Mode of Anesthesia and Bladder Management Following Orthopaedic Surgery in Children With Cerebral Palsy: A System Level Analysis.

Background: Postoperative urinary retention (POUR) is a surgical complication more prevalent in children with neurodisability and associated with an increase length of hospitalization. Risk factors include pre-existing bladder dysfunction, type and duration of surgery, anesthesia medications, postoperative opioid pain management, and patient demographics. The purpose of this investigation was (1) to determine the frequency of POUR following hip/lower limb orthopaedic procedures in which epidural analgesia was used for pain management; (2) to explore factors influencing postoperative bladder management.

A Longitudinal Study of Quantitative Muscle Strength and Functional Motor Ability in Ambulatory Boys with Duchenne Muscular Dystrophy.

Background: Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic disorder, that is characterized by progressive muscle degeneration and loss of ambulation between 7-13 years of age. Novel pharmacological agents targeting the genetic defects and disease mechanisms are becoming available; however, corticosteroid (CS) therapy remains the standard of care.

Objective: The purpose of this longitudinal study was to elucidate the effect of CS therapy on the rate of muscle strength and gross motor skill decline in boys with DMD and assess the sensitivity of selected outcome measures.

Longitudinal changes in energy cost during walking in boys with Duchenne Muscular Dystrophy (DMD).

Background: In boys with DMD, muscle weakness progresses in a proximal to distal pattern, leading to compensatory gait strategies, including hyperlordosis and equinus, that increase energy cost and accelerate the loss of walking capacity.

Research Question: The purpose of this study was to determine the changes in the energy cost of walking that occur with disease progression and to determine the optimal normalization scheme for the longitudinal assessment of the energy cost of walking in boys with DMD.

Methods: Energy cost was assessed with the COSMED K4b.

Efficacy of corticosteroid in decreasing scoliosis and extending time to loss of ambulation in a single clinic: an effectiveness trial.

Purpose: Pharmacologic doses of corticosteroid (CS) have been shown to ameliorate the progression of Duchenne muscular dystrophy (DMD) preserving strength, pulmonary function and ambulation as well as reducing the incidence of scoliosis. However, there are serious side effects of CS, which may impact dose tolerance. The purpose of this study was to compare the magnitude of positive CS effects on patients in our clinic to those reported in the literature.

Longitudinal community walking activity in Duchenne muscular dystrophy.

Introduction: Natural history studies for Duchenne muscular dystrophy (DMD) have not included measures of community ambulation.

Methods: Step activity (SA) monitors quantified community ambulation in 42 boys (ages 4-16 years) with DMD with serial enrollment up to 5 years by using a repeated-measures mixed model. Additionally, data were compared with 10-meter walk/run (10mWR) speed to determine validity and sensitivity.

Hip kinetics during gait are clinically meaningful outcomes in young boys with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is an X-linked genetic neuromuscular disorder characterized by progressive proximal to distal muscle weakness. The success of randomized clinical trials for novel therapeutics depends on outcome measurements that are sensitive to change. As the development of motor skills may lead to functional improvements in young boys with DMD, their inclusion may potentially confound clinical trials.

A comparison of the changes in the energy cost of walking between children with cerebral palsy and able-bodied peers over one year.

For ambulatory children with cerebral palsy, the assessment of walking energy efficiency is utilized to determine functional changes following surgical, pharmacologic, or orthotic interventions. While the assessment of energy efficiency is considered a useful outcome tool, minimal information exists about the changes in energy efficiency over one year in children with cerebral palsy at different gross motor function classification system (GMFCS) levels and whether the patterns of change are similar to their able-bodied peers. The purpose of this study was to determine whether energy efficiency variables change similarly over one year in children with cerebral palsy by GMFCS level and whether they differ from their age-matched peers.

Classification of the gait patterns of boys with Duchenne muscular dystrophy and their relationship to function.

Corticosteroids have recently been shown to reduce expected loss of muscle strength in patients with Duchenne muscular dystrophy and extend the time they can walk. We evaluated 43 boys with the condition to determine whether taking corticosteroids is associated with differences in gait pattern, gross motor skills, energy efficiency, and timed motor performance. We used the gait deviation index to quantify the degree of gait pathology and a single measure of gait quality.

Relationship between clinical outcome measures and parent proxy reports of health-related quality of life in ambulatory children with Duchenne muscular dystrophy.

In Duchenne muscular dystrophy, data directly linking changes in clinical outcome measures to patient-perceived well-being are lacking. This study evaluated the relationship between clinical outcome measures used in clinical trials of ambulatory Duchenne muscular dystrophy (Vignos functional grade, quantitative knee extension strength, timed functional performance measures, and gait velocity) and 2 health-related quality of life measures--the Pediatric Outcomes Data Collection Instrument and Pediatric Quality of Life Inventory-in 52 ambulatory Duchenne muscular dystrophy subjects and 36 controls. Those with the disease showed significant decrements in parent proxy-reported health-related quality of life measures versus controls across all domains.

Variability and minimum detectable change for walking energy efficiency variables in children with cerebral palsy.

For individuals with neuromuscular disorders, the assessment of walking energy efficiency is useful as a clinical outcome measure. Issues surrounding data collection methodology, normalization of the data, and variability and clinical utility of energy efficiency data preclude universal application. This study examined the variability and the clinical utility of velocity, energy efficiency index (EEI), gross cost, and net nondimensional cost (NNcost) in children and adolescents with spastic diplegic cerebral palsy (CP) in Gross Motor Function Classification System (GMFCS) levels I to III.

Walking energy expenditure in able-bodied individuals: a comparison of common measures of energy efficiency.

Assessment of walking energy efficiency is potentially useful for objectively quantifying gait efficiency; however, issues regarding methodology, day-to-day variability, inter subject variability (age and size differences) and clinical relevance prevents the assessment of walking energy efficiency from being used as a standard assessment tool. Volume of oxygen (VO(2)) and heart rate were assessed to determine the intra-subject (day-to-day) variability, the impact of age and body parameters and the clinical relevance of gross cost, Net non-dimensional Cost (NNcost) and Energy Efficiency Index (EEI) in able-bodied individuals. Gross cost demonstrated the least amount of day-to-day variability, in comparison to NNcost and EEI.

Comparing botulinum toxin A with casting for treatment of dynamic equinus in children with cerebral palsy.

The purpose of this study was to compare the cumulative efficacy (three treatment sessions) of botulinum toxin A (BTX-A) alone, casting alone, and the combination of BTX-A and casting in the management of dynamic equinus in ambulatory children with spastic cerebral palsy (CP). Thirty-nine children with spastic CP (mean age 5y 10mo, range 3 to 9y) were enrolled in the study. A multicenter, randomized, double blind, placebo-controlled prospective study was used.

A 2-year follow-up of outcomes following orthopedic surgery or selective dorsal rhizotomy in children with spastic diplegia.

The purpose of this study was to prospectively compare the effect of orthopedic surgery (OS) and selective dorsal rhizotomy (SDR) on muscle tone, range of motion, gait and energy efficiency in ambulatory children with spastic diplegia. Twenty-five children with a diagnosis of spastic diplegia, with a mean age of 73 months, were evaluated prior to surgery and 1 and 2 years postoperatively; however, only the preoperative and 2-year postoperative data are reported here. Eighteen children received SDR and seven received OS.

Comparison of three ankle-foot orthosis configurations for children with spastic diplegia.

This study compared the functional efficacy of three commonly prescribed ankle-foot orthosis (AFO) configurations (solid [SAFO], hinged [HAFO], and posterior leaf spring [PLS]). Sixteen independently ambulatory children (10 males, six females; mean age 8 years 4 months, SD 2 years 4 months; range 4 years 4 months to 11 years 6 months) with spastic diplegia participated in this study. Four children were classified at level I of the Gross Motor Function Classification System (GMFCS; Palisano et al.

Selective dorsal rhizotomy versus orthopedic surgery: a multidimensional assessment of outcome efficacy.

Objective: To compare the efficacy of selective dorsal rhizotomy (SDR) and orthopedic surgery using multidimensional (National Center for Medical Rehabilitation Research disablement framework) outcome measures.

Design: Prospective outcome study.

Setting: Pediatric orthopedic hospital.

Stair locomotion in children with spastic hemiplegia: the impact of three different ankle foot orthosis (AFOs) configurations.

The purpose of this study was to investigate the impact of three different ankle foot orthoses (AFO) configurations on the function and kinematics of stair locomotion in children with spastic hemiplegia. Nineteen children were evaluated barefoot and with a hinged, posterior leaf spring (PLS) and solid AFO during stair ascent and descent. Stair specific items from the Pediatric Evaluation of Disability Inventory (PEDI) were used to evaluate function, while a motion measurement system was used to evaluate kinematics.

Objective measurement of muscle strength in children with spastic diplegia after selective dorsal rhizotomy.

Objectives: To examine changes in isometric muscle strength at the elbow, knee, and ankle at 6 months and 1 year after selective dorsal rhizotomy (SDR) and to determine if SDR altered the frequency of muscle cocontraction.

Design: Prospective outcome study of a consecutive sample.

Setting: Children's hospital.