hip status was significant was determined using receiver operating characteristic curves. Logistic regression determined odds ratios for predictors of WHS. Eighty four patients were included. 37 (44%) had WHS postoperatively. ROC analysis and logistic regression demonstrated that the only spinopelvic alignment parameter that significantly correlated with WHS was lumbar hyperlordosis (T12-L5) > 60° (p = 0.028), OR = 2.77 (CI 1.10-6.94). All patients showed an increase in pre-to-postop LL. Change in LL pre-to-postop was no different between groups (p = 0.318), however the WHS group was more lordotic at BL and postop (pre44°/post58° vs pre32°/post51° in the no change group). Age, sex, Risser, hip position, levels fused, coronal parameters, global sagittal alignment (SVA), thoracic kyphosis, and reoperation were not associated with WHS. Postoperative hyperlordosis(> 60°) is a risk factor for WHS at 5years after spinal fusion in non-ambulant CP patients. WHS likely relates to anterior pelvic tilt and functional acetabular retroversion due to hyperlordosis, as well as loss of protective lumbopelvic motion causing anterior femoracetabular impingement. III.

Factors Influencing Outcomes of the Dysplastic Hip in Nonambulatory Children With Cerebral Palsy.

Hip dysplasia in the nonambulatory child with spastic cerebral palsy (CP) is a common condition not always effectively treated with conservative measures even when recognized early. Optimal surgical intervention strategies and timing are not clear from previous studies. Contralateral hips with less severe subluxation in these patients also often undergo surgery and little is known of outcomes of these less severe hips. This study aims to clarify treatment factors related to long term success following hip surgery for subluxation in nonambulatory children with CP. A total of 183 nonambulatory subjects with CP and a minimum of 2-year follow-up were included. All subjects underwent varus rotational osteotomy of the femur; other surgical factors considered were addition of pelvic osteotomy (PO), capsulorrhaphy, and soft tissue releases. Additional factors studied were age at index surgery, sex, and unilateral versus bilateral surgery. Severely subluxated (SS) hips, defined as having >50% migration, were studied separately from contralateral nonsevere hips. Surgeries were deemed successful if final follow-up indicated a migration of <25%; patients with any revision surgeries or >25% migration were categorized as failures. A 60% success rate was found in SS hips and a 68% success rate in nonsevere hips. Age at index surgery did not influence success rates in SS hips. In the nonsevere hips, success was associated with index surgery at older age. The addition of a PO was the only concomitant procedure demonstrated to improve outcomes. In SS hips, those with a successful outcome were 2.5 times more likely to have had a PO. The addition of capsulorrhaphy had a negative effect on the entire group, reducing odds of success to 0.8. No other factors were significant. The findings from this multicenter retrospective study suggest that PO be added to varus rotational osteotomies in patients with severe hip subluxation. Surgery should be undertaken for severe dysplasia without concern for age. The addition of capsulorrhaphy does not improve rate of success. Level III-retrospective comparative study.

Early Bony Hip Reconstructive Surgery for Hip Subluxation in Children With Severe Cerebral Palsy.

Hip subluxation occurs frequently in children with severe cerebral palsy. This retrospective study examined the effects of age and type of bony surgery on radiographic outcomes of children with severe cerebral palsy who were treated for hip subluxation. The study included nonambulatory children with cerebral palsy undergoing bony hip reconstructive surgery consisting of proximal femoral varus derotational osteotomy (VDRO) alone or combined with pelvic osteotomy. The migration index was recorded for preoperative, postoperative, and final follow-up (minimum 2 years) radiographs. Failure was defined as subsequent bony hip reconstructive surgery or final follow-up migration index greater than 50%. Seventy-seven hips in 40 patients younger than 6 years (55 VDRO alone, 22 combined) and 73 hips in 43 patients older than 6 years (27 VDRO alone, 46 combined) met inclusion criteria. For VDRO alone, the failure rate for patients younger than 6 years (33%) was significantly higher than for patients older than 6 years (7%). For combined procedures, failure rates between younger (0%) and older groups (9%) were not significantly different. Patients younger than 6 years undergoing combined procedures started with a significantly worse migration index than patients undergoing VDRO alone, 72% vs 46%, yet had significantly better final migration indices of 13% vs 31%, respectively. For surgeons performing bony reconstructive surgery to treat hip subluxation in younger children with severe cerebral palsy who are nonambulatory, the findings of this study support the use of combined VDRO and pelvic osteotomy. [Orthopedics. 2021;44(2):e294-e300.].

Locomotor and robotic assistive gait training for children with cerebral palsy.

To determine if robotic assisted gait training (RAGT) using surface muscle electrical stimulation and locomotor training enhances mobility outcomes when compared to locomotor training alone in children with cerebral palsy (CP). Forty children (18 females, 22 males; mean age 8y 1mo, SD 2y 1mo; range 5y 1mo-12y 11mo) with CP in Gross Motor Function Classification System levels (GMFCS) III, IV, and V were randomly assigned to the RAGT and locomotor training (RAGT+LT) group or locomotor training only group (dosage for both: three 1-hour sessions a week for 6weeks). Outcomes were assessed at baseline T1 (week 0), post-treatment T2 (week 6), and retention T3 (week 26). The primary outcome measure was the Goal Attainment Scale. Secondary outcome measures included the 10-metre walk test, children's functional independence measure mobility and self-care domain, the Canadian Occupational Performance Measure, and the Gross Motor Function Measure. There were no significant differences between the groups for both the primary and secondary outcome measures. All participants completed the intervention in their original group allocation. There were no reported adverse events. The addition of RAGT to locomotor training does not significantly improve motor outcomes in children with CP in GMFCS levels III, IV, and V. Future studies could investigate health and well-being outcomes after locomotor training. Marginally ambulant and non-ambulant children with cerebral palsy can participate in locomotor training. Robotic assisted gait training when added to locomotor training does not appear to be any more effective than locomotor training alone.

The use and perception of support walkers for children with disabilities: a United Kingdom survey

BackgroundSupport walkers are a type of assistive device that may enable non-ambulant children with disabilities to walk independently and promote improvements in bowel function, bone mineral density (BMD), mobility, independence, participation and social function. However, there is little evidence to support these benefits and there is a lack of research describing the use of support walkers in clinical practice. This study aimed to examine the use of support walkers for children with disabilities in clinical practice.MethodA survey was distributed via professional organisations, charities and schools associated with paediatric disabilities in the UK. Participants were recruited between January and March 2018. Populations of interest were those who prescribe support walkers to children with disabilities and those who work with children who use them.ResultsIn total, 125 people were included in the analysis; 107 responders prescribed support walkers and 18 responders worked with children who used support walkers. The population of children who use support walkers ranged from 6 months to 18 years and included children with cerebral palsy, chromosomal abnormalities and other medical conditions. Use of these devices was also reported in schools, at home and in the community for varying lengths of time. Numerous perceived benefits were noted, most frequent of which were increases in physical activity and enjoyment. By comparison, fewer perceived problems were identified but centred on lack of space and difficulty with transfers.ConclusionsThis study provides insight into the use of support walkers in the UK, particularly surrounding current practices, which may help to improve consistency in clinical settings. Perceived benefits and problems may provide a basis for identification of appropriate outcome measures to monitor effectiveness. These results should also provide a basis for designing future studies to examine effectiveness of support walkers for paediatric disabilities.

Hospitalizations in School-Aged Children with Cerebral Palsy and Population-Based Controls.

To compare hospitalizations among children with cerebral palsy (CP) and healthy controls and to identify factors associated with hospitalizations in children with CP. This retrospective cohort study linked data from a provincial CP Registry and administrative health databases. The CP cohort was comprised of children born from 1999 to 2002. Age, sex, and region-matched controls were identified from administrative health databases. Mean differences, relative risk (RR), and 95% confidence intervals (CIs) were calculated. A total of 301 children with CP were linked to administrative health data and matched to 6040 controls. Mean hospitalizations per child during the study period were higher in children with CP compared to controls (raw mean difference (RMD) 5.0 95% CI 4.7 to 5.2) with longer length of stay (RMD 2.8 95% CI 1.8 to 3.8) and number of diagnoses per hospitalization (RMD 1.6 95% CI 1.4 to 1.8). Increased risk of hospitalization was observed in non-ambulant children with CP (RR 1.12 95% CI 1.01 to 1.22) compared to ambulant children and among those with spastic tri/quadriplegic CP compared to other CP subtypes (RR 1.15, 95% CI 1.05 to 1.27). Feeding difficulties (RR 1.20 95% CI 1.13 to 1.27), cortical visual (RR 1.22 95% CI 1.13 to 1.32), cognitive (RR 1.16 95% CI 1.04 to 1.30), and communication impairment (RR 1.26 95% CI 1.10 to 1.44) were associated with increased hospitalizations. Children with CP face more frequent, longer hospital stays than peers, especially those with a more severe CP profile. Coordinated interdisciplinary care is needed in school-aged children with CP and medical complexity.

Hip Reconstruction in Nonambulatory Children With Cerebral Palsy: Identifying Risk Factors Associated With Postoperative Complications and Prolonged Length of Stay.

The purpose of this study is to examine the relationship between preoperative comorbidities, surgical complications, and length of stay (LOS) after hip reconstruction in nonambulatory children with cerebral palsy (CP). This single-center retrospective cohort study included 127 patients undergoing hip surgery between 2007 and 2016 who were diagnosed with CP (GMFCS IV/V). The cohort was 54% Gross Motor Function Classification System (GMFCS) V with an average age at surgery of 9 years (range, 3-19 y). Preoperative comorbidities included: presence of a gastrostomy tube, respiratory difficulty requiring positive-pressure ventilation or tracheostomy, history of seizures, and nonverbal status. Complications were dichotomized into major and minor complications according to severity. Multivariable general linear modeling was used to identify factors associated with complications and prolonged LOS. The median LOS in the hospital was 6 days (intequartile range, 5-9 d). The majority of procedures (72%) involved both the femur and acetabulum and 82% of surgeries were performed bilaterally. Patients who experienced a major complication were mostly GMFCS level V and were more likely to spend time in intensive care unit than postanesthetic care unit (P=0.001). Multivariable analysis for a major complication determined that the addition of each comorbid risk fact increased the odds of developing a major complication by 2.6 times (odds ratio, 2.64; 95% confidence interval, 1.56-4.47; P<0.001) regardless of GMFCS level. Multivariable analysis for prolonged LOS determined that major complications (P<0.001), bilaterality (P=0.01), age (P=0.02), female sex (P=0.01), and GMFCS V (P<0.001) were all factors that increased LOS. Migration percentage, acetabular index odds ratio, and pelvic obliquity were not associated with prolonged LOS or the presence of a major complication. From our analysis, the authors found that a patient's premorbid comorbidities were more predictive of the likelihood of sustaining a major complication than their GMFCS level. Identifying high-risk patients preoperatively may help reduce complications and LOS, which ultimately will improve the quality of care the authors deliver to nonambulatory children with CP undergoing hip reconstruction surgery. Level III-retrospective cohort study.

Manual ability and upper limb performance in nonambulatory stage of Duchenne muscular dystrophy

PurposeThe functional use of the upper extremities in daily living activities has become important in the later ages with the increasing life expectancy of patients with Duchenne muscular dystrophy (DMD). This study aimed to assess manual ability and upper limb performance of nonambulatory children with DMD, and to determine their relationship with factors that might affect ability and performance. MethodsManual ability was determined via the ABILHAND-Kids questionnaire and upper limb performance was assessed with the Performance of Upper Limb (PUL) test. Possible related factors such as functional level, steroid usage, upper limb range of motion (ROM), thumb opposition, upper limb muscular strength, and grip strength were evaluated. Correlations of related factors with manual ability and upper limb performance were analyzed. ResultsThe mean age of 23 nonambulatory DMD children was 13.04±1.39 years. Moderate impairments were determined according to the ABILHAND-Kids and PUL, with scores of 26.30±10.74 and 46.22±15.02, respectively. The functional level, steroid usage, duration of wheelchair use, upper extremity ROM, and global upper limb muscle strength of children were weak-to-strongly correlated with at least one score of ABILHAND-Kids and PUL (P<0.05). ConclusionChildren with DMD may already have severe proximal and mid-level upper extremity involvement, even reflected in distal functions, at the time when they progress to the nonambulatory stage. Besides muscular strength, many related factors should be taken into account for therapists to assess and treat upper limb performance at later stages of DMD.

Predictors of postoperative complications after selective dorsal rhizotomy.

Selective dorsal rhizotomy (SDR) reduces spasticity in children with cerebral palsy (CP). We analyzed potential preoperative predictors of complications after SDR via single-level laminectomy at the conus medullaris. One hundred and forty SDRs performed in children (2-17years) with CP were included in this retrospective study (March 2016 to July 2019). Of these children, 69% were ambulatory (Gross Motor Functional Classification System (GMFCS) II and III). Variables associated with wound dehiscence and infections, cerebrospinal fluid (CSF) leaks, and prolonged epidural pain management were analyzed statistically. Five children (3.6%) showed prolonged wound healing, which was associated with obesity (BMI z-score ≥ 1.64; odds ratio (OR) 24.4; 95% confidence interval (CI) 3-199; p= 0.003). Two cases (1.4%) had superficial surgical site infections (SSIs), which was associated with obesity (p= 0.004) and thrombocytopenia (< 180,000G/l; p= 0.028). The area under the curve at ≥ 1.55 BMI z-score for SSI was 0.97 (95% CI 0.93-0.99, p= 0.024), with a sensitivity and specificity for SSI of 100 and 94.9%, respectively. CSF leaks occurred in four (2.9%) children, associated with age ≤ 5years (p= 0.029). Fifteen (10.7%) children required prolonged (4-5days) epidural pain treatment, which was associated with non-ambulatory GMFCS levels (IV and V) (OR 3.6; 95% CI 1.2-10.8; p= 0.008). SDR is safe for all GMFCS levels. Obesity predicts prolonged wound healing and SSI. Prolonged pain management via epidural pain catheter is safe, but care should be taken with non-ambulatory children.

Possibilities of Minimally Invasive Treatment of Hip Subluxation in Children with Cerebral Palsy

Introduction: Hip subluxation is one of the main problems in children with cerebral palsy (CP) after the equines foot. Without treatment, we see a deterioration of around 7% per year. In addition to the delayed verticalization, the main cause is the pathological muscle tone of the muscles surrounding the hip. Children with restricted ability to walk and stand independently are most affected, which is assessed using the classification system for gross motor function (GMFCS). It is a common therapeutic measure to treat muscle imbalance by releasing the shortened soft tissue in early childhood. Here we prefer percutaneous myofasciotomy to lengthen short muscles. The Iliopsoas muscle cannot be lengthened percutaneously. Since muscle lengthening is not sufficient for older children with hips at risk, we use additional guided growth as a minimally invasive method to prevent further hip subluxation. In this regard, two other studies already showed a positive effect. Methods: This was a prospective follow-up study from 2010 to 2016 of 52 children aged 2.8 to 6.7 years (29 boys and 23 girls) who were to be treated surgically with a percutaneous muscle fascia release due to structural muscle shortening. In the preoperative examination, an X-ray image revealed a Reimer migration index (RMI) of over 25%. All children were classified according to their functional possibilities (GMFCS II 3 children, III 14 children, IV 18 children, V 17 children) and the mobility of both legs (ROM) was measured. The second group of 12 children over the age of 6 (6.1 to 12.7 years) (7 boys, 5 girls) underwent femoral head epiphysiodesis in addition to the release of the fascia between 2016 and 2018. The group was also examined according to the same criteria (III 2 children, IV 5 children, V 5 children). We followed these children postoperatively and reevaluated radiological and clinical findings. Results: For 99 observed hips of 52 children with CP GMFCS 3-5 younger than 6 years of age (average 4.2 years), we found an improvement in hip subluxation for 33%, deterioration for 5% and stabilization for 62%. For our patients older than 6 years, we combined the percutaneous myofasciotomy with a guided growth of the femoral head by percutaneous screw. For 16 hips of 12 children we found a mean improvement in the migration index of 5%, and an improvement in the femoral neck shaft angle of 9° within 16 months. Discussion: The minimally invasive release of muscle fascia appears to be as effective as the open release, as it is done with hip screening programs in many countries. The recovery of children who receive minimally invasive treatment is faster, however. Less surgical soft tissue damage, faster mobilization and fewer restrictions than varus osteotomy are strong arguments to consider guided growth as a treatment option for Coxa valga in spastic hip subluxation in non-ambulant children with CP. Conclusion: Further studies have to prove the effectiveness of this method in the long term.

Skeletal Muscle Mitochondrial Capacity Is Similar In Ambulatory And Non-ambulatory Children With Cerebral Palsy

PURPOSE: Cerebral palsy (CP) is the most common childhood movement dysfunction secondary to a brain injury around birth. These children can be classified as ambulatory or non-ambulatory based on their functional abilities. Importantly, they expend significantly increased energy expenditure during movement. Muscle mitochondria, specifically the electron transport system are responsible for oxidative capacity, energy production and are associated with functional capacity. Unfortunately, mitochondrial oxidative capacity in children with CP and its association with different functional levels is not known. We measured maximum mitochondrial respiration rates directly from biopsies in children with CP, compared across ambulatory levels. METHODS: Twenty children (6-16 years, 10 M/6F, Ambulatory-12), undergoing surgery participated in this study. Twenty-nine biopsies were obtained from adductors, vastus lateralis, gastrocnemius. Carbohydrate and fatty acid respirometry substrate-uncoupler-inhibitor titration (SUIT) protocols were performed on permeabilized muscle fiber bundles. In addition, muscle homogenate was used to measure citrate synthase activity as a marker of mitochondrial content. Ambulatory capacity was measured using 6-minute walk tests (n=7), muscle strength, and gait velocity (n=15) during routine therapy visits. RESULTS: Surprisingly, maximal mitochondrial phosphorylation capacity was similar across between functionally ambulatory and non-ambulatory children (77.1± 23.9 vs. 84.9± 24.0 pmol O2/s/mg). This was uncorrelated with mitochondrial content (p>0.1), as measured by citrate synthase. Mitochondria in children with CP still preferentially used carbohydrates over fatty acids based on state-3 respiration. Functionally ambulatory children showed positive associations between mitochondrial function and ambulatory capacity measures (r2 values for gait velocity=0.50, 6-meter walk test=0.33, p< 0.05). CONCLUSIONS: Mitochondrial function are typically associated with activity level. Surprisingly, functionally ambulatory children with CP did not have greater mitochondrial function compared to non-ambulatory children and might even be lower. Importantly, within ambulatory children walking capacity was related to maximal mitochondrial function.

What is the effect of increased standing time in non-ambulant children with cerebral palsy? a feasibility study

Key-Words cerebral palsy, Standing, Hip migration

Femoral shaft fractures in children with non-ambulatory neuromuscular disorders can be effectively treated using flexible intramedullary nails.

PurposeThere is little information in the literature regarding flexible intramedullary nails (FIN) for treating femur fractures in children with neuromuscular disorders. The purpose of this study is to investigate the outcomes of FIN for femoral shaft fractures in non-ambulatory children with neuromuscular disorders.MethodsA retrospective review was conducted on patients with femur fractures at a paediatric hospital between 2004 and 2018. Inclusion criteria were femoral shaft fracture treated with FIN. Outcomes were compared between patients with neuromuscular disorders (NM group) and a control group of those without neuromuscular disorders.ResultsA total of 37 patients with 37 femoral shaft fractures were studied (12 patients in the NM group and 25 in the control group). All NM group patients were non-ambulatory at baseline. Fractures were length stable in all 25 patients in the control group and in 2/12 (17%) patients in the NM group. All fractures healed in both groups. Three complications (all nail migrations) requiring reoperation before fracture union occurred in the NM group, yielding a major complication rate of 25% (3/12) in the NM group versus 0% (0/25) in controls (p = 0.03). Angular deformity occurred in 5/12 (42%) NM group patients and 1/25 (4%) control group patient (p = 0.009); none required reoperation.ConclusionFemur fractures in non-ambulatory children with neuromuscular disorders can be successfully treated with FIN. Angular deformities are common in this population, but had no functional impact in the non-ambulatory NM group patients. Surgeons must also be vigilant for implant prominence and skin breakdown in these patients.Level of evidenceIII