BACKGROUND:Childhood arterial ischemic stroke (AIS) is associated with substantial long-term morbidity. This study aims to describe factors associated with recurrence, mortality, and neurological disability. METHODS:Australian children with radiologically confirmed AIS were enrolled prospectively to the Royal Children's Hospital stroke registry from 2003 to 2017. Data collected included demographics, clinical and radiological variables, Pediatric National Institutes of Health Stroke Scale severity score, and the Childhood AIS Standardized Classification and Diagnostic Evaluation etiology. Neurological outcomes were categorized as good (normal) or poor (mild/moderate/severe) using the Pediatric Stroke Outcome Measure Severity Classification Scheme. RESULTS:A total of 172 children with AIS were identified; 11% died (2% stroke-related), 15% had recurrent strokes (5% transient ischemic attacks), and 8% developed epilepsy. Pediatric Stroke Outcome Measure follow-up data were available for 131 children at a median 8.9 years of follow-up (interquartile range, 6.0-11.9). Risk factors for recurrence were arteriopathy (hazard ratio [HR], 2.75; confidence interval [CI], 1.47-5.15, P = 0.002) and hemiparesis (HR, 1.34; CI, 1.07-1.69, P = 0.012); 43% of children had poor long-term outcomes, which were associated with altered conscious state (odds ratio [OR], 2.79; CI, 1.25-6.22, P = 0.012) and neurosurgical intervention (OR, 3.20; CI, 1.55-6.59, P = 0.002). CONCLUSIONS:Long-term neurological deficits are common in children with AIS in the prereperfusion therapy era. The use of pediatric-specific stroke assessment measures and etiologic classification systems enables this study to be a comparator for future stroke studies, measuring the impact of reperfusion therapies on outcomes in childhood AIS.

Background and Purpose:The aims of this study were to assess the prevalence of multidimensional fatigue symptoms 5 years after pediatric arterial ischemic stroke and identify factors associated with fatigue.Methods:Thirty-one children (19 males) with pediatric arterial ischemic stroke, participating in a larger prospective, longitudinal study, were recruited to this study at 5 years poststroke. Parent- and self-rated PedsQL Multidimensional Fatigue Scale scores were compared with published normative data. Associations between parent-rated PedsQL Multidimensional Fatigue Scale, demographics, stroke characteristics, and concurrent outcomes were examined.Results:Parent-rated total, general and cognitive fatigue were significantly poorer than population norms, with more than half of all parents reporting fatigue symptoms in their children. One-third of children also reported experiencing fatigue symptoms, but their ratings did not differ significantly from normative expectations, as such, all further analyses were on parent ratings of fatigue. Older age at stroke and larger lesion size predicted greater general fatigue; older age, female sex, and higher social risk predicted more sleep/rest fatigue. No significant predictors of cognitive fatigue were identified and only older age at stroke predicted total fatigue. Greater fatigue was associated with poorer adaptive functioning, motor skills, participation, quality of life, and behavior problems but not attention.Conclusions:Fatigue is a common problem following pediatric arterial ischemic stroke and is associated with the functional difficulties often seen in this population. This study highlights the importance of long-term monitoring following pediatric arterial ischemic stroke and the need for effective interventions to treat fatigue in children.

Background and Purpose: Childhood and adolescence coincide with rapid maturation of distributed brain networks supporting social cognition; however, little is known about the impact of early ischemic brain insult on the acquisition of these skills. This study aimed to examine the influence of arterial ischemic stroke (AIS) on facial emotion recognition and theory of mind (ToM) abilities of children and adolescents initially recruited to a single-center, prospective longitudinal study of recovery following AIS. Methods: The study involved 67 participants, including 30 children with AIS (mean time since stroke=5 years) and 37 age-matched typically developing controls who were assessed on measures of cognitive ToM, facial emotion recognition, and affective ToM. Acute clinical magnetic resonance imaging, including diffusion-weighted imaging sequences, were used to evaluate prospective structure-function relationships between acute lesion characteristics (size, location, and arterial territories affected) and long-term social cognitive abilities. Results: Relative to age-matched typically developing controls, children with AIS showed significantly worse performance on measures of basic facial emotion processing, cognitive ToM, and affective ToM. In univariate models, poorer ToM was associated with larger infarcts, combined cortical-subcortical pathology, and involvement of multiple arterial territories. In multivariate analyses, larger lesions and multiterritory infants were predictive of ToM processing but not facial emotion recognition. Poorer cognitive ToM predicted less frequent prosocial behavior and increased peer problems. Conclusions: Social cognitive skills appear vulnerable to disruption from early ischemic brain insult. In the first study to examine social cognition in a prospective cohort of children with AIS, our findings suggest that acute magnetic resonance imaging-based lesion characteristics may have predictive value for long-term social cognitive outcomes and may assist to identify children at elevated risk for social cognitive dysfunction.

Myelofibrosis is a rare disorder that is classified as one of the myeloproliferative disorders.This particular disorder results in the abnormal proliferation of hematopoietic stem cells in the bone marrow.In some cases, such as ours, pathologic fractures can occur due to skeletal manifestations.We report on a rare finding of rapidly progressive lytic lesions in multiple regions throughout the body.This presentation of myelofibrosis behaving in a metastatic-like fashion has not been previously described.

Background Pediatric patients with sarcomas are at risk of poor quality of life outcomes. The National Institutes of Health (NIH)-funded Patient-reported Outcomes Measurement Information System (PROMIS (R)) improves our ability to capture patient-reported outcomes. Do physical function, social, and mental health PROMIS outcomes for pediatric patients with non-metastatic malignant sarcomas differ from the U.S. pediatric population? Methods Six pediatric PROMIS short forms were collected for patient visits to orthopedic oncology at a tertiary referral center from September 1, 2016, to March 31, 2017. Mean T-scores differed from the reference population by a one-sample t-test. Results Of the 30 eligible patients, five had soft-tissue sarcomas and 25 (83%) had bone sarcomas. The mean age of the cohort was 13 years (5-17). The study cohort had a mean physical function T-score of 39.8 (SD 9.8), which was worse than the reference population. In contrast, the mean peer relationship T-score of 54.3 (SD 8.8) and mean depression T-score of 42.0 (SD 9.1) were better than the reference population. Conclusions Pediatric patients with non-metastatic sarcomas had a worse physical function but a better peer relationship and depression scores than the U.S. PROMIS reference population. Ceiling and flooring effects were reported. The level of evidence was III.

Background. The Patient Reported Outcomes Measurement Information System (PROMIS) is a tool developed by the National Institutes of Health that allows comparisons across conditions or even the United States (U.S.) general population. Objectives. Our purpose was to compare PROMIS outcomes between patients who underwent a planned resection to those who underwent an initial unplanned excision of their sarcoma followed by a definitive oncologic resection. We then compared these groups to the U.S. general population. Methods. Eighty-five patients were included and were divided into those who underwent an initial planned resection (67) and unplanned excision (18). These patients were then further categorized based on the length of follow-up since their last surgery, either early (<12 months) or late (>12 months). Results. We evaluated seven PROMIS domains and found no differences between patients who underwent planned resection versus those who underwent an initial unplanned excision followed by a wide resection of the previous wound bed. When compared to the U.S. population, both cohorts demonstrated significantly improved scores in several emotional health domains. Conclusions. Patients who undergo an unplanned excision followed by a definitive oncologic procedure have similar PROMIS scores compared to patients who undergo an initial planned resection.

Little is known about resilience after paediatric stroke (PS), or the factors that contribute to better outcomes. Rather, research emphasis has been on impairment, measured through cross-sectional or retrospective designs, often heavily weighted to children presenting for clinical or rehabilitation follow-up. Implementing a resilience framework, this study aimed to investigate cognitive recovery post-stroke and factors that contribute to cognitive resilience at 12 months following PS. In a single site, prospective, longitudinal study (baseline, 1, 6, 12 months post-stroke), 61 children (55.7% male) aged 0-18 years, with a diagnosis of acute arterial ischemic stroke were recruited. Neurological status, lesion and child characteristics were collected at diagnosis. Cognitive, language and motor skills were assessed directly using age-appropriate, standardised tools. Parents rated their mental health, and child social and adaptive abilities. Participants were classified as 'resilient' (74%) or 'vulnerable' based on 12-month cognitive scores. The resilient group demonstrated more intact acute neurological status and higher language and adaptive abilities 1-month post-stroke; 88% of the vulnerable group had strokes involving both cortical and subcortical regions. Neonatal stroke, large lesions, cortical-only lesions, and middle cerebral artery involvement were associated with poorer cognition over the 12 months post-stroke. Absence of seizures and older age at stroke predicted better cognitive outcomes. In summary, most children surviving PS are cognitively resilient at 12 months post-insult. Risk and protective factors identified may guide targeted clinical intervention for more vulnerable children. Future research is needed to explore cognitive resilience trajectories beyond 12 months post-stroke.

Background. The Patient Reported Outcomes Measurement Information System (PROMIS) is a patient-directed system that allows comparisons across medical conditions. With this tool, comparisons can now be made between rare conditions, such as sarcomas, and more common ailments, of the United States general population. This allows comparisons between rare conditions, such as sarcomas, to more common ailments, or even the United States (US) general population. Objectives. Our purpose was to use PROMIS to compare outcomes in patients that had undergone resection of a nonmetastatic sarcoma to the US population. Methods. One hundred thirty-eight patients were included in the analysis. These patients were divided into early (<2 years) and late follow-up (>2 years). Results. We evaluated results from seven health domains and found significantly lower scores in the physical function and depression domains. These differences were present in both the early and late cohorts when compared to the US population. Conclusion. While physical function was found to be worse in the sarcoma cohorts, we observed significantly improved levels of depression in these patients when compared to the US population. This finding was maintained over time and is an important reminder that a patient’s goals and desires change following a cancer diagnosis and must be taken into consideration when planning treatment and determining a successful outcome.

Introduction: The Patient-reported Outcomes Measurement Information System (PROMIS) is a scoring tool that allows comparisons between patients with rare conditions and more common ailments, or the general US population. PROMIS outcomes were compared between the limb salvage and amputee patients for nonmetastatic sarcomas to the US population. Methods: One hundred thirty-eight patients were included in the analysis. Patients were divided into the limb salvage and amputee cohorts, as well as based on the follow-up (1 to 11 or 12+ months). Results: Seven PROMIS domains were evaluated, and higher scores were found in both the limb salvage group and patients >12 months from surgery. The limb salvage group also had improved emotional health compared with the US population. Discussion: Improvements in PROMIS values are observed in limb salvage patients and in patients >12 months from surgery. Limb salvage patients demonstrate improved emotional health compared with the US population.

Pediatric stroke can result in long-term neurobehavioral impairments including cognitive, language, and motor deficits, all of which may disrupt the normal development of social skills. This study aimed to examine specific components of social function at 5-year poststroke at a group and individual level and explore the contribution of neurobehavioral impairment. Thirty-one children with arterial ischemic stroke participated in the study. Assessment included parent-rated questionnaires measuring social adjustment and social participation as well as behavior and fatigue. Children underwent testing of social cognition and neurobehavioral abilities (intellectual function, attention, pragmatic language, motor function, and neurological impairment). Group means for social function were generally within the normal range, with social adjustment poorer than normative expectations. Examination of impairment rates showed a significant proportion of children had impaired function across social domains. Childhood stroke was associated with poorer social adjustment and a range of neurobehavioral outcomes, compared to neonatal stroke. Social function was found to be impacted by fatigue and intellectual function, but not by attention, pragmatic language, behavior, motor function, or neurological impairment.

Background:Parosteal osteosarcoma is a rare tumor with increased survival compared to conventional high-grade osteosarcoma. Due to this increased survival comes the need for reconstructive options that provide good long-term functional results. Current treatment methods include geographic resection with allograft reconstruction versus resection and reconstruction with a distal femoral replacement.Purpose:Our purpose was to compare the long-term functional outcomes of distal femoral replacements to allograft reconstructions, using the musculoskeletal tumor society (MSTS) scoring system.Methods:After querying our database, 12 patients were identified and completed a MSTS questionnaire.Results:There was no difference in functional outcomes between the cohorts at an average of 14 years follow up for the endoprosthetic group and 25 years of follow up for the geographic resection group.Conclusion:At long-term follow-up, patients who undergo a distal femoral replacement for a parosteal osteosarcoma have no difference in functional outcomes compared to those who undergo an allograft reconstruction.Level of Evidence: IV.

Allograft reconstruction of large segmental intercalary bone defects after tumor resection is a well-accepted surgical technique. Although results generally have been satisfactory, nonunion at the allograft-host bone junction site remains a notable concern. Various reports have described attempts to enhance junctional healing with a variety of complex osteotomies, often complicating an already complex procedure. The use of an innovative reverse reaming technique can decrease the level of intraoperative difficulty mating the allograft-host junction and theoretically may improve junctional healing by enhancing stability and increasing the contact surface area.

OBJECTIVE:To evaluate the relationship between neurologic outcome at 1 month following diagnosis of pediatric arterial ischemic stroke (AIS) and motor and adaptive behavior outcomes at 12 months.METHODS:This prospective longitudinal observational cohort study recruited children from a single tertiary children's hospital diagnosed with first AIS between December 2007 and November 2013. Neurologic impairment was evaluated at 4 time points using the Pediatric Stroke Outcome Measure (PSOM) or Recovery and Recurrence Questionnaire following diagnosis of AIS (acute, 1, 6, and 12 months). Motor function and adaptive behavior were assessed at 12 months using standardized measures. Children were grouped for analysis, according to age at diagnosis (neonates vs preschool vs school-aged). The relationship between neurologic impairment and 12-month functional outcomes were examined.RESULTS:Sixty-four children were recruited (27 neonates, 19 preschool-aged, and 18 school-aged). Presence of impairment on the PSOM at 1 month was associated with lower 12-month fine motor z scores (p = 0.004), gross motor z scores (p = 0.001), and adaptive behavior standard scores (p = 0.004). One-month PSOM impairment score was more predictive than age group or lesion size of 12-month motor and adaptive behavior outcome.CONCLUSIONS:The PSOM has value as a predictive tool when used at 1 month after first AIS diagnosed acutely in relation to motor and adaptive behavior, with variation according to age group.

To describe 5‐year motor and functional outcomes after paediatric arterial ischaemic stroke (AIS) and to explore factors associated with poorer long‐term outcome.

Objective To evaluate the relationship between neurologic outcome at 1 month following diagnosis of pediatric arterial ischemic stroke (AIS) and motor and adaptive behavior outcomes at 12 months. Methods This prospective longitudinal observational cohort study recruited children from a single tertiary children's hospital diagnosed with first AIS between December 2007 and November 2013. Neurologic impairment was evaluated at 4 time points using the Pediatric Stroke Outcome Measure (PSOM) or Recovery and Recurrence Questionnaire following diagnosis of AIS (acute, 1, 6, and 12 months). Motor function and adaptive behavior were assessed at 12 months using standardized measures. Children were grouped for analysis, according to age at diagnosis (neonates vs preschool vs school-aged). The relationship between neurologic impairment and 12-month functional outcomes were examined. Results Sixty-four children were recruited (27 neonates, 19 preschool-aged, and 18 school-aged). Presence of impairment on the PSOM at 1 month was associated with lower 12-month fine motor z scores (p = 0.004), gross motor z scores (p = 0.001), and adaptive behavior standard scores (p = 0.004). One-month PSOM impairment score was more predictive than age group or lesion size of 12-month motor and adaptive behavior outcome. Conclusions The PSOM has value as a predictive tool when used at 1 month after first AIS diagnosed acutely in relation to motor and adaptive behavior, with variation according to age group.

AIM:Little is known about psychological and social outcomes after paediatric stroke. This study aimed to evaluate psychosocial outcomes in children 5 years after paediatric stroke and explore the contribution of early presenting factors. METHOD:Thirty-one children (19 males, 12 females) with arterial ischemic stroke were involved in this prospective, longitudinal study. Children underwent intellectual assessment at 12 months poststroke and parents completed questionnaires rating their own mental health and their child's functioning at 12 months and 5 years poststroke. RESULTS:At 5-year follow-up, psychological and social function were significantly poorer than normative expectations. Exploration of early predictive factors showed poorer cognitive and psychological function at 12 months poststroke and older age at stroke onset was associated with poorer psychosocial function at 5 years. Larger lesion size was also associated with poorer psychological function at 5 years poststroke. INTERPRETATION:These early predictors of poorer psychosocial outcome suggest that screening children within the first year after stroke may identify children most at risk of later problems and facilitate early intervention.

BACKGROUND:Neuromotor impairments are common after pediatric stroke, but little is known about functional motor outcomes. We evaluated motor function and how it changed over the first 12 months after diagnosis. We also examined differences in outcome according to age at diagnosis and whether fine motor (FM) or gross motor (GM) function at 12 months was associated with adaptive behavior.METHODS:This prospective, longitudinal study recruited children (N = 64) from The Royal Children's Hospital, Melbourne who were diagnosed with acute arterial ischemic stroke (AIS) between December 2007 and November 2013. Motor assessments were completed at 3 time points after the diagnosis of AIS (1, 6, and 12 months). Children were grouped as follows: neonates (n = 27), preschool-aged (n = 19), and school-aged (n = 18).RESULTS:A larger lesion size was associated with poorer GM outcomes at 12 months (P = .016). Neonatal AIS was associated with better FM and GM function initially but with a reduction in z scores over time. For the preschool- and school-aged groups, FM remained relatively stable over time. For GM outcomes, the preschool- and the school-aged age groups displayed similar profiles, with gradual recovery over time. Overall, poor FM and GM outcomes at 12 months were associated with poorer adaptive behavior scores.CONCLUSIONS:Motor outcomes and the trajectory of recovery post-AIS differed according to a child's age at stroke onset. These findings indicate that an individualized approach to surveillance and intervention may be needed that is informed in part by age at diagnosis.