Motor Function Classification System Score Research Articles

Navigating being a young adult with cerebral palsy: a qualitative study.

Young adults with cerebral palsy (CP) face potential challenges. The transition to young adulthood is characterized by significant changes in roles and responsibilities. Furthermore, young adults with chronic conditions face a transfer from pediatric care to adult healthcare. This study explores how living with CP affects young adults in general, and specifically which psychosocial, medical and healthcare needs are particularly important during this phase of life. A qualitative study with data from individual, semi-structured, in-depth interviews with six young adults with CP (ages 21-31 years) were transcribed verbatim and analyzed. The participants were selected to provide a maximum variation in age, gender, Gross Motor Function Classification System score and educational background. A descriptive thematic analysis was used to explore patterns and identify themes. Three themes were identified: "Being a Young Adult", "Development in Physical Disability and New Challenges in Adulthood" and "Navigating the Healthcare System". The three themes emerged from 15 sub-themes. Our findings emphasized that young adults with CP faced psychosocial challenges in social relationships, participation in education and work settings and striving towards independence. The transition to young adulthood led to a series of new challenges that the young adults were not prepared for. Medical challenges included managing CP-related physical and cognitive symptoms and navigating adult health care services, where new physicians with insufficient knowledge regarding CP were encountered. The young adults with CP were not prepared for the challenges and changes they faced during their transition into adulthood. They felt that they had been abandoned by the healthcare system and lacked a medical home. Better transitional care is urgently needed to prepare them for the challenges in young adulthood.

Alternating Hemiplegia of Childhood: gastrointestinal manifestations and correlation with neurological impairments

BackgroundAlternating Hemiplegia of Childhood (AHC) is caused by mutations of the ATP1A3 gene which is expressed in brain areas that include structures controling autonomic, gastrointestinal, gut motility and GABAergic functions. We aimed to investigate, in a cohort of 44 consecutive AHC patients, two hypotheses: 1) AHC patients frequently manifest gastrointestinal, particularly motility, problems. 2) These problems are often severe and their severity correlates with neurological impairments.Results41/44 (93%) exhibited gastrointestinal symptoms requiring medical attention. For these 41 patients, symptoms included constipation (66%), swallowing problems (63%), vomiting (63%), anorexia (46%), diarrhea (44%), nausea (37%), and abdominal pain (22%). Symptoms indicative of dysmotility occurred in 33 (80%). The most common diagnoses were oropharyngeal dysphagia (63%) and gastroesophageal reflux (63%). 16 (39%) required gastrostomy and two fundoplication. Severity of gastrointestinal symptoms correlated with non-paroxysmal neurological disability index, Gross Motor Function Classification System scores, and with the presence/absence of non-gastrointestinal autonomic dysfunction (p = 0.031, 0.043, Spearman correlations and 0.0166 Cramer’s V, respectively) but not with the paroxysmal disability index (p = 0.408).ConclusionsMost AHC patients have gastrointestinal problems. These are usually severe, most commonly are indicative of dysmotility, often require surgical therapies, and their severity correlates with that of non-paroxysmal CNS manifestations. Our findings should help in management-anticipatory guidance of AHC patients. Furthermore, they are consistent with current understandings of the pathophysiology of AHC and of gastrointestinal dysmotility, both of which involve autonomic and GABAergic dysfunction.

Structural network performance for early diagnosis of spastic cerebral palsy in periventricular white matter injury

Periventricular white matter injury (PWMI) is a common cause of spastic cerebral palsy (SCP). Diffusion tensor imaging (DTI) shows high sensitivity but moderate specificity for predicting SCP. The limited specificity may be due to the diverse and extensive brain injuries seen in infants with PWMI. We enrolled 72 infants with corrected age from 6 to 18months in 3 groups: PWMI with SCP (n = 20), non-CP PWMI (n = 19), and control (n = 33) groups. We compared DTI-based brain network properties among the three groups and evaluated the diagnostic performance of brain network properties for SCP in PWMI infants. Our results show abnormal global parameters (reduced global and local efficiency, and increased shortest path length), and local parameters (reduced node efficiency) in the PWMI with SCP group. On logistic regression, the combined node efficiency of the bilateral precentral gyrus and right middle frontal gyrus had a high sensitivity (90%) and specificity (95%) for differentiating PWMI with SCP from non-CP PWMI, and significantly correlated with the Gross Motor Function Classification System scores. This study confirms that DTI-based brain network has great diagnostic performance for SCP in PWMI infants, and the combined node efficiency improves the diagnostic accuracy.

Reducing opioid usage: a pilot study comparing postoperative selective dorsal rhizotomy protocols.

Selective dorsal rhizotomy (SDR) is a surgical procedure used to treat spasticity in children with spastic cerebral palsy. Currently, there is a lack of work examining the efficacy of optimizing pain management protocols after single-level laminectomy for SDR. This pilot study aimed to compare the clinical outcomes of SDR completed with a traditional pain management protocol versus one designed for opioid dosage reduction. The Texas Comprehensive Spasticity Center prospective database was queried for all patients who underwent SDR between 2015 and 2018. Demographic, surgical, and postoperative data for all patients who underwent SDR were collected from medical records. The study was designed as a retrospective study between the patient-controlled analgesia (PCA) and dexmedetomidine infusion (INF) groups with 80% power to detect a 50% difference at a significance level of 0.05. Patients in the INF group received perioperative gabapentin, intraoperative dexmedetomidine infusion, and scheduled acetaminophen and NSAIDs postoperatively. Medication administration records, pain scores, and therapy notes were collected for 30 patients. Patients who underwent SDR between June 2015 and the end of December 2017 received traditional pain management (PCA group, n = 14). Patients who underwent SDR between January 2018 and the end of December 2018 received modified pain management (INF group, n = 16). No patients were lost to follow-up. Differences in age, weight, height, preoperative Gross Motor Function Classification System scores, operative duration, hospital length of stay, and sex distribution were not statistically different between the 2 groups (p > 0.05). Analysis of analgesic medication doses demonstrated that the INF group required fewer doses and lower amounts of opioids overall, and also fewer NSAIDs than the PCA group. When converted to the morphine milligram equivalent, the patients in the INF group used fewer doses and lower amounts of opioids overall than the PCA group. These differences were either statistically significant (p < 0.05) or trending toward significance (p < 0.10). Both groups participated in physical and occupational therapy similarly postoperatively (p > 0.05). Pain scores were comparable between the groups (p > 0.05) despite patients in the INF group requiring fewer opioids. Infusion with dexmedetomidine during SDR surgery combined with perioperative gabapentin and scheduled acetaminophen and NSAIDs postoperatively resulted in similar pain scores to traditional pain management with opioids. In addition, this pilot study demonstrated that patients who received the INF pain management protocol required reduced opioid dosages and were able to participate in therapy similarly to the control PCA group.

Improvement in gross motor function and muscle tone in children with cerebral palsy related to neonatal icterus: an open-label, uncontrolled clinical trial

BackgroundAlthough stem cell transplantation has been successfully performed for cerebral palsy (CP) related to oxygen deprivation, clinical trials involving the use of stem cell transplantation for CP related to neonatal icterus have not been reported. The aim of this study was to evaluate the effectiveness of transplantation of autologous bone marrow mononuclear cell (BMMC) for improving gross motor function and muscle tone in children with CP related to neonatal icterus.MethodsThis open-label, uncontrolled clinical trial, which included 25 patients with CP related to neonatal icterus who had a Gross Motor Function Classification System (GMFCS) score between level II and level V, was conducted between July 2014 and July 2017 at Vinmec International Hospital (Vietnam). BMMC were harvested from the patients’ iliac crests. Two procedures involving BMMC transplantation via the intrathecal route were performed: the first transplantation was performed at baseline, and the second transplantation was performed 6 months after the first transplantation. Gross motor function and muscle tone were measured at three time points (baseline, 6 months, and 12 months) using the Gross Motor Function Measure (GMFM) and the Modified Ashworth Scale.ResultsIn this trial, we observed significant improvement in gross motor function and a significant decrease in muscle tone values. Total score on the 88-item GMFM (GMFM-88), scores on each GMFM-88 domain, and the 66-item GMFM (GMFM-66) percentile were significantly enhanced at 6 months and 12 months after the first transplantation compared with the corresponding baseline measurements (p-values < 0.05). In addition, a significant reduction was observed in muscle tone score after the transplantations (p-value < 0.05).ConclusionAutologous BMMC transplantation can improve gross motor function and muscle tone in children with CP related to neonatal icterus.Trial registrationClinicalTrials.gov identifier: NCT03123562. Retrospectively registered on December 26, 2017.

Accuracy of Activity Monitors for Measuring Ambulation in Children with Cerebral Palsy

Purpose: Commercially available activity tracking devices present the opportunity to quantify functional changes in ambulatory status of patients with cerebral palsy (CP). The purpose of this study was to assess the accuracy of a waist-based and a wrist-based wearable device for tracking steps and walking distance of children with CP in a controlled setting. Methods: Participants were 39 children, mean age of 9.6 years (range, 4-15), with CP and Gross Motor Function Classification System (GMFCS) score between I-III. Fifteen children …

Outcomes of Extremely Preterm Infants With Birth Weight Less Than 400 g

Birth weight (BW) is an important predictor of mortality and morbidity. At extremely early gestational ages (GAs), BW may influence decisions regarding initiation of resuscitation. To characterize outcomes of liveborn infants with a BW less than 400 g. This retrospective multicenter cohort study analyzed extremely preterm infants born between January 2008 and December 2016 within the National Institute of Child Health and Human Development Neonatal Research Network. Infants with a BW less than 400 g and a GA of 22 to 26 weeks were included. Active treatment was defined as the provision of any potentially lifesaving intervention after birth. Survival was analyzed for the entire cohort; neurodevelopmental impairment (NDI) was examined for those born between January 2008 and December 2015 (birth years with outcomes available for analysis). Neurodevelopmental impairment at 18 to 26 months' corrected age (CA) was defined as a Bayley Scales of Infant and Toddler Development, Third Edition, cognitive composite score less than 85, a motor composite score less than 85, moderate or severe cerebral palsy, gross motor function classification system score of 2 or greater, bilateral blindness, and/or hearing impairment. Data were analyzed from September 2017 to October 2018. Birth weight less than 400 g. The primary outcome was survival to discharge among infants who received active treatment. Analysis of follow-up data was limited to infants born from 2008 to 2015 to ensure children had reached assessment age. Within this cohort, neurodevelopmental outcomes were assessed for infants who survived to 18 to 26 months' CA and returned for a comprehensive visit. Of the 205 included infants, 121 (59.0%) were female, 133 (64.9%) were singletons, and 178 (86.8%) were small for gestational age. Almost half (101 of 205 [49.3%]) received active treatment at birth. A total of 26 of 205 infants (12.7%; 95% CI, 8.5-18.9) overall survived to discharge, and 26 of 101 actively treated infants (25.7%; 95% CI, 17.6-35.4) survived to discharge. Within the subset of infants with a BW less than 400 g and a GA of 22 to 23 weeks, 6 of 36 actively treated infants (17%; 95% CI, 6-33) survived to discharge. Among infants born between 2008 and 2015, 23 of 90 actively treated infants (26%; 95% CI, 17-36) survived to discharge. Two infants died after discharge, and 2 were lost to follow-up. Thus, 19 of 90 actively treated infants (21%; 95% CI, 13-31) were evaluated at 18 to 26 months' CA. Moderate or severe NDI occurred in 14 of 19 infants (74%). Infants born with a BW less than 400 g are at high risk of mortality and significant morbidity. Although 21% of infants survived to 18 to 26 months' CA with active treatment, NDI was common among survivors.

Oropharyngeal Dysphagia in Adults With Dyskinetic Cerebral Palsy and Cervical Dystonia: A Preliminary Study

ObjectivesTo investigate the characteristics of oropharyngeal dysphagia in adults with dyskinetic cerebral palsy (DCP) and cervical dystonia (CD). DesignExploratory observational cross-sectional study. SettingUniversity hospital. ParticipantsSeventeen patients with DCP (8 men, 9 women; age, 45.7±6.3y) enrolled in a randomized controlled trial on the effects of botulinum toxin injection on CD. InterventionsNot applicable. Main Outcome MeasuresBaseline clinical assessments and videofluoroscopic swallowing studies (VFSSs) were conducted. VFSS findings were evaluated using the videofluoroscopic dysphagia scale (VDS) and the penetration-aspiration scale (PAS). The Gross Motor Function Classification System (GMFCS) and Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores were also assessed. Relationships between outcomes were evaluated using Spearman’s rank correlation. ResultsThe clinical assessment revealed abnormalities in chewing (n=10, 58.8%), tongue movement (n=10, 58.8%), and laryngeal elevation (n=8, 47.1%). The most common abnormality on the VDS was inadequate mastication (n=13, 76.5%), followed by premature bolus loss, vallecular residue, and penetration/aspiration (all: n=10, 58.8%). A maximum PAS score of 8 was observed in 8 of 17 patients (47.1%). Total and pharyngeal VDS scores were significantly correlated with TWSTRS scores (ρ=0.543, P=.024 and ρ=0.539, P=.026, respectively); the VDS oral score did not correlate with the TWSTRS score (ρ=0.446, P=.073). There was no significant correlation between VDS score and GMFCS level (ρ=0.212, P=.414). ConclusionsThis preliminary observational study presents the characteristics of oropharyngeal dysphagia in adults with DCP and CD. Pharyngeal stage difficulties were negatively correlated with severity of CD, but not with GMFCS level. Screening for dysphagia may be recommended in adults with DCP and severe CD.

Simultaneous Selective Dorsal Rhizotomy and Baclofen Pump Removal Improve Ambulation in Patients with Spastic Cerebral Palsy.

Background: Intrathecal baclofen (ITB) administration via an implanted programmable pump and selective dorsal rhizotomy (SDR) are both used for the treatment of cerebral palsy (CP) spasticity.Objective: To examine whether SDR can improve ambulation in children who have been receiving ITB therapy for spastic cerebral palsy.Methods: We reviewed 13 patients who received prior ITB placement with subsequent simultaneous SDR and ITB removal. Patients also completed a follow-up survey to document long-term motor function.Results: In our 13-patient cohort, patients received ITB treatment for an average of 4.4 pm 1.8 years and the mean age of ITB removal/SDR was 12.5 pm 5.8 years. The follow-up period ranged from 3 to 19 months (mean duration: 6.9 pm 5 months). Pre-operatively, all patients had Gross Motor Function Classification System (GMFCS) scores between 2 and 4. Nine patients were diagnosed with spastic diplegia, two had spastic triplegia and two had spastic quadriplegia. SDR and ITB removal led to improved lower limb spasticity and ambulation. GMFCS scores remained stable in all patients. One patient developed a cerebrospinal fluid (CSF) collection in the abdominal wall due to a CSF leak from the baclofen pump site. All 11 patients who completed the follow-up survey noted improved motor function.Conclusion: This study demonstrates that SDR can reduce spasticity and improve mobility after years of ITB treatment for spastic cerebral palsy.

Clinical characteristics of children with cerebral palsy in Chengdu, China: A cross-sectional study

Objective: To describe the subtypes, motor function, and comorbidities of cerebral palsy (CP) in children in Chengdu, China.Methods: A cross-sectional survey of children with CP was performed in 2013 and participants were enrolled from the branches of China Disabled Persons’ Federation in Chengdu. Diagnosis, clinical subtypes, gross and fine motor function outcomes, and comorbidities of CP were determined through caregiver interviews, evaluation of medical records, and thorough physical examinations.Results: Four-hundred and twenty-two children were diagnosed with CP (mean age, 7.7 ± 3.8 years). Spastic diplegia was the predominating subtype (35%). Mild impairments in gross and fine motor function were present in 45% and 52% of the patients, respectively. Cognitive impairment (60%) was the most common comorbidity, and was followed by language disorder (41%) and microcephaly (35%). About 79% of children with cerebral palsy had at least one comorbidity, and the number of cumulative comorbidities was positively correlated with Gross Motor Function Classification System and Manual Ability Classification System scores. Preterm birth (52%) was the most common risk factor, and was followed by low birth weight (42%) and birth asphyxia (27%).Conclusion: Distributions of CP subtypes, motor functions, and comorbidities in a Chinese population were similar to those in developed countries, but different from those in other developing countries. The trend for decreased numbers of children with preterm CP since 2009 indicates an improvement in neonatal care. However, perinatal healthcare should still be improved to reduce the occurrence of birth asphyxia.

Surgical reconstruction of hip subluxation and dislocation in children with cerebral palsy.

This study aims to review the efficacy of femoral varus derotation osteotomy (VDRO) and Dega transiliac osteotomy in the treatment of hip subluxation and dislocation of cerebral palsy (CP) patients. This retrospective study included 25 hips of 22 CP patients (9 males, 13 females; mean age 8.7 years; range 4 to 18 years) who were operated due to hip subluxation and dislocation between July 2010 and December 2015. The mean follow-up period was 36.1±10.4 months (range, 20 to 65.6 months). Femoral VDRO and Dega transiliac osteotomy were performed in all cases. None of the patients were administered cast immobilization postoperatively. Patients were evaluated clinically with gross motor function classification system preoperatively and at the follow-up period. Acetabular index (AI), migration percentage (MP), and neck-shaft angle (NSA) were measured and documented by pelvic radiographs taken pre- and postoperatively and at the follow-up period. Intra- and postoperative complications were recorded. Gross motor function classification system scores improved in 16 patients. Mean AI was 33.2° preoperatively and 20.4° postoperatively. In preoperative period, MP and NSA were 72.7% and 160°, respectively, which improved to 24.3% and 130°, respectively, postoperatively. The postoperative improvement in AI, NSA and MP were statistically significant (p<0.001). We performed revision surgery due to implant failure in two patients and detected hip subluxation due to increased pelvic obliquity in one patient who had thoracolumbar scoliosis. In CP patients, reconstruction of hip subluxation and dislocation with femoral VDRO and Dega transiliac osteotomy establish femoroacetabular congruency. Without any cast immobilization, early physical therapy is encouraged for immediate recovery.

Therapeutic hypothermia for neonatal hypoxic-ischemic encephalopathy: magnetic resonance imaging findings and neurological outcomes in a Brazilian cohort

Objective: To determine the neurodevelopment outcomes after therapeutic hypothermia for neonatal hypoxic-ischemic encephalopathy (HIE) and identify the neonatal magnetic resonance imaging (MRI) findings associated with neurological outcome in a middle-income country.Study design: All infants born after 35 completed weeks’ gestation with signs of moderate to severe encephalopathy and evidence of perinatal asphyxia before 6 hours of life were submitted to whole-body hypothermia and were imaged at 18 ± 8.4 days of life (range 7–33 days) after birth. Surviving infants had the neurodevelopment outcome assessed at 12 to 18 months of age by trained professional masked to MRI findings.Results: Forty-eight infants included, MRI scans were obtained from 34 infants; 14 (29.1%) patients died during hospitalization before MRI was performed. Nine (64.3%) of 14 patients were classified as severe encephalopathy presented Posterior Limb Internal Capsule (PLIC) sign at the MRI, 10 (71.4%) thalamus and basal ganglia (TBG) lesion, 9 (64.3%) white matter (WM) lesion, and 7 (50.0%) cortical lesion. Severe encephalopathy was associated with the motor delay at 12–18 months by Bayley III, Alberta Infant Motor Scale (AIMS), and Gross Motor Function Classification System (GMFCS) scores (p = .020, p = .048, p = .033, respectively), but not for the cognitive (p = .167) or language skills (p = .309). Lower BSID-III motor, cognitive, and language composite scores were associated with PLIC sign (p = .047; p = .006 and p = .003, respectively). TBG lesion (p = .051) and cortical lesion (p = .030) were associated with lower language composite score. Motor delay by AIMS and the presence of PLIC sign, TBG lesion, WM lesion, and Cortical lesion on MRI were observed (p < .001; p = .002; p = .001 and p = .027, respectively); as well as higher GMFCS score were associated with the presence of PLIC sign, TBG lesion, WM lesion, and Cortical lesion on MRI (p < .001; p = .001; p = .001, and p = .011, respectively).Conclusions: Brain MRI in neonates with HIE after therapeutic hypothermia is a valuable tool for diagnosis of encephalopathy cerebral abnormalities and is an early predictor of outcome in infants treated with whole body hypothermia for HIE in the Brazilian experience.

A Review on Stem Cell Therapy in Cerebral Palsy with a Focus on Motor Function Improvement

: The current knowledge on how to use stem cells therapeutically for improving motor function in patients with cerebral palsy is growing. The present review aimed at assessing clinical trials related to beneficial effects of stem cells in patients with cerebral palsy. Electronic searches including “stem cell” and “cerebral palsy” as keywords were conducted using PubMed, Ovid, Web of Science, Scopus, Cochrane library, and CINAHL till February 2016. Two assessors reviewed the methodological quality and eligibility of the retrieved articles, independently. Among 77 studies initially reviewed based on the keywords, only 6 clinical trials were identified that met the inclusion criteria. Pooled response rate of stem cell therapy to treat cerebral palsy was estimated by assessing the percentage of improvement with a gross motor function classification system score. The resulting pooled estimate indicated a 30.7% (95% CI: 25.80% to 35.69%) increase in the score, 1 to 6 months after cell transplantation. In this regard, the test for heterogeneity was statistically significant (I2 = 87.9%, χ2 = 41.22, P < 0.0001). Stem cell therapy has the potential to improve motor function in patients with cerebral palsy. However, promising results from limited studies warrant further research.

Profile of Pediatric Patients with Cerebral Palsy at the Department of Rehabilitation Medicine, Philippine General Hospital

Objectives: Data is necessary for a hospital-wide cerebral palsy (CP) profile, this being the leading pediatric diagnosis at the PGH Rehabilitation Medicine Out Patient clinic. This study aims to identify clinical features, severity of disability and common interventions in pediatric CP patients. Methods: CP profile data collection forms were accomplished from September 2014 to December 2015. Primary Results: Among 125 participants: 55% were &lt; 5 years old, 47% were delivered vaginally, 42% had perinatal onset of condition, 34% had normal birth weight and 23% were moderately preterm. Most were quadriplegic (36%) and spastic (50%). Primary caregivers were mostly mothers (45%) and &lt;20 years old (38%). Mobility-wise, 29% had Gross Motor Function Classification System score of 3. For hand function, 31% scored 2 in the Manual Ability Classification System and 26% were ‘below average’ in the Peabody Developmental Motor Skills-2. Common problems were: feeding (100%), communication (63%), seizure (58%), visual (56%) and auditory (54%). Majority (73%) had access to government medical facilities; 18% had no access. Physical (64%) and occupational (29%) therapy were common interventions; 29% received special education. Conclusion: A hospital-wide cerebral palsy profile should be established to monitor CP, given its multifactorial cause and complex functional impact. Trends should be correlated with maternal and patient factors, healthcare provision and socioeconomics.

Urine hydroxyproline correlates with progression of spasticity in cerebral palsy

Introduction:Most Cerebral Palsy (CP) patients develop muscle spasticity which is characterized by jerky movements and muscle and joint stiffness. This increase of muscle stiffness in spastic CP has been correlated with the accumulation of collagen in the muscle as detected by the increase in muscle hydroxyproline, a major component of collagen.Material and Methods:This was a cross sectional comparative study, conducted in the tertiary hospital, Malaysia from June’2012 to December’2014. Children with spastic CP (6 to 18 years) who were scheduled for muscle/tendon lengthening as part of the on going management and children with pure spasticity were included in this study. Normal children who are aged and sex matched to the CP children were included. Muscle biopsy and urine samples were collected for MH and UH analysis respectively.Results:A total of 48 children, aged 6 to 18 years (17 normal; 16 spastic CP without contracture, 15 spastic CP with contracture) were included in this study. Muscle biopsy (only for CP children with contracture) and urine samples were collected. A significant negative correlation was noted between the MH (261.894±69.077ng/ml) and UH (13.266±7.999ng/ml) levels (p=0.031). There was a statistically significant correlation between UH levels and the MAS score (p=0.01), and GMFCS score (p=0.015).Conclusions:UH quantification may be an objective tool to estimate the severity and progression of spasticity in CP.Objectives:The objective of the study is to determine if there is any correlation between muscle and urine hydroxyproline levels in spastic CP. Further, to determine if Urine Hydroxyproline levels are different between spastic CP with and without contracture. Finally to determine if UH levels can be correlated with severity of CP as determined by Modified Ashworth Scale (MAS) and Gross Motor Function Classification System (GMFCS) scores.

Neurodevelopmental and Behavioral Outcomes in Extremely Premature Neonates With Ventriculomegaly in the Absence of Periventricular-Intraventricular Hemorrhage

Studies of cranial ultrasonography and early childhood outcomes among cohorts of extremely preterm neonates have linked periventricular-intraventricular hemorrhage and cystic periventricular leukomalacia with adverse neurodevelopmental outcomes. However, the association between nonhemorrhagic ventriculomegaly and neurodevelopmental and behavioral outcomes is not fully understood. To characterize the outcomes of extremely preterm neonates younger than 27 weeks' gestational age who experienced nonhemorrhagic ventriculomegaly that was detected prior to 36 weeks' postmenstrual age. This longitudinal observational study was conducted at 16 centers of the Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network. Infants born prior to 27 weeks' gestational age in any network facility between July 1, 2006, and June 30, 2011, were included if they had a cranial ultrasonogram performed prior to 36 weeks' postmenstrual age. Comparisons were made between those with ventriculomegaly and those with normal cranial sonograms. Data analysis was completed from August 2013 to August 2017. The main outcome was neurodevelopmental impairment, defined as a Bayley Scales of Infant and Toddler Development III cognitive score less than 70, moderate/severe cerebral palsy, a Gross Motor Function Classification System score of level 2 or more, vision impairment, or hearing impairment. Secondary outcomes included Bayley Scales of Infant and Toddler Development III subscores, components of neurodevelopmental impairment, behavioral outcomes, and death/neurodevelopmental impairment. Logistic regression was used to evaluate the association of ventriculomegaly with adverse outcomes while controlling for potentially confounding variables and center differences as a random effect. Linear regression was used similarly for continuous outcomes. Of 4193 neonates with ultrasonography data, 300 had nonhemorrhagic ventriculomegaly (7%); 3045 had normal cranial ultrasonograms (73%), 775 had periventricular-intraventricular hemorrhage (18.5%), and 73 had cystic periventricular leukomalacia (1.7%). Outcomes were available for 3008 of 3345 neonates with ventriculomegaly or normal scans (90%). Compared with normal cranial ultrasonograms, ventriculomegaly was associated with lower gestational age, male sex, and bronchopulmonary dysplasia, late-onset sepsis, meningitis, necrotizing enterocolitis, and stage 3 retinopathy of prematurity. After adjustment, neonates with ventriculomegaly had higher odds of neurodevelopmental impairment (odds ratio [OR], 3.07; 95% CI, 2.13-4.43), cognitive impairment (OR, 3.23; 95% CI, 2.09-4.99), moderate/severe cerebral palsy (OR, 3.68; 95% CI, 2.08-6.51), death/neurodevelopmental impairment (OR, 2.17; 95% CI, 1.62-2.91), but not death alone (OR, 1.09; 95% CI, 0.76-1.57). Behavioral outcomes did not differ. Nonhemorrhagic ventriculomegaly is associated with increased odds of neurodevelopmental impairment among extremely preterm neonates.

Long-term Outcome of External Tibial Derotation Osteotomies in Children With Cerebral Palsy.

Internal tibial torsion (ITT) is a common boney 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 after external TDO performed to correct ITT in ambulatory children with CP. Following IRB approval, gait kinematics and passive range of motion measurements were retrospectively evaluated in children with spastic CP who underwent TDO due to ITT 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 the subjects mean tibial rotation (MTR) in gait to a group of typically developing children. Age at surgery, preop MTR (at E0), preop gait velocity (at E0), gross motor function classification system score, and foot deformity were evaluated to determine their influence on long-term results. The study sample consisted of 36 legs (with E0 and E2) and 17 legs (with E0, E1, and E2). The mean age at surgery was 7.4±2.8 (range, 4 to 16.6) years. Comparing the changes over time, kinematic MTR improved from 17±11 degrees initially (E0) to -10±14 degrees short term (E1) and progressed to -23±13 degrees long term (E2) (P<0.05 E0/E1/E2; internal rotation is positive). At E2, 16 legs (44%) were found to be in the kinematic corrected group and 20 legs (56%) in the kinematic overcorrected group. There were no significant differences between the corrected and overcorrected groups of children in respect to age of surgery, gross motor function classification system, E0 MTR, gait velocity, or foot deformity. Although external TDO is an accepted form of treatment in children with CP, in the long term a tendency to move into external tibial torsion is common. Therefore, caution is warranted with children who initially present with ITT to avoid overcorrection. Level IV-therapeutic study.

Long-term Outcome of Internal Tibial Derotation Osteotomies in Children With Cerebral Palsy.

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. 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. 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. Although internal TDO improves ETT in the short term, recurrence is frequent with an apparent developmental trend toward external rotation of the tibia. Level IV-therapeutic study.

Botulinum toxin injections for chronic sialorrhoea in children are effective regardless of the degree of neurological dysfunction: A single tertiary institution experience

ObjectiveTo determine the effectiveness of submandibular salivary gland Botulinum Toxin Type-A (BTX-A) injection in the treatment of drooling in children with varying degrees of neurological dysfunction. MethodsA retrospective review of pre- and post-procedure drooling frequency and severity scores of patients receiving BTX-A between January 2008 and January 2013. Stratification to different subgroups of neurological impairment was performed according to Gross Motor Function Classification System (GMFCS) score. Drooling severity was assessed using Thomas-Stonell and Greenberg symptom questionnaires administered at time of initial consultation and 3 months after treatment. Results48 sets of BTX-A injections in 26 patients with an average age of 9.45 years (range 7 months–18 years) were included in the study. Marked improvement in drooling was seen in 60.4% of patients, a marginal or brief improvement was seen in 20.8% and there was no improvement in 18.8%. No adverse events were reported following any of the BTX-A injections. BTX-A was safe and effective in the eight patients with pre-existing swallowing dysfunction. Subsequent drooling surgery was performed in 15 (57.7%) of the cohort, all 15 patients responded to BTX-A injections.In patients with Cerebral Palsy, there was no correlation between the severity of the neurological dysfunction as measured by the Gross Motor Function Classification System (GMFCS) score and the response to BTX-A treatment. ConclusionsInjection of BTX-A to the submandibular glands of children with neurological disorders is a safe procedure and results in a reduction in drooling in the majority of patients. Children with severe neurological dysfunction respond to BTX-A injections as effectively as their less impaired peers and the degree of response does not appear to be associated with the severity of neurological disability. BTX-A injection is a good initial procedure when drooling surgery is being considered.

Intensive gait training in toddlers with cerebral palsy: A pilot study

Background: Reduced muscle growth may be involved in the development of contractures in children with cerebral palsy (CP). Here, we report data from a pilot study of intensive gait training in CP toddlers. Methods: Five children with CP aged 8-30 months performed activity-based gait training for one hour/day, five days/week for three consecutive months. Included children were diagnosed with spastic CP, had a Gross Motor Function Classification System (GMFCS) score of I–II, and were not epileptic. All children wore pedometers during training. Before and after the training period, kinematic and qualitative gait analysis, clinical and objective evaluation of spasticity, Gross Motor Function Measure-66 (GMFM-66), and ultrasound of the affected medial gastrocnemius (MG) muscle were performed. Two children were also tested before and after three months of receiving only standard care (SC). Results: On average 1410 steps/session were logged during 63 days of training. More steps were achieved at home than at a central facility. During training, MG muscle volume increased significantly, while it decreased for SC children. Gait improved qualitatively in all children, and GMFM-66 score improved in four of the five children. Similar improvements were seen among the SC children. Two children had pathologically increased muscle stiffness prior to training, which was reduced during training. Reflex stiffness was unchanged in all five children. Conclusions: This pilot study suggests that intensive gait training may increase muscle volume, improve walking skills and reduce passive muscle stiffness in toddlers with CP.