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Sacrococcygeal teratoma is the most common extragonadal germ cell tumor in the pediatric population, and accounts for approximately 70% of all teratomas in childhood.1,2 They present in two distinct phases, with most cases seen in neonates with large predominately exophytic tumors, often detected in utero on prenatal sonography or at birth. A smaller cohort presents in older infants and children with primarily hidden tumors in the pelvis which have a much higher rate of malignancy. The primary surgical objective is complete tumor resection without compromise to critical structures or function. Herein we outline the critical elements of tumor resection and management of sacrococcygeal germ cell tumors with a focus on the technical aspects of this tumor across a range of presentations.
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Neoplasias Embrionárias de Células Germinativas , Teratoma , Lactente , Recém-Nascido , Gravidez , Feminino , Humanos , Criança , Idoso , Região Sacrococcígea/cirurgia , Região Sacrococcígea/patologia , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Neoplasias Embrionárias de Células Germinativas/cirurgia , Teratoma/diagnóstico , Teratoma/cirurgia , Teratoma/patologia , Ultrassonografia Pré-NatalRESUMO
OBJECTIVE: Perinatal mental health and substance use disorders (PMHSUD) often go unrecognized and untreated. This study examined the use of the Project ECHO model to teach obstetric, primary care, and mental health clinicians about screening, diagnosis, and treatment of PMHSUD. METHODS: Participants in 3 years of the University of Washington's Moms' Access Project (MAP) ECHO program (2019-2022) completed pre- and post-program surveys. Nine participants in year 1 completed qualitative interviews. Dedoose was used for qualitative analysis of interviews. RESULTS: Of 136 participants, 62.5% (15/24) completed both pre- and post-surveys in year 1, 56% (28/50) in year 2, and 32.2% (20/62) in year 3. Most respondents agreed or strongly agreed that they were glad to have participated (96.8%; 60/62) and that they had used information learned in the program in treating a patient (95.1%; 58/61). In all years, respondents endorsed increased confidence regarding learning objectives of the program. Qualitative interviews following year 1 yielded themes of hierarchy of competence, motivation versus results of participation, connection, and politics of change: position and practice type. CONCLUSIONS: Findings supported the feasibility, acceptability, and self-reported effectiveness of the ECHO model for workforce development in PMHSUD.
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Importance: Prostacyclin (PGI2) is a therapeutic option to treat congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension in neonates. Its use may decrease the need for extracorporeal life support (ECLS). Objective: To evaluate the association of early PGI2 therapy with ECLS use and outcomes among patients with CDH. Design, Setting, and Participants: This was a cohort study from the CDH Study Group (CDHSG) registry of patients born from January 2007 to December 2019. Patients were from 88 different tertiary pediatric referral centers worldwide that contributed data to the CDHSG. Patients were included in the study if they were admitted within the first week of life. Propensity score matching was performed using estimated gestational age, birth weight, transfer status, 1-minute and 5-minute Apgar scores, highest and lowest partial pressure of arterial carbon dioxide in the first 24 hours of life, and degree of pulmonary hypertension as covariates to generate a matched cohort of exposed and unexposed patients. Data were analyzed from January 2021 to December 2022. Exposures: Early PGI2 therapy was defined as initiation of PGI2 within the first week of life. Patients who received ECLS were included in the early PGI2 group if PGI2 was started prior to ECLS. Main Outcomes and Measures: The primary outcome of the study was the proportion of patients receiving ECLS in the exposed and unexposed groups. Results: Of 6227 patients who met inclusion criteria (mean [SD] gestational age, 37.4 [2.36] weeks; 2618 [42%] female), 206 (3.3%) received early PGI2 therapy. ECLS was used in 46 of 206 patients who received PGI2 (22.2%) and 1682 of 6021 who did not (27.9%). After propensity score matching, there were 147 patients in the treatment and control groups. Thirty-four patients who received PGI2 (23.3%) and 63 who did not (42.9%) received ECLS. Those who received PGI2 were less likely to receive ECLS (adjusted odds ratio, 0.39; 95% CI, 0.22-0.68) and had shorter mean (SD) duration of ECLS (8.6 [3.73] days vs 12.6 [6.61] days; P < .001), although there was no significant difference in in-hospital mortality. Conclusions and Relevance: In this study, there was decreased use of ECLS and decreased ECLS duration among patients with CDH who started PGI2 therapy during the first week of life. These results identify a potential advantage of early prostacyclin therapy in this population.
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Oxigenação por Membrana Extracorpórea , Hérnias Diafragmáticas Congênitas , Hipertensão Pulmonar , Recém-Nascido , Humanos , Feminino , Criança , Adulto , Masculino , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/terapia , Estudos de Coortes , Hipertensão Pulmonar/terapia , Epoprostenol/uso terapêutico , Estudos RetrospectivosRESUMO
PURPOSE: Off-label use of prostacyclins to manage congenital diaphragmatic hernia-associated pulmonary hypertension (CDH-PHTN) has been described over recent years, but use is not standardized across institutions. This study aims to describe trends in use of these medications in the CDH Study Group (CDHSG) patients. METHODS: The CDHSG was queried for all patients born from 2007 to 2019. Records were reviewed to describe the number of patients receiving prostacyclins, the day of life on which the agent was started, start time relative to ECLS, the duration of medication use, and continuation of the medication at the time of discharge. Finally, trends in use by year of birth were evaluated to assess for changes in use over time. RESULTS: There were 6439 patients identified from the registry who were born during the study period. 4372 (68%) patients received medications to treat pulmonary hypertension. Of these, 604 (14%) received a prostacyclin at some point during their care. The median start time for prostacyclins was 7.5 days of life (mean 16.9 days, SD 32.5 days), and the median duration was 12.5 days (mean 25.1 days, SD 49.1 days). Among patients who received prostacyclins, 340 patients required ECLS during care, 53 (15.5%) of whom started the prostacyclin prior to ECLS, and 159 (46.8%) of whom started prostacyclin therapy during their ECLS run. Only a small cohort (26/604, 4.3%) required continuation of the prostacyclin at the time of discharge. The proportion of patients receiving a prostacyclin remained relatively stable over the study period. CONCLUSIONS: While the proportion of patients receiving a prostacyclin for management of CDH-PHTN has remained relatively stable over the last 13 years, there is significant variation in timing of initiation and duration of use especially in the pre-ECLS period that warrants further investigation to describe optimal use in these patients.
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Hérnias Diafragmáticas Congênitas , Hipertensão Pulmonar , Epoprostenol/uso terapêutico , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Sistema de Registros , Estudos RetrospectivosRESUMO
BACKGROUND: Published data demonstrate that management of uncomplicated pediatric appendicitis with antibiotics-alone is safe and frequently successful. Randomized controlled trials (RCT) comparing antibiotics-alone to appendectomy are lacking, alongside insight into drivers of failure. We sought to validate the antibiotics-alone approach and identify barriers to success using an RCT design. METHODS: Patients aged 6-17 years with uncomplicated appendicitis were randomized to appendectomy or intravenous piperacillin/tazobactam for 24-48 h followed by 10 days of oral ciprofloxacin/metronidazole. Enrollment required symptoms <48 h, WBC<18, appendiceal diameter <11 mm, and radiographic absence of perforation. Lack of clinical improvement or persistently elevated WBC resulted in appendectomy. Primary outcomes were 1-year success rate of antibiotics-alone and quality-of-life measures. RESULTS: Among 39 children enrolled over 31 months, 20 were randomized to antibiotics-alone and 19 to surgery. At 1 year, 6 nonoperative patients underwent appendectomy (70% success). Four cases were not true antibiotic failures but instead reflected "pragmatic" challenges to executing nonoperative algorithms. Only 2 cases represented recurrent/refractory appendicitis, suggesting a 90% adjusted 1-year success rate. Parental PedsQL™ scores were similar between treatment cohorts (91.3 vs 90.2, P = 0.32). Children treated with antibiotics-alone had faster return to activity (2.0 vs 12 days, P = 0.001) and fewer parental missed work days (0.0 vs 2.5, P = 0.03). CONCLUSIONS: These data corroborate findings from non-randomized studies suggesting 70-90% of uncomplicated pediatric appendicitis can be treated with antibiotics-alone, with fewer disability days. Failures appear multifactorial, often reflecting practical hurdles and not antibiotic limitations. As surgeons consider nonoperative protocols for uncomplicated appendicitis, these data further inform the variability of treatment success. LEVEL OF EVIDENCE: 1; randomized controlled trial.
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Apendicite , Doença Aguda , Antibacterianos/uso terapêutico , Apendicectomia , Apendicite/tratamento farmacológico , Apendicite/cirurgia , Criança , Seguimentos , Humanos , Resultado do TratamentoRESUMO
PURPOSE: Complications associated with use of dual-lumen venovenous extracorporeal membrane oxygenation (VV-ECMO) cannulas are of concern in pediatric patients. While the risk of atrial perforation is believed to be higher with bicaval cannulas, direct comparison of complication rate between atrial and bicaval cannulas has not been conducted in this population. METHODS: A retrospective review was conducted at a free-standing children's hospital of all patients 0-18 years old, placed on VV-ECMO with a dual-lumen cannula from January 2009 to December 2018. Patients were grouped based on cannula type. Complications were assessed over the entire duration of the ECMO run. Logistic regression analyses were used to evaluate for an association between cannula type and risk of pericardial effusion or cannula-related complication requiring median sternotomy or pericardial drain placement. RESULTS: During the study period 119 patients were placed on VVECMO using a dual-lumen cannula. Eighty-two patients (69%) were <2 years old, 19 (16%) were 2-10 years old, and 18 (15%) were 11-18 years old. Seventy-three were cannulated with an atrial cannula and 46 patients received a bicaval cannula. Pericardial effusions were seen in 30% and 24% of these patients respectively while severe complications were seen in 9.6% and 8.7% of patients respectively. Compared to patients treated with a bicaval cannula, those who received an atrial cannula had similar odds of effusions (OR: 1.41, 95% CI: 0.62-3.36) and severe complications (OR 0.89, 95% CI: 0.27-3.18). After adjusting for age, weight, cannula and circuit manipulations, and use of echocardiography, the OR of effusion was 1.91 (95% CI: 0.65-6.42), and the adjusted OR of severe complication was 0.69 (95% CI: 0.16-3.33). CONCLUSIONS: There were no significant differences in frequency of pericardial effusions or severe cannula-related complications between the treatment groups across all pediatric patients and within the subgroup of patients under 2 years of age.
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Oxigenação por Membrana Extracorpórea , Traumatismos Cardíacos , Derrame Pericárdico , Adolescente , Cânula/efeitos adversos , Criança , Pré-Escolar , Oxigenação por Membrana Extracorpórea/efeitos adversos , Átrios do Coração , Humanos , Lactente , Recém-Nascido , Derrame Pericárdico/etiologiaRESUMO
PURPOSE: There is a growing body of literature regarding long-term pulmonary outcomes in children with congenital diaphragmatic hernia (CDH). Oral feeding skills in these children are often delayed. Chronic descending aspiration due to uncoordinated swallowing can further insult the already compromised lung parenchyma in these children. This study describes patterns of swallowing dysfunction and aspiration in patients with CDH. METHODS: Records of all children treated for CDH at our institution from January 2014 to December 2019 were reviewed. Concern for swallowing dysfunction was marked by performance of a video-fluoroscopic swallow study (VFSS). We determined the frequency of aspiration on VFSS and how frequently that finding changed patient management. We also evaluated for association between clinical suspicion of swallow dysfunction and descriptors of CDH severity. RESULTS: Sixty-nine patients were treated during this 6-year time period. Of those, 10 (14%) had a VFSS as an inpatient, and 25 (36%) had one as an outpatient. Eight (80%) inpatient and 17 (68%) outpatient studies identified aspiration. VFSS results changed management in 80% of patients, often by altering the consistency of oral feeds. There were no associations between CDH side, defect size or need for a patch and need for a VFSS. CONCLUSIONS: The frequency of aspiration in the CDH population is high. Identification of aspiration on VFSS leads to changes in treatment aimed at protecting the lungs. Additionally, the severity of the CDH was not associated with aspiration on VFSS.
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Transtornos de Deglutição/etiologia , Hérnias Diafragmáticas Congênitas/complicações , Criança , Transtornos de Deglutição/diagnóstico por imagem , Transtornos de Deglutição/epidemiologia , Fluoroscopia , Humanos , IncidênciaAssuntos
Teste para COVID-19 , COVID-19/diagnóstico , Gastroenteropatias/virologia , SARS-CoV-2 , Adolescente , COVID-19/complicações , Reações Falso-Negativas , Gastroenteropatias/diagnóstico , Gastroenteropatias/diagnóstico por imagem , Gastroenteropatias/etiologia , Humanos , Masculino , Nasofaringe , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration. METHODS: Sites from the Pediatric Surgery Oncology Research Collaborative performed a retrospective review of children with NA over a 6-year period. RESULTS: Sixty-six children, with a median age of 11 years (range 1-17), were identified with NA while undergoing cancer treatment. The most common cancer diagnoses were leukemia (62%) and brain tumor (12%). Upfront appendectomy was performed in 41% of patients; the remainder had initial nonoperative management. Rates of abscess or perforation at diagnosis were equivalent in the groups (30% vs 24%; P = .23). Of patients who had initial nonoperative management, 46% (17 of 37) underwent delayed appendectomy during the same hospitalization. Delayed appendectomy was due to failure of initial nonoperative management in 65% (n = 11) and count recovery in 35% (n = 6). Cancer therapy was delayed in 35% (n = 23). Initial nonoperative management was associated with a delay in cancer treatment (46% vs. 22%, P = .05) and longer LOS (29 vs 12 days; P = .01). Patients who had initial nonoperative management and delayed appendectomy had a higher rate of postoperative complications (P < .01). CONCLUSIONS: In pediatric patients with NA from oncologic treatment, upfront appendectomy resulted in lower complication rates, reduced LOS, and fewer alterations in chemotherapy regimens compared to initial nonoperative management.
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Apendicectomia/tendências , Apendicite/terapia , Neutropenia Febril Induzida por Quimioterapia/terapia , Neoplasias/terapia , Conduta Expectante/tendências , Adolescente , Apendicite/diagnóstico , Apendicite/epidemiologia , Neutropenia Febril Induzida por Quimioterapia/diagnóstico , Neutropenia Febril Induzida por Quimioterapia/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Neoplasias/diagnóstico , Neoplasias/epidemiologia , Estudos Retrospectivos , Conduta Expectante/métodosAssuntos
Avaliação Geriátrica , Geriatria/métodos , Cuidados Pré-Operatórios/métodos , Procedimentos Cirúrgicos Operatórios , Procedimentos Desnecessários/estatística & dados numéricos , Idoso , Idoso de 80 Anos ou mais , Envelhecimento , Cognição , Comorbidade , Delírio , Feminino , Idoso Fragilizado , Avaliação Geriátrica/métodos , Humanos , Masculino , Saúde Mental , Avaliação Nutricional , Estado Nutricional , Polimedicação , Guias de Prática Clínica como Assunto , Cuidados Pré-Operatórios/estatística & dados numéricosRESUMO
Angelman syndrome (AS) is a neurodevelopmental disorder associated with developmental delay, lack of speech, motor dysfunction, and epilepsy. In the majority of the patients, AS is caused by the deletion of small portions of maternal chromosome 15 harboring the UBE3A gene. This results in a lack of expression of the UBE3A gene because the paternal allele is genetically imprinted. The UBE3A gene encodes an enzyme termed ubiquitin ligase E3A (E6-AP) that targets proteins for degradation by the 26S proteasome. Because neurodegenerative disease and other neurodevelopmental disorders have been linked to oxidative stress, we asked whether mitochondrial reactive oxygen species (ROS) played a role in impaired synaptic plasticity and memory deficits exhibited by AS model mice. We discovered that AS mice have increased levels of superoxide in area CA1 of the hippocampus that is reduced by MitoQ 10-methanesuflonate (MitoQ), a mitochondria-specific antioxidant. In addition, we found that MitoQ rescued impairments in hippocampal synaptic plasticity and deficits in contextual fear memory exhibited by AS model mice. Our findings suggest that mitochondria-derived oxidative stress contributes to hippocampal pathophysiology in AS model mice and that targeting mitochondrial ROS pharmacologically could benefit individuals with AS. SIGNIFICANCE STATEMENT: Oxidative stress has been hypothesized to contribute to the pathophysiology of neurodevelopmental disorders, including autism spectrum disorders and Angelman syndrome (AS). Herein, we report that AS model mice exhibit elevated levels of mitochondria-derived reactive oxygen species in pyramidal neurons in hippocampal area CA1. Moreover, we demonstrate that the administration of MitoQ (MitoQ 10-methanesuflonate), a mitochondria-specific antioxidant, to AS model mice normalizes synaptic plasticity and restores memory. Finally, our findings suggest that antioxidants that target the mitochondria could be used therapeutically to ameliorate synaptic and cognitive deficits in individuals with AS.
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Síndrome de Angelman/complicações , Hipocampo , Mitocôndrias/metabolismo , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/patologia , Superóxidos/metabolismo , Sinapses/fisiologia , Análise de Variância , Animais , Condicionamento Psicológico , Modelos Animais de Doenças , Estimulação Elétrica , Medo , Hipocampo/metabolismo , Hipocampo/patologia , Hipocampo/ultraestrutura , Técnicas In Vitro , Camundongos , Camundongos Endogâmicos C57BL , Atividade Motora/fisiologia , Compostos Organofosforados/metabolismo , Ubiquinona/análogos & derivados , Ubiquinona/metabolismoRESUMO
Angelman syndrome (AS) is associated with symptoms that include autism, intellectual disability, motor abnormalities, and epilepsy. We recently showed that AS model mice have increased expression of the alpha1 subunit of Na/K-ATPase (α1-NaKA) in the hippocampus, which was correlated with increased expression of axon initial segment (AIS) proteins. Our developmental analysis revealed that the increase in α1-NaKA expression preceded that of the AIS proteins. Therefore, we hypothesized that α1-NaKA overexpression drives AIS abnormalities and that by reducing its expression these and other phenotypes could be corrected in AS model mice. Herein, we report that the genetic normalization of α1-NaKA levels in AS model mice corrects multiple hippocampal phenotypes, including alterations in the AIS, aberrant intrinsic membrane properties, impaired synaptic plasticity, and memory deficits. These findings strongly suggest that increased expression of α1-NaKA plays an important role in a broad range of abnormalities in the hippocampus of AS model mice.