Publications by authors named "Freeman Miller"

157 Publications

Comparison of Surgical Outcomes for Distal Rectus Femoris Transfer and Resection Surgeries in Children With Cerebral Palsy With Stiff Knee Gait.

J Pediatr Orthop 2021 Jul 19. Epub 2021 Jul 19.

Gait Analysis Laboratory, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Children with cerebral palsy (CP) often present with a stiff knee gait pattern because of rectus femoris (RF) spasticity and/or contracture. Rectus femoris transfers (RFTs) and resections are surgical procedures aimed at reducing muscle stiffness, thereby improving knee flexion during the swing phase of gait. Previous research has consistently demonstrated objective benefits of rectus transfer using instrumented gait analysis (IGA). Rectus femoris resection (RFR), a relatively simpler procedure, shows similar improvement in knee range of motion during gait. The objective of this study was to compare surgical outcomes between rectus transfers and resections using 3-dimensional IGA.

Methods: Children with spastic CP who had RFTs or resections were retrospectively matched by walking speed and preoperative knee kinematics from 3-dimensional IGA (peak and timing of peak knee flexion in swing). Secondary outcomes included knee range of motion and maximum knee extension during gait.

Results: Twenty-eight children were included in both the transfer group [age 9.4±2 y; Gross Motor Function Classification System (GMFCS) I (3 children), II (15 children), III (8 children), and IV (2 children)] and the resection group [age 10.6±2.5 y; GMFCS I (1 child), II (14 children), and III (13 children)]. Both surgical groups showed statistically significant short-term postsurgical improvements in peak knee flexion during swing (P<0.001 for the transfer group and P=0.003 for the resection group) and Duncan-Ely test (P=0.004 for the transfer group and P<0.001 for the resection group). Further analysis by GMFCS level showed children at GMFCS levels III/IV had a greater tendency to crouch after RFT when compared with children at GMFCS levels I/II. This tendency was not observed in the RFR group.

Conclusions: Both transfer and resection surgeries significantly improved gait kinematics short-term outcomes in children with spastic CP who present with stiff knee gait pattern. Further studies are required to compare long-term outcomes of both surgeries.

Level Of Evidence: Level III-retrospective matched-cohort study.
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http://dx.doi.org/10.1097/BPO.0000000000001886DOI Listing
July 2021

Single-event multilevel surgery in cerebral palsy: Value added by a co-surgeon.

Medicine (Baltimore) 2021 Jun;100(24):e26294

National Academy of Medicine Fellowship, American Osteopathic Association, Chicago, IL, USA.

Abstract: The aim of this study was to compare outcomes for single-event multilevel surgery (SEMLS) in cerebral palsy (CP) performed by 1 or 2 attending surgeons.A retrospective review of patients with CP undergoing SEMLS was performed. Patients undergoing SEMLS performed by a single senior surgeon were compared with patients undergoing SEMLS by the same senior surgeon and a consistent second attending surgeon. Due to heterogeneity of the type and quantity of SEMLS procedures included in this study, a scoring system was utilized to stratify patients to low and high surgical burden. The SEMLS events scoring less than 18 points were categorized as low burden surgery and SEMLS scoring 18 or more points were categorized as high burden surgery. Operative time, estimated blood loss, hospital length of stay, and operating room (OR) utilization costs were compared.In low burden SEMLS, 10 patients had SEMLS performed by a single surgeon and 8 patients had SEMLS performed by 2 surgeons. In high burden SEMLS, 10 patients had SEMLS performed by a single surgeon and 12 patients had SEMLS performed by 2 surgeons. For high burden SEMLS, operative time was decreased by a mean of 69 minutes in cases performed by 2 co-surgeons (P = 0.03). Decreased operative time was associated with an estimated savings of $2484 per SEMLS case. In low burden SEMLS, a trend toward decreased operative time was associated for cases performed by 2 co-surgeons (182 vs 221 minutes, P = 0.11). Decreased operative time was associated with an estimated savings of $1404 per low burden SEMLS case. No difference was found for estimated blood loss or hospital length of stay between groups in high and low burden SEMLS.Employing 2 attending surgeons in SEMLS decreased operative time and OR utilization cost, particularly in patients with a high surgical burden. These findings support the practice of utilizing 2 attending surgeons for SEMLS in patients with CP.Level of Evidence: Level III.
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http://dx.doi.org/10.1097/MD.0000000000026294DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8213317PMC
June 2021

The influence of preoperative knee flexion contracture severity on short-term outcome of orthopedic surgery in ambulatory children with bilateral cerebral palsy.

BMC Musculoskelet Disord 2021 May 25;22(1):481. Epub 2021 May 25.

Department of Orthopedics, Örebro University Hospital, Södra Grev Rosengatan, Örebro, Sweden.

Background: Indications and cutoff value of deformities to determine surgical procedures for flexed knee gait are not clear. The aim was to determine the influence of none or mild, and moderate preoperative knee flexion contracture on the improvement of gait after orthopedic surgery in children with bilateral cerebral palsy (CP).

Methods: Inclusion criteria; bilateral CP, Gross Motor Function Classification System level I-III, and pre- and post operative-gait analysis. The 132 individuals identified were categorized into 2 groups based on the severity of knee flexion contracture (group 1: none or less than 11°; group 2: greater than or equal to 11°), and then matched according to the exact same soft tissue and/or bony orthopedic surgical procedures performed. The indication for surgery was to prevent progressive development of knee flexion contracture and stance phase flexed knee gait. Pre- and postoperative physical examination and gait analysis data were analyzed retrospectively.

Results: Sixty (30 + 30) children, with mean age 10.6 years in each group, were included. The average follow-up time was 17 months. Gait Deviation Index (GDI) improved in group 1 from mean 66 (SD 19) to 74 (15), p = 0.004, and in group 2 from 60 (13) to 69 (15), p = 0.001. Knee flexion in stance improved in group 1 from 21.4 (16.1) to 12.1 (16.0) degrees, p = 0.002, and in group 2 from 32.2 (14.2) to 17.0 (15.9), p = 0.001. Step length improved in both groups, p = 0.017 and p = 0.008, respectively. Only in group 2 significant improvement was noted in walking speed, p = 0.018 and standing function, Gross Motor Function Measure (GMFM-D), p = 0.001. Knee flexion contracture decreased in group 1 from mean 4.6 (5.3) to 2.1 (8.3) degrees, p = 0.071 and in group 2 from 17.2 (4.9) to 9.6 (9.3), p = 0.001. There was no statistical difference between groups in pre-post improvement of GDI or other variables, except GMFM-D.

Conclusions: Relative mild to moderate preoperative knee flexion contracture does not influence the short-term improvement of gait after orthopedic surgery in children with bilateral CP.
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http://dx.doi.org/10.1186/s12891-021-04362-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8152141PMC
May 2021

The Impact of Spinal Fusion on Hip Displacement in Cerebral Palsy.

Indian J Orthop 2021 Feb 9;55(1):176-182. Epub 2020 Oct 9.

Department of Orthopedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE USA.

Background And Study Aims: The aims of this study were to determine the risk of progressive hip subluxation in children with CP after spinal fusion for scoliosis and how frequent the hips follow-up should be scheduled.

Patients And Methods: Pelvis radiography [migration index (MI) and pelvic obliquity (PO)] of Gross Motor Function Classification System (GMFCS) levels IV and V children with CP who received spinal fusion and pelvic fixation were reviewed retrospectively. This population was categorized into three groups based on the MI at spinal fusion: G1 = 0-29%; G2 = 30-59%; and G3 = 60-100%.

Results: Fifty children (age 7.5-15.0 years) and categorized into 3 groups (G1 = 19, G2 = 23, G3 = 8; 100 hips in total). Preoperative and last follow-up MI were 22 ± 7% and 30 ± 20% (G1), 41 ± 9% and 43 ± 22% (G2), 92 ± 15% and 97 ± 10% (G3). The MIs at spinal fusion between groups were statistically different ( < 0.001). In G1, the mean MI progression was 5% and 25% at 12 months and 62 months, respectively. In G2, the mean MI progression was 9% and 25% at 12 months and 32 months, respectively. The progression more than 10% occurred within 2 years in G1 and within 1 year in G2. There was no difference between groups based on preoperative degree of PO ( = 0.653) and correction rate in PO ( = 0.421).

Conclusions: In GMFCS IV and V children with the highest risk for progression occurred with increasing preoperative MI, especially over 50%. Hips should be monitored continuously after spinal fusion until hip stability is documented.
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http://dx.doi.org/10.1007/s43465-020-00271-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7851297PMC
February 2021

The construct and concurrent validity of brief standing sway assessments in children with and without cerebral palsy.

Gait Posture 2021 02 7;84:293-299. Epub 2021 Jan 7.

Department of Kinesiology and Applied Physiology, University of Delaware, Newark, DE, USA. Electronic address:

Background: Standing postural sway is often quantified from center of pressure trajectories. During assessments of longer durations, children may fidget, thus limiting the feasibility and validity of sway recordings.

Research Question: Do postural sway sample durations less than 30 s maintain construct and concurrent validity?

Methods: In this case-control, observational study, we measured postural sway in 41 children (age 5-12 years, 23 typically developing (TD); 18 with spastic cerebral palsy (CP), 13 diplegic and 5 hemiplegic, 11 GMFCS level I and 7 level II) for 30-second eyes-opened and eyes-closed conditions. From a single recording, 5-second incremental durations of 5-30 s were considered in this analysis. We quantified anteroposterior, mediolateral, and transverse-plane sway using seven time-domain variables: root-mean-square error, total excursion, mean frequency, mean distance, sway area, and 95 % confidence circle and ellipse areas. Variables were calculated in eyes-opened and eyes-closed conditions, as well as the ratio of the two. Construct validity was evaluated by the persistence of large effect sizes (Glass's Δ ≥ 0.80) between CP and TD participants at shorter durations than 30 s. Concurrent validity was evaluated by the correlations of shorter duration measures to the 30 s measure.

Results: Seven sway measures had large between-group effects (Glass's Δ ≥ 1.02) for the 30 s measure that persisted (Glass's Δ ≥ 0.81) at shorter durations (5-25 s) and also maintained concurrent validity (r ≥ 0.83). Six of these seven measures were taken in the eyes-closed condition, and all seven measures were in the mediolateral direction or transverse plane.

Significance: Our analysis suggests that sway durations less than 30 s can uphold construct and concurrent validity. These measures were primarily in the eyes-closed conditions and mediolateral direction. These results are a promising indicator that shorter-duration sway measures may be of utility when fidgeting prevents longer recordings.
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http://dx.doi.org/10.1016/j.gaitpost.2020.12.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7902400PMC
February 2021

Orthopaedic manifestations of glutaric acidemia Type 1.

J Child Orthop 2020 Oct;14(5):473-479

Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.

Purpose: Glutaric acidemia type 1 (GA1), a rare hereditary metabolic disease caused by biallelic mutations of can result in acute or insidious striatal degeneration within the first few years of life. We reviewed the orthopaedic sequelae and management of 114 neurologically injured patients with a confirmed molecular diagnosis of GA1.

Methods: We performed a retrospective chart review spanning 28 years identifying 114 GA1 patients, most from the Old Order Amish population of Lancaster County, Pennsylvania, who were homozygous for a pathogenic founder variant of (c.1262C>T). We collected demographics, medical comorbidities, muscle tone patterns, Gross Motor Function Classification System level, gastrostomy tube status, seizure history, inpatient events, orthopaedic diagnoses and operative characteristics.

Results: Over an average follow-up of 4.7 ± 3.4 years, 24 (21%) of 114 patients had musculoskeletal problems requiring orthopaedic consultation. Scoliosis (n = 14), hip dislocation (n = 8/15 hips), hip subluxation (n = 2/three hips), and windswept hip deformity (n = 2) in the spine and hip joint were most common. In total, 35 orthopaedic surgeries were performed in 17 (71%) patients. The most common primary operations were one-stage procedures with proximal femoral varus derotation osteotomy and/or pelvic osteotomy (n = 8/14 hips) for subluxation or dislocation. In all, 11 patients had posterior spinal fusion for severe scoliosis. With the recommended metabolic management, there were no disease-specific complications in this cohort.

Conclusions: Children with GA1 who have static striatal lesions are at risk for musculoskeletal complications, especially scoliosis and hip dislocation, and appropriate operative management requires consultation with a metabolic specialist with specific considerations for fluid management and nutrition.

Level Of Evidence: IV.
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http://dx.doi.org/10.1302/1863-2548.14.200059DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7666789PMC
October 2020

Glutaric acidemia type 1: Treatment and outcome of 168 patients over three decades.

Mol Genet Metab 2020 11 4;131(3):325-340. Epub 2020 Oct 4.

Clinic for Special Children, Strasburg, PA, USA; Department of Pediatrics, Penn Medicine-Lancaster General Hospital, Lancaster, PA, USA; Central Pennsylvania Clinic, Belleville, PA, USA.

Glutaric acidemia type 1 (GA1) is a disorder of cerebral organic acid metabolism resulting from biallelic mutations of GCDH. Without treatment, GA1 causes striatal degeneration in >80% of affected children before two years of age. We analyzed clinical, biochemical, and developmental outcomes for 168 genotypically diverse GA1 patients managed at a single center over 31 years, here separated into three treatment cohorts: children in Cohort I (n = 60; DOB 2006-2019) were identified by newborn screening (NBS) and treated prospectively using a standardized protocol that included a lysine-free, arginine-enriched metabolic formula, enteral l-carnitine (100 mg/kg•day), and emergency intravenous (IV) infusions of dextrose, saline, and l-carnitine during illnesses; children in Cohort II (n = 57; DOB 1989-2018) were identified by NBS and treated with natural protein restriction (1.0-1.3 g/kg•day) and emergency IV infusions; children in Cohort III (n = 51; DOB 1973-2016) did not receive NBS or special diet. The incidence of striatal degeneration in Cohorts I, II, and III was 7%, 47%, and 90%, respectively (p < .0001). No neurologic injuries occurred after 19 months of age. Among uninjured children followed prospectively from birth (Cohort I), measures of growth, nutritional sufficiency, motor development, and cognitive function were normal. Adherence to metabolic formula and l-carnitine supplementation in Cohort I declined to 12% and 32%, respectively, by age 7 years. Cessation of strict dietary therapy altered plasma amino acid and carnitine concentrations but resulted in no serious adverse outcomes. In conclusion, neonatal diagnosis of GA1 coupled to management with lysine-free, arginine-enriched metabolic formula and emergency IV infusions during the first two years of life is safe and effective, preventing more than 90% of striatal injuries while supporting normal growth and psychomotor development. The need for dietary interventions and emergency IV therapies beyond early childhood is uncertain.
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http://dx.doi.org/10.1016/j.ymgme.2020.09.007DOI Listing
November 2020

Evaluation of Risk Factors for Cerebrospinal Leakage in Pediatric Patients With Cerebral Palsy Treated With Intrathecal Baclofen.

J Pediatr Orthop 2020 Jul;40(6):e522-e526

Department of Orthopaedic Surgery, Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Insertion of an intrathecal baclofen (ITB) pump can provide significant benefits in patients with cerebral palsy (CP). However, there are little data describing the risk of complications. Specifically, there is a lack of data describing the incidence of cerebrospinal fluid (CSF) leakage and risk factors following ITB placement. The purpose of our study was to describe risk factors for developing CSF leak in pediatric patients with CP treated with ITB and to report the treatment and outcome of CSF leaks.

Methods: Following institutional review board approval, 720 ITB procedures in 341 children with CP were identified retrospectively over a 15-year study period. Patients' demographic characteristics, medical comorbidities, muscle tone patterns, feeding tube status, seizure history, inpatient events, ITB-related CSF leak and headache complaints and their management, and other complications were evaluated.

Results: Eighty-five (24.9%) patients experienced 90 CSF leak episodes over a follow-up time of 6.3±3.9 years. There were 72 episodes of headache as a result of CSF leakage in 61 (71.7%) of these 85 patients. There was a positive correlation between the risk of CSF leak and preoperative comorbidities such as epilepsy/seizure history, feeding tube, mixed type CP, and dystonic type CP. The risk of CSF leak after primary ITB administration was 5.8% (20/341), and the risk after secondary ITB procedures due to complications was 24.2% (32/132). There was no significant relationship between CSF leak and primary ITB (P=0.21), but the risk of CSF leak was positively correlated to the secondary ITB due to complications (P<0.05).

Conclusions: CSF leak was fairly common (25% incidence), and it correlated with epilepsy/seizure history, feeding tube, mixed type CP, and dystonic type CP. Recurrent ITB procedures were a risk factor for CSF leak. Half of these patients had self-limited symptoms that improved with conservative medical treatment, and the epidural blood patch was successful in resistant cases. Successful treatment of CSF leakage complications allows patients to continue ITB.

Level Of Evidence: Level III.
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http://dx.doi.org/10.1097/BPO.0000000000001472DOI Listing
July 2020

What's New in the Orthopaedic Treatment of Ambulatory Children With Cerebral Palsy Using Gait Analysis.

J Pediatr Orthop 2020 Jul;40(6):e498-e503

Department of Orthopaedics.

Background: Limb deformities in ambulatory children with cerebral palsy (CP) are common. The natural history of lower extremity deformities is variable and the impact on gait is managed with many treatment modalities. Effective interventions must consider the underlying pathophysiology, patient-specific goals, and incorporate objective outcome assessment. Evaluation and treatment include observation, tone management multilevel orthopaedic surgery to address muscle contractures and bony deformities, and the use of gait analysis for preoperative and postoperative assessment.

Methods: A PubMed search of the orthopaedic literature for studies published between January 2016 and February 2019 was performed. Eligible abstracts included the use of 3-dimensional instrumented gait analysis in the evaluation and treatment of the lower extremities in ambulatory children with CP. Seven hundred twenty abstracts were reviewed, with 84 papers identified as eligible, of which 45 full manuscripts were included for detailed review.

Results: The review summarized recent advances regarding the treatment of torsional alignment, knee deformities and clinical gait evaluation with visual assessment tools compared with instrumented gait analysis.

Conclusions: Gait analysis of ambulatory children with CP remains essential to evaluation and surgical decision-making. Promising results have been reported with the goal of maintaining or reaching a higher level of function and increased endurance.

Level Of Evidence: Level IV-literature review.
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http://dx.doi.org/10.1097/BPO.0000000000001461DOI Listing
July 2020

Management of Hip Disorders in Patients with Cerebral Palsy.

JBJS Rev 2020 03;8(3):e0148

Motion Analysis Laboratory, Alfred I. DuPont Institute, Wilmington, Delaware.

Hip disorders are the second most common musculoskeletal abnormality seen in children with cerebral palsy, affecting nearly 1 in 3. The goal of surgical intervention is to provide a mobile, located, and painless hip. Reconstructive procedures are indicated for children with migration percentages of >40%. Reconstructive procedures typically result in a long-term satisfactory outcome, although recurrence is seen particularly in nonambulatory children and when the reconstruction is performed earlier than 6 years of age. Salvage procedures are performed when reconstructive procedures are no longer an option and degenerative changes have occurred. They include proximal femoral valgus osteotomy, proximal femoral resection, and hip arthroplasty.
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http://dx.doi.org/10.2106/JBJS.RVW.19.00148DOI Listing
March 2020

Brain Stiffness Relates to Dynamic Balance Reactions in Children With Cerebral Palsy.

J Child Neurol 2020 06 23;35(7):463-471. Epub 2020 Mar 23.

Department of Biomedical Engineering, University of Delaware, Newark, DE, USA.

Cerebral palsy is a neurodevelopmental movement disorder that affects coordination and balance. Therapeutic treatments for balance deficiencies in this population primarily focus on the musculoskeletal system, whereas the neural basis of balance impairment is often overlooked. Magnetic resonance elastography (MRE) is an emerging technique that has the ability to sensitively assess microstructural brain health through measurements of neural tissue stiffness. Using magnetic resonance elastography, we have previously measured significantly softer grey matter in children with cerebral palsy as compared with typically developing children. To further allow magnetic resonance elastography to be a clinically useful tool in rehabilitation, we aim to understand how brain stiffness in children with cerebral palsy is related to dynamic balance reaction performance as measured through anterior and posterior single-stepping thresholds, defined as the standing perturbation magnitudes that elicit anterior or posterior recovery steps. We found that global brain stiffness is significantly correlated with posterior stepping thresholds ( = .024) such that higher brain stiffness was related to better balance recovery. We further identified specific regions of the brain where stiffness was correlated with stepping thresholds, including the precentral and postcentral gyri, the precuneus and cuneus, and the superior temporal gyrus. Identifying brain regions affected in cerebral palsy and related to balance impairment can help inform rehabilitation strategies targeting neuroplasticity to improve motor function.
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http://dx.doi.org/10.1177/0883073820909274DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7550076PMC
June 2020

Quantifying bone marrow fat using standard T1-weighted magnetic resonance images in children with typical development and in children with cerebral palsy.

Sci Rep 2020 03 9;10(1):4284. Epub 2020 Mar 9.

Department of Kinesiology, University of Georgia, Athens, GA, USA.

Excess bone marrow adiposity may have a negative effect on bone growth and development. The aim of this study was to determine whether a procedure using standard T1-weighted magnetic resonance images provides an accurate estimate of bone marrow fat in children with typical development and in children with mild spastic cerebral palsy (CP; n = 15/group; 4-11 y). Magnetic resonance imaging was used to acquire T1-weighted images. It was also used to acquire fat and water images using an iterative decomposition of water and fat with echo asymmetry and least-squares estimation (IDEAL) technique. Bone marrow fat volume and fat fraction in the middle-third of the tibia were determined using the standard T1-weighted images (BMFV and BMFF, respectively) and the fat and water images (BMFV and BMFF, respectively). In both groups, BMFV was highly correlated with (both r > 0.99, p < 0.001) and not different from (both p > 0.05) BMFV. In both groups, BMFF was moderately correlated with (both r = 0.71, p < 0.01) and not different from (both p > 0.05) BMFF. There was no group difference in BMFV or BMFV (both p > 0.05). BMFF was higher in children with CP (p < 0.05), but there was no group difference in BMFF (p > 0.05). We conclude that a procedure using standard T1-weighted magnetic resonance images can produce estimates of bone marrow fat volume similar to estimates from the IDEAL technique in children. However, it is less sensitive to variation in the bone marrow fat fraction.
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http://dx.doi.org/10.1038/s41598-019-57030-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7062906PMC
March 2020

Anteroposterior balance reactions in children with spastic cerebral palsy.

Dev Med Child Neurol 2020 06 2;62(6):700-708. Epub 2020 Mar 2.

Department of Kinesiology, University of Georgia, Athens, GA, USA.

Aim: To compare anterior and posterior standing balance reactions, as measured by single-stepping thresholds, in children with and without spastic cerebral palsy (CP).

Method: Seventeen ambulatory children with spastic CP (eight males, nine females) and 28 typically developing children (13 males, 15 females; age range 5-12y, mean [SD] 9y 2mo [2y 3mo]), were included in this cross-sectional, observational study. Balance reaction skill was quantified as anterior and posterior single-stepping thresholds, or the treadmill-induced perturbations that consistently elicited a step in that direction. In order to understand the underlying mechanisms of between-group differences in stepping thresholds, dynamic stability was quantified using the minimum margin of stability. Ankle muscle activation latency, magnitude, and co-contraction were assessed with surface electromyography.

Results: We observed an age and group interaction for anterior thresholds (p=0.001, partial η =0.24). At older (≈11y; p<0.001, partial η =0.48), but not younger (≈7y; p=0.33, partial η =0.02) ages, typically developing children had larger anterior thresholds than those with CP. In response to near-threshold anterior perturbations, older typically developing children recovered from more instability than their peers with CP (p=0.004, partial η =0.18). Older children had no between-group differences in ankle muscle activity. No between-group differences were observed in posterior thresholds.

Interpretation: The effects of CP on balance reactions are age- and direction-specific. Older typically developing children are more able or willing to withhold a step when unstable.

What This Paper Adds: Children with spastic cerebral palsy have age- and direction-specific balance-reaction impairments. Lower anterior stepping thresholds were observed in older, but not younger children. Older typically developing children withheld a forward step at higher levels of instability. No between-group differences were seen in posterior stepping thresholds.
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http://dx.doi.org/10.1111/dmcn.14500DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7916524PMC
June 2020

Preferential deficit of fat-free soft tissue in the appendicular region of children with cerebral palsy and proposed statistical models to capture the deficit.

Clin Nutr 2020 05 2;39(5):1541-1550. Epub 2019 Jul 2.

Department of Kinesiology, University of Georgia, Athens, GA, USA. Electronic address:

Background: Cerebral palsy (CP) is a neurological disorder characterized by a profound skeletal muscle deficit. However, whether there is a regional-specific skeletal muscle deficit in children with CP is unknown. The purpose of this study was to determine whether fat-free soft tissue mass (FFST), a commonly used surrogate for skeletal muscle mass, is more compromised in the limbs than in the trunk in children with CP. A second purpose was to determine whether physical characteristics can be used to accurately estimate appendicular FFST (AFFST) in children with CP.

Methods: Forty-two children with CP (4-13 y) and 42 typically developing children matched to children with CP for sex, age and race were studied. Whole body FFST (FFST), FFST in the upper limbs (FFST), FFST in the lower limbs (FFST), the ratio of AFFST to height (AFFST/ht), the ratio of AFFST to height (AFFST/ht) and non-appendicular FFST were estimated from dual-energy X-ray absorptiometry. Statistical models were developed to estimate AFFST, AFFST/ht and AFFST/ht in both groups of children, and the leave-one-out method was used to validate the models.

Results: Children with CP had 21% lower FFST, 30% lower AFFST, 34% lower FFST, 14% lower non-appendicular FFST, 23% lower AFFST/ht, 19% lower AFFST/ht and 9% lower AFFST/FFST (all p < 0.05). Statistical models developed using data from typically developing children overestimated AFFST, AFFST/ht and AFFST/ht by 35%, 30% and 21% (all p < 0.05), respectively, in children with CP. Separate models developed using data from children with CP yielded better accuracy, with the estimated results highly correlated (r = 0.78, 0.66 and 0.50, respectively; all p < 0.001) and not different from calculated AFFST, AFFST/ht and AFFST/ht (all p > 0.99). However, when the difference in estimated values and measured values of AFFST, AFFST/ht and AFFST/ht were plotted against measured values, there was an inverse relationship (r = -0.38, -0.47 and -0.61, respectively, all p < 0.05).

Conclusion: Children with CP have a remarkable deficit in FFST that is more pronounced in the appendicular than in the non-appendicular region and more pronounced in the lower than in the upper limbs. Preliminary models developed using data from children with CP can provide reasonable estimates of AFFST and indexes of AFFST relative to height, but further development of the models may be needed.
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http://dx.doi.org/10.1016/j.clnu.2019.06.020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8249227PMC
May 2020

The effectiveness of epidural blood patch in patients with cerebral palsy treated with intrathecal baclofen implantation.

Paediatr Anaesth 2020 02 14;30(2):153-160. Epub 2020 Jan 14.

Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

Background: Cerebrospinal fluid leak and postdural puncture spinal headache following intrathecal baclofen therapy are known complications. Although primary treatments are conservative, epidural blood patch is an alternative in patients with persistent and severe symptoms.

Aim: The purpose of this article is to review the effectiveness of epidural blood patch for the treatment of spinal headache and cerebrospinal fluid leak associated with intrathecal baclofen treatment in children with cerebral palsy.

Methods: Our database was reviewed for epidural blood patch in 341 pediatric patients with cerebral palsy who underwent primary intrathecal baclofen treatment from 2004 to 2018 at one institution. The number of patches, time frame of treatment, and effectiveness of the epidural blood patch were collected. All patients treated with epidural blood patch were evaluated for primary and secondary intrathecal baclofen-related procedures, and subsequent treatment of intrathecal baclofen associated with cerebrospinal fluid leak and spinal headache.

Results: Twenty-nine epidural blood patch procedures were performed on 26 patients who had received intrathecal baclofen procedures. Of these 26 patients, four had a secondary epidural blood patch. The incidence of spinal headache/cerebrospinal fluid leak was 31% (107/341), and 81/107 (76%) patients with spinal headache/cerebrospinal fluid leak responded to conservative treatments. Success rate for initial epidural blood patch was 79.3% (23/29). The second epidural blood patch was performed in four patients after failure of initial epidural blood patch. Second epidural blood patch success rate was 75% (3/4).

Conclusion: Spinal headache and cerebrospinal fluid leak are known complications after intrathecal baclofen treatment in children with cerebral palsy. When conservative treatments are unsuccessful, epidural blood patch can be used with confidence for these patients. In patients with ongoing symptoms, it is possible to obtain success by repeating the epidural blood patch to continue intrathecal baclofen treatment and avoid aggressive surgery.
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http://dx.doi.org/10.1111/pan.13791DOI Listing
February 2020

Long-term effects of selective dorsal rhizotomy in children with cerebral palsy: a systematic review.

Dev Med Child Neurol 2020 05 24;62(5):554-562. Epub 2019 Jul 24.

Al duPont Hospital for Children, Wilmington, DE, USA.

Aim: To evaluate the long-term effects of selective dorsal rhizotomy (SDR) 10 years or more after the procedure and complications observed any time after SDR in children with cerebral palsy (CP).

Method: Embase, PubMed, and the Cochrane Library were searched from their individual dates of inception through 1st June 2018 for full-text original articles in English that described long-term follow-up after SDR in children with CP. The authors independently screened publications to determine whether they met inclusion criteria; thereafter all authors extracted data on patient characteristics, the proportion of the original cohort being followed-up, and the reported outcomes.

Results: Of the 199 studies identified, 16 were included in this evaluation: 14 were case series and two studies reported a retrospectively assigned comparison group. Evidence concerning function was limited by study design differences, clinical variability, loss to follow-up, and heterogeneity across trials.

Interpretation: At 10 years or more follow-up, available studies generate low-level evidence with considerable bias. No functional improvement of SDR over routine therapy is documented. Furthermore, the long-term effects of SDR with respect to spasticity reduction is unclear, with many studies reporting a high amount of add-on spasticity treatment. More long-term follow-up using robust scientific protocols is required before it can be decided whether the use of SDR as routine therapy for children with CP is to be recommended or not.

What This Paper Adds: Ten years after selective dorsal rhizotomy, available studies supply inconclusive evidence on functional outcomes. The long-term effect on spasticity is uncertain, studies reported a substantial need for add-on treatment. Short- and long-term complications seem frequent but are not reported in a consistent manner.
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http://dx.doi.org/10.1111/dmcn.14320DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7187377PMC
May 2020

The cross-sectional relationships between age, standing static balance, and standing dynamic balance reactions in typically developing children.

Gait Posture 2019 09 3;73:20-25. Epub 2019 Jul 3.

Department of Kinesiology and Applied Physiology, University of Delaware, Newark, DE, United States. Electronic address:

Background: Static balance performance is a common metric for evaluating the development of postural control in children. Less is known about the potentially independent development of dynamic balance performance.

Research Question: How does age relate to static (i.e. postural sway) and dynamic (i.e. stepping thresholds) standing balance performance, and what is the relationship between postural sway and stepping thresholds?

Methods: Twenty-six typically developing children (12 males, 14 females; 5-12 years of age) were recruited for this cross-sectional study. Static balance performance was quantified as the total path length during a postural sway assessment using a force platform with conditions of eyes open and eyes closed. Dynamic balance performance was quantified using a single-stepping threshold assessment, whereby participants attempted to prevent a step in response to treadmill-induced perturbations in the anterior and posterior directions. Relationships between age and body-size scaled measures of static and dynamic balance performance were assessed using Spearman rank correlations.

Results: There was a weak correlation between age and postural sway (|r| < 0.10, p >  0.68), but a moderate-to-strong correlation between age and single-stepping thresholds (r > 0.68, p < 0.001). A weak correlation was found between postural sway and single-stepping thresholds (|r| < 0.20, p >  0.39).

Significance: Dynamic, but not static standing balance performance, may improve with typical development between the ages of 5 and 12 years. Static and dynamic balance should be considered as unique constructs when assessed in children.
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http://dx.doi.org/10.1016/j.gaitpost.2019.07.128DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6707867PMC
September 2019

Perioperative management of patients with cerebral palsy undergoing scoliosis surgery: Survey of surgeon practices.

J Pediatr Rehabil Med 2019 ;12(2):205-212

Department of Orthopedics, Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

Purpose: Progressive scoliosis significantly impacts the quality of life in patients with cerebral palsy (CP). Spinal fusion is the mainstay of treatment of progressive spinal curves. The current study aims to identify approaches used by pediatric spine surgeons to optimize care of patients with CP undergoing scoliosis surgery.

Methods: A 33-question survey was distributed electronically to 181 POSNA/SRS members with an established interest in pediatric spinal deformity surgery. Eighty one responses were obtained (45%). Using the Delphi consensus guidelines, agreement > 75% was considered as consensus.

Results: There was a consensus on 15 out of 33 questions (46%). 97% of responders identified nutrition status as a comorbidity which could be optimized. However, the timing and method of obtaining nutritional assessment varied. 92% of the surgeons stated that they used shared decision making with the family but only 22% used a formal decision aid. 83% use antifibrinolytics routinely, 81% used a surgical site infection prevention protocol, 78% obtained preoperative pulmonary consult, and 88% took steps postoperatively to prevent pulmonary complications.

Conclusion: There is significant variability in the current practices of perioperative management of patients with CP undergoing scoliosis surgery. This data can be used in future studies to create a standardized integrated care pathway.
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http://dx.doi.org/10.3233/PRM-170504DOI Listing
May 2020

Dynamic stability during walking in children with and without cerebral palsy.

Gait Posture 2019 07 11;72:182-187. Epub 2019 Jun 11.

540 S College Ave, Department of Kinesiology and Applied Physiology, University of Delaware, Newark, DE, USA. Electronic address:

Background: Cerebral palsy (CP) is associated with a high risk of falling during walking. Many gait abnormalities associated with CP likely alter foot placement and center of mass (CoM) movement in a way that affects anterior or lateral dynamic stability, in turn influencing fall risk.

Research Question: Do children with CP demonstrate altered anterior or lateral dynamic stability compared to typically-developing (TD) children?

Methods: In this case-control, observational study, we measured gait kinematics of two groups of children (15 CP, 11 GMFCS level I, 4 GMFCS level II; 14 TD; age 5-12) in walking conditions of a preferred speed, a fast speed, and a preferred speed while completing a cognitive task. For dominant and non-dominant limbs, the margin of stability (MoS), a spatial measure of dynamic stability, was calculated as the distance between the edge of the base of support and the CoM position after accounting for scaled velocity. Statistical comparisons of were made using mixed factorial ANOVAs. Post hoc comparisons were Sidak adjusted.

Results: The anterior MoS before foot strike and at mid-swing differed between each condition but not between groups. Based on the minimum lateral MoS, children with CP had more stability when bearing weight on their non-dominant limb compared to TD children. These differences were not apparent when on the dominant limb.

Significance: This high-functioning group of children with CP exhibited a more conservative lateral stability strategy during walking when bearing weight with the non-dominant limb. This strategy may be protective against lateral falls. We observed no between-group differences in anterior stability. Because CP has been previously associated with impaired anterior balance reactions, and there was no observed compensation in anterior gait stability, this lack of group differences could contribute to a higher risk of falling in that direction.
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http://dx.doi.org/10.1016/j.gaitpost.2019.06.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6684400PMC
July 2019

Valgus Hip Osteotomy in Children With Spondyloepiphyseal Dysplasia Congenita: Midterm Results.

J Pediatr Orthop 2019 Jul;39(6):282-288

Department of Orthopedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Coxa vara has been frequently reported in spondyloepiphyseal dysplasia congenita (SEDC), and proximal femoral osteotomy has been described as a useful treatment. The aim of this study was to discuss the clinical, radiographic, and gait outcomes after valgus extension osteotomy of the proximal femur. Changes of lumbar lordosis, associated with coxa vara correction, are reported as well as the outcome differences between different ages.

Methods: Records of children with SEDC, who were followed at our institution between 2004 and 2014, were reviewed; and children had hip surgery were identified. Hip pain and passive range of motion, radiographic neck shaft angle (NSA), and Hilgenreiner trochanteric (H-T) angle, sagittal spinopelvic parameters, and gait data were recorded. Preoperative and last follow-up data were compared. Outcomes were also compared between 3 age groups.

Results: Of the 79 children with SEDC, 26 children (12 boys and 14 girls) had hip osteotomy in 48 hips. Mean age at surgery was 9.6 years and the mean follow-up was 5 years. Preoperative hip pain was noted in 30 hips. At the last follow-up, 3 hips were painful at the extreme range of motion. Passive range of motion, NSA, and H-T improved postoperatively. Although NSA was maintained over the follow-up, H-T deterioration was noted. Spinopelvic measurements changed significantly and gait data remained stable except pelvic tilt that reduced significantly after surgery. The changes of radiographic measurements in each age group were similar to the total group of patients.

Conclusions: In children with SEDC, surgical correction of coxa vara, by proximal femoral valgus osteotomy, is an effective treatment that improves hip pain and range of motion in addition to the radiographic alignment of the proximal femur and the sagittal spinopelvic alignment. Children are expected to maintain their level of function after surgery and to have good results over the midterm regardless of their age at surgery.

Level Of Evidence: Level IV-therapeutic study.
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http://dx.doi.org/10.1097/BPO.0000000000000945DOI Listing
July 2019

The long-term outcome of pelvic asymmetry during gait in children with cerebral palsy following unilateral femoral derotation osteotomy.

J Pediatr Orthop B 2019 Jul;28(4):320-326

Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.

In this retrospective study, children with cerebral palsy underwent a unilateral femoral derotation osteotomy and had a preoperative (PO), short-term postoperative (1-3 years), and a long-term postoperative (≥5 years) gait analysis. Patients were subdivided into groups by the PO pelvic presentation and Gross Motor Function Classification System level. In children with PO pelvic external rotation, femoral derotation osteotomy decreased the hip internal rotation and decreased the pelvic external rotation. These results could influence surgical planning to achieve long-term pelvic asymmetry.
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http://dx.doi.org/10.1097/BPB.0000000000000613DOI Listing
July 2019

Increased femoral anteversion-related biomechanical abnormalities: lower extremity function, falling frequencies, and fatigue.

Gait Posture 2019 05 27;70:336-340. Epub 2019 Mar 27.

Nemours/Alfred I. duPont Hospital for Children, Turkey.

Background: Increased femoral anteversion (IFA) is defined as forwardly rotated femoral head relative to the transcondylar knee axis which may have a potential to reduce the functional quality of adolescents. Therefore, the aim of our study was to investigate the effects of IFA on lower-extremity function, falling frequency, and fatigue onset in neurologically intact children.

Research Question: Does increased femoral anteversion influence lower extremity function, falling frequency and fatigue on set in healthy children?

Methods: Sixty-five participants with increased femoral anteversion (IFA) and thirty-two healthy peers as control were included into the study. For the function, the lower extremity function form (LEFF) which is adapted from Lower Extremity Function Test used. Falling frequency and fatigue onset time were assessed by a Likert-type scale. In addition, the activities which cause frequently fall for the participants were questioned.

Results: Lower extremity function was found deteriorated (p= 0.02) and falling frequency was higher (p = 0.00) in IFA than in controls. Fatigue onset time was not different between groups, although lower extremity function was strongly correlated with fatigue onset (rho = -0.537, p < 0.001). IFA children fall four times more during running (60%), three times more during fast walking (21.42%) than their healthy peers (14.28%, 7.14% respectively).

Significance: IFA leads functional problems, especially in the form of high falling frequencies. According to the LEFF score, the most difficult functional parameters for these children were walking long distances, becoming tired, walking more than a mile, and standing on one spot. Also, shorter fatigue onset time may worsen the lower-extremity function secondarily. Because of the higher frequency of falling and functional problems, children with IFA may be more defenseless to injuries, especially in high-motor-skill activities such as running and soccer.
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http://dx.doi.org/10.1016/j.gaitpost.2019.03.027DOI Listing
May 2019

Altered brain tissue viscoelasticity in pediatric cerebral palsy measured by magnetic resonance elastography.

Neuroimage Clin 2019 7;22:101750. Epub 2019 Mar 7.

Department of Biomedical Engineering, University of Delaware, Newark, DE, United States; Department of Psychological and Brain Sciences, University of Delaware, Newark, DE, United States. Electronic address:

Cerebral palsy (CP) is a neurodevelopmental disorder that results in functional motor impairment and disability in children. CP is characterized by neural injury though many children do not exhibit brain lesions or damage. Advanced structural MRI measures may be more sensitively related to clinical outcomes in this population. Magnetic resonance elastography (MRE) measures the viscoelastic mechanical properties of brain tissue, which vary extensively between normal and disease states, and we hypothesized that the viscoelasticity of brain tissue is reduced in children with CP. Using a global region-of-interest-based analysis, we found that the stiffness of the cerebral gray matter in children with CP is significantly lower than in typically developing (TD) children, while the damping ratio of gray matter is significantly higher in CP. A voxel-wise analysis confirmed this finding, and additionally found stiffness and damping ratio differences between groups in regions of white matter. These results indicate that there is a difference in brain tissue health in children with CP that is quantifiable through stiffness and damping ratio measured with MRE. Understanding brain tissue mechanics in the pediatric CP population may aid in the diagnosis and evaluation of CP.
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http://dx.doi.org/10.1016/j.nicl.2019.101750DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6416970PMC
January 2020

Statistical Models to Assess Leg Muscle Mass in Ambulatory Children With Spastic Cerebral Palsy Using Dual-Energy X-Ray Absorptiometry.

J Clin Densitom 2019 Jul - Sep;22(3):391-400. Epub 2018 Dec 15.

Department of Kinesiology, University of Georgia, Athens, GA, USA. Electronic address:

Cerebral palsy (CP) is a movement disorder associated with small and weak muscles. Methods that accurately assess muscle mass in children with CP are scarce. The purpose of this study was to determine whether dual-energy X-ray absorptiometry (DXA) accurately estimates midleg muscle mass in ambulatory children with spastic CP. Ambulatory children with spastic CP and typically developing children 5-11 y were studied (n = 15/group). Fat-free soft tissue mass (FFST) and fat mass at the middle third of the tibia (i.e., midleg) were estimated using DXA. Muscle mass (muscle) and muscle mass corrected for intramuscular fat (muscle) in the midleg were estimated using magnetic resonance imaging (MRI). Statistical models were created to predict muscle and muscle using DXA. Children with CP compared to typically developing children had lower FFST (38%), muscle (40%) and muscle (47%) (all p < 0.05) and a lower ratio of muscle to FFST (17%, p < 0.05). DXA-based models developed using data from typically developing children overestimated muscle (13%) and muscle (22%) (both p < 0.05) in children with CP. DXA-based models developed using data from children with CP explained 91% of the variance in muscle and 90% of the variance in muscle in children with CP (both p < 0.05). Moreover, the estimates were not different from muscle and muscle (both p > 0.99). We conclude that DXA-based statistical models accurately estimate midleg muscle mass in children with CP when the models are composed using data from children with CP rather than typically developing children.
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http://dx.doi.org/10.1016/j.jocd.2018.12.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7333971PMC
July 2020

Long-term outcome of knee hyperextension in children with cerebral palsy.

J Pediatr Orthop B 2019 Jul;28(4):345-350

Department of Orthopedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.

This study evaluated knee hyperextension course in children with cerebral palsy over a 5-year follow-up. Knee hyperextension was identified in 308 knees, of which 97 had follow-up greater than 5 years. Between the tests, 40% of limbs had plantar flexor lengthening (PFL). Overall, knee flexion and ankle dorsiflexion in stance increased (P<0.0001). Similar changes were noted between limbs that had PFL and those that did not. PFL is indicated in cases associated with equinus. However, the role of multilevel surgery in the treatment of knee hyperextension needs to be further determined.
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http://dx.doi.org/10.1097/BPB.0000000000000564DOI Listing
July 2019

Greater Visceral Fat but No Difference in Measures of Total Body Fat in Ambulatory Children With Spastic Cerebral Palsy Compared to Typically Developing Children.

J Clin Densitom 2020 Jul - Sep;23(3):459-464. Epub 2018 Sep 22.

Department of Kinesiology, University of Georgia, Athens, GA, USA. Electronic address:

Background: Individuals with cerebral palsy (CP) are at increased risk for obesity and obesity-related complications. Studies of total body fat in those with CP are inconsistent and studies of abdominal fat are lacking in children with CP. The objective of this study was to determine if ambulatory children with spastic CP have greater central adiposity compared to typically developing children.

Methodology: Eighteen ambulatory children with spastic CP (n = 5 girls; 8.6 ± 2.9 yr) and 18 age-, sex-, and race-matched typically developing children (controls; 8.9 ± 2.1 yr) participated in this cross-sectional study. Children with CP were classified as I or II using the Gross Motor Function Classification System. Dual-energy X-ray absorptiometry assessed body composition, including total body, trunk and abdominal fat mass, fat-free mass, fat mass index (FMI), and fat-free mass index (FFMI).

Results: There were no group differences in fat mass, fat-free mass, FMI, and FFMI in the total body, fat mass, fat-free mass, and FFMI in the trunk, or fat mass, visceral fat mass, and subcutaneous fat mass in the abdomen (p > 0.05). Compared to controls, children with CP had higher trunk FMI, abdominal FMI, and visceral FMI (p < 0.05). Although marginally insignificant (p = 0.088), children with CP had higher subcutaneous FMI.

Conclusions: Ambulatory children with spastic CP have elevated central adiposity, especially in the visceral region, despite no differences in measures of total body fat. How this relates to cardiometabolic disease progression in those with CP requires further investigation.
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http://dx.doi.org/10.1016/j.jocd.2018.09.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8081403PMC
September 2018

BMI does not capture the high fat mass index and low fat-free mass index in children with cerebral palsy and proposed statistical models that improve this accuracy.

Int J Obes (Lond) 2019 01 4;43(1):82-90. Epub 2018 Sep 4.

Department of Kinesiology, University of Georgia, Athens, GA, USA.

Background/objectives: Children with cerebral palsy (CP) are at risk for having a misclassified overweight/obesity status based on BMI thresholds due to their lower fat-free mass and similar fat mass compared with typically developing children. The primary objective was to determine if BMI could predict fat mass index (FMI) and fat-free mass index (FFMI) in children with CP.

Subjects/methods: Forty-two children with CP and 42 typically developing children matched to children with CP for age and sex participated in the study. Dual-energy X-ray absorptiometry was used to assess body composition. Children with CP who could ambulate without assistance were considered ambulatory (ACP) and the rest were considered nonambulatory (NACP).

Results: Children with CP had higher percent body fat (%Fat) and FMI and lower fat-free mass and FFMI than controls (p < 0.05) but no difference in fat mass (p = 0.10). When BMI was statistically controlled, NACP had higher %Fat, fat mass and FMI and lower FFMI than ACP and controls (p < 0.05). NACP also had lower fat-free mass than controls (p < 0.05). ACP had higher %Fat and FMI and lower fat-free mass and FFMI than controls (p < 0.05). BMI was a strong predictor of FMI (r= 0.83) and a moderately strong predictor of FFMI (r= 0.49) in children with CP (both p < 0.01). Prediction of FMI (R= 0.86) and FFMI (R= 0.66) from BMI increased (p < 0.05) when age, sex and ambulatory status were included.

Conclusion: Compared with typically developing children, children with CP have a higher FMI and lower FFMI for a given BMI, which is more pronounced in NACP than ACP. The finding suggests that the prevalence of overweight/obesity status may be underestimated in children with CP.
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http://dx.doi.org/10.1038/s41366-018-0183-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8007077PMC
January 2019

Preliminary Safety and Tolerability of a Novel Subcutaneous Intrathecal Catheter System for Repeated Outpatient Dosing of Nusinersen to Children and Adults With Spinal Muscular Atrophy.

J Pediatr Orthop 2018 Nov/Dec;38(10):e610-e617

Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Many patients with spinal muscular atrophy (SMA) who might benefit from intrathecal antisense oligonucleotide (nusinersen) therapy have scoliosis or spinal fusion that precludes safe drug delivery. To circumvent spinal pathology, we designed a novel subcutaneous intrathecal catheter (SIC) system by connecting an intrathecal catheter to an implantable infusion port.

Methods: Device safety and tolerability were tested in 10 SMA patients (age, 5.4 to 30.5 y; 80% with 3 copies of SMN2); each received 3 sequential doses of nusinersen (n=30 doses). Pretreatment disease burden was evaluated using the Revised Hammersmith Scale, dynamometry, National Institutes of Health pegboard, pulmonary function testing, electromyography, and 2 health-related quality of life tools.

Results: Device implantation took ≤2 hours and was well tolerated. All outpatient nusinersen doses were successfully administered via SIC within 20 minutes on the first attempt, and required no regional or systemic analgesia, cognitive distraction, ultrasound guidance, respiratory precautions, or sedation. Cerebrospinal fluid withdrawn from the SIC had normal levels of glucose and protein; cerebrospinal fluid white blood cells were slightly elevated in 2 (22%) of 9 specimens (median, 1 cell/µL; range, 0 to 12 cells/µL) and red blood cells were detected in 7 (78%) specimens (median, 4; range, 0 to 2930 cells/µL).

Discussion: Preliminary observations reveal the SIC to be relatively safe and well tolerated in SMA patients with advanced disease and spinal fusion. The SIC warrants further study and, if proven effective in larger trials of longer duration, could double the number of patients able to receive nusinersen worldwide while reducing administration costs 5- to 10-fold.
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http://dx.doi.org/10.1097/BPO.0000000000001247DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6211782PMC
December 2018

Gait Analysis Parameters and Walking Activity Pre- and Postoperatively in Children With Cerebral Palsy.

Pediatr Phys Ther 2018 07;30(3):203-207

Nemours/Alfred I duPont Hospital for Children, Wilmington, Delaware.

Purpose: This study examined the relationship between the Gait Deviation Index (GDI) and walking activity preoperatively and postoperatively.

Methods: The GDI and walking activity from 74 youth with cerebral palsy (CP) were included in the analysis. The preoperative GDI was calculated using gait parameters collected during a clinical gait analysis 1 to 16 months prior to surgery. The postoperative GDI was calculated using gait parameters collected during a clinical gait analysis 10 to 26 months following surgery.

Results: A weak correlation was present between the change in the average GDI and the change in strides. A moderate correlation was found between the change in the Surgery GDI and the change in strides.

Conclusion: Single-event multilevel surgery improves gait deviations in children with CP. However, the improvement in gait pattern has limited correlation with postoperative change in walking activity. Our results demonstrate a need to pair surgical with additional intervention to affect long-term improvements in walking activity.
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http://dx.doi.org/10.1097/PEP.0000000000000512DOI Listing
July 2018

Comparison of three-dimensional multi-segmental foot models used in clinical gait laboratories.

Gait Posture 2018 06 16;63:236-241. Epub 2018 May 16.

Department of Orthopedics, Nemours/A.I. duPont Hospital for Children, Wilmington, DE, USA.

Background: Many skin-mounted three-dimensional multi-segmented foot models are currently in use for gait analysis. Evidence regarding the repeatability of models, including between trial and between assessors, is mixed, and there are no between model comparisons of kinematic results.

Research Question: This study explores differences in kinematics and repeatability between five three-dimensional multi-segmented foot models. The five models include duPont, Heidelberg, Oxford Child, Leardini, and Utah.

Methods: Hind foot, forefoot, and hallux angles were calculated with each model for ten individuals. Two physical therapists applied markers three times to each individual to assess within and between therapist variability. Standard deviations were used to evaluate marker placement variability. Locally weighted regression smoothing with alpha-adjusted serial T tests analysis was used to assess kinematic similarities.

Results: All five models had similar variability, however, the Leardini model showed high standard deviations in plantarflexion/dorsiflexion angles. P-value curves for the gait cycle were used to assess kinematic similarities. The duPont and Oxford models had the most similar kinematics.

Conclusions: All models demonstrated similar marker placement variability. Lower variability was noted in the sagittal and coronal planes compared to rotation in the transverse plane, suggesting a higher minimal detectable change when clinically considering rotation and a need for additional research. Between the five models, the duPont and Oxford shared the most kinematic similarities. While patterns of movement were very similar between all models, offsets were often present and need to be considered when evaluating published data.
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http://dx.doi.org/10.1016/j.gaitpost.2018.05.013DOI Listing
June 2018
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