Clinical Presentation Of Pediatric Patients Research Articles

Navigating postextubation bronchospasm in pediatric patients with intraoperative inhalation agents: A comprehensive review

Post-extubation bronchospasm poses a considerable challenge in pediatric anesthesia, necessitating effective management strategies to mitigate its potential complications. This review provides an overview of post-extubation bronchospasm, including its etiology, risk factors, and clinical presentation in pediatric patients. We emphasize the critical role of intraoperative inhalation agents in managing bronchospasm, discussing their mechanisms of action and comparative efficacy. Through a comprehensive analysis of existing literature and clinical evidence, we highlight best practices for administering and monitoring inhalation agents during pediatric anesthesia. Furthermore, we present case studies and clinical outcomes to illustrate the practical implications of utilizing these agents in bronchospasm management. Our review underscores the importance of optimizing intraoperative inhalation agents to improve patient outcomes and safety in pediatric anesthesia, emphasizing the need for evidence-based approaches and tailored interventions in clinical practice.

Idiopathic intracranial hypertension. Review of our experience in the last eight years (2016-2023)

Idiopathic intracranial hypertension (IIH) or benign intracranial hypertension is a rare disease in childhood. The clinical presentation in pediatric patients can be very variable, being more unespecific in younger patients. A retrospective descriptive study was carried out on patients diagnosed of IIH in the last eight years (2016-2023) in the neuropediatrics unit of a tertiary hospital. In the present study, the clinical-epidemiological characteristics and the diagnostic-therapeutic procedure carried out in each case were analyzed. We studied 14 patients, 57% were women. The average age at diagnosis was 9 years, headache was the most common reason for consultation. In all patients, papilledema was found in the fundus and neuroimaging didn´t find alterations. Optical coherence tomography has been carried out in 78.5% of the sample, > 80% of patients showed thickening of the retinal nerve fiber layer. All patients had a high cerebrospinal fluid opening pressure (>25 cmH2O). 57% of patients required treatment with acetazolamide, a carbonic anhydrase inhibito. In all patients the resolution was complete, however almost 30% of them have presented recurrences during follow-up. In recent years there has been an increase in the incidence of this entity, making early diagnosis and treatment essential to avoid possible irreversible damage.

Comparison of pathological findings in head computed to-mography with clinical presentation of pediatric patients in the Emergency Department

Pediatric trauma is a cause for over 750.000 visits to emergency departments each year. Of all types of traumas, head injuries represent the group at most risk. Computed tomography (CT) is still the most frequently used advanced imaging method to distinguish cases of severe injuries out of thousands of mild or apparently trivial injuries. The aim of this study was to review the results of CT in terms of clinical prediagnosis and radiological findings in children categorized into four age groups, who underwent a CT scan in the Pediatric Emergency Department (ED). Among all CT studies, no findings were found in 552 (80.23%) patients, whereas CT findings were detected in 98 (14.2%) boys and 38 (5.5%) girls from all participants. The incidence of lesions in the male group is 2 times higher than in the female group. The most common abnormality was hematoma representing 26.47% of all confirmed lesions and 5.23% among all head CTs performed from the ED. Head CT is an incredibly useful tool in the evaluation of some head emergencies. However, in the majority of cases, the clinical presentation with which children present to the ED does not correlate with the severity of neuroimaging results.

Clinical features of early-onset pediatric traumatic glaucoma and predictive factors for the need of early glaucoma surgery.

To assess the clinical presentation of pediatric patients having early traumatic glaucoma and to analyze early predictors for the need of filtration surgery. Patients with early traumatic glaucoma after close globe injury (CGI) from January 2014 to December 2020 were retrospectively reviewed. Clinical features, treatment provided (medical and surgical), and visual outcomes were documented. Patients were divided into two groups based on the management required: group A- trabeculectomy and group B- medication + minor surgery. A total of 85 patients were studied after applying the necessary inclusion and exclusion criteria. Out of these, 46 underwent trabeculectomy for the control of intraocular pressure (IOP) and the remaining 39 were managed with antiglaucoma medications. Significant male predominance of 9.6:1 was observed. Patients presented to the hospital after a mean duration of 8.5 days posttrauma. Wooden objects were most commonly responsible for trauma. Mean best corrected visual acuity at presentation was 1.91 log of minimum angle of resolution (logMAR). Mean IOP at presentation was 40 mmHg. The common anterior segment finding were severe anterior chamber (AC) reaction (63.5%), followed by angle recession (56.4%). Severe AC reaction (P = 0.0001) and corneal microcystic edema (P = 0.04) were significant predictive factors for early need of trabeculectomy. Need of trabeculectomy was higher in patients with severe AC reaction and corneal microcystic edema. The threshold to perform trabeculectomy should be lower, as glaucoma is often relentless, severe, and may result in irreversible vision loss.

Clinical presentation of pediatric patients with symptomatic SARS-CoV-2 infection during the first months of the COVID-19 pandemic in a single center in Mexico City.

BackgroundThe clinical spectrum of COVID-19 is broad, from asymptomatic to severe cases and death. The objective of this study is to analyze the clinical course of patients attended during the first months of the SARS-CoV-2 pandemic in a third-level pediatric hospital.MethodsDesign: prospective cohort study. Patients with viral respiratory disease or suspected cases of COVID-19 were evaluated at the Pediatric Hospital, National Medical Center XXI Century, Mexico City, from 21 March 2020 to 13 January 2021. Statistical analysis: Chi-square test and Fisher’s exact test were used for comparisons; a logistic regression model was constructed to identify clinical or laboratory characteristics associated with critical disease. A p-value < 0.05 was considered statistically significant.ResultsA total of 697 patients met the operational definition of viral respiratory disease or suspected cases of COVID-19 and underwent real-time reverse transcription polymerase chain reaction (rRT-PCR) SARS-CoV-2 testing. Patients with a positive result were included. Of the 181 patients (26%), 121 (66.8%) had mild disease and were treated as outpatients and 60 (33.1%) were hospitalized. A total of six patients met the criteria for multisystem inflammatory syndrome in children (MIS-C). Of the 60 inpatients, 65% were males, and 82% had one or more comorbidities. The main comorbidities were cancer (42%) and overweight (15%). The median hospital stay was 9 days. The inpatients had a higher frequency of fever, general malaise, dyspnea, chills, polypnea, and cyanosis than the outpatients (p < 0.05). Only 21.4% of the outpatients had one or more comorbidities, which were lower than in the hospitalized patients (p < 0.001). Laboratory data at admission were similar between critically ill and those with moderate and severe disease. The patients who developed pneumonia were at higher risk of critical disease, while older age was associated with a better prognosis. A total of 13 of the 60 inpatients died (mortality 7.1%). All but one had one or more comorbidities: four had cancer, four congenital heart disease, one chronic kidney disease and epilepsy, one Epstein–Barr virus-induced hemophagocytic lymphohistiocytosis, one obesity, and one diabetes mellitus.ConclusionHospital mortality is high, especially in children with comorbidities. Despite 2 years having passed since the beginning of the COVID-19 pandemic, the epidemiological and clinical data on children are still helpful to improve their prognosis.

Platelet functional abnormalities and clinical presentation in pediatric patients with germline RUNX1, ANKRD26, and ETV6 mutations.

Not available.

Postural Orthostatic Tachycardia Syndrome (POTS): Characteristics of Youth Enrolled in an Outpatient Interdisciplinary Pediatric Chronic Pain Program

Postural Orthostatic Tachycardia Syndrome (POTS) is a common form of orthostatic intolerance that affects about 3,000,000 people in the United States. For youth, significant functional impairment is noted, especially related to school attendance, limited participation in social and physical activities, and lower quality of life. Although previous research highlights the unique challenges for youth with chronic pain, fewer studies have focused on the clinical presentation of pediatric patients with POTS. This study aimed to explore characteristics of youth with POTS presenting to an interdisciplinary pediatric chronic pain program. Twenty-nine adolescents (14.7 +/- 1.7 years; 79% female) diagnosed with POTS presenting with their caregiver to an initial visit at an interdisciplinary pediatric chronic pain management program at a large Northeastern children's hospital in the United States were asked to complete questionnaires assessing demographic and pain characteristics, pain acceptance, and functional impairment and anxiety (PROMIS; t-scores). Number of visits within the program were gathered from medical record. Youth were primarily Caucasian/White (86.2%), Other (6.9%), AAPI (3.4%) and Mixed Race (3.4%). Youth reported overall low pain acceptance (M=34.1, SD=9.3), moderate anxiety (M=57.1, SD=13.1) and high pain interference (M=63, SD =7.4). Caregivers reported observing moderate anxiety (M=59.1, SD =11.5) and high pain interference (M=65.1, SD=4.6) for youth. Adolescents were observed to attend a wide range of visits within the program, including medical (1.7 +/- 2.5), physical therapy (3.4 +/- 4.9), occupational therapy (.5 +/- 1.7), and psychology (4.3 +/- 3.6). Less is known about youth with POTS who present for treatment. Additionally, youth with POTS meet with multiple doctors before receiving a diagnosis leading to disparities in access to treatment and the type of treatment that is received. Data here highlights that youth with POTS are experiencing significant impairment and more research targeting this population is necessary to improve outcomes. Postural Orthostatic Tachycardia Syndrome (POTS) is a common form of orthostatic intolerance that affects about 3,000,000 people in the United States. For youth, significant functional impairment is noted, especially related to school attendance, limited participation in social and physical activities, and lower quality of life. Although previous research highlights the unique challenges for youth with chronic pain, fewer studies have focused on the clinical presentation of pediatric patients with POTS. This study aimed to explore characteristics of youth with POTS presenting to an interdisciplinary pediatric chronic pain program. Twenty-nine adolescents (14.7 +/- 1.7 years; 79% female) diagnosed with POTS presenting with their caregiver to an initial visit at an interdisciplinary pediatric chronic pain management program at a large Northeastern children's hospital in the United States were asked to complete questionnaires assessing demographic and pain characteristics, pain acceptance, and functional impairment and anxiety (PROMIS; t-scores). Number of visits within the program were gathered from medical record. Youth were primarily Caucasian/White (86.2%), Other (6.9%), AAPI (3.4%) and Mixed Race (3.4%). Youth reported overall low pain acceptance (M=34.1, SD=9.3), moderate anxiety (M=57.1, SD=13.1) and high pain interference (M=63, SD =7.4). Caregivers reported observing moderate anxiety (M=59.1, SD =11.5) and high pain interference (M=65.1, SD=4.6) for youth. Adolescents were observed to attend a wide range of visits within the program, including medical (1.7 +/- 2.5), physical therapy (3.4 +/- 4.9), occupational therapy (.5 +/- 1.7), and psychology (4.3 +/- 3.6). Less is known about youth with POTS who present for treatment. Additionally, youth with POTS meet with multiple doctors before receiving a diagnosis leading to disparities in access to treatment and the type of treatment that is received. Data here highlights that youth with POTS are experiencing significant impairment and more research targeting this population is necessary to improve outcomes.

Impact of Psychological Disorders on Clinical Presentation of Pediatric Patients Following a Concussion

To examine differences in clinical symptom measures between pediatric patients with a history of depression and/or anxiety and no history of psychological disorders (PD) following a concussion. Limited information exists regarding impact of pre-existing psychological disorders on initial clinical presentation in pediatric patients following concussion. Data were prospectively collected from participants aged 5-18 diagnosed with a concussion between August 2015 and March 2020. Demographics and clinical measures from initial presentation were reviewed, including SCAT-5 Symptom, Patient Health Questionnaire (PHQ-8), Generalized Anxiety Disorder (GAD-7) scale, and Brief Resiliency Scale (BRS). Participants were separated into 4 groups based on self-reported prior diagnosis: depression, anxiety, both, and no PD. One thousand seven hundred seventy participants included: 50 depression, 82 anxiety, 84 both, and 1,554 no PD history. There was no significant difference in age, sex, prior concussion history, or time to presentation between the depression and no PD group, or the anxiety and no PD group. The both group was older, had more females and prior concussions, and presented later than the no PD group. The depression, anxiety, and both groups had higher rates of learning disorders than the no PD group (40%, 47.6%, 46.4% vs 16.4%, all p < 0.001). Each PD group had higher symptom severity scores than the no PD group. The PD groups all reported higher GAD-7 and PHQ-8 scores and lower BRS scores compared to the no PD group. The both group had the highest symptom severity, GAD-7, and PHQ-8 scores along with the lowest BRS score. All findings p = 0.001. Differences were seen in participants with a history of depression and/or anxiety at initial clinical presentation, including history of learning disability, symptom severity scores, and screening tests for depression, anxiety, and resiliency compared to those without a history of PD. Understanding differences at initial presentation may urge providers to engage multidisciplinary teams in facilitating patient recovery.

Pediatric Avoidant-Restrictive Food Intake Disorder and gastrointestinal-related Somatic Symptom Disorders: Overlap in clinical presentation.

Certain presentations of Avoidant/Restrictive Food Intake Disorder (ARFID) and Somatic Symptom and Related Disorders (SSRDs) have conceptual overlap, namely, distress and impairment related to a physical symptom. This study compared characteristics of pediatric patients diagnosed with ARFID to those with gastrointestinal (GI)-related SSRD. A 5-year retrospective chart review at a tertiary care pediatric hospital comparing assessment data of patients with a diagnosis of ARFID (n = 62; 69% girls, Mage = 14.08 years) or a GI-related SSRD (n = 37; 68% girls, Mage = 14.25 years). Patients diagnosed with ARFID had a significantly lower percentage of median BMI than those with GI-related SSRD. Patients diagnosed with ARFID were most often assessed in the Eating Disorders Program, whereas patients diagnosed with an SSRD were most often assessed by Consultation-Liaison Psychiatry. Groups did not differ on demographics, psychiatric diagnoses, illness duration, or pre-assessment services/medications. GI symptoms were common across groups. Patients diagnosed with an SSRD had more co-occurring medical diagnoses. A subset (16%) of patients reported symptoms consistent with both diagnoses. Overlap is observed in the clinical presentation of pediatric patients diagnosed with ARFID or GI-related SSRD. Some group differences emerged, including anthropometric measurements and co-occurring medical conditions. Findings may inform diagnostic classification and treatment approach.

INITIAL CLINICAL PRESENTATION OF PEDIATRIC PATIENTS WITH PSYCHOLOGICAL DISORDERS FOLLOWING CONCUSSION

Background:There is limited evidence regarding the impact of pre-existing psychological disorders on the initial clinical presentation in pediatric patients following concussion.Hypothesis/Purpose:To examine differences in clinical symptom measures between pediatric patients with a history of depression and/or anxiety and no history of psychological disorder (PD) following a concussion.Methods:Data were prospectively collected from participants enrolled in the North Texas Concussion Network Registry (ConTex) between August 2015 and March 2020. Participants aged 5-18 years diagnosed with a concussion were included. Demographic variables and a range of clinical measures from initial presentation were reviewed, including SCAT-5 Symptom Log, Patient Health Questionnaire (PHQ-8), Generalized Anxiety Disorder (GAD-7) scale, and Brief Resiliency Scale (BRS). Participants were separated into four groups based on self-reported prior diagnosis: depression, anxiety, depression+anxiety, and no PD.Results:A total of 1770 participants were included: 50 with depression, 82 with anxiety, 84 with both, and 1554 with no history of PD. There was no significant difference in age, sex, prior concussion history, or time to presentation between the depression and no PD group, or between the anxiety and no PD group. A significant difference was found between the depression+anxiety group and no PD group in the following variables: age (15.11±1.8 vs 13.68±2.61 years, p<0.001), prior concussion history (40.5% vs 23.9%, p=0.001), and time to presentation (31.47±25.82 vs 19.85±26.33 days, p=0.01). Additionally, there were more females in the depression+anxiety group than the no PD group (71.4% vs 47.8%, p<0.001). The depression, anxiety, and depression+anxiety groups had significantly higher rates of learning disorders than the no PD group (40%, 47.6%, 46.4% vs 16.4%, all p<0.001).A significant difference in SCAT-5 symptom severity scores between the depression, anxiety, and depression+anxiety groups compared to the no PD group was found. The PD groups all reported higher GAD-7 and PHQ-8 scores and lower BRS scores compared to the no PD group. The depression+anxiety group had the highest symptom severity, GAD-7, and PHQ-8 scores along with the lowest BRS score. Table 1.1 summarizes these significant findings.Conclusion:Differences were seen in participants with a history of depression and/or anxiety at initial clinical presentation, including history of learning disability, SCAT-5 symptom severity scores, and common screening tests for depression, anxiety, and resiliency compared to those without a history of PD. Understanding these differences at initial presentation may urge providers to engage multidisciplinary teams early in facilitating patient recovery.Tables/Figures:

Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis

ObjectiveTo evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. MethodsCase series of ten patients (median age 13.2 years), followed‐up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow‐up. ResultsAll patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. ConclusionIn this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time.

Pediatric Patients with SARS-CoV-2 Infection: Clinical Characteristics in the United States from a Large Global Health Research Network

BackgroundFew reports have been published on the clinical presentation of pediatric patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We aim to shed more light on the clinical presentation of pediatric patients infected with coronavirus disease 2019 (COVID-19), and also potential risk factors for more severe clinical case presentation.MethodsWe used a large global health research network to gather clinical data extracted from the electronic medical records of pediatric patients aged < 18 years with confirmed SARS-CoV-2 from January 1, 2020 to May 7, 2020. Clinical symptoms at presentation, hospitalization status, associated co-morbidities, and treatments received were reviewed.ResultsA total of 627 patients with COVID-19 diagnosis (334 were outpatient, 293 were inpatient) were included from a total of 20 organizations across the United States. The mean age of patients was seven years, 48% were females. Inpatients were younger than outpatients (mean age of 5.6 years vs 8.2 years, p<0.001). Sixty-one percent of patients in the inpatient group were < 5 years of age vs. 44% in the outpatient group. Amongst 293 inpatients, 90% (n=265) were non-severe and 10% (n=28) were classified as severe. The percentage of patients <5 years was higher in severe inpatients vs. non-severe (71% vs 60%.) Significantly more patients with a severe illness vs. non-severe illness had a history of co-morbidity including non-congenital heart disease (50% vs 11%, p<0.001) and disease of the respiratory system (86% vs 53%, p< 0.001).ConclusionClinicians should closely monitor young children with underlying conditions and COVID-19, as they may be more likely to be hospitalized and have a higher severity of the disease.

Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis

ObjectiveTo evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. MethodsCase series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up. ResultsAll patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. ConclusionIn this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time.

Acute mastoiditis: 20 years of experience with a uniform management protocol

ObjectivesTo characterize the clinical presentation of pediatric patients who, upon AM diagnosis, also had imaging-diagnosed ICCs (ID-ICCs); to define the group of AM patients at risk of developing ID-ICCs; and to update knowledge about organisms causing AM. Study designAnalysis of all AM patients admitted between 1997 and 2018 and treated according to an obligatory protocol including both brain imaging and sampling for bacterial culture upon clinical diagnosis of AM. ResultsOf 166 admitted patients (0.5–19 years old) 22 (13%) already had ID-ICCs. In patients who, on admission, had already received antibiotics for acute otitis media (AOM) and also had CRP (C-reactive protein) levels above 93.5 mg/L, the risk of ID-CC was increased by 22.5-fold (P < 0.0001). Bacterial culture results were available for all patients and were positive in 115 (69%). Organisms most commonly found in patients without prior antibiotic treatment were group A Streptococcus pyogenes (53%), Streptococcus pneumoniae (23%), and Haemophylus influenzae (11%), while with prior antibiotic treatment they were Fusobacterium necrophorum (21%), Streptococcus pyogenes (18%) and Pseudomonas aeruginosa (18%). ConclusionsSince the risk of ID-ICC in patients with the abovementioned CRP and prior antibiotic treatment was significantly higher than in the others, these high-risk patients should undergo diagnostic imaging on admission. Antibiotic treatment prior to AM development may promote growth of non-AOM pathogen.

Pediatric Anaphylaxis in the Emergency Department: Clinical Presentation, Quality of Care, and Reliability of Consensus Criteria.

The objective of this study was to describe the quality of emergency department (ED) care for pediatric patients with anaphylaxis. The secondary objectives were to describe (1) the clinical presentation of pediatric patients with anaphylaxis including the proportion of patients meeting each of the National Institute of Allergy and Infectious Diseases (NIAID) consensus criteria and subcriteria and (2) the Interrater reliability (IRR) for applying the NIAID criteria. We conducted a retrospective cross-sectional chart review of patients seen in a pediatric ED during a 2-year period. All charts were reviewed by a trained chart reviewer with 10% abstracted by a second reviewer. Data were analyzed using descriptive statistics including proportions, medians, and interquartile range. Interrater reliability was calculated using Cohen unweighted κ or percent agreement. Of the 250 charts reviewed, 84% (211) met the NIAID criteria for anaphylaxis (IRR, 1.0). Only 68% of patients received epinephrine in the ED or within 3 hours of the ED visit. Adherence was poor and IRR was variable, for measures reflecting documentation of discharge instructions and follow-up with a specialist. The IRR of reviewers for determining which patients met the NIAID criteria overall and for each subcriterion was high. Our findings highlight a gap between best practice and ED care. In addition, our results suggest that the NIAID criteria can reliably be used to retrospectively identify pediatric patients with anaphylaxis. Accurately identifying cases is a prerequisite for measuring gaps in management and developing interventions to improve care.

Early Functional Outcomes for Pediatric Patients Diagnosed with Anti-N-Methyl-D-Aspartate Receptor Encephalitis during Inpatient Rehabilitation.

The aims of the current study were to characterize the demographic and clinical presentation of pediatric patients diagnosed with anti-N-methyl-D-aspartate receptor encephalitis who require inpatient rehabilitation, to examine early functional outcomes, and to investigate predictors of early recovery. A retrospective chart review was conducted for 27 pediatric patients diagnosed with anti-N-methyl-D-aspartate receptor encephalitis who received intensive inpatient neurorehabilitation. On average, patients were 10.6 yrs of age (range, 2-18 yrs) at the time of symptom onset. Average time to treatment from symptom onset was 27.2 days (range, 5-91 days). Patients displayed significant improvements between admission and discharge Functional Independence Measure for Children (WeeFIM) Developmental Functional Quotient (DFQ) scores across patients (P < 0.01). Mean Functional Independence Measure for Children Total Developmental Functional Quotient score at admission was 28.6 (range, 15.0-62.6) and at discharge was 54.3 (range, 14.2-91.9). Younger age at onset, seizures, and number of treatments received were associated with worse functional outcomes at discharge. Time to initiate treatment was not found to be associated with early functional outcomes. Pediatric patients diagnosed with anti-N-methyl-D-aspartate receptor encephalitis displayed significant functional gains during inpatient rehabilitation, despite persistent functional deficits at discharge, suggesting the need for ongoing monitoring and intervention. Complete the self-assessment activity and evaluation online at http://www.physiatry.org/JournalCME CME OBJECTIVES: Upon completion of this article, the reader should be able to (1) Recognize the clinical presentation of anti-N-methyl-D-aspartate receptor encephalitis in pediatric patients, (2) Appreciate the role of rehabilitation in the care of the pediatric patient with anti-N-methyl-D-aspartate receptor encephalitis, and (3) Identify demographic and clinical variables that predict poor functional outcomes after rehabilitation in pediatric patients with anti-N-methyl-D-aspartate receptor encephalitis. Advanced. The Association of Academic Physiatrists is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.The Association of Academic Physiatrists designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest

To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R). This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014. A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT). Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest.

Periventricular Location as a Risk Factor for Hemorrhage and Severe Clinical Presentation in Pediatric Patients with Untreated Brain Arteriovenous Malformations.

The morphologic features of brain arteriovenous malformations differ between children and adults; therefore, our aim was to analyze various features of brain arteriovenous malformations to assess the risk of hemorrhage in children. We identified all consecutive children admitted to Beijing Tiantan Hospital for brain arteriovenous malformations between July 2009 and April 2014. The effects of demographic characteristics and brain arteriovenous malformation morphology on hemorrhage presentation, annual bleeding rates, postnatal hemorrhage, and immediate posthemorrhagic neurologic outcomes were studied by using univariate and multivariable regression analyses. A total of 108 pediatric brain arteriovenous malformation cases were identified, 66 (61.1%) of which presented with hemorrhage. Of these, 69.7% of ruptured brain arteriovenous malformations were in a periventricular location. Periventricular nidus location (OR, 3.443; 95% CI, 1.328-8.926; P = .011) and nidus size (OR, 0.965; 95% CI, 0.941-0.989; P = .005) were independent predictors of hemorrhagic presentation. The annual hemorrhage rates in children with periventricular brain arteriovenous malformations were higher at 6.88% (OR, 1.965; 95% CI, 1.155-3.341; P < .05). The hemorrhage-free survival rates were also lower for children with periventricular brain arteriovenous malformations (log-rank, P = .01). Periventricular location (hazard ratio, 1.917; 95% CI, 1.131-3.250; P = .016) and nidus size (hazard ratio, 0.983; 95% CI, 0.969-0.997; P = .015) were associated with hemorrhage after birth in pediatric brain arteriovenous malformations. An ordinal analysis showed lower immediate posthemorrhage mRS in patients with periventricular brain arteriovenous malformations (OR for greater disability, 2.71; 95% CI, 1.03-7.11; P = .043). Small periventricular brain arteriovenous malformations were associated with increased hemorrhage risk in pediatric patients. Cautious follow-up of children with untreated periventricular brain arteriovenous malformations is recommended because of a higher hemorrhage risk and potentially more severe neurologic outcomes.

Endoscopic Approach for the Treatment of Pineal Region Tumors

Tumors of the pineal region account for 3 to 8% of the tumors involving the central nervous system. The most common are germ cell tumors (39%). Less common examples include teratomas, primitive neuroectodermic tumors, astrocytomas, and choriocarcinomas. Clinical presentation in pediatric patients is in direct relation to the anatomical structures surrounding the pineal region and to the associated hydrocephalus that is present in almost 90% of cases. The diagnosis of a tumor in the pineal region is based on radiological findings and the presence of tumor markers such as α-fetoprotein and human chorionic gonadotrophic hormone subfraction β. Neuroendoscopy is considered to be one of the minimally invasive techniques useful for the management of such patients. This minimally invasive approach to pineal region tumors allows the treatment of hydrocephalus in a safe and effective way, avoiding the complications derived from other procedures such as external ventricular drainage or shunt surgery.

Macrophagic Myofasciitis in Children Is a Localized Reaction to Vaccination

Macrophagic myofasciitis is a novel, "inflammatory myopathy" described after a variety of vaccinations, almost exclusively in adults. We examined the relevance of histological findings of this myopathy to the clinical presentation in pediatric patients. Muscle biopsies from 8 children (7 months to 6 years old) with histological features of macrophagic myofasciitis were reviewed and correlated with the clinical manifestations. Patients underwent quadriceps muscle biopsy for suspected mitochondrial disease (4 patients), spinal muscular atrophy (2 patients), myoglobinuria (1 patient), and hypotonia with motor delay (1 patient). All biopsies showed identical granulomas composed of periodic acid-Schiff-positive and CD68-positive macrophages. Characteristic aluminum hydroxide crystals were identified by electron microscopy in 2 cases. The biopsy established diagnoses other than macrophagic myofasciitis in 5 patients: spinal muscular atrophy (2), Duchenne muscular dystrophy (1), phospho-glycerate kinase deficiency (1), and cytochrome c oxidase deficiency (1). Three children with manifestations and/or a family history of mitochondrial disease had otherwise morphologically normal muscle. All children had routine vaccinations between 2 months and 1 year before the biopsy, with up to 11 intramuscular injections, including the biopsy sites. There was no correlation between histological findings of macrophagic myofasciitis in biopsies and the clinical symptoms. We believe that macrophagic myofasciitis represents a localized histological hallmark of previous immunization with the aluminum hydroxide adjuvants contained in vaccines, rather than a primary or distinct inflammatory muscle disease.