RESUMO
Microglia are CNS-resident macrophages that scavenge debris and regulate immune responses. Proliferation and development of macrophages, including microglia, requires Colony Stimulating Factor 1 Receptor (CSF1R), a gene previously associated with a dominant adult-onset neurological condition (adult-onset leukoencephalopathy with axonal spheroids and pigmented glia). Here, we report two unrelated individuals with homozygous CSF1R mutations whose presentation was distinct from ALSP. Post-mortem examination of an individual with a homozygous splice mutation (c.1754-1G>C) demonstrated several structural brain anomalies, including agenesis of corpus callosum. Immunostaining demonstrated almost complete absence of microglia within this brain, suggesting that it developed in the absence of microglia. The second individual had a homozygous missense mutation (c.1929C>A [p.His643Gln]) and presented with developmental delay and epilepsy in childhood. We analyzed a zebrafish model (csf1rDM) lacking Csf1r function and found that their brains also lacked microglia and had reduced levels of CUX1, a neuronal transcription factor. CUX1+ neurons were also reduced in sections of homozygous CSF1R mutant human brain, identifying an evolutionarily conserved role for CSF1R signaling in production or maintenance of CUX1+ neurons. Since a large fraction of CUX1+ neurons project callosal axons, we speculate that microglia deficiency may contribute to agenesis of the corpus callosum via reduction in CUX1+ neurons. Our results suggest that CSF1R is required for human brain development and establish the csf1rDM fish as a model for microgliopathies. In addition, our results exemplify an under-recognized form of phenotypic expansion, in which genes associated with well-recognized, dominant conditions produce different phenotypes when biallelically mutated.
Assuntos
Anormalidades Congênitas/etiologia , Leucoencefalopatias/genética , Leucoencefalopatias/patologia , Microglia/patologia , Mutação , Receptores de Fator Estimulador das Colônias de Granulócitos e Macrófagos/genética , Adulto , Animais , Criança , Anormalidades Congênitas/patologia , Proteínas de Homeodomínio/genética , Proteínas de Homeodomínio/metabolismo , Homozigoto , Humanos , Lactente , Recém-Nascido , Microglia/metabolismo , Linhagem , Fenótipo , Proteínas Tirosina Quinases/genética , Proteínas Tirosina Quinases/metabolismo , Receptores Proteína Tirosina Quinases , Proteínas Repressoras/genética , Proteínas Repressoras/metabolismo , Fatores de Transcrição/genética , Fatores de Transcrição/metabolismo , Adulto Jovem , Peixe-Zebra , Proteínas de Peixe-Zebra/genética , Proteínas de Peixe-Zebra/metabolismoRESUMO
OBJECTIVE. This article reviews the ultrasound characteristics of pediatric slow-flow vascular malformations and underscores findings that significantly impact diagnosis and treatment. Key imaging features are discussed including lesion size, malformation location, morphology, and mimics. CONCLUSION. Ultrasound findings affect the management of slow-flow vascular malformations and should be emphasized in lesion diagnosis. Superficial, focal lesions with well-defined margins are ideal for ultrasound evaluation.
Assuntos
Ultrassonografia , Malformações Vasculares/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo , Criança , Humanos , Malformações Vasculares/fisiopatologia , Malformações Vasculares/terapiaRESUMO
BACKGROUND: The safety and efficacy of US-guided lumbar puncture in children has been described. In the pediatric setting, children are frequently referred to interventional radiology only after a failed landmark-based attempt. Routine pre-procedure US in these children is useful to determine a safe level for subarachnoid access and to optimize success. OBJECTIVE: To determine whether pre-procedure US improves technical success and safety of US-guided lumbar puncture. MATERIALS AND METHODS: We included 47 children. Inclusion criteria were urgent US-guided lumbar puncture in pediatric patients <18 years old. Exclusion criteria were non-urgent lumbar punctures, children referred without an antecedent landmark-based attempt, lumbar punctures performed with fluoroscopic guidance, and procedures performed prior to introducing the diagnostic approach in 2017. We did not evaluate data pertaining to successful landmark-based lumbar punctures performed without subsequent need for additional attempts. We recorded technical successes, adverse events and relevant abnormalities identified on pre-procedural US. RESULTS: Thirty-six US-guided lumbar punctures were performed with 100% technical success. Eleven children referred to interventional radiology did not undergo lumbar puncture because of unfavorable US findings or interval clinical improvement obviating the need for lumbar puncture. Thirty-six children underwent US evaluation of the thecal sac prior to potential intervention. Of these 36 with pre-procedural US studies, 12 demonstrated paucity of cerebrospinal fluid and 14 demonstrated an epidural hematoma. Fifteen children who underwent lumbar puncture had a "traumatic tap," classified as a mild adverse event. No moderate or severe adverse events were recorded. CONCLUSION: Limited spinal US following failed landmark-based lumbar punctures frequently identifies procedure-related complications and can augment patient selection for future image-guided lumbar punctures.
Assuntos
Hematoma Epidural Craniano , Punção Espinal , Criança , Fluoroscopia , Humanos , Coluna Vertebral , Ultrassonografia , Ultrassonografia de IntervençãoRESUMO
BACKGROUND: Ultrasound (US)-guided tunneled femoral peripherally inserted central catheters (PICCs) are a safe central venous access option in infants and neonates. Studies have shown, however, that femoral central venous access has the potential for high central line-associated bloodstream infection (CLABSI) rates with a significant increase in risk around line day 30, though no studies have evaluated these risks exclusively for tunneled femoral PICCs. OBJECTIVE: The primary purpose of this study was to evaluate the relationship between line duration and the risk of CLABSI in tunneled femoral PICCs in children. MATERIALS AND METHODS: Four hundred forty-five patients (196 females, 249 males; median age: 49.4 days; median weight: 3.7 kg) who underwent 573 tunneled femoral PICC placements or exchanges from Jan. 1, 2017, to Jan. 31, 2020, were included in the study. All tunneled femoral PICCs were placed using US technique and catheter specifications, including catheter size (French) and length (cm), were retrieved from the electronic medical record. The location of the PICC placement, the number of lumens, the laterality of placement, and the patient's age and weight were also recorded. Only non-mucosal barrier injury CLABSIs, according to the Centers for Disease Control and Prevention (CDC) definitions, were counted as CLABSI for this study. The number of central line days until a CLABSI event was analyzed with an accelerated failure time model using the exponential, Weibull, and log-normal distributions to determine the probability of a CLABSI over time, taking into consideration the recorded covariates. RESULTS: Tunneled femoral PICC placements accounted for 14,855 line days, during which 20 non-mucosal barrier injury CLABSIs (CLABSI rate of 1.35 per 1,000 line days) occurred during the study period. The highest CLABSI rate occurred in PICCs placed in the neonatal intensive care unit (NICU) at 2.01 per 1,000 line days and the lowest occurred in PICCs placed in interventional radiology at 0.26 per 1,000 line days. Overall, PICCs placed outside of interventional radiology had a CLABSI rate of 1.72 per 1,000 line days. The CLABSI rate during the first 30 days a line was in situ was lower than the rate after 30 days (0.51 per 1,000 line days vs. 3.06 per 1,000 line days, respectively). Statistical modeling and hazard estimation using the Akaike information criterion corrected for small sample size (AICc)-average of log-normal, Weibull and exponential distributions demonstrate the daily risk of CLABSI rapidly increases from day 1 to day 30, with the risk remaining high for the duration of line days. CONCLUSION: While tunneled femoral PICCs are a relatively safe and effective central venous access alternative, the rate of CLABSI appears to rapidly increase with increasing line days until around day 30 and then remains high thereafter.
Assuntos
Infecções Relacionadas a Cateter , Cateterismo Venoso Central , Cateterismo Periférico , Cateteres Venosos Centrais , Sepse , Infecções Relacionadas a Cateter/diagnóstico por imagem , Infecções Relacionadas a Cateter/epidemiologia , Cateterismo Venoso Central/efeitos adversos , Cateterismo Periférico/efeitos adversos , Cateteres Venosos Centrais/efeitos adversos , Criança , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de RiscoRESUMO
BACKGROUND: Ultrasonography may reliably visualize both appropriately positioned and malpositioned femoral-approach catheter tips. Radiography may be used to confirm catheter tip position after placement, but its utility following intraprocedural ultrasound (US) catheter tip verification is unclear. OBJECTIVES: To report the utility of confirmatory radiographs after US-guided tunneled femoral central venous catheter (CVC) placements by interventional radiology in pediatric patients. MATERIALS AND METHODS: A total of 484 pediatric patients underwent bedside US-guided tunneled femoral CVC placements in an intensive care setting at a single tertiary children's hospital between Jan. 1, 2016, and April 20, 2020. Technical success, adverse events, post-procedure radiographic practices and inter-modality catheter tip concordance were recorded. All radiographs were performed within 12 h of catheter placement. RESULTS: The mean patient age was 175±508 days (range: 1 day to 19 years), including 257 (53.1%) males and 227 (46.9%) females. Of the 484 attempted placements, 472 (97.5%) were primary placements. Four hundred eighty-one (99.4%) placements were technically successful. There were three (0.6%) technical failures due to previously undiagnosed iliofemoral venous occlusive disease. Five (1.0%) adverse events occurred. Radiographs were obtained within 12 h of CVC placement in 171 (35.3%) patients, in 120 (70.2%) of whom the indication was recent catheter placement. All 171 (100%) post-placement radiographs showed catheter tip location concordance with the intra-procedural US. In one (0.2%) patient, in whom there was nonvisualization of a guidewire and clinical concern for malposition during US-guided placement, post-procedure radiographs, coupled with multiplanar venography, demonstrated inadvertent paravertebral venous plexus catheter placement. CONCLUSION: The concordance between intra-procedural US and confirmatory post-procedure radiographs of CVC placements by interventional radiology obviates the need for routine radiographs. Radiographs may be obtained in instances of proceduralist uncertainty or clinical concern.
Assuntos
Cateterismo Venoso Central , Cateteres Venosos Centrais , Cateterismo Venoso Central/efeitos adversos , Criança , Feminino , Humanos , Lactente , Masculino , Radiografia , Radiologia Intervencionista , Ultrassonografia , Ultrassonografia de IntervençãoRESUMO
We report a patient without known preexisting liver disease who presented with hepatopulmonary syndrome (HPS) due to aberrant intrahepatic portal venous development leading to portosystemic shunting. Liver transplantation resulted in resolution of portal hypertension and HPS and sildenafil was safely tolerated in the treatment of persistent fatigue and hypoxemia. Twelve months later, patient has normal allograft function and has returned to normal activity.
Assuntos
Síndrome Hepatopulmonar/diagnóstico , Hipóxia/tratamento farmacológico , Transplante de Fígado , Complicações Pós-Operatórias/tratamento farmacológico , Citrato de Sildenafila/uso terapêutico , Malformações Vasculares/diagnóstico , Vasodilatadores/uso terapêutico , Criança , Fadiga/tratamento farmacológico , Fadiga/etiologia , Síndrome Hepatopulmonar/etiologia , Síndrome Hepatopulmonar/fisiopatologia , Síndrome Hepatopulmonar/cirurgia , Humanos , Hipóxia/etiologia , Masculino , Veia Porta/anormalidades , Cuidados Pós-Operatórios/métodos , Malformações Vasculares/fisiopatologia , Malformações Vasculares/cirurgiaRESUMO
BACKGROUND: Accurate and reproducible means of measuring the portosystemic gradient are essential for risk stratification and treatment of portal hypertension. OBJECTIVE: To report the reliability of hepatic venous pressure gradients in children with intrahepatic veno-venous collateralization. MATERIALS AND METHODS: Between January 2012 and December 2019 (96 months), 39 patients with native livers underwent wedge hepatic venography and hepatic venous pressure gradient measurements at a tertiary pediatric center. All archived images were reviewed for balloon isolation of the hepatic vein and hepatic vein-to-hepatic vein (HV-HV) collaterals. HV-HV collaterals were categorized as present on the basis of non-catheterized segmental venous opacification despite appropriate balloon isolation. Hepatic venous pressure gradient was defined as the difference of wedge and free hepatic venous pressures. Wedge portosystemic gradient was defined as the difference between wedge hepatic venous pressure and right atrial (RA) pressures. For patients subsequently undergoing portal venous catheterization, portosystemic gradient was defined as the difference between main portal vein and RA pressures. RESULTS: Thirteen of 39 (33.3%) patients demonstrated HV-HV collaterals on wedge hepatic venography. The mean hepatic venous pressure gradient was 5.2±3.8 mmHg (range: 0-15 mmHg). The mean hepatic venous pressure gradient was 3.6±2.6 mmHg (range: 0-9 mmHg) in the presence of HV-HV collaterals and 5.9±4.2 mmHg (range: 1-15 mmHg) in the absence of HV-HV collaterals (P=0.043). Twelve (30.8%) patients were found to have varices: 10 gastroesophageal, 1 rectal and 1 stomal. The mean hepatic venous pressure gradient in patients with varices was 5.4±47 mmHg (range: 0-15 mmHg). For patients with varices, mean hepatic venous pressure gradient was 3.0±2.7 mmHg (range: 0-9 mmHg) in the presence of HV-HV collaterals and 10.3±4.1 mmHg (range: 5-15 mmHg) in the absence of HV-HV collaterals (P=0.004). Four (10.3%) patients had extrahepatic portal vein occlusion: 3 with cavernous transformation and 1 with type Ib Abernethy malformation. All patients with extrahepatic portal vein occlusion demonstrated HV-HV collaterals compared with 8 of 35 (22.9%) patients without extrahepatic portal vein occlusion (P=0.002). Four of 39 (10.3%) patients underwent direct portal pressure measurements: 3 via transhepatic and 1 via trans-splenic portal access. All had demonstrated HV-HV collaterals on wedged imaging. One had extrahepatic portal vein occlusion. The mean time between wedge portosystemic gradient and portosystemic gradient measurement was 3.75 days (range: 0-8 days). The mean wedge portosystemic gradient was 4.5±3.1 mmHg (range: 2-9 mmHg) and the mean portosystemic gradient was 14.5±3.7 mmHg (range: 12-20 mmHg) (P=0.006). CONCLUSION: HV-HV collateralization is frequently observed in children undergoing wedged portal venography and leads to misrepresentative hepatic venous pressure gradients. All patients undergoing hepatic venous pressure gradient measurement should have wedged venography to identify HV-HV collaterals and to qualify measured pressures. Additional techniques to obtain representative pressures in the presence of HV-HV collaterals warrant further investigation.
Assuntos
Hipertensão Portal/diagnóstico por imagem , Biópsia Guiada por Imagem , Flebografia/métodos , Pressão na Veia Porta , Sistema Porta/diagnóstico por imagem , Adolescente , Cateterismo , Criança , Pré-Escolar , Circulação Colateral , Feminino , Humanos , Hipertensão Portal/fisiopatologia , Hipertensão Portal/terapia , Lactente , Masculino , Sistema Porta/fisiopatologia , Derivação Portossistêmica Transjugular Intra-Hepática , Radiografia Intervencionista , Reprodutibilidade dos TestesRESUMO
Protocol biopsies are defined as sampling of allograft tissue at predetermined times regardless of function. This procedure can be justified due to the lack of non-invasive methods to reliably diagnose rejection (acute or subclinical). Changes in creatinine are not seen with subclinical rejection or early acute rejection and do not always correlate with efficacy of treatment. Parents and providers are still hesitant to pursue protocol biopsy due to the potential complications and lack of definitive evidence of a benefit from doing this procedure. Importantly, the rate of transplant renal biopsy complications requiring additional intervention is low. It is unclear if detection and treatment of subclinical rejection detected on protocol biopsy will lead to improved graft survival. Our goal is to review the literature on this topic and share some of the experience in our center. Definition, indications, and complications of diagnostic transplant renal biopsies are not included in this review.
Assuntos
Biópsia/efeitos adversos , Rejeição de Enxerto/diagnóstico , Transplante de Rim/efeitos adversos , Rim/patologia , Fatores Etários , Biópsia/economia , Análise Custo-Benefício , Rejeição de Enxerto/economia , Rejeição de Enxerto/imunologia , Rejeição de Enxerto/patologia , Sobrevivência de Enxerto , Custos de Cuidados de Saúde , Humanos , Rim/imunologia , Transplante de Rim/economia , Valor Preditivo dos Testes , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Cone-beam CT is increasingly used in pediatric interventional radiology procedures. However, the feasibility or safety of using this mode of imaging guidance for percutaneous lung nodule biopsy in children has not been assessed. OBJECTIVE: To retrospectively evaluate safety and diagnostic accuracy of percutaneous lung nodule biopsy in people treated at a pediatric hospital using cone-beam CT with navigational overlay. MATERIALS AND METHODS: Thirty-six consecutive patients from two large tertiary-care children's hospitals with lung nodules of 48 mm or smaller underwent percutaneous lung nodule biopsy using cone-beam CT with navigational overlay. We evaluated patient demographics, pre- and post-biopsy diagnoses, number of biopsy passes, complications, radiation exposure and technical success. RESULTS: Percutaneous lung nodule biopsy was performed for 37 nodules in 36 patients (23 males, 13 females, median age 15.5 years, range 8 months to 23 years). One patient underwent biopsy of two nodules at a single procedure. Median patient weight was 55 kg (range 8-97 kg). Pre-biopsy diagnoses included metastatic sarcoma or other solid tumor (n=11), leukemia/lymphoma (n=13), infection (n=10), chronic granulomatous disease (n=2) and post-transplant lymphoproliferative disorder (PTLD; n=1). Mean number of passes was 5 (range 2-15). Mean pre-procedure international normalized ratio (INR) was 1.1 and platelet count 193 × 109/L. Diagnostic specimens were obtained in 32 of 36 patients (89%). Thirteen complications were encountered in 12 patients (33% of cohort), including 9 pneumothoraces (4 requiring chest tubes); 1 hemothorax, which required a chest tube; and 3 self-limited pulmonary hemorrhages. Mean and median radiation doses were as follows: fluoroscopy time 3 min and 2.4 min, dose-area product (DAP) (recorded in 31 patients) 23,402 Gy·cm2 and 12,453 Gy·cm2, and air kerma 88 mGy and 58 mGy. CONCLUSION: Percutaneous lung nodule needle biopsy can be performed accurately using cone-beam CT with navigational overlay.
Assuntos
Tomografia Computadorizada de Feixe Cônico , Biópsia Guiada por Imagem , Nódulo Pulmonar Solitário/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Image-guided percutaneous microwave ablation has been used to treat adult osteoid osteomas but has not been thoroughly evaluated in the pediatric population. OBJECTIVE: To evaluate the technical feasibility and clinical efficacy of microwave ablation to treat osteoid osteomas in pediatric patients. MATERIALS AND METHODS: The electronic medical record and imaging archive were reviewed for 24 consecutive patients who had undergone microwave ablation of osteoid osteomas between January 1, 2015, and May 31, 2018, at a single tertiary care pediatric hospital. All patients were diagnosed by clinical and imaging criteria, and referred by a pediatric orthopedic surgeon after failing conservative management with pain medication. The average age of the patients was 13.3 years (range: 3-18 years), and the average size of the osteoid osteoma nidus was 8.8 mm (range: 5-22 mm). Technical success was defined as placement of the microwave antenna at the distal margin of the lesion nidus and achievement of the target ablation temperature. Clinical findings were assessed pre- and post-ablation and clinical success was defined as complete relief of pain without pain medication at 1-month follow-up. The number and severity of complications were also documented. RESULTS: Clinical success was achieved in 100% of patients (24/24), with all reporting complete cessation of pain medication use 1 week after treatment and 0/10 pain at 1 month. There were 4 minor complications (17%) including access site numbness and a minor soft-tissue infection. There were no major complications. CONCLUSION: Microwave ablation is a technically feasible and clinically effective treatment for pediatric osteoid osteomas.
Assuntos
Neoplasias Ósseas/cirurgia , Ablação por Cateter/métodos , Micro-Ondas/uso terapêutico , Osteoma Osteoide/cirurgia , Radiografia Intervencionista/métodos , Adolescente , Criança , Pré-Escolar , Tomografia Computadorizada de Feixe Cônico , Feminino , Humanos , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: The use of arterial closure devices in achieving femoral hemostasis has been well documented in adults but insufficiently studied in the pediatric population. An earlier study from our institution of 40 Angio-Seal devices in 38 patients concluded that the arterial closure device is safe in children with only a single minor complication. Ongoing experience with this device at our institution, however, suggests a higher rate of complication. OBJECTIVE: To retrospectively evaluate the safety and efficacy of the Angio-Seal in a pediatric population. MATERIALS AND METHODS: A retrospective analysis reviewed all cases in which the Angio-Seal was deployed from June 2011 to September 2017. Peri-procedural documentation was reviewed for pre-procedure labs, clinical effectiveness in achieving hemostasis and complications related to the use of this device. Logistic regression analysis was also used to evaluate the relationship between patient demographic, vessel size and indication for angiography, and the presence or absence of complications. RESULTS: During the study period, 48 additional Angio-Seal devices were deployed in 41 consecutive patients. Five patients were excluded for being older than 18 years. The mean age of the patients was 13.3 years (range: 4-18 years) with 18 patients female. The mean common femoral artery diameter was 5.98 mm in short axis diameter (range: 4-9 mm). Complications were present in 6/43 cases (14%) including 3 minor and 3 major complications that included additional procedures. No significant relationship was identified between vessel size, age and the indication for angiography, and the rate of complication on logistic regression analysis. CONCLUSION: While percutaneous arterial closure devices can be efficacious for achieving hemostasis, our experience demonstrates a higher rate of complications in children, contrary to a previous report. The deployment of such devices should be performed with prejudice in this population.
Assuntos
Artéria Femoral/cirurgia , Hemostasia Cirúrgica/instrumentação , Adolescente , Angiografia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Punções , Estudos RetrospectivosRESUMO
BACKGROUND: Published data describing the endovascular treatment of dysfunctional mesoportal and portosystemic shunts in the pediatric population are limited. OBJECTIVE: We sought to describe the treatment and follow-up of such shunts managed by interventional radiology at a single pediatric hospital. We hypothesized that stenotic and occluded pediatric portosystemic and mesoportal shunts can be maintained patent by interventional radiology in the moderate term. MATERIALS AND METHODS: We conducted a single-center retrospective study at a tertiary pediatric hospital. We included children with surgical mesoportal (meso-Rex) or portosystemic (mesocaval, splenorenal or splenocaval) shunts treated with attempted angioplasty or stenting from 2010 to 2018. Technical success was defined as catheterization and intervention upon the shunt with venographic evidence of flow improvement. The primary outcome variables were shunt patency at 1 month, 6 months, 12 months and 24 months post-procedure and freedom from reintervention. RESULTS: Twenty pediatric patients (11 boys, 9 girls; mean age 8.25 years, range 1.3-17 years) met inclusion criteria. Fifty-two interventions (primary and reintervention) on 13 splenorenal, 3 meso-Rex, 2 mesocaval and 2 splenocaval shunts were performed because of evidence of shunt failure, including gastrointestinal bleeding, hypersplenism, or radiographic evidence of a flow defect. The 11 stenotic shunts were treated with 100% technical success, while the remaining 9 occluded shunts were treated with 66.7% technical success. The mean number of reinterventions was 1.9 (standard deviation [SD] = 3.1) per child, which did not differ between stenotic and occluded shunts (P=0.24). Primary patency at 1-month, 6-month, 12-month and 24-months follow-up visits was 17/17 (100%), 10/16 (62.5%), 7/15 (46.7%) and 4/10 (40%), respectively. However, 100% of shunts were either primary patent or primary-assisted patent by endovascular reintervention. There were no cases of shunt occlusion following initial technical success. Finally, the median freedom from reintervention duration was 387 days (SD=821 days). CONCLUSION: Dysfunctional portosystemic surgical shunts are effectively managed by endovascular methods. While many shunts require reintervention, combined primary patency and assisted primary patency rates are excellent.
Assuntos
Procedimentos Endovasculares/métodos , Oclusão de Enxerto Vascular/diagnóstico por imagem , Oclusão de Enxerto Vascular/terapia , Derivação Portossistêmica Cirúrgica , Radiologia Intervencionista/métodos , Adolescente , Angioplastia , Cateterismo , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estudos Retrospectivos , Stents , Resultado do TratamentoRESUMO
PURPOSE: To describe safety and efficacy of catheter-directed thrombolysis (CDT) for portal vein thrombosis (PVT) in children. MATERIALS AND METHODS: Retrospective review was performed of 10 consecutive patients (7 girls, 3 boys; mean age 11.9 y; range, 3-17 y) with PVT undergoing CDT at a single tertiary children's hospital between August 2005 and March 2016. PVT was categorized by etiology and extent (intrahepatic, extrahepatic, or both). CDT was performed with infusion catheters placed via percutaneous transhepatic (PTH) and/or transjugular intrahepatic (TJ) approaches, with or without the use of adjunctive maneuvers, including balloon maceration and suction and rheolytic thrombectomy. Degree of thrombolysis on portal venography, presence of portal vein thrombus on available follow-up imaging, and complication rates were recorded. RESULTS: In 10 patients, 13 CDT procedures were performed for PVT, with 3 patients requiring repeat CDT. Portal access was achieved with PTH (n = 6), TJ (n = 2), or combined (n = 2) approaches. All cases were successful in re-establishing patency and hepatopetal flow on portal venography with complete thrombolysis achieved in 10 of 13 cases (77%). Two major complications (20%) occurred, both with PTH access. Mean follow-up time for 9 patients was 2.6 years (range, 51-1,902 d) with long-term patency achieved in 6 (67%). CONCLUSIONS: CDT can be safe and effective for PVT in children. Portal access considerations and early initiation of thrombolysis may further increase safety and efficacy.
Assuntos
Cateterismo Periférico/métodos , Fibrinolíticos/administração & dosagem , Veia Porta/efeitos dos fármacos , Terapia Trombolítica/métodos , Ativador de Plasminogênio Tecidual/administração & dosagem , Trombose Venosa/tratamento farmacológico , Administração Intravenosa , Adolescente , Cateterismo Periférico/efeitos adversos , Criança , Pré-Escolar , Feminino , Fibrinolíticos/efeitos adversos , Humanos , Circulação Hepática/efeitos dos fármacos , Masculino , Flebografia , Veia Porta/diagnóstico por imagem , Veia Porta/fisiopatologia , Estudos Retrospectivos , Fatores de Risco , Terapia Trombolítica/efeitos adversos , Fatores de Tempo , Ativador de Plasminogênio Tecidual/efeitos adversos , Resultado do Tratamento , Grau de Desobstrução Vascular/efeitos dos fármacos , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/fisiopatologiaRESUMO
Massive hemoptysis occurs in a minority of patients with cystic fibrosis, with an annual incidence of 1%. Although rare, massive hemoptysis can be a severe and potentially fatal complication of this disease. Beyond the acute life-threatening event, hemoptysis in patients with cystic fibrosis has been associated with faster decline in lung function, accelerated need for lung transplant, and increased mortality. The bronchial arteries are the culprit vessels in over 90% of cases of hemoptysis. This normally quiescent vascular system undergoes remarkable hypertrophy, collateralization, and angiogenesis before the onset of hemoptysis, introducing numerous pitfalls for the interventionalist. However, in experienced hands, bronchial artery embolization is a safe and potentially lifesaving therapy. Preprocedural noninvasive imaging, specifically computed tomographic angiography, has been repeatedly validated for helping to localize the likely site of bleeding, characterizing pertinent arterial anatomy, and promoting efficient and effective intervention; it has been recommended for all stable patients with hemoptysis. Success in the angiographic suite requires a thorough understanding of normal and variant bronchial arterial anatomy, appropriate patient selection, and a meticulous embolization technique. A meticulous approach to imaging and intervention, conscientious of both visualized and nonvisualized collateral pathways and nontarget vessels, can minimize potentially devastating complications. This review summarizes the current literature, modern procedural techniques, and emerging controversies, serving to guide an evolving approach to management of patients with cystic fibrosis and hemoptysis. ©RSNA, 2018.
Assuntos
Angiografia/métodos , Artérias Brônquicas , Fibrose Cística/complicações , Embolização Terapêutica/métodos , Hemoptise/etiologia , Hemoptise/terapia , Radiografia Intervencionista/métodos , Artérias Brônquicas/anatomia & histologia , Diagnóstico Diferencial , Hemoptise/diagnóstico por imagem , HumanosRESUMO
BACKGROUND: Despite the demonstrated feasibility of the single-stick technique in the femoral vein, its use in neonates and infants for placing central lines in internal and external jugular veins has not been reported. OBJECTIVE: Describe and assess the safety and efficacy of tunneled jugular central venous catheter placement performed under ultrasound (US) and fluoroscopic guidance in neonates and infants weighing <5 kg using the single-stick technique at three tertiary pediatric hospitals. MATERIALS AND METHODS: Thirty-three children weighing less than 5 kg received tunneled central venous access in either internal or external jugular veins using the single-stick technique. Patient history, procedural records and clinical follow-up documents were retrospectively reviewed. Complication rates were compared to those of 41 patients receiving single-stick femoral central lines. RESULTS: Technical complications occurred during one (3.0%) jugular placement with the patient having a failed right-side attempt with subsequent successful left-side placement. The catheters did not last the entire course of treatment in three (9.1%) patients with jugular lines. One patient had the catheter removed due to concern for infection, one catheter was accidentally removed during dressing changes, and one catheter was displaced and subsequently exchanged. Of patients receiving femoral central lines, 1 (2.4%) had a technical complication and 5 catheters (12.2%) did not last the entire course of treatment. CONCLUSION: The placement of tunneled central venous catheters in neonates/infants <5 kg is safe and technically feasible using the internal/external jugular vein via the single-stick technique. By theoretically reducing the risks of catheter infection by avoiding the diaper area and thrombosis by using larger veins, it may be preferable in certain patient populations.
Assuntos
Peso Corporal , Cateterismo Venoso Central/métodos , Veias Jugulares , Ultrassonografia de Intervenção , Remoção de Dispositivo , Estudos de Viabilidade , Feminino , Fluoroscopia , Humanos , Lactente , Recém-Nascido , Masculino , Resultado do TratamentoAssuntos
Nefrostomia Percutânea/normas , Pediatria/normas , Radiografia Intervencionista/normas , Radiologia Intervencionista/normas , Cateterismo Urinário/normas , Doenças Urológicas/terapia , Criança , Pré-Escolar , Consenso , Humanos , Lactente , Recém-Nascido , Melhoria de Qualidade/normas , Indicadores de Qualidade em Assistência à Saúde/normas , Doenças Urológicas/diagnóstico por imagemRESUMO
The objective of this study was to determine which transabdominal ultrasound parameters correlate with portal vein stenosis (PVS) on percutaneous transhepatic portal venography in pediatric liver transplant patients. A retrospective review was performed of percutaneous transhepatic portal venograms performed between 2005 and 2013. The findings were compared to those from ultrasounds performed before venography and at the baseline. Patients were stratified on the basis of the presence of significant PVS (group 1, >50% stenosis; group 2, ≤50% stenosis) on portal venography. Findings were compared to those for age-matched controls. Twenty portal venograms were performed for 12 pediatric patients. Thirteen of the 20 patients (65%) demonstrated significant PVS (>50%). The mean peak anastomotic velocity (PAV) was 253.6±96 cm/s in group 1, 169.7±48 cm/s in group 2, and 51.3±20 cm/s in the control group. PAV (r=0.672, P=0.002) was the only ultrasound variable that correlated with the presence of significant PVS. A receiver operating characteristic curve was generated from PAV and PVS data (area under the curve=0.75, P=0.08). A threshold velocity of 180 cm/s led to a sensitivity of 83% and a specificity of 71% in predicting significant PVS on portal venography. At the baseline, the mean PAV was 155.8±90 cm/s for group 1 and 69.5±33 cm/s for group 2 (P=0.08); for control subjects, it was 78.9±53 cm/s (P=0.06). PAV is the only measured ultrasound parameter that correlates with significant PVS on portal venography in pediatric liver transplant patients. An elevated baseline PAV may increase the risk of developing PVS.
Assuntos
Transplante de Fígado/efeitos adversos , Flebografia/métodos , Veia Porta/cirurgia , Portografia/métodos , Ultrassonografia Doppler , Doenças Vasculares/diagnóstico por imagem , Anastomose Cirúrgica , Angiografia Digital , Área Sob a Curva , Velocidade do Fluxo Sanguíneo , Pré-Escolar , Constrição Patológica , Feminino , Georgia , Humanos , Circulação Hepática , Masculino , Veia Porta/diagnóstico por imagem , Veia Porta/fisiopatologia , Valor Preditivo dos Testes , Curva ROC , Estudos Retrospectivos , Fatores de Risco , Centros de Atenção Terciária , Resultado do Tratamento , Doenças Vasculares/etiologia , Doenças Vasculares/fisiopatologiaRESUMO
Mycotic pseudoaneurysm is a rare complication of systemic infection in children. We report a case of a previously healthy 11-year-old female with methicillin-resistant staph aureus (MRSA) bacteremia who developed both pulmonary and systemic arterial pseudoaneurysms. These were detected on magnetic resonance (MR) and computed tomography (CT) imaging and treated with coil embolization.
RESUMO
Somatic activating variants in PIK3CA, the gene that encodes the p110α catalytic subunit of phosphatidylinositol 3-kinase (PI3K), have been previously detected in â¼80% of lymphatic malformations (LMs).1 , 2 We report the presence of somatic activating variants in BRAF in individuals with LMs that do not possess pathogenic PIK3CA variants. The BRAF substitution p.Val600Glu (c.1799T>A), one of the most common driver mutations in cancer, was detected in multiple individuals with LMs. Histology revealed abnormal lymphatic channels with immunopositivity for BRAFV600E in endothelial cells that was otherwise indistinguishable from PIK3CA-positive LM. The finding that BRAF variants contribute to low-flow LMs increases the complexity of prior models associating low-flow vascular malformations (LM and venous malformations) with mutations in the PI3K-AKT-MTOR and high-flow vascular malformations (arteriovenous malformations) with mutations in the RAS-mitogen-activated protein kinase (MAPK) pathway.3 In addition, this work highlights the importance of genetic diagnosis prior to initiating medical therapy as more studies examine therapeutics for individuals with vascular malformations.
RESUMO
This retrospective, single center, case report describes the first use of the Ellipsys Vascular Access System for percutaneous arteriovenous fistula (pAVF) creation in children. Two adolescent (<20 year of age) patients (18 and 19-year-old females), one of whom was developmentally delayed, were not considered candidates for traditional surgical arteriovenous fistula creation. pAVF creation was successful in both patients using the Ellipsys device and physiologic maturation of the fistula was achieved within 8 weeks of creation with subsequent 2 needle cannulation. No complications or adverse events were encountered. pAVF creation with the Ellipsys device can be safely performed in adolescents. Further studies will be needed to validate the expanded use of these devices in children.