RESUMEN
BACKGROUND: Clinical decision making for pediatric neck trauma is challenging because data and reports are sparse. We present a case report showing current recommendations for managing pediatric neck injuries. CASE PRESENTATION: This is the case of an 11-year-old boy who presented to our Level I trauma center after a slip and fall on a metal boat cleat (metal fixture used to secure rope). He suffered a penetrating injury to his neck, requiring operative exploration. This case report provides an overview on the rare incidence of pediatric penetrating neck trauma and treatment options. CONCLUSION: This case highlights penetrating neck injuries, which are uncommon in the pediatric population. This case report is unusual due to the method of injury and nature of the object. Neck trauma via a metal boat cleat is particularly rare. A comprehensive understanding of the anatomy of the neck, mechanism of injury, thorough clinical examination, and proper workup are essential to providing effective care.
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Traumatismos del Cuello , Heridas Penetrantes , Masculino , Niño , Humanos , Navíos , Estudios Retrospectivos , Traumatismos del Cuello/etiología , Traumatismos del Cuello/cirugía , Traumatismos del Cuello/diagnóstico , Heridas Penetrantes/etiología , Heridas Penetrantes/cirugía , Centros TraumatológicosRESUMEN
Multi-component lipid emulsions, rather than soy-oil emulsions, prevent cholestasis by an unknown mechanism. Here, we quantified liver function, bile acid pools, and gut microbial and metabolite profiles in premature parenterally fed pigs given a soy-oil lipid emulsion, Intralipid (IL), a multi component lipid emulsion, SMOFlipid (SMOF), a novel emulsion with a modified fatty-acid composition [experimental emulsion (EXP)], or a control enteral diet (ENT) for 22 days. We assayed serum cholestasis markers, measured total bile acid levels in plasma, liver, and gut contents, and analyzed colonic bacterial 16S rRNA gene sequences and metabolomic profiles. Serum cholestasis markers (i.e., bilirubin, bile acids, and γ-glutamyl transferase) were highest in IL-fed pigs and normalized in those given SMOF, EXP, or ENT. Gut bile acid pools were lowest in the IL treatment and were increased in the SMOF and EXP treatments and comparable to ENT. Multiple bile acids, especially their conjugated forms, were higher in the colon contents of SMOF and EXP than in IL pigs. The colonic microbial communities of SMOF and EXP pigs had lower relative abundance of several gram-positive anaerobes, including Clostridrium XIVa, and higher abundance of Enterobacteriaceae than those of IL and ENT pigs. Differences in lipid and microbial-derived compounds were also observed in colon metabolite profiles. These results indicate that multi-component lipid emulsions prevent cholestasis and restore enterohepatic bile flow in association with gut microbial and metabolomic changes. We conclude that sustained bile flow induced by multi-component lipid emulsions likely exerts a dominant effect in reducing bile acid-sensitive gram-positive bacteria.
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Ácidos y Sales Biliares/metabolismo , Colestasis/metabolismo , Colestasis/microbiología , Metabolismo de los Lípidos , Microbiota , Nacimiento Prematuro/metabolismo , Nacimiento Prematuro/microbiología , Animales , Colestasis/complicaciones , Nutrición Parenteral , PorcinosRESUMEN
New generation, multicomponent parenteral lipid emulsions provide key fatty acids for brain growth and development, such as docosahexaenoic acid (DHA) and arachidonic acid (AA), yet the content may be suboptimal for preterm infants. Our aim was to test whether DHA and AA-enriched lipid emulsions would increase activity, growth, and neurodevelopment in preterm piglets and limit brain inflammation. Cesarean-delivered preterm pigs were given three weeks of either enteral preterm infant formula (ENT) or TPN with one of three parenteral lipid emulsions: Intralipid (IL), SMOFlipid (SMOF) or an experimental emulsion (EXP). Activity was continuously monitored and weekly blood sampling and behavioral field testing performed. At termination of the study, whole body and tissue metrics were collected. Neuronal density was assessed in sections of hippocampus (HC), thalamus, and cortex. Frontal cortex (FC) and HC tissue were assayed for fatty acid profiles and expression of genes of neuronal growth and inflammation. After 3â¯weeks of treatment, brain DHA content in SMOF, EXP and ENT pigs was higher (Pâ¯<â¯0.01) in FC but not HC vs. IL pigs. There were no differences in brain weight or neuron density among treatment groups. Inflammatory cytokine TNFα and IL-1ß expression in brain regions were increased in IL pigs (Pâ¯<â¯0.05) compared to other groups. Overall growth velocity was similar among groups, but IL pigs had higher percent body fat and increased insulin resistance compared to other treatments (Pâ¯<â¯0.05). ENT pigs spent more time in higher physical activity levels compared to all TPN groups, but there were no differences in exploratory behavior among groups. We conclude that a soybean oil emulsion increased select brain inflammatory cytokines and multicomponent lipid emulsions enriched with DHA and AA in parenteral lipids results in increased cortical DHA and improved body composition without affecting short term neurodevelopmental outcomes.
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Ácidos Docosahexaenoicos , Recien Nacido Prematuro , Animales , Composición Corporal , Encéfalo , Emulsiones , Femenino , Aceites de Pescado , Humanos , Recién Nacido , Aceite de Oliva , Embarazo , Aceite de Soja , Porcinos , TriglicéridosRESUMEN
INTRODUCTION: Fetal magnetic resonance imaging (MRI) has been used to stratify severity of congenital diaphragmatic hernia (CDH) after ultrasound diagnosis. The purpose of this study was to determine if timing of MRI influenced prediction of severity of outcome in CDH. METHODS: A single institution retrospective review of all CDH referred to our institution from February 2004 to May 2017 was performed. Patients were included if they underwent at least 2 fetal MRIs prior to delivery. Prenatal MRI indices including observed-to-expected total fetal lung volume (o/e TFLV) were evaluated. Indices were categorized by trimester, either 2nd (20-27 weeks gestation) or 3rd trimester (>28 weeks gestation) and further analyzed for outcome predictability. Primary outcomes were survival, extracorporeal membrane oxygenation (ECMO), and pulmonary hypertension (PAH). Student t test and logistic and linear regression were used for data analyses. RESULTS: Of 256 fetuses evaluated for CDH, 197 were further characterized by MRI with 57 having both an MRI in the 2nd and 3rd trimesters. There was an average of 9.95 weeks (±4.3) between the 1st and 2nd MRI. Second trimester o/e TFLV was the only independent predictor of survival by logistic regression (OR 0.890, p < 0.01). Third trimester MRI derived lung volumes were associated with, and independent predictors of, severity of PAH and need for ECMO. Interval TFLV growth was a strong predictor of PAH postnatally (OR 0.361, p < 0.01). Overall cohort survival was 79%. CONCLUSION: Accuracy of MRI lung volumes to predict outcomes is dependent on the -gestational age at the time of exam. While MRI lung volumes at either the 2nd or 3rd trimester are predictive of morbidity, 2nd trimester lung volumes strongly correlated with mortality.
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Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Imagen por Resonancia Magnética , Diagnóstico Prenatal , Femenino , Hernias Diafragmáticas Congénitas/mortalidad , Hernias Diafragmáticas Congénitas/patología , Humanos , Tiempo de Internación , Pulmón/patología , Tamaño de los Órganos , Embarazo , Segundo Trimestre del Embarazo , Tercer Trimestre del Embarazo , Estudios Retrospectivos , Texas/epidemiologíaRESUMEN
Postnatal growth of lean mass is commonly blunted in preterm infants and may contribute to short- and long-term morbidities. To determine whether preterm birth alters the protein anabolic response to feeding, piglets were delivered at term or preterm, and fractional protein synthesis rates (Ks) were measured at 3 days of age while fasted or after an enteral meal. Activation of signaling pathways that regulate protein synthesis and degradation were determined. Relative body weight gain was lower in preterm than in term. Gestational age at birth (GAB) did not alter fasting plasma glucose or insulin, but when fed, plasma insulin and glucose rose more slowly, and reached peak value later, in preterm than in term. Feeding increased Ks in longissimus dorsi (LD) and gastrocnemius muscles, heart, pancreas, and kidney in both GAB groups, but the response was blunted in preterm. In diaphragm, lung, jejunum, and brain, feeding increased Ks regardless of GAB. Liver Ks was greater in preterm than term and increased with feeding regardless of GAB. In all tissues, changes in 4EBP1, S6K1, and PKB phosphorylation paralleled changes in Ks. In LD, eIF4E·eIF4G complex formation, phosphorylation of TSC2, mTOR, and rpS6, and association of mammalian target of rapamycin (mTOR1) complex with RagA, RagC, and Rheb were increased by feeding and blunted by prematurity. There were no differences among groups in LD protein degradation markers. Our results demonstrate that preterm birth reduces weight gain and the protein synthetic response to feeding in muscle, pancreas, and kidney, and this is associated with blunted insulin- and/or amino acid-induced translation initiation signaling.
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Animales Recién Nacidos , Ingestión de Alimentos , Biosíntesis de Proteínas , Transducción de Señal , Animales , Peso al Nacer , Glucemia/metabolismo , Femenino , Edad Gestacional , Riñón/metabolismo , Músculo Esquelético/metabolismo , Fenómenos Fisiológicos de la Nutrición , Páncreas/metabolismo , Porcinos , Serina-Treonina Quinasas TOR/metabolismo , Aumento de PesoRESUMEN
Enteroendocrine L cells and glucagon-like peptide 2 (GLP-2) secretion are activated in the intestinal adaptation process following bowel resection in patients with short bowel syndrome. We hypothesized that enteral activation of Takeda G protein-coupled receptor 5 (TGR5), expressed in enteroendocrine L cells, could augment endogenous GLP-2 secretion and the intestinal adaptation response. Our aim was to assess the efficacy of different TGR5 agonists to stimulate GLP-2 secretion and intestinal adaptation in a piglet short-bowel model. In study 1, parenterally fed neonatal pigs (n = 6/group) were gavaged with vehicle, olive extract (OE; 10 or 50 mg/kg), or ursolic acid (UA; 10 mg/kg), and plasma GLP-2 was measured for 6 h. In study 2, neonatal pigs (n = 6-8/group) were subjected to transection or 80% mid-small intestine resection and, after 2 days, assigned to treatments for 10 days as follows: 1) transection + vehicle (sham), 2) resection + vehicle (SBS), 3) resection + 30 mg UA (SBS + UA), and 4) resection + 180 mg/kg OE (SBS + OE). We measured plasma GLP-2, intestinal histology, cell proliferation, and gene expression, as well as whole body citrulline-arginine kinetics and bile acid profiles. In study 1, GLP-2 secretion was increased by UA and tended to be increased by OE. In study 2, SBS alone, but not additional treatment with either TGR5 agonist, resulted in increased mucosal thickness and crypt cell proliferation in remnant jejunum and ileum sections. SBS increased biliary and ileal concentration of bile acids and expression of inflammatory and farnesoid X receptor target genes, but these measures were suppressed by UA treatment. In conclusion, UA is an effective oral GLP-2 secretagogue in parenterally fed pigs but is not capable of augmenting GLP-2 secretion or the intestinal adaptation response after massive small bowel resection. NEW & NOTEWORTHY Therapeutic activation of endogenous glucagon-like peptide 2 (GLP-2) secretion is a promising strategy to improve intestinal adaptation in patients with short bowel syndrome. This study in neonatal pigs showed that oral supplementation with a selective Takeda G protein-coupled receptor 5 (TGR5) agonist is an effective approach to increase GLP-2 secretion. The results warrant further study to establish a more potent oral TGR5 agonist that can effectively improve intestinal adaptation in pediatric patients with SBS.
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Péptido 2 Similar al Glucagón/metabolismo , Mucosa Intestinal/metabolismo , Receptores Acoplados a Proteínas G , Síndrome del Intestino Corto , Adaptación Fisiológica , Animales , Animales Recién Nacidos , Proliferación Celular , Modelos Animales de Enfermedad , Células Enteroendocrinas/fisiología , Intestino Delgado/metabolismo , Nutrición Parenteral/métodos , Receptores Acoplados a Proteínas G/agonistas , Receptores Acoplados a Proteínas G/metabolismo , Síndrome del Intestino Corto/metabolismo , Síndrome del Intestino Corto/terapia , Porcinos , Resultado del TratamientoRESUMEN
BACKGROUND: The purpose of this study was to identify an optimal definition of massive transfusion in civilian pediatric trauma with severe traumatic brain injury (TBI) METHODS: Severely injured children (age ≤18 y) with severe TBI in the Trauma Quality Improvement Program research data sets 2015-2016 that received blood products were identified. Data were analyzed using descriptive statistics, Wilcoxon rank-sum, chi-square, and logistic regression. Continuous variables are presented as median (interquartile range). Massive transfusion thresholds were determined based on receiver operating curves and optimization of sensitivity and specificity RESULTS: Of the 460 included children, the mortality rate was 43%. There were no differences in demographics, heart rate at presentation, or injury severity score between children that lived or died. However, those who died had lower Glasgow coma scores (3 [3, 8] versus 3 [3, 3]; P < 0.01), were more likely to have had a penetrating injury (20% versus 11%; P < 0.01) and were more likely to be hypotensive for age (62% versus 34%; P < 0.01). Total blood products infused were greater in those who died (34 mL/kg/4-h [17, 65] versus 22 [12, 44]; P < 0.01). Sensitivity and specificity for delayed mortality was optimized at 40 mL/kg/4 h, and for the need for a hemorrhage control procedure at 50 mL/kg/4 h. These thresholds predicted delayed mortality (OR 2.12; 95% CI 1.28-3.50; P < 0.01) and the need for hemorrhage control procedures (5.47; 95% CI 2.82-10.61; P < 0.01) CONCLUSIONS: For children with TBI, a massive transfusion threshold of 40 mL/kg/4-h of total administered blood products may be used to identify at-risk patients, improve resource utilization, and guide future research methodology.
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Transfusión Sanguínea/estadística & datos numéricos , Lesiones Traumáticas del Encéfalo/terapia , Hemorragia/terapia , Selección de Paciente , Adolescente , Lesiones Traumáticas del Encéfalo/diagnóstico , Lesiones Traumáticas del Encéfalo/mortalidad , Niño , Preescolar , Femenino , Hemorragia/diagnóstico , Hemorragia/mortalidad , Humanos , Puntaje de Gravedad del Traumatismo , Masculino , Valor Predictivo de las Pruebas , Sistema de Registros/estadística & datos numéricos , Estudios Retrospectivos , Medición de Riesgo/métodos , Sensibilidad y Especificidad , Análisis de Supervivencia , Factores de TiempoRESUMEN
Necrotizing enterocolitis (NEC) is associated with low plasma arginine and vascular dysfunction. It is not clear whether low intestinal citrulline production, the precursor for arginine synthesis, occurs before and thus predisposes to NEC or if it results from tissue damage. This study was designed to test the hypothesis that whole body rates of citrulline, arginine, and nitric oxide synthesis are low in premature pigs and that they precede NEC. Piglets delivered by cesarean section at 103 days [preterm (PT)], 110 days [near-term (NT)], or 114 days [full-term (FT)] of gestation were given total parenteral nutrition and after 2 days orogastrically fed infant formula for 42 h to induce NEC. Citrulline and arginine fluxes were determined before and during the feeding protocol. Gross macroscopic and histological NEC scores and plasma fatty acid binding protein (iFABP) concentration were determined as indicators of NEC. Intestinal gene expression for enzymes of the arginine pathway were quantitated. A lower ( P < 0.05) survival rate was observed for PT (8/27) than for NT (9/9) and FT pigs (11/11). PT pigs had higher macroscopic gross ( P < 0.05) and histological NEC ( P < 0.05) scores and iFABP concentration ( P < 0.05) than pigs of more advanced gestational age. PT pigs had lower citrulline production and arginine fluxes ( P < 0.05) throughout and a reduced gene expression in genes of the citrulline-arginine pathway. In summary, intestinal enzyme expression and whole body citrulline and arginine fluxes were reduced in PT pigs compared with animals of more advance gestational age and preceded the development of NEC. NEW & NOTEWORTHY Arginine supplementation prevents necrotizing enterocolitis (NEC), the most common gastrointestinal emergency of prematurity. Citrulline (precursor for arginine) production is reduced during NEC, and this is believed to be a consequence of intestinal damage. In a swine model of NEC, we show that intestinal gene expression of the enzymes for citrulline production and whole body citrulline and arginine fluxes are reduced and precede the onset of NEC in premature pigs. Reduced citrulline production during prematurity may be a predisposition to NEC.
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Arginina/metabolismo , Citrulina/metabolismo , Enterocolitis Necrotizante/etiología , Desarrollo Fetal , Mucosa Intestinal/metabolismo , Óxido Nítrico/metabolismo , Animales , Enterocolitis Necrotizante/metabolismo , Enterocolitis Necrotizante/patología , Mucosa Intestinal/crecimiento & desarrollo , Mucosa Intestinal/patología , PorcinosRESUMEN
PURPOSE: To develop a quantifiable approach to identify a dome shape of the repaired diaphragm using post-operative chest radiograph and to determine if a dome-shaped prosthetic patch repair is associated with a decreased rate of CDH recurrence. METHODS: We conducted a retrospective review of all neonates evaluated at our institution from January 2004 to August 2017 with left- and right-sided CDH with at least 6 months of follow-up after CDH repair. Patch use, post-operative imaging and postnatal outcomes were analyzed. Neonates with patch repair were divided into two groups based on the presence of a dome. Using postoperative chest radiographs, the presence of a dome was classified as having a vertical-horizontal diaphragm ratio (VHDR) greater than 20%. Primary outcome was CDH recurrence after repair. RESULTS: We identified 192 neonates who met our inclusion criteria. Cohort survival was 96%, recurrence rate was 15%, 78% had a left-sided CDH; 54% received a patch repair, of which 58% had a type C defect. Of the 104 infants with patch repairs, the CDH recurrence rate was 22% (n = 23) at a median age of 8.5 months (3.8, 20.1). Although neonates with a dome repair had more ECMO use and longer hospital stay, their recurrence rate was about half of those with a non-dome repair (14% vs 28%, p = 0.07). CONCLUSIONS: A dome-shaped repair may reduce recurrence following prosthetic patch repair of congenital diaphragmatic hernia. A larger, multi-institutional study is needed to statistically validate this clinically significant observation. TYPE OF STUDY: Retrospective review. LEVEL OF EVIDENCE: III.
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Hernias Diafragmáticas Congénitas , Estudios de Cohortes , Diafragma/cirugía , Hernias Diafragmáticas Congénitas/complicaciones , Hernias Diafragmáticas Congénitas/cirugía , Humanos , Lactante , Recién Nacido , Recurrencia , Estudios Retrospectivos , Tórax , Resultado del TratamientoRESUMEN
PURPOSE: The purpose of this study was to compare the accuracy of prenatal and postnatal imaging modalities for evaluation and management of congenital lung malformations (CLMs). METHODS: A retrospective review was performed of all fetuses evaluated for a CLM between December 2001 and January 2018. Pre and postnatal imaging findings, operative treatment, and patient outcomes were collected. Patients were included in analysis if they had fetal imaging (US and/or fetal MRI), a postnatal chest CT, and surgical pathology. RESULTS: Over the study period, we identified 157 patients with prenatal imaging that also had a follow-up with postnatal chest CT at a median age of 2.1 [1.4, 3.2] months. Of these, 75% (nâ¯=â¯117) had surgical resection. Diagnostic accuracy (DA) for localization of unilobar lesions was 100% for pre- and postnatal imaging and 97% vs 98% for multilobar disease, respectively. On comparison for identification of aberrant vasculature and pathology prediction, pre- and postnatal imaging DAs were similar. However, postnatal CT had the highest specificity for diagnosing lesions overall (pâ¯<â¯0.05). CONCLUSION: Prenatal imaging provides valuable information for counseling and possible fetal intervention. However, this study suggests that postnatal CT scan continues to provide important information for preoperative counseling and surgical management. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level IV.
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Enfermedades Pulmonares/diagnóstico por imagen , Pulmón/diagnóstico por imagen , Femenino , Humanos , Lactante , Recién Nacido , Pulmón/anomalías , Enfermedades Pulmonares/congénito , Imagen por Resonancia Magnética , Masculino , Embarazo , Diagnóstico Prenatal , Anomalías del Sistema Respiratorio/cirugía , Estudios Retrospectivos , Sensibilidad y Especificidad , Tomografía Computarizada por Rayos X , Ultrasonografía PrenatalRESUMEN
PURPOSE: The purpose of this study was to identify an optimal definition of massive transfusion (MT) in civilian pediatric trauma. METHODS: Severely injured children (age ≤18â¯years, injury severity score ≥25) in the Trauma Quality Improvement Program research datasets 2014-2015 that received blood products were identified. Children with traumatic brain injury and non-survivable injuries were excluded. Early mortality was defined as death within 24 h and delayed mortality as death after 24 h from hospital admission. Receiver operating curves and sensitivity and specificity analysis identified an MT threshold. Continuous variables are presented as median [IQR]. RESULTS: Of the 270 included children, the overall mortality was 27% (Nâ¯=â¯74). There were no differences in demographics or mechanism of injury between children that lived or died. Sensitivity and specificity for early mortality was optimized at a 4-h transfusion volume of 37â¯ml/kg. After controlling for other significant variables, a threshold of 37â¯ml/kg/4 h predicted the need for a hemorrhage control procedure (OR 8.60; 95% CI 4.25-17.42; pâ¯<â¯0.01) and early mortality (OR 4.24; 95% CI 1.96-9.16; pâ¯<â¯0.01). CONCLUSION: An MTP threshold of 37â¯mL/kg/4 h of transfused blood products predicted the need for hemorrhage control procedures and early mortality. This threshold may provide clinicians with a timely prognostic indicator, improve research methodology, and resource utilization. TYPE OF STUDY: Diagnostic Test. LEVEL OF EVIDENCE: III.
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Transfusión Sanguínea , Hemorragia/terapia , Heridas y Lesiones/mortalidad , Heridas y Lesiones/terapia , Adolescente , Niño , Bases de Datos Factuales , Femenino , Hemorragia/mortalidad , Humanos , Puntaje de Gravedad del Traumatismo , Masculino , Curva ROC , Factores de Tiempo , Estados Unidos/epidemiologíaRESUMEN
PURPOSE: The purpose of this study was to describe the epidemiology and evaluate the clinical significance of traumatic sternal fractures. METHODS: Patients age ≤18 years with sternal fractures in the National Trauma Database research datasets from 2007-2014 were identified. Patient demographics, injuries, procedures, and outcomes were analyzed using descriptive statistics and logistic regression. RESULTS: Three thousand one hundred sixty patients with sternal fracture were identified. Ninety percent of injuries occurred in patients between 12 and 18 years old. Median injury severity score (ISS) was 17 [9,29]. Exploratory thoracotomy was performed in 1%. Thirty-nine percent were admitted to the intensive care unit (ICU). On multivariate regression, predictors of ICU stay >1 day were increasing ISS, lack of the use of protective devices, decreasing Glasgow Coma Score (GCS), tachycardia, and pulmonary contusion. Median hospital length of stay was 4 [2, 9] days. In-hospital mortality was 8%. Predictors of mortality were lower GCS, increasing ISS, decreasing oxygen saturation, hypotension, and cardiac arrest. Use of protective devices and seat belts did not affect mortality. CONCLUSION: Sternal fractures in patients increase in incidence with age, and poor outcomes are impacted by associated injuries and complications. The presence of a sternal fracture should trigger a careful diagnostic evaluation. LEVEL OF EVIDENCE: III STUDY TYPE: Treatment Study.
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Fracturas Óseas/epidemiología , Mortalidad Hospitalaria , Unidades de Cuidado Intensivo Pediátrico/estadística & datos numéricos , Tiempo de Internación/estadística & datos numéricos , Esternón/lesiones , Adolescente , Factores de Edad , Niño , Contusiones/epidemiología , Bases de Datos Factuales , Femenino , Fracturas Óseas/cirugía , Escala de Coma de Glasgow , Humanos , Incidencia , Puntaje de Gravedad del Traumatismo , Lesión Pulmonar/epidemiología , Masculino , Cinturones de Seguridad , Taquicardia/epidemiología , Toracotomía/estadística & datos numéricos , Estados Unidos/epidemiologíaRESUMEN
PURPOSE: To examine postsurgical outcomes of a consecutive series of children treated with elective operations for congenital lung malformations (CLM). METHODS: A retrospective review was performed on a prospectively collected dataset of all fetuses evaluated for a CLM between July 2001 and June 2016. Prenatal findings, operative treatment and postnatal outcomes were collected. Children having elective operations were divided in two groups based on age at time of surgery. RESULTS: Of 220 fetuses, 143 had operations and follow-up at our center. Six had open fetal lobectomy, 17 had EXIT-to-resection, 16 infants had urgent resection for symptoms and 110 with asymptomatic lesions had elective resection. Of these 110, the median fetal maximum CVR was 0.8 [range 0.1-2.2], and median age at operation was 4 (1.5-60) months (58% had resection at ≤4â¯months). Overall complication rate, including air-leak and pleural effusion, was 15%. When comparing those having resection at ≤4â¯months to those >4â¯months, there were no significant differences in complication rates or length of stay. Operative time was shorter for patients with early resection (154⯱â¯59 vs 181⯱â¯89, pâ¯=â¯0.05). No infant having resection at ≤4â¯months was readmitted. Overall, children survived with good recovery. CONCLUSION: Early elective resection of congenital lung malformations prior to 4â¯months of age is feasible and not associated with increased operative risk. TYPE OF STUDY: Restropective study. LEVEL OF EVIDENCE: Level III.
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Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Procedimientos Quirúrgicos Electivos/métodos , Terapias Fetales/métodos , Neumonectomía/métodos , Tiempo de Tratamiento/estadística & datos numéricos , Factores de Edad , Procedimientos Quirúrgicos Electivos/efectos adversos , Femenino , Terapias Fetales/efectos adversos , Humanos , Lactante , Neumonectomía/efectos adversos , Complicaciones Posoperatorias/epidemiología , Embarazo , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
PURPOSE: The purpose of this study was to determine the accuracy of fetal echocardiogram (ECHO) for detecting cardiac structural anomalies that may impact Extracorporeal Membrane Oxygenation (ECMO) candidacy in infants with Congenital Diaphragmatic Hernia (CDH). METHODS: A retrospective review was performed on fetuses with CDH (January 2007-June 2017). Inclusion criteria were inborn and at least one prenatal and postnatal ECHO. ECHOs were evaluated for structural heart defects. Primary outcomes were accuracy of prenatal fetal ECHO and identify differences between prenatal and postnatal ECHO. Descriptive statistics and Chi-square analysis were performed. RESULTS: 131 inborn patients were identified. Mean gestational age of fetal ECHO was 26.6⯱â¯5.5â¯weeks. The median time to postnatal ECHO was DOL 1 [0-30]. Fetal ECHO had 92% accuracy, 83% sensitivity, 93% specificity, PPV of 95%, NPV of 92%, and a 90% accuracy for visualization of at least one pulmonary vein into the left atrium on the contralateral (non-CDH) side. Thirty-five percent of patients received ECMO, and 26% had an associated cardiac anomaly. All ECMO patients had an accurate structural fetal ECHO. CONCLUSION: Fetal ECHO is sufficient for identifying major structural heart defects at large volume centers with trained pediatric cardiologists and may be used to guide clinical management, particularly in regards to ECMO candidacy. LEVEL OF EVIDENCE: Level III.
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Ecocardiografía , Oxigenación por Membrana Extracorpórea , Cardiopatías Congénitas/diagnóstico por imagen , Hernias Diafragmáticas Congénitas/terapia , Ultrasonografía Prenatal , Femenino , Edad Gestacional , Cardiopatías Congénitas/complicaciones , Hernias Diafragmáticas Congénitas/complicaciones , Humanos , Recién Nacido , Masculino , Selección de Paciente , Periodo Posparto , Valor Predictivo de las Pruebas , Estudios RetrospectivosRESUMEN
PURPOSE: The purpose of this study was to evaluate treatment and surgical outcomes of patients of Beckwith-Wiedemann Syndrome (BWS) treated at a tertiary children's hospital. METHODS: A retrospective review of infants evaluated at Texas Children's Hospital for BWS from August 2000 to December 2016 was performed. Data collected included demographic information, clinical presentation, genetic evaluation, fetal imaging, operative treatment, and outcomes. RESULTS: Forty-seven children with a diagnosis of BWS were identified. Sixty-four percent (n=30) had a genetic mutation in an imprinting domain of chromosome 11p15. Thirty-two patients (68%) underwent at least one operation related to BWS with a median of 2 [range: 0-8] surgical procedures per patient. Sixteen underwent omphalocele repair, 12 had partial glossectomies-, 7 underwent surgeries related to hemihypertrophy, and 6 had resection of an embryonal tumor (two adrenal cortical adenoma, one Wilms' tumor, two hepatoblastoma). Overall, survival was 100% with feeding difficulty (47%) being the most frequent complication. CONCLUSION: A substantial number of patients with Beckwith-Wiedemann Syndrome will require surgery. However, overall outcomes are similar between those that require surgery and those that do not. LEVEL OF EVIDENCE: Level III.
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Síndrome de Beckwith-Wiedemann/cirugía , Diagnóstico por Imagen/métodos , Complicaciones Posoperatorias/epidemiología , Procedimientos Quirúrgicos Operativos/métodos , Síndrome de Beckwith-Wiedemann/diagnóstico , Síndrome de Beckwith-Wiedemann/mortalidad , Femenino , Edad Gestacional , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Texas/epidemiología , Resultado del TratamientoRESUMEN
PURPOSE: The purpose of this study was to evaluate the characteristics of patients with congenital heart disease (CHD) who developed necrotizing enterocolitis (NEC). METHODS: A retrospective review of neonates with CHD at a tertiary care center between January 2006 and January 2016 was performed. Diagnosis of NEC was based on modified Bell's criteria. Patients were grouped by Risk Adjustment for Congenital Heart Surgery (RACHS-1) or by ductal-dependent (DD) lesions that require a patent ductus arteriosus to supply pulmonary or systemic circulation. RESULTS: Of 1811 neonates with CHD, 3.4% (n=61) developed NEC. Eighteen (30%) of these required surgical management. The rate of NEC among DD patients was 5% (n=33/653), compared to 2.4% (n=28/1158) in the non-DD group (p=0.003). RACHS-1 score>2 had a higher rate of NEC 6.2% (41/658) compared to RACHS-1≤2 cases, 1.7% (20/1153) (p=0.005). DD patients and complex patients with RACHS-1>2 were more likely to develop NEC after cardiac surgery. Hypoplastic left heart syndrome patients had a rate of 9% (n=16/185). Surgical NEC was more prevalent in the non-DD group. Mortality was similar among groups. CONCLUSION: CHD patients with ductal-dependent lesions or complex cases (RACHS-1 score>2) have higher rates of NEC than non-ductal-dependent patients or RACHS-1 score of 2 or less. Mortality is similar regardless of ductal dependence, but surgical NEC was more prevalent in non-DD patients. LEVEL OF EVIDENCE: Level IIb.
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Enterocolitis Necrotizante/etiología , Cardiopatías Congénitas/complicaciones , Adulto , Procedimientos Quirúrgicos Cardíacos , Enterocolitis Necrotizante/diagnóstico , Enterocolitis Necrotizante/mortalidad , Femenino , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/cirugía , Humanos , Recién Nacido , Enfermedades del Recién Nacido , Masculino , Embarazo , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia/tendencias , Estados Unidos/epidemiologíaRESUMEN
BACKGROUND & OBJECTIVES: Congenital Diaphragmatic Hernia (CDH) is associated with significant morbidity and mortality. This study compares the efficacy of the highest oxygenation index in the first 48 h (HiOI) versus current prenatal indices to predict survival and morbidity. METHODS: Medical records of 50 prenatally diagnosed, isolated, left-sided CDH patients treated from January 2011 to April 2016 were reviewed. Data abstracted included HiOI, lung to head ratio (LHR), observed to expected total fetal lung volume (O/E TFLV), percent liver herniation (%LH), 6 month survival, respiratory support at discharge, ventilator days and length of stay. Data were analyzed using parametric and nonparametric tests and regression analyses as appropriate. RESULTS: HiOI was associated with significantly increased LOS (p<0.001), respiratory support at discharge (p<0.001), greater ventilator days (p=0.001) and higher odds of death (p=0.004) with risk of death increasing by 5% for every one-unit increase in OI. HiOI was statistically a better predictor of LOS than O/E TFLV (p=0.007) and %LH (p=0.02). CONCLUSIONS: In isolated, left-sided CDH patients, HiOI is associated with higher mortality, greater length of stay, more ventilator days and increased respiratory support at discharge. HiOI is a better predictor of length of stay than O/E TFLV and %LH. TYPE OF STUDY: Retrospective Study LEVEL OF EVIDENCE: II.
Asunto(s)
Hernias Diafragmáticas Congénitas/diagnóstico , Oxígeno/metabolismo , Biomarcadores/metabolismo , Femenino , Hernias Diafragmáticas Congénitas/metabolismo , Hernias Diafragmáticas Congénitas/mortalidad , Hernias Diafragmáticas Congénitas/terapia , Humanos , Lactante , Recién Nacido , Masculino , Valor Predictivo de las Pruebas , Pronóstico , Análisis de Regresión , Estudios RetrospectivosRESUMEN
INTRODUCTION: This study tests the hypothesis that renal tissue oxygen saturation as measured by Near Infrared Spectroscopy (NIRS) would correlate with urine output in neonates with congenital diaphragmatic hernia (CDH) on extracorporeal membrane oxygenation (ECMO). METHODS: Between 2012 and 2015, neonates with CDH were enrolled as part of a comprehensive study that provided renal/cerebral/abdominal NIRS monitoring for the duration of ECMO support. Continuous NIRS measurements, mean arterial pressure, and urine output were recorded. Periods of anuria (NU), adequate urine output >1ml/kg/h (AU), and low urine output <1ml/kg/h (LU) were noted and analyzed. RESULTS: Over 1500h of continuous renal NIRS were obtained from six neonates. NIRS values were significantly different during periods of AU, LU, and anuria (84±6%, 76±3%, and 67±6%, p<0.01). ROC curves identified NIRS >76% as highly predictive of adequate urine output (AUC=0.96). MAP was significantly lower only in anuric patients, 36.42±10.26, compared to patients with AU and LU - 42.99±5.25 and 42.85±7.4, respectively (p<0.001). CONCLUSION: Renal NIRS measurements correlate with urine production. Lower values are noted as urine output declines and precedes a decline in MAP. Renal NIRS may have promise as a non-invasive means of determining adequacy of renal perfusion and urine output in neonates with complex fluid shifts. LEVEL OF EVIDENCE: IIb.
Asunto(s)
Anuria/etiología , Oxigenación por Membrana Extracorpórea , Hernias Diafragmáticas Congénitas/terapia , Riñón/diagnóstico por imagen , Oxígeno/metabolismo , Espectroscopía Infrarroja Corta , Anuria/diagnóstico , Anuria/metabolismo , Biomarcadores/metabolismo , Femenino , Hernias Diafragmáticas Congénitas/complicaciones , Hernias Diafragmáticas Congénitas/metabolismo , Hernias Diafragmáticas Congénitas/orina , Humanos , Recién Nacido , Riñón/metabolismo , Masculino , Curva ROC , Estudios Retrospectivos , Método Simple Ciego , Resultado del TratamientoRESUMEN
INTRODUCTION: The purpose of this study was to evaluate the accuracy of prenatal diagnosis for fetuses with gastrointestinal (GI) obstruction with correlation to postnatal outcomes. METHODS: Fetuses diagnosed with GI obstruction (excluding esophageal and duodenal) were reviewed for those evaluated between 2006 and 2016. Prenatal diagnosis and imaging studies were compared to postnatal findings. Outcomes evaluated included diagnostic accuracy, rate of other anomalies, neonatal length of stay, incidence of short bowel syndrome, and discharge with TPN or gastrostomy. RESULTS: Forty-eight patients were diagnosed prenatally with obstruction. Six patients were excluded owing to incomplete records and follow-up. Twelve fetuses were diagnosed with ultrasound alone, and thirty-four with ultrasound and MRI. A diagnosis of obstruction was accurate in 88.1% (n=37/42) with a positive predictive value of 91.3%, while US with MRI had an accuracy of 84.4%. Associated anomalies were highest among fetuses with anorectal obstruction (90.1%), compared to large (50%) or small bowel obstruction (28%). Survival rate was lowest for anorectal obstruction (54.5%), compared to large or small bowel obstruction (100% for both). CONCLUSION: Fetal MRI is an accurate modality in the diagnosis of fetal GI obstruction and can complement findings characterized by ultrasound. Fetuses with anorectal obstruction have a higher rate of associated anomalies and the lowest survival. LEVEL OF EVIDENCE: IIb.
Asunto(s)
Obstrucción Intestinal/diagnóstico por imagen , Diagnóstico Prenatal , Femenino , Estudios de Seguimiento , Gastrostomía , Humanos , Recién Nacido , Obstrucción Intestinal/congénito , Obstrucción Intestinal/mortalidad , Obstrucción Intestinal/terapia , Imagen por Resonancia Magnética , Masculino , Nutrición Parenteral Total , Embarazo , Diagnóstico Prenatal/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Ultrasonografía Prenatal/métodosRESUMEN
BACKGROUND/PURPOSE: The purpose of this study was to develop a computational algorithm that would predict the need for ECMO in neonates with congenital diaphragmatic hernia (CDH). METHODS: CDH patients from August 2010 to 2016 were enrolled in a study to continuously measure cerebral tissue oxygen saturation (cStO2) of left and right cerebral hemispheres. NIRS devices utilized were FORE-SIGHT, CASMED and INVOS 5100, Somanetics. Using MATLAB©, a data randomization function was used to deidentify and blindly group patient's data files as follows: 12 for the computational model development phase (6 ECMO and 6 non-ECMO) and the remaining patients for the validation phase. RESULTS: Of the 56 CDH patients enrolled, 22 (39%) required ECMO. During development of the algorithm, a difference between right and left hemispheric cerebral oxygenation via NIRS (ΔHCO) was noted in CDH patients that required ECMO. Using ROC analysis, a ΔHCO cutoff >10% was predictive of needing ECMO (AUC: 0.92; sensitivity: 85%; and specificity: 100%). The algorithm predicted need for ECMO within the first 12h of life and at least 6h prior to the clinical decision for ECMO with 88% sensitivity and 100% specificity. CONCLUSION: This computational algorithm of cerebral NIRS predicts the need for ECMO in neonates with CDH. LEVEL OF EVIDENCE: II.