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1.
J Physiol ; 602(14): 3489-3504, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39008710

ABSTRACT

Cerebral palsy (CP) describes some upper motoneuron disorders due to non-progressive disturbances occurring in the developing brain that cause progressive changes to muscle. While longer sarcomeres increase muscle stiffness in patients with CP compared to typically developing (TD) patients, changes in extracellular matrix (ECM) architecture can increase stiffness. Our goal was to investigate how changes in muscle and ECM architecture impact muscle stiffness, gait and joint function in CP. Gracilis and adductor longus biopsies were collected from children with CP undergoing tendon lengthening surgery for hamstring and hip adduction contractures, respectively. Gracilis biopsies were collected from TD patients undergoing anterior cruciate ligament reconstruction surgery with hamstring autograft. Muscle mechanical testing, two-photon imaging and hydroxyproline assay were performed on biopsies. Corresponding data were compared to radiographic hip displacement in CP adductors (CPA), gait kinematics in CP hamstrings (CPH), and joint range of motion in CPA and CPH. We found at matched sarcomere lengths muscle stiffness and collagen architecture were similar between TD and CP hamstrings. However, CPH stiffness (R2 = 0.1973), collagen content (R2 = 0.5099) and cross-linking (R2 = 0.3233) were correlated to decreased knee range of motion. Additionally, we observed collagen fibres within the muscle ECM increase alignment during muscular stretching. These data demonstrate that while ECM architecture is similar between TD and CP hamstrings, collagen fibres biomechanics are sensitive to muscle strain and may be altered at longer in vivo sarcomere lengths in CP muscle. Future studies could evaluate the impact of ECM architecture on TD and CP muscle stiffness across in vivo operating ranges. KEY POINTS: At matched sarcomere lengths, gracilis muscle mechanics and collagen architecture are similar in TD patients and patients with CP. In both TD and CP muscles, collagen fibres dynamically increase their alignment during muscle stretching. Aspects of muscle mechanics and collagen architecture are predictive of in vivo knee joint motion and radiographic hip displacement in patients with CP. Longer sarcomere lengths in CP muscle in vivo may alter collagen architecture and biomechanics to drive deficits in joint mobility and gait function.


Subject(s)
Cerebral Palsy , Collagen , Humans , Cerebral Palsy/physiopathology , Cerebral Palsy/pathology , Child , Male , Female , Collagen/metabolism , Biomechanical Phenomena , Adolescent , Gracilis Muscle , Range of Motion, Articular , Muscle, Skeletal/physiology , Muscle, Skeletal/physiopathology , Gait/physiology , Hamstring Muscles/physiology , Hamstring Muscles/physiopathology , Extracellular Matrix/physiology
2.
Dev Med Child Neurol ; 2024 Jun 27.
Article in English | MEDLINE | ID: mdl-38937924

ABSTRACT

AIM: To evaluate the mechanosensitivity of muscle satellite cells (MuSCs) and fibro-adipogenic progenitors (FAPs) in cerebral palsy (CP) and the efficacy of the drug verteporfin in restoring cells' regenerative capacity. METHOD: Muscle biopsies were collected from six children with CP and six typically developing children. MuSCs and FAPs were isolated and plated on collagen-coated polyacrylamide gels at stiffnesses of 0.2 kPa, 8 kPa, and 25 kPa. Cells were treated with verteporfin to block mechanosensing or with dimethyl sulfoxide as a negative control. MuSC differentiation and FAP activation into myofibroblasts were measured using immunofluorescence staining. RESULTS: Surprisingly, MuSC differentiation was not affected by stiffness; however, stiff substrates resulted in large myonuclear clustering. Across all stiffnesses, MuSCs from children with CP had less differentiation than those of their typically developing counterparts. FAP activation into myofibroblasts was significantly higher in children with CP than their typically developing peers, but was not affected by stiffness. Verteporfin did not affect differentiation or activation in either cell population, but slightly decreased myonuclear clustering on stiff substrates. INTERPRETATION: Cells from children with CP were less regenerative and more fibrotic compared to those of their typically developing counterparts, with MuSCs being sensitive to increases in stiffness. Therefore, the mechanosensitivity of MuSCs and FAPs may represent a new target to improve differentiation and activation in CP muscle.

3.
J Biomech ; 168: 112092, 2024 May.
Article in English | MEDLINE | ID: mdl-38669795

ABSTRACT

Gait for individuals with movement disorders varies widely and the variability makes it difficult to assess outcomes of surgical and therapeutic interventions. Although specific joints can be assessed by fewer individual measures, gait depends on multiple parameters making an overall assessment metric difficult to determine. A holistic, summary measure can permit a standard comparison of progress throughout treatments and interventions, and permit more straightforward comparison across varied subjects. We propose a single summary metric (the Shriners Gait Index (SGI)) to represent the quality of gait using a deep learning autoencoder model, which helps to capture the nonlinear statistical relationships among a number of disparate gait metrics. We utilized gait data of 412 individuals under the age of 18 collected from the Motion Analysis Center (MAC) at the Shriners Children's - Chicago. The gait data includes a total of 114 features: temporo-spatial parameters (7), lower extremity kinematics (64), and lower extremity kinetics (43) which were min-max normalized. The developed SGI score captured more than 89% variance of all 144 features using subject-wise cross-validation. Such summary metrics holistically quantify an individual's gait which can then be used to assess the impact of therapeutic interventions. The machine learning approach utilized can be leveraged to create such metrics in a variety of contexts depending on the data available. We also utilized the SGI to compare overall changes to gait after surgery with the goal of improving mobility for individuals with gait disabilities such as Cerebral Palsy.


Subject(s)
Cerebral Palsy , Gait , Humans , Cerebral Palsy/surgery , Cerebral Palsy/physiopathology , Child , Gait/physiology , Female , Male , Biomechanical Phenomena , Adolescent , Child, Preschool , Gait Analysis/methods , Treatment Outcome , Deep Learning , Lower Extremity/surgery , Lower Extremity/physiopathology
4.
J Pediatr Orthop ; 44(6): e518-e529, 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38515131

ABSTRACT

Posttraumatic cubitus varus is a multiplanar deformity that results from an improperly reduced supracondylar humerus fracture. The prevention of posttraumatic cubitus varus hinges on the stable restoration of all 3 columns of the distal humerus while avoiding malrotation. The collapse of any column leads to varying degrees of deformity in the coronal, sagittal, and/or axial plane. The purpose of this article is to explain the pattern of the deformity and use this to summarize preventative tactics for avoiding its described sequelae. We also summarize, illustrate, and present case examples for the various osteotomies used to correct the deformity, and speculate future directions.


Subject(s)
Elbow Injuries , Elbow Joint , Humeral Fractures , Osteotomy , Humans , Humeral Fractures/surgery , Humeral Fractures/complications , Osteotomy/methods , Elbow Joint/surgery , Joint Deformities, Acquired/etiology , Joint Deformities, Acquired/surgery , Child
5.
Nucleic Acids Res ; 52(D1): D1333-D1346, 2024 Jan 05.
Article in English | MEDLINE | ID: mdl-37953324

ABSTRACT

The Human Phenotype Ontology (HPO) is a widely used resource that comprehensively organizes and defines the phenotypic features of human disease, enabling computational inference and supporting genomic and phenotypic analyses through semantic similarity and machine learning algorithms. The HPO has widespread applications in clinical diagnostics and translational research, including genomic diagnostics, gene-disease discovery, and cohort analytics. In recent years, groups around the world have developed translations of the HPO from English to other languages, and the HPO browser has been internationalized, allowing users to view HPO term labels and in many cases synonyms and definitions in ten languages in addition to English. Since our last report, a total of 2239 new HPO terms and 49235 new HPO annotations were developed, many in collaboration with external groups in the fields of psychiatry, arthrogryposis, immunology and cardiology. The Medical Action Ontology (MAxO) is a new effort to model treatments and other measures taken for clinical management. Finally, the HPO consortium is contributing to efforts to integrate the HPO and the GA4GH Phenopacket Schema into electronic health records (EHRs) with the goal of more standardized and computable integration of rare disease data in EHRs.


Subject(s)
Biological Ontologies , Humans , Phenotype , Genomics , Algorithms , Rare Diseases
6.
Bioinformatics ; 39(12)2023 12 01.
Article in English | MEDLINE | ID: mdl-38001031

ABSTRACT

MOTIVATION: Methods for concept recognition (CR) in clinical texts have largely been tested on abstracts or articles from the medical literature. However, texts from electronic health records (EHRs) frequently contain spelling errors, abbreviations, and other nonstandard ways of representing clinical concepts. RESULTS: Here, we present a method inspired by the BLAST algorithm for biosequence alignment that screens texts for potential matches on the basis of matching k-mer counts and scores candidates based on conformance to typical patterns of spelling errors derived from 2.9 million clinical notes. Our method, the Term-BLAST-like alignment tool (TBLAT) leverages a gold standard corpus for typographical errors to implement a sequence alignment-inspired method for efficient entity linkage. We present a comprehensive experimental comparison of TBLAT with five widely used tools. Experimental results show an increase of 10% in recall on scientific publications and 20% increase in recall on EHR records (when compared against the next best method), hence supporting a significant enhancement of the entity linking task. The method can be used stand-alone or as a complement to existing approaches. AVAILABILITY AND IMPLEMENTATION: Fenominal is a Java library that implements TBLAT for named CR of Human Phenotype Ontology terms and is available at https://github.com/monarch-initiative/fenominal under the GNU General Public License v3.0.


Subject(s)
Algorithms , Language , Humans , Sequence Alignment , Electronic Health Records , Publications
7.
Article in English | MEDLINE | ID: mdl-37684057

ABSTRACT

We identified a de novo heterozygous transient receptor potential cation channel subfamily M (melastatin) member 3 (TRPM3) missense variant, p.(Asn1126Asp), in a patient with developmental delay and manifestations of cerebral palsy (CP) using phenotype-driven prioritization analysis of whole-genome sequencing data with Exomiser. The variant is localized in the functionally important ion transport domain of the TRPM3 protein and predicted to impact the protein structure. Our report adds TRPM3 to the list of Mendelian disease-associated genes that can be associated with CP and provides further evidence for the pathogenicity of the variant p.(Asn1126Asp).


Subject(s)
Cerebral Palsy , Intellectual Disability , Nervous System Malformations , TRPM Cation Channels , Humans , Cerebral Palsy/genetics , Intellectual Disability/genetics , Mutation, Missense/genetics , Phenotype , TRPM Cation Channels/genetics
9.
Dev Med Child Neurol ; 65(11): 1486-1492, 2023 11.
Article in English | MEDLINE | ID: mdl-37143284

ABSTRACT

AIM: To assess the accuracy, reliability, and discriminatory ability of a mobile app for measurement of migration percentage in hip surveillance radiographs of children with cerebral palsy (CP). METHOD: The free mobile app HipScreen (www.hipscreen.org) was utilized by a diverse group of users to measure the migration percentage of 40 hips at two time points after completing an online tutorial and competency test. The mean absolute error (MAE) was calculated against the reference standard obtained on a radiology workstation. Statistical analyses included linear regression, intraclass correlation coefficient (ICC), and area under receiver-operating characteristic curve (AUC). RESULTS: Thirty-seven users completed the study, with 30 having a healthcare professional background, but only 15 with x-ray interpretation expertise. The overall MAE of migration percentage measurement using the HipScreen app was 5.72% (95% confidence interval [CI]: 5.38-6.06), with good reliability between time points (ICC = 0.83). With a migration percentage less than 30% considered as a positive case, the HipScreen app had a sensitivity of 94% (95% CI: 87-97), specificity of 66% (95% CI: 61-77), and an AUC of 0.92 (95% CI: 0.88-0.96). INTERPRETATION: Users from a broad range of backgrounds can utilize the HipScreen app to measure hip surveillance radiographs with clinically acceptable accuracy, reliability, and discriminatory ability. WHAT THIS PAPER ADDS: The HipScreen app can accurately and reliably measure migration percentage. As a screening tool, HipScreen has excellent sensitivity and discriminatory ability. A broad range of HipScreen users achieve clinically acceptable performance.


Subject(s)
Cerebral Palsy , Hip Dislocation , Mobile Applications , Child , Humans , Cerebral Palsy/diagnosis , Reproducibility of Results , Hip
10.
J Pediatr Orthop ; 43(2): 91-98, 2023 Feb 01.
Article in English | MEDLINE | ID: mdl-36607920

ABSTRACT

BACKGROUND: Tendo Achilles lengthening (TAL) for the management of equinus contractures in ambulatory children with cerebral palsy (CP) is generally not recommended due to concerns of over-lengthening, resulting in weakness and plantar flexor insufficiency. However, in some cases, surgical correction of severe equinus deformities can only be achieved by TAL. The goal of this study is to assess the outcomes following TAL in these cases. METHODS: A retrospective cohort study of children with CP with severe equinus contractures (ankle dorsiflexion with the knee extended of -20 degrees or worse) who underwent TAL as part of a single event multilevel surgery, with preoperative and postoperative gait analysis studies. Continuous data were analyzed by paired t test, and categorical data by McNemar Test. RESULTS: There were 60 subjects: 42 unilateral, 18 bilateral CP; 41 GMFCS II, 17 GMFCS I; mean age at surgery was 10.6 years, mean follow-up was 1.3 years. Ankle dorsiflexion with the knee extended improved from -28 to 5 degrees (P<0.001). The ankle Gait Variable Score improved from 34.4 to 8.6 (P<0.001). The ankle moment in terminal stance improved from 0.43 to 0.97 Nm/kg (P<0.001). Significant improvements (P<0.001) were seen in radiographic measures of foot alignment following surgery. There were few significant differences in the outcome parameters between subjects with unilateral versus bilateral CP (eg, only the bilateral group showed improved but persistent increased knee flexion in mid-stance). CONCLUSIONS: The outcomes following TAL for the management of severe equinus deformity in ambulatory children with CP were favorable 1 year after surgery, with significant improvements in all domains measured. SIGNIFICANCE: This study does not advocate for the widespread use of TAL to correct equinus deformity in children with CP. However, it does show that good short-term outcomes following TAL are possible in properly selected subjects with severe contractures when the dosing of the surgery is optimal (correction of contracture to between 0 and 5 degrees of dorsiflexion with the knee extended) and the procedure is performed in the setting of single event multilevel surgery with subsequent proper orthotic management and rehabilitation.


Subject(s)
Cerebral Palsy , Contracture , Equinus Deformity , Humans , Child , Equinus Deformity/etiology , Equinus Deformity/surgery , Retrospective Studies , Cerebral Palsy/complications , Cerebral Palsy/surgery , Tenotomy/methods , Gait
11.
J Child Orthop ; 16(6): 442-453, 2022 Dec.
Article in English | MEDLINE | ID: mdl-36483640

ABSTRACT

Purpose: In children with cerebral palsy, flexion deformities of the knee can be treated with a distal femoral extension osteotomy combined with either patellar tendon advancement or patellar tendon shortening. The purpose of this study was to establish a consensus through expert orthopedic opinion, using a modified Delphi process to describe the surgical indications for distal femoral extension osteotomy and patellar tendon advancement/patellar tendon shortening. A literature review was also conducted to summarize the recent literature on distal femoral extension osteotomy and patellar tendon shortening/patellar tendon advancement. Method: A group of 16 pediatric orthopedic surgeons, with more than 10 years of experience in the surgical management of children with cerebral palsy, was established. The group used a 5-level Likert-type scale to record agreement or disagreement with statements regarding distal femoral extension osteotomy and patellar tendon advancement/patellar tendon shortening. Consensus for the surgical indications for distal femoral extension osteotomy and patellar tendon advancement/patellar tendon shortening was achieved through a modified Delphi process. The literature review, summarized studies of clinical outcomes of distal femoral extension osteotomy/patellar tendon shortening/patellar tendon advancement, published between 2008 and 2022. Results: There was a high level of agreement with consensus for 31 out of 44 (70%) statements on distal femoral extension osteotomy. Agreement was lower for patellar tendon advancement/patellar tendon shortening with consensus reached for 8 of 21 (38%) of statements. The literature review included 25 studies which revealed variation in operative technique for distal femoral extension osteotomy, patellar tendon advancement, and patellar tendon shortening. Distal femoral extension osteotomy and patellar tendon advancement/patellar tendon shortening were generally effective in correcting knee flexion deformities and extensor lag, but there was marked variation in outcomes and complication rates. Conclusion: The results from this study will provide guidelines for surgeons who care for children with cerebral palsy and point to unresolved questions for further research. Level of evidence: level V.

12.
Gait Posture ; 98: 355-361, 2022 10.
Article in English | MEDLINE | ID: mdl-36308864

ABSTRACT

BACKGROUND: Gait classification systems (GCS) may enable clinicians to differentiate gait patterns into clinically significant categories that assist in clinical decision-making and assessment of outcomes. Davids and Bagley in 2014 [1] described a GCS for children with cerebral palsy (GCS-CP). The purpose of our study was to use the GCS-CP for the first time on a sample of patients with CP and to evaluate the reliability and utility of the classification system. METHODS: The gait of 131 children with CP was retrospectively reviewed and classified according to Davids and Bagley's classification using two-dimensional (2D) video and three-dimensional (3D) lower limb kinematics and kinetics. Gross Motor Function Classification System (GMFCS) levels were determined, and the Gait Profile Scores (GPS) calculated to characterize the sample concerning gait classification. The comparison between the groups was performed using the Kruskal-Wallis test with respect to the non-normal distribution of the data. The intrarater and interrater reliability was determined using the Kappa index (k) statistics with 95% CI. RESULTS: All GCS-CP groups were represented within the evaluated sample. Of the 131 cases evaluated, 127 (96.95%) were able to be classified with respect to sagittal plane stance phase gait deviations. All patients in the sample were able to be classified with respect to sagittal plane swing phase and transverse plane gait deviations. The interrater reliability was 0.596 and 0.485 for the first and second levels of the classification, respectively, according to the Fleiss's Kappa statistics. Intrarater reliability was 0.776 and 0.714 for the raters one and two, respectively, according to the Cohen's Kappa statistics. SIGNIFICANCE: The GCS-CP exhibited clinical utility, successfully classifying almost all subjects with CP in two planes, based upon kinematic and kinetic data. The classification is valid and has moderate interrater and moderate to substantial intrarater reliability.


Subject(s)
Cerebral Palsy , Movement Disorders , Child , Humans , Reproducibility of Results , Retrospective Studies , Gait , Biomechanical Phenomena
13.
J Am Acad Orthop Surg ; 30(22): e1419-e1430, 2022 Nov 15.
Article in English | MEDLINE | ID: mdl-36084329

ABSTRACT

Toe walking is a common presenting report to an orthopaedic practice. Evaluation of a child with toe walking includes a thorough history and physical examination to elucidate the diagnosis. When no other diagnosis is suspected, a diagnosis of idiopathic toe walking is often given. Despite the high prevalence of the condition, there is notable controversy of the nomenclature of the disease. Recent research has shed more light on both the natural history and the genetic basis. The use of motion analysis, including EMG and multisegment foot model, may help both differentiate children with mild forms of cerebral palsy and evaluate outcomes after treatment. Early treatment for young children with adequate range of motion (ROM) is generally reassurance because most young children with idiopathic toe walking are expected to resolve spontaneously. When the toe walking persists, treatment options include both surgical and nonsurgical management. Nonsurgical management centers on obtaining ROM through stretching and serial casting, followed by gait retraining and bracing treatment. Surgical lengthening at either the Achilles or gastrocnemius level improves the ROM of the ankle and then similarly requires therapy and bracing treatment to obtain a more normalized gait.


Subject(s)
Toes , Walking , Child , Humans , Child, Preschool , Gait , Ankle Joint , Range of Motion, Articular
14.
J Child Orthop ; 16(2): 111-120, 2022 Apr.
Article in English | MEDLINE | ID: mdl-35620124

ABSTRACT

Purpose: The purpose of this study was to establish consensus for the assessment of foot alignment and function in ambulatory children with cerebral palsy, using expert surgeon's opinion through a modified Delphi technique. Methods: The panel used a five-level Likert-type scale to record agreement or disagreement with 33 statements regarding the assessment of foot alignment and function. Consensus was defined as at least 80% of responses being in the highest or lowest of two of the five Likert-type ratings. General agreement was defined as 60%-79% falling into the highest or lowest two ratings. There was no agreement if neither threshold was reached. Results: Consensus was achieved for 25 (76%) statements, general agreement for 4 (12%) statements, and lack of consensus for 4 (12%) of the statements. There was consensus that the functional anatomy of the foot is best understood by dividing the foot into three segments and two columns. Consensus was achieved concerning descriptors of foot segmental alignment for both static and dynamic assessment. There was consensus that radiographs of the foot should be weight-bearing. There was general agreement that foot deformity in children with cerebral palsy can be classified into three levels based on soft tissue imbalance and skeletal malalignment. Conclusion: The practices identified in this study can be used to establish best care guidelines, and the format used will be a template for future Delphi technique studies on clinical decision-making for the management of specific foot segmental malalignment patterns commonly seen in children with cerebral palsy. Level of Evidence: V.

15.
J Child Orthop ; 16(1): 55-64, 2022 Feb.
Article in English | MEDLINE | ID: mdl-35615393

ABSTRACT

Purpose: There is marked variation in indications and techniques for hamstring surgery in children with cerebral palsy. There is particular uncertainty regarding the indications for hamstring transfer compared to traditional hamstring lengthening. The purpose of this study was for an international panel of experts to use the Delphi method to establish consensus indications for hamstring surgery in ambulatory children with cerebral palsy. Methods: The panel used a five-level Likert-type scale to record agreement or disagreement with statements regarding hamstring surgery, including surgical indications and techniques, post-operative care, and outcome measures. Consensus was defined as at least 80% of responses being in the highest or lowest two of the five Likert-type ratings. General agreement was defined as 60%-79% falling into the highest or lowest two ratings. There was no agreement if neither of these thresholds was reached. Results: The panel reached consensus or general agreement for 38 (84%) of 45 statements regarding hamstring surgery. The panel noted the importance of assessing pelvic tilt during gait when considering hamstring surgery, and also that lateral hamstring lengthening is rarely needed, particularly at the index surgery. They noted that repeat hamstring lengthening often has poor outcomes. The panel was divided regarding hamstring transfer surgery, with only half performing such surgery. Conclusion: The results of this study can help pediatric orthopedic surgeons optimize decision-making in their choice and practice of hamstring surgery for ambulatory children with cerebral palsy. This has the potential to reduce practice variation and significantly improve outcomes for ambulatory children with cerebral palsy. Level of evidence: level V.

16.
J Child Orthop ; 16(1): 65-74, 2022 Feb.
Article in English | MEDLINE | ID: mdl-35615394

ABSTRACT

Purpose: The purpose of this study was to develop consensus for the surgical indications of anterior distal femur hemiepiphysiodesis in children with cerebral palsy using expert surgeon opinion through a modified Delphi technique. Methods: The panel used a 5-level Likert-type scale to record agreement or disagreement with 27 statements regarding anterior distal femur hemiepiphysiodesis. Consensus was defined as at least 80% of responses being in the highest or lowest 2 of the Likert-type ratings. General agreement was defined as 60%-79% falling into the highest or lowest 2 ratings. Results: For anterior distal femur hemiepiphysiodesis, 27 statements were surveyed: consensus or general agreement among the panelists was achieved for 22 of 27 statements (22/27, 82%) and 5 statements had no agreement (5/27, 18%). There was general consensus that anterior distal femur hemiepiphysiodesis is indicated for ambulatory children with cerebral palsy, with at least 2 years growth remaining, and smaller (<30 degrees) knee flexion contractures and for minimally ambulatory children to aid in standing/transfers. Consensus was achieved regarding the importance of close radiographic follow-up after screw insertion to identify or prevent secondary deformity. There was general agreement that percutaneous screws are preferred over anterior plates due to the pain and irritation associated with plates. Finally, it was agreed that anterior distal femur hemiepiphysiodesis was not indicated in the absence of a knee flexion contracture. Conclusion: Anterior distal femur hemiepiphysiodesis can be used to treat fixed knee flexion contractures in the setting of crouch gait, but other associated lever arm dysfunctions must be addressed by single-event multilevel surgery. Level of evidence: V.

17.
J Pediatr Rehabil Med ; 15(1): 3-11, 2022.
Article in English | MEDLINE | ID: mdl-35275572

ABSTRACT

PURPOSE: The purpose of this study is to describe the efficacy and adverse events of neuraxial anesthesia for post-operative pain control in non-ambulatory children with cerebral palsy with pre-existing intrathecal baclofen (ITB) pumps undergoing hip reconstructive or palliative surgery. METHODS: Twelve children (mean age 11.25 years) were included in the study with the following neuraxial anesthesia methods: indwelling epidural catheter (8 patients), neuraxial opioids administered through the side port of the ITB pump (3 patients), and single injection spinal anesthetic (1 patient). Observational pain scores and opioid requirements were quantified for all patients. RESULTS: There were no ITB pump or surgical complications at a mean follow-up of 2.2 years. The average length of stay was 6 days. Patients had good post-operative pain control with a mean observational pain score of 0.7 and mean morphine equivalent use of 0.26mg/kg/day. Four patients required anti-emetics to control nausea and three patients had urinary retention requiring repeat catheterization, but all medical complications resolved prior to discharge. CONCLUSION: Neuraxial anesthesia can effectively control post-operative pain in children with a pre-existing ITB pump. Utilizing the side port of the ITB pump for administration of neuraxial opioids is an option when epidural or spinal anesthesia is not possible.


Subject(s)
Anesthesia , Cerebral Palsy , Muscle Relaxants, Central , Baclofen , Cerebral Palsy/complications , Child , Humans , Infusion Pumps, Implantable/adverse effects , Muscle Relaxants, Central/therapeutic use , Muscle Spasticity/complications , Muscle Spasticity/drug therapy , Pain, Postoperative/chemically induced , Pain, Postoperative/complications , Pain, Postoperative/drug therapy , Retrospective Studies
18.
Clin Rheumatol ; 40(12): 5033-5040, 2021 Dec.
Article in English | MEDLINE | ID: mdl-34350521

ABSTRACT

INTRODUCTION/OBJECTIVES: The aim of this study was to investigate the validity and reliability of "Shriners Hospital for Children Upper Extremity Evaluation (SHUEE)" for children with rheumatic diseases. METHODS: The study was carried out after obtaining the necessary permissions and retrospectively registered. The psychometric properties evaluated were reliability and concurrent validity. Reliability was determined by intra- and inter-observer agreement. Concurrent validity was performed using the Jebsen Taylor Hand Function Test (JTHFT), Abilhand-Rheumatoid Arthritis (Abilhand-RA), and Children Health Assessment Questionnaire (CHAQ). The validity and reliability of the evaluation were determined after the retest 1 week later. RESULTS: Twenty children with rheumatic diseases were participated in to study. Intraclass coefficients ranged from 0.82 to 0.97 and the intraobserver reliability for SHUEE total and subscales were considered "excellent." Interobserver reliability was considered "excellent" for the SHUUE total score, spontaneous functional analysis and dynamic positional analysis, and "moderate" for grasp-release. A moderate negative correlation was determined between Spontaneous Functional Analysis and JTHFT (r = - 0.63; p = 0.003). CONCLUSION: SHUEE is a valid and reliable evaluation for children with rheumatic diseases. ClinicalTrials.org NCT04685434/21.12.2020 Key Points • SHUEE tends to be appropriate and acceptable to children with rheumatic diseases. • SHUEE can be used safely in the pediatric rheumatology group and it is beneficial in the clinical decision-making process. • SHUEE is a pioneering performance test that evaluates the quality of movement in pediatric rheumatology on a joint basis.


Subject(s)
Arthritis, Rheumatoid , Cerebral Palsy , Child , Disability Evaluation , Hospitals , Humans , Psychometrics , Reproducibility of Results , Surveys and Questionnaires , Upper Extremity
19.
J Child Orthop ; 15(3): 270-278, 2021 Jun 01.
Article in English | MEDLINE | ID: mdl-34211604

ABSTRACT

PURPOSE: The purpose of this study was for an international panel of experts to establish consensus indications for distal rectus femoris surgery in children with cerebral palsy (CP) using a modified Delphi method. METHODS: The panel used a five-level Likert scale to record agreement or disagreement with 33 statements regarding distal rectus femoris surgery. The panel responded to statements regarding general characteristics, clinical indications, computerized gait data, intraoperative techniques and outcome measures. Consensus was defined as at least 80% of responses being in the highest or lowest two of the five Likert ratings, and general agreement as 60% to 79% falling into the highest or lowest two ratings. There was no agreement if neither threshold was reached. RESULTS: Consensus or general agreement was reached for 17 of 33 statements (52%). There was general consensus that distal rectus femoris surgery is better for stiff knee gait than is proximal rectus femoris release. There was no consensus about whether the results of distal rectus femoris release were comparable to those following distal rectus femoris transfer. Gross Motor Function Classification System (GMFCS) level was an important factor for the panel, with the best outcomes expected in children functioning at GMFCS levels I and II. The panel also reached consensus that they do distal rectus femoris surgery less frequently than earlier in their careers, in large part reflecting the narrowing of indications for this surgery over the last decade. CONCLUSION: This study can help paediatric orthopaedic surgeons optimize decision-making for, and outcomes of, distal rectus femoris surgery in children with CP. LEVEL OF EVIDENCE: V.

20.
J Pediatr Orthop ; 41(5): e356-e366, 2021.
Article in English | MEDLINE | ID: mdl-33734198

ABSTRACT

BACKGROUND: Crouch gait is a frequent gait abnormality observed in children with cerebral palsy. Distal femoral extension osteotomy (DFEO) with the tightening of the extensor mechanism is a common treatment strategy to address the pathologic knee flexion contracture and patella alta. The goal of this study was to review the results of a patellar tendon imbrication (PTI) strategy to address quadriceps insufficiency in the setting of children undergoing DFEO. METHODS: After institutional review board approval, all patients with crouch gait treated at a single institution with DFEO and PTI were identified. Clinical, radiographic, and instrumented gait analysis data were analyzed preoperatively and at 1 year following surgery. RESULTS: Twenty-eight patients (54 extremities) with a diagnosis of cerebral palsy and crouch gait were included. Significant improvements were appreciated in the degree of knee flexion contracture, quadriceps strength, knee extensor lag, and popliteal angle (P<0.01). Knee flexion at initial contact and during mid-stance improved significantly (P<0.0001), and knee moments in late stance were significantly reduced (P<0.01). The anterior pelvic tilt, however, significantly increased postoperatively (P<0.0001). Radiographic improvements were seen in the knee flexion angle and patellar station as assessed by the Koshino Sugimoto Index (P<0.0001). Four patients (14.2%) developed a recurrence of knee flexion contracture requiring further intervention. CONCLUSIONS: PTI is a simplified and safe technique to address quadriceps insufficiency when performing DFEO. The short-term results of patients who underwent DFEO with PTI demonstrated improvements in clinical, radiographic, and gait analysis variables of the knee. Investigating long-term outcomes, comparing techniques, and assessing quality of life measures are important next steps in research. LEVEL OF EVIDENCE: Level IV-case series.


Subject(s)
Femur/surgery , Gait Disorders, Neurologic/surgery , Osteotomy , Patellar Ligament/surgery , Quadriceps Muscle/physiopathology , Adolescent , Cerebral Palsy/complications , Child , Contracture/etiology , Contracture/physiopathology , Contracture/surgery , Female , Gait , Gait Analysis , Gait Disorders, Neurologic/etiology , Gait Disorders, Neurologic/physiopathology , Humans , Knee Joint/diagnostic imaging , Knee Joint/physiopathology , Male , Muscle Strength , Patella/diagnostic imaging , Patella/physiopathology , Postoperative Period , Preoperative Period , Range of Motion, Articular , Retrospective Studies , Young Adult
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