RESUMEN
BACKGROUND: Happiness, comfort, and motor function contribute to satisfaction with life for individuals with cerebral palsy (CP). Evidence-based medical care can improve motor function and physical health of youth with CP. Less is known about medical care and its relationship to health-related quality of life (HRQOL) in adolescents and young adults with CP. This study aimed to describe HRQOL among adolescents with CP to examine differences between adolescent (self) and parent (proxy) reports of HRQOL and to explore associations of pain, age, and gross motor function with HRQOL. METHODS: This is a retrospective study including adolescents with CP classified as Gross Motor Function Classification System levels I to V, ages 11 to 20 years, reading ≥ a fourth-grade level, and who completed the self-reported Pediatric Outcomes Data Collection Instrument (PODCI). Parents completed the PODCI concurrently or within 12 months and scores were compared. In addition, self-reported scores were compared between age bands, across Gross Motor Function Classification System levels, with typically developing youth (TDY), and between youth with/without pain. RESULTS: PODCI scores from 102 adolescents [59 males; 15.0 (SD: 2.6) years old] were examined. Scores from 50 adolescents and parents were matched. Mean self-reported scores were significantly higher than mean parent-reported scores in 4 domains: upper extremity and physical function ( P =0.018), sports and physical function ( P =0.005), happiness ( P =0.023), and global functioning ( P =0.018). All domains, except Happiness, were significantly < TDY ( P <0.01). The presence of pain was associated with lower scores in all domains ( P <0.05). CONCLUSION: Examining HRQOL with the PODCI revealed significant limitations in physical function and higher pain in adolescents with CP compared with TDY. Self- and parent-reported PODCI results should be considered separately. Adolescents report higher HRQOL compared with parent proxy. Recognizing and validating the perspectives of youth and their parents presents an opportunity for providers to discuss different points of view with families. Such engagement can help promote self-efficacy in youth with CP as they transition to the responsibility of guiding their own care in adulthood. LEVEL OF EVIDENCE: III, Retrospective comparative study.
Asunto(s)
Parálisis Cerebral , Masculino , Adulto Joven , Niño , Humanos , Adolescente , Preescolar , Calidad de Vida , Autoinforme , Estudios Retrospectivos , Dolor/etiologíaRESUMEN
AIM: To report survival probability of a large cohort of children with cerebral palsy (CP) after spinal fusion. METHOD: All children with CP who had spinal fusion between 1988 and 2018 at the reporting facility were reviewed for survival. Death records of the institutional CP database, institutional electronic medical records, publicly available obituaries, and the National Death Index through the US Centers for Disease Control were searched. Survival probabilities with different surgical eras, comorbidities, ages, and curve severities were compared using Kaplan-Meier curves. RESULTS: A total of 787 children (402 females, 385 males) had spinal fusion at a mean age of 14 years 1 month (standard deviation 3 years 2 months). The 30-year estimated survival was approximately 30%. Survival decreased for children who had spinal fusion at younger ages, longer postoperative hospital stays, longer postoperative intensive care unit stays, gastrostomy tubes, and pulmonary comorbidities. INTERPRETATION: Children with CP who required spinal fusions had reduced long-term survival compared with an age-matched typically developing cohort; however, a substantial number survived 20 to 30 years after the surgery. This study had no comparison group of children with CP scoliosis; therefore, we do not know whether correction of scoliosis affected their survival.
Asunto(s)
Parálisis Cerebral , Escoliosis , Fusión Vertebral , Masculino , Femenino , Humanos , Niño , Adolescente , Estudios de Seguimiento , Estudios Retrospectivos , Parálisis Cerebral/cirugía , Escoliosis/cirugía , Resultado del Tratamiento , ParálisisRESUMEN
BACKGROUND: The Shriners Hospital Upper Extremity Evaluation (SHUEE) is a video-based measure designed to assess upper extremity function in people with cerebral palsy (CP). The SHUEE completes both dynamic positional analysis (DPA; position during functional activities) and spontaneous functional analysis (spontaneous use of the involved limb). Although the SHUEE has been suggested as a measure for planning upper limb interventions and evaluating outcomes, limited evidence of its ability to detect change exists. Thus, this study aimed to describe responsiveness of the SHUEE to detect change after orthopaedic surgery. METHODS: In this Institutional Review Board-approved retrospective cohort study, we identified children with CP who were administered SHUEE on≥2 encounters. We formed pairs of initial and follow-up visits between temporally adjacent visits. Pairs were assigned to a surgery or non-surgery group based on intervening upper limb orthopaedic surgery. We compared differences in baseline SHUEE scores between groups and differences in temporally adjacent SHUEE scores within groups using Welch unequal variances t tests and paired t tests, respectively. RESULTS: Nineteen people (7 female) with hemiplegic CP had≥2 SHUEE assessments; Manual Ability Classification System levels I (3), II (8), III (7), IV (1); Gross Motor Function Classification System levels I (10), II (7), IV (2); mean age at baseline 11.9 (5.1 to 19.1) years; and follow-up at 13.4 (5.5 to 19.7) years. Six people had≥2 visits leading to 14 surgical pairs and 10 non-surgical pairs. At baseline, DPA of the wrist and forearm were significantly lower in the surgical group ( P <0.05). At follow-up, no significant difference between the groups existed in DPA measures ( P >0.05). After surgical intervention, there was a significant change in overall and wrist DPA ( P <0.05). CONCLUSIONS: The DPA measures demonstrated responsiveness to expected positional changes in the arm after orthopaedic surgery in people with CP. The SHUEE was useful in identifying abnormal segmental alignment pre-surgically and documenting changes in alignment postoperatively. As orthopaedic surgery does not address limb neglect or bimanual ability, spontaneous functional analysis scores were as expected-unchanged. LEVEL OF EVIDENCE: Level III, retrospective cohort study.
Asunto(s)
Parálisis Cerebral , Procedimientos Ortopédicos , Niño , Humanos , Femenino , Adolescente , Adulto Joven , Adulto , Estudios Retrospectivos , Hemiplejía/diagnóstico , Hemiplejía/etiología , Extremidad SuperiorRESUMEN
PURPOSE: To examine the role of multiple factors, including therapy dose, on recovery of mobility function during post-single-event multilevel surgery (SEMLS) rehabilitation in youth with cerebral palsy. METHODS: Several factors expected to influence postoperative change in Gross Motor Function Measure (GMFM) were examined: age, Gross Motor Function Classification System (GMFCS) level, cognition, number of osteotomies, surgical complications, medical comorbidities, number of therapy sessions, and preoperative measures of gait, balance, and gross motor function. RESULTS: Sixty-nine youth with cerebral palsy, GMFSC levels I-IV, who had undergone SEMLS and rehabilitation had on average 2.6 osteotomies and 89 postoperative therapy sessions. Fewer osteotomies, higher therapy dose, higher preoperative GMFM, and lower GMFM at postoperative admission were significant in determining GMFM change. CONCLUSIONS: The most relevant factors on post-SEMLS recovery were therapy dose, surgical burden, and level of gross motor function immediately before and after surgery.
Asunto(s)
Parálisis Cerebral , Adolescente , Humanos , Niño , Resultado del Tratamiento , Parálisis Cerebral/rehabilitación , Marcha , Periodo Posoperatorio , Destreza MotoraRESUMEN
AIM: To determine how surgical burden and preoperative factors affect the recovery of walking activity after multilevel orthopedic surgery (MLS). METHOD: In this retrospective study, inclusion criteria were a diagnosis of cerebral palsy, MLS, and walking activity monitoring using a StepWatch device within 12 months pre-MLS and 24 months post-MLS. The outcome measure was total mean strides per day normalized to age and Gross Motor Function Classification System level. Pre- and postoperative walking activity were compared using unpaired t-tests; the effects of preoperative predictors and surgical burden on the recovery of walking activity were evaluated using regression analysis. RESULTS: Participants included 178 children (mean age 12 years 10 months [SD 8 years 7 months; range 4-20 years]; 91 males, 87 females). On average, children returned to baseline walking activity 3 months after low-burden surgery and 1 year 2 months after high-burden surgery. Postoperative walking activity was higher for children who had surgery at a younger age and those with a higher preoperative mobility function. INTERPRETATION: The burden of MLS was found to be inversely related to the time to recovery of postoperative walking activity. These findings provide evidence to help clinicians set expectations for return to function post-MLS. Further study is necessary to investigate the impact of postoperative factors on walking activity recovery. WHAT THIS PAPER ADDS: High-burden surgeries lead to longer recovery than low-burden surgeries. Younger children recover walking activity faster after multilevel orthopedic surgery. Children with high preoperative mobility function recover walking activity faster after surgery.
OBJETIVO: Determinar como a carga cirúrgica e os fatores pré-operatórios afetam a recuperação da atividade de caminhada após cirurgia ortopédica multinível (MLS). MÉTODOS: Neste estudo retrospectivo, os critérios de inclusão foram um diagnóstico de paralisia cerebral, MLS e monitoramento da atividade de caminhada usando um dispositivo Step Watch dentro de 12 meses pré-MLS e 24 meses pós-MLS. A medida de resultado foi o total de passos médios por dia normalizados para idade e nível do Sistema de Classificação da Função Motora Grossa. A atividade de caminhada pré e pós-operatória foi comparada usando testes t não pareados; os efeitos dos preditores pré-operatórios e da carga cirúrgica na recuperação da atividade de caminhada foram avaliados por meio de análise de regressão. RESULTADOS: Os participantes incluíram 178 crianças (idade média de 12 anos e 10 meses [DP 8 anos e 7 meses; intervalo de 4 a 20 anos]; 91 meninos, 87 meninas). Em média, as crianças retornaram à atividade de caminhada inicial 3 meses após a cirurgia de baixa carga e 1 ano e 2 meses após a cirurgia de alta carga. A atividade de caminhada pós-operatória foi maior para crianças que foram operadas em idade mais jovem e aquelas com maior função de mobilidade pré-operatória. INTERPRETAÇÃO: A carga de MLS foi inversamente relacionada ao tempo de recuperação da atividade de caminhada pós-operatória. Esses achados fornecem evidências objetivas para ajudar os médicos a definir as expectativas de retorno à função pós-MLS. Mais estudos são necessários para investigar o impacto dos fatores pós-operatórios na recuperação da atividade de caminhada.
Asunto(s)
Parálisis Cerebral , Procedimientos Ortopédicos , Parálisis Cerebral/cirugía , Niño , Femenino , Humanos , Lactante , Masculino , Evaluación de Resultado en la Atención de Salud , Estudios Retrospectivos , CaminataRESUMEN
BACKGROUND: The Ponseti method effectively treats idiopathic clubfoot, but its effectiveness in treating the stiffer clubfoot associated with arthrogryposis is less clear. The purpose of this study was to assess the comparative effectiveness of the Ponseti method in 5-year-old children with either idiopathic clubfoot or clubfoot due to arthrogryposis. METHODS: The outcomes of the Ponseti method were retrospectively evaluated in children with idiopathic clubfoot and clubfoot associated with arthrogryposis. The children with clubfoot were seen at our hospital between 2012 and 2019 and were 4.0 to 6.9 years old at the time of their evaluation. Outcomes of the 2 groups of children with clubfoot were assessed using passive range of motion, foot pressure analysis, the Gross Motor Function Measure Dimension-D, and parent report using the Pediatric Outcomes Data Collection Instrument. These results were also compared with the same measures from a group of typically developing children. Surgical and bracing history was also recorded. RESULTS: A total of 117 children were included (89 idiopathic clubfoot and 28 associated with arthrogryposis) with an average age of 4.8±0.8 years. The historical gait analyses of 72 typically developing children were used as a control, with an average age of 5.2±0.8 years. Significant residual equinovarus was seen in both children with idiopathic clubfoot and associated with arthrogryposis according to passive range of motion and foot pressure analysis when compared with normative data. Children with arthrogryposis demonstrated limited transfer and basic mobility, sports functioning, and global functioning while children with idiopathic clubfoot were significantly different from their typically developing peers in only transfer and basic mobility. CONCLUSIONS: Although children with idiopathic clubfoot continue with some level of residual deformity, the Ponseti method is effective in creating a pain-free, highly functional foot. In children with clubfoot associated with arthrogryposis, the Ponseti method is successful in creating a braceable foot that can delay the need for invasive surgical intervention. LEVEL OF EVIDENCE: Level III, Therapeutic Studies-Investigating the Results of Treatment.
Asunto(s)
Artrogriposis , Moldes Quirúrgicos , Pie Equinovaro , Procedimientos Ortopédicos , Tenotomía , Articulación del Tobillo/fisiopatología , Artrogriposis/complicaciones , Artrogriposis/fisiopatología , Artrogriposis/terapia , Preescolar , Pie Equinovaro/complicaciones , Pie Equinovaro/fisiopatología , Pie Equinovaro/terapia , Pie Equino/diagnóstico , Pie Equino/etiología , Femenino , Análisis de la Marcha , Humanos , Masculino , Procedimientos Ortopédicos/efectos adversos , Procedimientos Ortopédicos/instrumentación , Procedimientos Ortopédicos/métodos , Evaluación de Resultado en la Atención de Salud/métodos , Evaluación de Resultado en la Atención de Salud/estadística & datos numéricos , Rango del Movimiento Articular , Recuperación de la Función , Estudios Retrospectivos , Tenotomía/efectos adversos , Tenotomía/métodosRESUMEN
Rhizomelic chondrodysplasia punctata (RCDP) is a class of peroxisomal disorders characterized by defective plasmalogen biosynthesis. There are multiple recognized types of RCDP, all of which have autosomal recessive inheritance, and their associated genes are known: RCDP type 1 with PEX7, RCDP type 2 with GNPAT, RCDP type 3 with AGPS, RCDP type 4 with FAR1, and RCDP type 5 with PEX5. Among other medical/developmental issues, plasmalogen deficiency has a direct effect on bone growth and results in postnatal growth failure, the severity of which corresponds to the degree of plasmalogen deficiency. In order to document growth in patients with RCDP, we present detailed growth curves for length, weight, and head circumference derived from retrospective data from 23 individuals with RCDP types 1 and 2 confirmed by molecular and/or biochemical studies. We stratified growth curves by age as well as by plasmalogen level, with those with higher plasmalogens grouped as "non-classic." The growth charts presented here provide guidance to families and physician caretakers on the natural course of growth in individuals with RCDP during infancy into early childhood, and thus will have particular utility in setting expectations and guiding optimal feeding interventions in this population.© 2016 Wiley Periodicals, Inc.
Asunto(s)
Condrodisplasia Punctata Rizomélica/diagnóstico , Estudios de Asociación Genética , Gráficos de Crecimiento , Adolescente , Peso Corporal , Niño , Preescolar , Condrodisplasia Punctata Rizomélica/genética , Femenino , Humanos , Lactante , Masculino , Sistema de Registros , Estudios RetrospectivosRESUMEN
BACKGROUND: Excessive hip internal rotation is frequently seen in children with cerebral palsy (CP). Femoral derotational osteotomy (FDO) is effective in the short term, but factors associated with long-term correction remain unclear. The purposes of this study were to define the incidence of persistence and recurrence of hip internal rotation following FDO in ambulatory children with CP and to evaluate factors that influence outcome. METHODS: Following IRB approval, kinematic and passive range of motion (PROM) variables were retrospectively evaluated in children with spastic CP who had FDO to correct hip internal rotation as part of clinical care at a children's specialty hospital. Children included had a preoperative evaluation (Vpre), a short-term postoperative evaluation (Vshort, 1 to 3 y post), and, in some cases, a long-term postoperative evaluation (Vlong, ≥5 y post). Age at surgery, physical exam measures, and kinematics variables were evaluated as predictors for dynamic and static recurrence. RESULTS: Kinematic hip rotation improved from 14±12 degrees (Vpre; internal positive) to 4±13 degrees (Vshort) and relapsed to 9±15 degrees long term (P<0.05 Vpre/Vshort/Vlong; 99 limbs). Hip PROM midpoint improved from 23±9 degrees (Vpre) to 8±11 degrees (Vshort) and relapsed to 14±13 degrees (P<0.01 Vpre/Vshort/Vlong). Persistent hip internal rotation was noted in 41% (kinematics) and 18% (PROM) of limbs at Vshort (105 children, 178 limbs). Of limbs that showed initial improvement at Vshort (62 children, 95 limbs), recurrence was seen in 40% (kinematic hip rotation) and 39% (hip midpoint) at Vlong. Comparing children who had recurrent hip internal rotation and those who maintained long-term correction, we saw higher levels of spasticity and lower gait velocity in the recurrent group (P<0.05). CONCLUSIONS: Although FDO is an accepted treatment in children with CP, persistence and recurrence of hip internal rotation can occur. Recurrence is associated with spasticity and slower gait velocity. Predictor variables may be useful for surgeons during preoperative discussions of expected outcome with families of FDO candidates. LEVEL OF EVIDENCE: Level III.
Asunto(s)
Parálisis Cerebral/complicaciones , Fémur/anomalías , Trastornos Neurológicos de la Marcha/cirugía , Osteotomía/métodos , Anomalía Torsional/cirugía , Adolescente , Niño , Femenino , Trastornos Neurológicos de la Marcha/etiología , Humanos , Masculino , Espasticidad Muscular/complicaciones , Periodo Posoperatorio , Rango del Movimiento Articular , Recurrencia , Estudios Retrospectivos , Anomalía Torsional/etiología , Resultado del TratamientoRESUMEN
BACKGROUND: External tibial torsion (ETT) is a common bony deformity in children with cerebral palsy (CP). The current recommended treatment is tibial derotation osteotomy (TDO) to improve gait biomechanics. Satisfactory short-term results after TDO have been reported but long-term results have not been studied. The purpose of this study was to evaluate the long-term outcome following TDO to correct ETT in ambulatory children with CP. METHODS: Following IRB approval, gait kinematics and passive range of motion measurements were retrospectively evaluated in children with spastic CP who underwent TDO due to ETT comparing preoperative (E0), short-term postoperative (E1; 1 to 3 y post), and long-term postoperative (E2; >5 y post) results. Limbs were categorized as corrected, undercorrected, or overcorrected at both E1 and E2, by comparing mean tibial rotation (MTR) in gait to a group of typically developing children. Age at surgery, E0 MTR, E0 gait velocity, gross motor function classification system (GMFCS) score, and foot deformity were evaluated to determine their influence on long-term results. RESULTS: The study sample consisted of 43 legs (with E0 and E2) and 22 legs (with E0, E1, and E2). The mean age at surgery was 10.3±3.4 years (range, 6 to 19.2 y). In the group MTR trended toward improvement moving from -26±17 degrees (E0, external negative) to -16±16 degrees (E1) and relapsed to -23±17 degrees at the long term (P=0.071, E0/E1; P=0.589, E0/E2). Improvement was also seen in the transmalleolar axis (P=0.074), mean ankle rotation, and mean foot orientation (P<0.05, E0/E2). At the long-term evaluation, 16 legs (37%) were found to be in the kinematic corrected group, 25 legs (58%) in the kinematic undercorrected group, and 2 legs (5%) in the kinematic overcorrected group. There were no significant differences between the corrected and undercorrected groups of children with respect to age at surgery, GMFCS, E0 MTR, gait velocity, or foot deformity. CONCLUSIONS: Although internal TDO improves ETT in the short term, recurrence is frequent with an apparent developmental trend toward external rotation of the tibia. LEVELS OF EVIDENCE: Level IV-therapeutic study.
Asunto(s)
Parálisis Cerebral/complicaciones , Marcha , Osteotomía/métodos , Tibia/anomalías , Anomalía Torsional/cirugía , Adolescente , Adulto , Parálisis Cerebral/fisiopatología , Niño , Preescolar , Femenino , Humanos , Masculino , Periodo Posoperatorio , Estudios Retrospectivos , Tibia/fisiopatología , Tibia/cirugía , Anomalía Torsional/etiología , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Pediatric pedestrian motor vehicle-associated of injuries correlated with a particular census tract's trauma is a significant public health concern for children. demographic composition. GIS mapping software was used We aimed to use geographic information systems (GIS) to examine the relationship between motor vehicle pedestrian injuries in children and the demographics of the region in which they occurred for the state of Delaware. METHODS: This is a retrospective analysis of collected data from the Delaware State Trauma Registry form January 1, 2002, to December 31, 2012. The records of all patients younger than 18 years who went to one of the state's six trauma centers during the study were reviewed. For each injury event, patient demographic information was recorded, and latitude/longitude coordinates of the injury site were determined. Median income, minority population, education level, and percentage of males and children in the census tract were obtained from state census data. Analysis of variance was used to characterize how the frequency of injuries correlated with a particular census tract's demographic composition. GIS mapping software was used to identify specific "hot spots" throughout the state where the examine the relationship between motor vehicle pedestrian frequency of traffic crash events was the highest. RESULTS: Urban and poorer areas had tile highest number of injury events, with Wilmington having the highest frequency Methods: This is a retrospective analysis of collected data of injuries per capita. Census tracts with low median income, from the Delaware State Trauma Registry from January 1, lack of high school degree, and increased percentage of 2002, to December 31, 2012. The records of all patients African Americans and females had significantly higher injury younger than 18 years who went to one of the state's six counts compared with other census tracts. CONCLUSIONS: In the state of Delaware, children in urban and poor areas are disproportionately affected by motor vehicle-associated pedestrian injuries. Specific risk factors for accidents in these areas need to be identified to facilitate the development of focused prevention strategies.
Asunto(s)
Accidentes de Tránsito/estadística & datos numéricos , Sistemas de Información Geográfica , Peatones , Niño , Delaware/epidemiología , Femenino , Humanos , Masculino , Estudios Retrospectivos , Factores de RiesgoRESUMEN
Purpose: Foot deformities are prevalent in children with cerebral palsy, but there is limited research on the progression of foot posture during growth. Our study aimed to evaluate the change in dynamic foot posture in children with cerebral palsy. Methods: Children with cerebral palsy, aged 17-40 months, were recruited to participate in this Institutional Review Board-approved prospective longitudinal study by having serial foot posture evaluations. The coronal plane index and foot segmental impulses were measured with dynamic pedobarography. Data were compared between children stratified by Gross Motor Function Classification System level and typically developing children using serial Welch's t-tests across time with Holm correction for multiple comparisons. Results: In total, 33 children (54 limbs) were included in the analysis (21 bilateral and 12 unilateral; Gross Motor Function Classification System: I-13, II-14, III-4, IV-2. Children completed 16.9 (± 4.4) evaluations (initial age 2.9 (± 0.7) and final age 18.6 (± 1.7) years)). Early valgus foot posture normalizes in children at Gross Motor Function Classification System levels I/II and persists in children at levels III/IV who do not have foot surgery. For most young children, foot posture development is variable. Conclusion: Foot posture in young children with cerebral palsy begins in valgus and tends to normalize in youth who walk without an assistive device. Conservative management of foot deformity is recommended in early childhood. Level of evidence: Level II, prognostic study.
RESUMEN
Microcephalic primordial dwarfism (MPD) is a class of disorders characterized by intrauterine growth restriction (IUGR), impaired postnatal growth and microcephaly. Majewski osteodysplastic primordial dwarfism type II (MOPD II) is one of the more common conditions within this group. MOPD II is caused by truncating mutations in pericentrin (PCNT) and is inherited in an autosomal recessive manner. Detailed growth curves for length, weight, and OFC are presented here and derived from retrospective data from 26 individuals with MOPD II confirmed by molecular or functional studies. Severe pre- and postnatal growth failure is evident in MOPD II patients. The length, weight, and OFC at term (when corrected for gestational age) were -7.0, -3.9, and -4.6 standard deviation (SD) below the population mean and equivalent to the 50th centile of a 28-29-, 31-32-, and 30-31-week neonate, respectively. While at skeletal maturity, the height, weight, and OFC were -10.3, -14.3, and -8.5 SD below the population mean and equivalent to the size of 3-year 10- to 11-month-old, a 5-year 2- to 3-month-old, and 5- to 6-month-old, respectively. During childhood, MOPD II patients grow with slowed, but fairly constant growth velocities and show no evidence of any pubertal growth spurt. Treatment with human growth hormone (n = 11) did not lead to any significant improvement in final stature. The growth charts presented here will be of assistance with diagnosis and management of MOPD II, and should have particular utility in nutritional management of MOPD II during infancy.
Asunto(s)
Antígenos , Enanismo/diagnóstico , Enanismo/genética , Retardo del Crecimiento Fetal/diagnóstico , Retardo del Crecimiento Fetal/genética , Gráficos de Crecimiento , Microcefalia/diagnóstico , Microcefalia/genética , Mutación , Osteocondrodisplasias/diagnóstico , Osteocondrodisplasias/genética , Adolescente , Adulto , Estatura , Peso Corporal , Niño , Preescolar , Enanismo/tratamiento farmacológico , Femenino , Retardo del Crecimiento Fetal/tratamiento farmacológico , Hormona de Crecimiento Humana/uso terapéutico , Humanos , Masculino , Microcefalia/tratamiento farmacológico , Osteocondrodisplasias/tratamiento farmacológico , Adulto JovenRESUMEN
BACKGROUND: Previous studies have looked at the short-term effectiveness of conservative and surgical treatment of children with cerebral palsy (CP), but few have explored the long-term outcomes into adulthood using gait analysis and patient-reported outcome measures. RESEARCH QUESTION: How do gait, mobility, and patient-reported outcomes in adults with CP who received specialized pediatric orthopedic care change from adolescence? METHODS: We identified 645 adults with 1) CP, 2) age 25-45 years, and 3) an adolescent instrumented gait analysis (IGA) at our center. Measurement outcomes included physical examination, IGA, and select domains of the Patient-Reported Outcomes Measurement Information System (PROMIS). RESULTS: Participants included 136 adults with CP; Gross Motor Function Classification System levels I (21 %), II (51 %), III (22 %), and IV (7%); 57 % males; and average age 16 ± 3/29 ± 3 years (adolescent/adult visits). There was no significant difference in gait deviation index, stride length, or gross motor function between adolescent and adult visits. There were statistically significant but not clinically meaningful declines in gait velocity. At adulthood, PROMIS results revealed limitations in physical function compared with a normative sample but no differences in depression, participation, or pain interference. SIGNIFICANCE: In this relatively homogeneous group of adults with CP who received orthopedic care from one center, gait and gross motor function showed no clinically meaningful change from adolescence, which differs from recent reports of declining mobility in adulthood. Expert orthopedic care, guided by IGA, may prevent losses in functional mobility for adults with CP.
Asunto(s)
Parálisis Cerebral , Trastornos Neurológicos de la Marcha , Adolescente , Adulto , Parálisis Cerebral/complicaciones , Niño , Femenino , Marcha , Análisis de la Marcha , Trastornos Neurológicos de la Marcha/etiología , Humanos , Masculino , Persona de Mediana Edad , Medición de Resultados Informados por el PacienteRESUMEN
BACKGROUND: This prospective study used instrumented gait analysis, patient-reported outcomes, and portable accelerometers to examine walking activity in adults with cerebral palsy (CP). RESEARCH QUESTION: This study aimed to provide objective data and evaluate factors associated with walking activity in adults with CP. METHODS: Participants with CP (ages 25-45 years) completed instrumented gait analysis and patient-reported outcomes, including the Patient Reported Outcome Measurement Information System (PROMIS) and Satisfaction with Life Score (SWLS), and wore a StepWatch for 8 days. Average strides per day, stratified by Gross Motor Function Classification System (GMFCS), were compared with nondisabled adults ages 30-39 years utilizing Welch's t-tests with Bonferroni corrections. Correlation coefficients and stepwise multiple linear regression analyses examined relationships between walking activity and GMFCS, gait deviation index (GDI), gait velocity, PROMIS physical function, SWLS, body mass index (BMI), and employment. RESULTS: Participants included 109 adults with CP, ages 29 ± 4 years, classified at GMFCS levels I/II (73 %) and III/IV (27 %). Compared with nondisabled adults, daily stride count was significantly lower in both groups of adults with CP (p < 0.00025), with a progressive decline according to GMFCS level. Walking activity correlated with PROMIS physical function (r = .42), GDI (r = .48), and gait velocity (r = .58). Association for employment was lower (r = 0.27) but significant, while age, SWLS, and BMI were not individually correlated with walking activity. Stepwise, multiple linear regression modeled with Akaike information criterion explained 40.9 % of the observed variability in walking activity in this cohort of adults with CP. SIGNIFICANCE: Physical function, as classified by GMFCS or measured by PROMIS and self-selected walking velocity, has the strongest association with and is the most significant predictor of walking activity in adults with CP. After accounting for physical function, a small amount of the variation in walking activity can be explained by GDI, employment, and age.
Asunto(s)
Parálisis Cerebral , Trastornos Neurológicos de la Marcha , Adulto , Marcha , Humanos , Persona de Mediana Edad , Estudios Prospectivos , CaminataRESUMEN
BACKGROUND: Microcephalic osteodysplastic primordial dwarfism type II (MOPDII) is the most common form of primordial dwarfism, caused by bialleic mutations in the pericentrin gene (PCNT). Aside from its classic features, there are multiple associated medical complications, including a well-documented risk of neurovascular disease. Over the past several years, it has become apparent that additional vascular issues, as well as systemic hypertension and kidney disease may also be related to MOPDII. However, the frequency and extent of the vasculopathy was unclear. To help address this question, a vascular substudy was initiated within our Primordial Dwarfism Registry. RESULTS: Medical records from 47 individuals, living and deceased, ranging in age from 3 to 41 years of age were interrogated for this purpose. Of the total group, 64% were diagnosed with moyamoya, intracranial aneurysms, or both. In general, the age at diagnosis for moyamoya was younger than aneurysms, but the risk for neurovascular disease was throughout the shortened lifespan. In addition to neurovascular disease, renal, coronary and external carotid artery involvement are documented. 43% of the total group was diagnosed with hypertension, and 17% had myocardial infarctions. A total of 32% of the entire cohort had some form of chronic kidney disease, with 4% of the total group necessitating a kidney transplant. In addition, 38% had diabetes/insulin resistance. Ages of diagnoses, treatment modalities employed, and location of vasculopathies were notated as available and applicable, as well as frequencies of other comorbidities. CONCLUSIONS: It is now clear that vascular disease in MOPDII is global and screening of the cardiac and renal vessels is warranted along with close monitoring of blood pressure. We recommend a blood pressure of 110/70 mmHg as a starting point for an upper limit, especially if the individual has a history of neurovascular disease, chronic kidney disease and/or diabetes. Additionally, providers need to be at high alert for the possibility of myocardial infarctions in young adults with MOPDII, so that appropriate treatment can be initiated promptly in an acute situation.
Asunto(s)
Enanismo , Microcefalia , Osteocondrodisplasias , Enfermedades Vasculares , Adolescente , Adulto , Niño , Preescolar , Enanismo/genética , Retardo del Crecimiento Fetal , Humanos , Osteocondrodisplasias/genética , Enfermedades Vasculares/genética , Adulto JovenRESUMEN
The effect of a continuous intrathecal infusion of baclofen (CITB) was retrospectively studied in 19 ambulatory children with cerebral palsy (aged 12.4±4.9 years at CITB initiation). The mean clinical follow-up was 5.1±2.4 years and the mean follow-up gait analysis was 2.8±1.9 years. Spastic cerebral palsy diagnosis [14 (74%)] was most frequent. Most patients [11 (58%)] were Gross Motor Function Classification System level III. CITB significantly improved muscle tone and knee flexion at initial contact (P<0.05), but it did not lead to improved gait speed or gross motor function.
Asunto(s)
Baclofeno/administración & dosificación , Parálisis Cerebral/diagnóstico , Parálisis Cerebral/tratamiento farmacológico , Relajantes Musculares Centrales/administración & dosificación , Caminata/fisiología , Adolescente , Parálisis Cerebral/fisiopatología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Inyecciones Espinales , Masculino , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
This study aimed to assess the effects of plantar flexor lengthening (PFL) on dynamic foot pressures of children with cerebral palsy using pedobarographs. Of 97 enrolled, 13 children with 18 legs had PFL. Age at surgery was 4.7 (2.8-8.8) years. A significant increase in ankle dorsiflexion and heel impulse was achieved postoperatively and was maintained at 5 years. The coronal plane pressure index increased postoperatively, but reverted to preoperative levels at the 5-year follow-up. Children tend to have more valgus after PFL. In young children, there caution should be exercised to avoid over treating varus at the time of equinus correction to avoid overcorrection.
Asunto(s)
Parálisis Cerebral/cirugía , Pie/fisiología , Pie/cirugía , Músculo Esquelético/fisiología , Músculo Esquelético/cirugía , Caminata/fisiología , Parálisis Cerebral/fisiopatología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Estudios Longitudinales , Masculino , Procedimientos Ortopédicos/efectos adversos , Procedimientos Ortopédicos/tendencias , Presión , Estudios Prospectivos , Resultado del TratamientoRESUMEN
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.
Asunto(s)
Pie/fisiología , Marcha/fisiología , Modelos Anatómicos , Adolescente , Adulto , Fenómenos Biomecánicos , Femenino , Humanos , Masculino , Rango del Movimiento Articular/fisiología , Reproducibilidad de los Resultados , Adulto JovenRESUMEN
Crouched gait is common in children with cerebral palsy (CP), and there are various treatment options. This study evaluated the effectiveness of single-event multilevel surgery including posterior knee capsulotomy or distal femoral extension osteotomy to correct knee flexion contracture in children with CP. Gait analyses were carried out to evaluate gait preoperatively and postoperatively. Significant improvements were found in physical examination and kinematic measures, which showed that children with CP and crouched gait who develop knee flexion contractures can be treated effectively using single-event multilevel surgery including a posterior knee capsulotomy or distal femoral extension osteotomy.
Asunto(s)
Parálisis Cerebral/cirugía , Contractura/cirugía , Trastornos Neurológicos de la Marcha/cirugía , Marcha , Articulación de la Rodilla/cirugía , Osteotomía , Capsulotomía Posterior , Adolescente , Fenómenos Biomecánicos , Parálisis Cerebral/complicaciones , Niño , Investigación sobre la Eficacia Comparativa , Contractura/complicaciones , Femenino , Trastornos Neurológicos de la Marcha/complicaciones , Humanos , Masculino , Periodo Posoperatorio , Rango del Movimiento Articular , Estudios Retrospectivos , Resultado del Tratamiento , Infección de HeridasRESUMEN
BACKGROUND: Stress ultrasound (SUS) of the elbow has demonstrated changes in the anterior band of the ulnar collateral ligament (UCL) in professional and high school-aged pitchers. However, there have been no large reports correlating pitching history data with SUS changes in youth and adolescent baseball pitchers. HYPOTHESIS: Changes of the UCL on SUS will correlate with pitching volume in youth and adolescent baseball pitchers. STUDY DESIGN: Cross-sectional study; Level of evidence, 3. METHODS: SUS of the elbow was performed in both elbows of 102 youth and adolescent baseball pitchers. UCL thickness and the width of the ulnohumeral joint, at rest and with 150 N of valgus stress, were measured using a standardized, instrumented device. Demographic data, arm measurements, and a pitching history questionnaire were recorded as well. The pitchers were separated into 2 groups based on age: group 1 (12-14 years) and group 2 (15-18 years). SUS findings of the dominant elbows were compared between the 2 groups. Correlation analysis and linear regression were used to identify relationships between SUS findings and pitching history data. RESULTS: In all pitchers, the mean UCL thickness was 4.40 mm in the dominant elbow and 4.11 mm in the nondominant elbow (P =.03). There was no significant difference between elbows in any joint space characteristics. A comparison of group 1 versus group 2 demonstrated significant differences in UCL thickness (4.13 vs 4.96 mm; P < .001), resting joint space width (6.56 vs 4.04 mm; P < .001), and stressed joint space width (7.68 vs 4.07 mm; P < .001). There was no difference in the change in joint space width between the 2 groups (1.11 vs 0.76 mm; P = .05). The UCL was significantly thicker in pitchers who threw more than 67 pitches per appearance (4.69 vs 4.14 mm), who pitched more than 5 innings per appearance (4.76 vs 4.11 mm), and who had more than 5.5 years of pitching experience (4.71 vs 4.07 mm; P < .001). Linear regression demonstrated that age, weight, and pitches per appearance (R 2 = 0.114, 0.370, and 0.326, respectively) significantly correlated with UCL thickness. CONCLUSION: These findings suggest that UCL thickness increases as pitchers get older and heavier and as they increase their pitch volumes.