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1.
Childs Nerv Syst ; 40(2): 555-561, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37796295

RESUMO

INTRODUCTION: Polymorphous low grade neuroepithelial tumor of the young (PLNTY) is a newly described epileptogenic tumor first reported by Jason. T. Huse et al. in 2016. Only a very few cases have been reported so far and has been recently incorporated in the World Health Organization (WHO) Central Nervous System Classification of tumours, 5th edition, 2021. Here we report a rare case of PLNTY which closely resembles DNET (Dysembryoplastic neuroepithelial tumor) with plenty of interesting findings which would otherwise go unnoticed resulting in a nonspecific or misclassified diagnosis. CASE REPORT: A 12 year old boy presented to the Neurosurgery OPD with seizures for the past five years and was given multiple antiepileptics for the same. Magnetic resonance imaging (MRI) showed a well-defined lobulated cortical mass with T1 hypo intensity and T2 hyperintensity in the left temporal lobe measuring 2.1 × 2 × 1.3 cm suggesting a DNET. Left temporal craniotomy and excision of the lesion was done. Frozen section showed features of a low grade glial neoplasm. Routine sections demonstrated polymorphous findings including oligodendroglia like features, neuronal nuclear pleomorphism, spindled astroglial elements, perivascular rosettes, calcification, and vascular mineralization. By immunohistochemistry (IHC), the tumor cells were diffusely positive for GFAP and CD34.Ki67 labelling index was low. A final diagnosis of PLNTY was made based on the above findings. The child has been epilepsy free since the past one-month post-surgery and is on follow up. DISCUSSION/CONCLUSION: PLNTY is a newly discovered distinct pediatric low grade glial neoplasm which was earlier grouped into nonspecific forms of DNET. It is characterized morphologically and molecularly by the presence of oligodendroglial component, CD34 expression, BRAFV600E mutation and alterations in the MAP kinase pathway. They are known to behave in a low-grade fashion amenable to control by excision with occasional cases of recurrence reported. It is important to recognize and report similar tumors to determine the long-term risk of recurrence and create a more complete understanding on their radiology and molecular genetics.


Assuntos
Neoplasias Encefálicas , Epilepsia , Glioma , Neoplasias Neuroepiteliomatosas , Masculino , Humanos , Criança , Neoplasias Neuroepiteliomatosas/diagnóstico por imagem , Neoplasias Neuroepiteliomatosas/cirurgia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Convulsões
2.
Pediatr Radiol ; 54(1): 181-196, 2024 01.
Artigo em Inglês | MEDLINE | ID: mdl-37962604

RESUMO

BACKGROUND: The management of pediatric trauma with trans-arterial embolization is uncommon, even in level 1 trauma centers; hence, there is a dearth of literature on this subject compared to the adult experience. OBJECTIVE: To describe a single-center, level 1 trauma center experience with arterial embolization for pediatric trauma. MATERIALS AND METHODS: A retrospective review was performed to identify demographics, transfusion requirements, pre-procedure imaging, procedural details, adverse events, and arterial embolization outcomes over a 19-year period. Twenty children (age 4.5 months to 17 years, median 13.5 years; weight 3.6 to 108 kg, median 53 kg) were included. Technical success was defined as angiographic resolution of the bleeding-related abnormality on post-embolization angiography or successful empiric embolization in the absence of an angiographic finding. Clinical success was defined as not requiring additional intervention after embolization. RESULTS: Seventy-five percent (n=15/20) of patients required red blood cell transfusions prior to embolization with a mean volume replacement 64 ml/kg (range 12-166 ml/kg) and the median time from injury to intervention was 3 days (range 0-16 days). Technical success was achieved in 100% (20/20) of children while clinical success was achieved in 80% (n=16/20). For the 4 children (20%) with continued bleeding following initial embolization, 2 underwent repeat embolization, 1 underwent surgery, and 1 underwent repeat embolization and surgery. Mortality prior to discharge was 15% (n=3). A post-embolization mild adverse event included one groin hematoma, while a severe adverse event included one common iliac artery pseudoaneurysm requiring open surgical ligation. CONCLUSIONS: In this single-center experience, arterial embolization for hemorrhage control in children after trauma is feasible but can be challenging and the clinical failure rate of 20% in this series reflects this complexity. Standardization of pre-embolization trauma assessment parameters and embolic techniques may improve outcomes.


Assuntos
Traumatismos Abdominais , Embolização Terapêutica , Adulto , Humanos , Criança , Lactente , Embolização Terapêutica/métodos , Hemorragia/diagnóstico por imagem , Hemorragia/terapia , Hemorragia/etiologia , Estudos Retrospectivos , Centros de Traumatologia , Resultado do Tratamento
3.
Catheter Cardiovasc Interv ; 101(5): 853-862, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36877806

RESUMO

PURPOSE: The contribution of thoracic duct obstruction to lymphatic flow disorders has not been well-characterized. We describe imaging findings, interventions, and outcomes in patients with suspected duct obstruction by imaging or a lympho-venous pressure gradient (LVPG). MATERIALS AND METHODS: Clinical, imaging, and interventional data, including the LVPG, of patients with flow disorders and imaging features of duct obstruction who underwent lymphatic intervention were retrospectively reviewed, collated, and analyzed with descriptive statistics. RESULTS: Eleven patients were found to have obstruction, median age 10.4 years (interquartile range: 8-14.9 years). Pleural effusions were seen in 8/11 (72%), ascites in 8/11 (72%), both in 5/11 (45%), and protein-losing enteropathy in 5 (45%). Eight patients (72%) had congenital heart disease. The most common site of obstruction was at the duct outlet in 7/11 patients (64%). Obstruction was secondary to extrinsic compression or ligation 4 patients (36%). Nine patients (82%) underwent interventions, with balloon dilation in 7/9 (78%), massive lymphatic malformation drainage and sclerotherapy in 1, and lympho-venous anastomosis in 1. There was resolution of symptoms in 7/9 (78% who underwent intervention, with worsening in 1 patient and no change in 1. In these patients, preprocedure mean LVPG was 7.9 ± 5.7 mmHg and postprocedure gradient was 1.6 ± 1.9 mmHg (p = 0.014). Five patients in this series underwent intervention solely to alleviate duct obstruction and in 4/5 (80%) this led to resolution of symptoms (p = 0.05). CONCLUSION: Duct obstruction may be seen in lymphatic flow disorders and can occur from intrinsic and extrinsic causes. Stenosis at the outlet was most common. Obstruction can be demonstrated by an elevated LVPG, and interventions to alleviate the obstruction can be beneficial.


Assuntos
Cardiopatias Congênitas , Doenças Linfáticas , Humanos , Criança , Ducto Torácico/diagnóstico por imagem , Estudos Retrospectivos , Resultado do Tratamento , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/terapia
4.
J Vasc Interv Radiol ; 34(10): 1815-1821, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37336489

RESUMO

PURPOSE: To describe a single-center experience of placing percutaneous nephrostomy (PCN) tubes in neonates and young infants aged ≤3 months. MATERIALS AND METHODS: This retrospective study evaluated PCN placement during a 19-year period. Medical records were reviewed for patient demographics, indications, procedure details, catheter-related adverse events, and outcomes. A total of 45 primary PCN insertions were attempted in 29 children (median age, 11 days [range, first day of life to 3 months]; median weight, 3.5 kg [range, 1.4-7.0 kg]). Salvage procedures resulted in 13 secondary catheters in 6 children. The most common indication was ureteropelvic junction obstruction (40.0%), and the most common urinary tract dilation classification was P3 (88.9%). RESULTS: Technical success for primary placements was 95.6%; both technical failures were due to loss of access in the same patient. Of primary placements, 76.7% were electively removed, 6.9% were dislodged but not replaced, and the remaining 16.3% required salvage procedures. Mechanical adverse events occurred in 20.9% of primary and 53.8% of secondary catheters, including partial retraction, complete dislodgement, and occlusion. Urinary tract infections (UTIs) occurred in 18.6% of primary and 15.4% of secondary catheters. Urosepsis occurred in 2.3% of primary and 7.7% of secondary catheters. Median primary catheter dwell time was 41 days (range, 1-182 days) and median secondary catheter dwell time was 31 days (range, 10-107 days). CONCLUSION: PCN placement in neonates and young infants has a high technical success rate, although not without particular procedural and management challenges of catheter malfunction and UTI.


Assuntos
Nefrostomia Percutânea , Obstrução Ureteral , Infecções Urinárias , Recém-Nascido , Criança , Humanos , Lactente , Nefrostomia Percutânea/efeitos adversos , Nefrostomia Percutânea/métodos , Estudos Retrospectivos , Catéteres , Infecções Urinárias/etiologia , Obstrução Ureteral/diagnóstico por imagem , Obstrução Ureteral/etiologia , Obstrução Ureteral/cirurgia
5.
Pediatr Radiol ; 53(2): 324-331, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36104540

RESUMO

Primary percutaneous gastrostomy and gastrojejunostomy tube placements are fundamental procedures performed in pediatric interventional radiology, with both antegrade and retrograde techniques described. In pediatric patients, however, challenges may arise due to smaller patient size and anatomical variations. Several adjunctive techniques may facilitate safe percutaneous access in the setting of a limited percutaneous gastric access window. These include the intra-procedural use of cone beam computed tomography (CT), percutaneous needle decompression in the setting of distended air-filled bowel interposed between the stomach and abdominal wall, post-pyloric balloon occlusion to facilitate gastric distension, ultrasound-guided gastric puncture, and intra-gastric contrast-enhanced ultrasound (ceUS) to define the relationship of the gastric wall and the anterior abdominal wall. Adjunctive techniques may increase successful primary percutaneous gastroenteric tube placement and may improve operator confidence in safe placement.


Assuntos
Derivação Gástrica , Gastrostomia , Humanos , Criança , Gastrostomia/métodos , Fluoroscopia/métodos , Estômago , Nutrição Enteral , Derivação Gástrica/métodos
6.
Pediatr Radiol ; 53(7): 1380-1390, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35980463

RESUMO

The lymphatic system has been poorly understood and its importance neglected for decades. Growing understanding of lymphatic flow pathophysiology through peripheral and central lymphatic flow imaging has improved diagnosis and treatment options in children with lymphatic diseases. Flow dynamics can now be visualized by different means including dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), the current standard technique to depict central lymphatics. Novel imaging modalities including intranodal, intrahepatic and intramesenteric DCMRL are quickly evolving and have shown important advances in the understanding and guidance of interventional procedures in children with intestinal lymphatic leaks. Lymphatic imaging is gaining importance in the radiologic and clinical fields and new techniques are emerging to overcome its limitations.


Assuntos
Doenças Linfáticas , Vasos Linfáticos , Criança , Humanos , Meios de Contraste , Vasos Linfáticos/patologia , Sistema Linfático/diagnóstico por imagem , Doenças Linfáticas/diagnóstico por imagem , Doenças Linfáticas/patologia , Linfografia/métodos
7.
Pediatr Surg Int ; 39(1): 257, 2023 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-37653245

RESUMO

PURPOSE: Central lymphatic obstructions are associated with anasarca and high mortality. We hypothesized that opening dilated cutaneous lymphatic channels by creating a lymphocutaneous fistula (LCF) would decompress the lymphatic circulation and improve anasarca. METHODS: We reviewed all patients that had at least one LCF created between 9/2019 and 12/2022. LCF efficacy was determined by changes in weight, urine/diuresis, ventilation, and clinical status. RESULTS: We created eleven LCFs in four infants. LCFs initially drained 108 cc/kg/d (IQR68-265 cc/kg/d). Weights significantly decreased after LCF creation (6.9 [IQR6.1-8.1] kg vs. 6.1 [IQR 4.9-7.6] kg, P = 0.042). Ventilatory support decreased significantly in all patients after at least one LCF was created, and 3/4 patients (75%) had significantly lower peak inspiratory pressures (28 [IQR 25-31] cmH2O vs. 22 [IQR 22-24] cmH2O, P = 0.005; 36 [IQR36-38] cmH2O vs. 33 [IQR 33-35] cmH2O, P = 0.002; 36 [IQR 34-47] cmH2O vs. 28 [28-31] cmH2O, P = 0.002). LCFs remained patent for 29d (IQR 16-49d). LCFs contracted over time, and 6/11 (54.5%) were eventually revised. There were no complications. Two patients died from overwhelming disease, one died from unrelated causes, and one remains alive 29 months after their initial LCF. CONCLUSION: LCFs provide safe and effective temporary lymphatic decompression in patients with central lymphatic obstruction. While LCFs are not a cure, they can serve as a bridge to more definitive therapies or spontaneous lymphatic remodeling. LEVEL OF EVIDENCE: IV.


Assuntos
Fístula , Sistema Linfático , Humanos , Lactente , Fístula/cirurgia , Sistema Linfático/cirurgia
8.
Eur Radiol ; 32(4): 2564-2571, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35001156

RESUMO

PURPOSE: To assess the feasibility of direct intra-lymphatic administration of diluted ferumoxytol as a T1-positive contrast agent for dynamic contrast-enhanced MR lymphangiography (DCMRL) imaging of the central lymphatics in children with renal disease. METHODS: In vitro scan of dilute ferumoxytol was initially performed using time-resolved and high-resolution 3D gradient echo (GRE) sequences with short TE values (1 to 1.5 ms). A ferumoxytol concentration of 0.25 to 0.40 mg/mL was found to retain high signal in the T1-weighted sequences. DCMRL was then performed in 4 children with renal disease with the same 3D GRE sequences administrating diluted ferumoxytol via intra-mesenteric (IM), intra-hepatic (IH), and intra-nodal (IN) routes (6 to 9 mL to each site; average total dose of 0.75 mg/kg) by slow hand injection (0.5 to 1.0 mL/min). The signal-to-noise ratio (SNR) of the lymphatics was measured for quantitative evaluation. RESULTS: Ferumoxytol-enhanced DCMRL was technically successful in all patients. Contrast conspicuity within the lymphatics was sufficient without subtraction. The mean SNR was significantly higher than the muscle (50.1 ± 12.2 vs 13.2 ± 2.8; t = 15.9; p < .001). There were no short-term complications attributed to the administration of ferumoxytol in any of the four patients. CONCLUSION: Magnetic resonance lymphangiography using ferumoxytol via IN, IH, and IM access is a new method to directly visualize the central lymphatic system and can be applied safely in patients with renal failure based on our preliminary report of four cases. Ferumoxytol-enhanced DCMRL shows diagnostic image quality by using 3D GRE sequences with short TE values and appropriate dilution of ferumoxytol. KEY POINTS: • MR lymphangiography using ferumoxytol via intra-nodal, intra-hepatic, and intra-mesenteric access is a new method to directly visualize the central lymphatic system from the groin to the venous angle. • FDCMRL can be applied safely in patients with renal failure based on our preliminary report of four cases. • FDCMRL shows diagnostic image quality by using 3D GRE sequences with short TE values and appropriate dilution of the ferumoxytol.


Assuntos
Óxido Ferroso-Férrico , Insuficiência Renal Crônica , Criança , Meios de Contraste/farmacologia , Estudos de Viabilidade , Humanos , Linfografia/métodos , Imageamento por Ressonância Magnética/métodos , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/diagnóstico por imagem
9.
Eur Radiol ; 32(1): 112-121, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34165621

RESUMO

OBJECTIVES: To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention. METHODS: In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 - April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed. RESULTS: During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p < .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p < .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6 months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics. CONCLUSION: We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal. KEY POINTS: • We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. • Imaging patterns were correlated with disease presentation (normal - chylothorax/PB, hepatoperitoneal - ascites/chylothorax, hepatopulmonary - chylothorax/PB, hepatoduodenal - PLE). • Abnormal imaging patterns correlated with increased morbidity.


Assuntos
Quilotórax , Doenças Linfáticas , Vasos Linfáticos , Humanos , Fígado/diagnóstico por imagem , Doenças Linfáticas/diagnóstico por imagem , Linfografia , Estudos Retrospectivos
10.
J Pediatr Gastroenterol Nutr ; 74(1): 38-45, 2022 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-34406998

RESUMO

OBJECTIVES: Protein-losing enteropathy (PLE) is a disorder of intestinal lymphatic flow resulting in leakage of protein-rich lymph into the gut lumen. Our primary aim was to report the imaging findings of dynamic contrast magnetic resonance lymphangiography (DCMRL) in patients with PLE. Our secondary objective was to use these imaging findings to characterize lymphatic phenotypes. METHODS: Single-center retrospective cohort study of patients with PLE unrelated to single-ventricle circulation who underwent DCMRL. We report imaging findings of intranodal (IN), intrahepatic (IH), and intramesenteric (IM) access points for DCMRL. RESULTS: Nineteen patients 0.3-58 years of age (median 1.2 years) underwent 29 DCMRL studies. Primary intestinal lymphangiectasia (PIL) was the most common referring diagnosis (42%). Other etiologies included constrictive pericarditis, thoracic insufficiency syndrome, and genetic disorders. IN-DCMRL demonstrated a normal central lymphatic system in all patients with an intact thoracic duct and localized duodenal leak in one patient (1/19, 5%). IH-DCMRL detected a duodenal leak in 12 of 17 (71%), and IM-DCMRL detected duodenal leak in 5 of 6 (83%). Independent of etiology, lymphatic leak was only visualized in the duodenum. CONCLUSIONS: In patients with PLE, imaging via DCMRL reveals that leak is localized to the duodenum regardless of etiology. Comprehensive imaging evaluation with three access points can provide detailed information about the site of duodenal leak.


Assuntos
Linfografia , Enteropatias Perdedoras de Proteínas , Duodeno/diagnóstico por imagem , Humanos , Lactente , Sistema Linfático , Linfografia/métodos , Espectroscopia de Ressonância Magnética , Enteropatias Perdedoras de Proteínas/diagnóstico por imagem , Enteropatias Perdedoras de Proteínas/etiologia , Estudos Retrospectivos
11.
Pediatr Radiol ; 52(2): 285-294, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33830292

RESUMO

Lymphatic flow disorders include a broad spectrum of abnormalities that can originate in the lymphatic or the venous system. The development of these disorders is multifactorial and is most commonly associated with congenital heart diseases and palliative surgeries that these patients undergo. Central lymphatic disorders might be secondary to traumatic leaks, lymphatic overproduction, conduction abnormalities or lymphedema, and they can progress to perfusion anomalies. Several imaging modalities have been used to visualize the lymphatic system. However, the imaging of central lymphatic flow has always been challenging. Dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) allows for visualization of central lymphatic flow disorders and has been recently applied for the assessment of plastic bronchitis, protein-losing enteropathy, chylothorax and chylopericardium, among other lymphatic disorders. The hepatic and mesenteric accesses are innovative and promising techniques for better identification and understanding of these abnormalities. The main objectives of this review are to discuss the physiology and anatomy of the lymphatic system and review the current uses of DCMRL in the diagnosis and management of lymphatic flow disorders.


Assuntos
Meios de Contraste , Linfografia , Humanos , Sistema Linfático/diagnóstico por imagem , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética
12.
Eur Radiol ; 30(10): 5777-5784, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32462442

RESUMO

OBJECTIVES: To report on our initial experience with intramesenteric (IM) dynamic contrast magnetic resonance lymphangiography (DCMRL) for evaluation of the lymphatics in patients with concern for mesenteric lymphatic flow disorders and to compare IM-DCMRL with intrahepatic (IH) and intranodal (IN) DCMRL. METHODS: This is a retrospective review of imaging findings in 15 consecutive patients who presented with protein losing enteropathy (PLE) and/or ascites undergoing IM-DCMRL, IH-DCMRL, and IN-DCMRL. The IM-DCMRL technique involves the injection of a gadolinium contrast agent into the mesenteric lymphatic ducts or lymph nodes followed by imaging of the abdomen and chest with dynamic time-resolved MR lymphangiography. RESULTS: IM-DCMRL was successfully performed in 14/15 (93%) of the patients. When comparing IN-DCMRL with IM-DCMRL, there was a significant difference in the visualization of dermal backflow (p = 0.014), duodenal perfusion (p = 0.003), duodenal leak (p = 0.014), and peritoneal leak (p = 0.003). IM-DCMRL demonstrated peritoneal leak in 7 patients in contrast to IH-DCMRL which demonstrated leak in 4 patients and IN-DCMRL which did not demonstrate any peritoneal leaks. Duodenal leaks were seen by IH-DCMRL in 9 patients versus 5 with IM-DCMRL and none with IN-DCMRL. In one patient with congenital PLE, the three modalities showed different disconnected flow patterns with duodenal leak only seen by IM-DCMRL. There were no short-term complications from the procedures. CONCLUSIONS: IM-DCMRL is a feasible imaging technique for evaluation of the mesenteric lymphatics. In certain mesenteric lymphatic flow abnormalities, such as PLE and ascites, this imaging may be helpful for diagnosis and the planning of interventions and warrants further studies. KEY POINTS: • Intramesenteric dynamic contrast magnetic resonance lymphangiography (IM-DCMRL) is a new imaging technique to evaluate mesenteric lymphatic flow disorders such as ascites. • IM-DCMRL is able to image lymphatic leaks in patients with ascites and protein losing enteropathy not seen with intranodal (IN-DCMRL) imaging.


Assuntos
Angiografia/métodos , Meios de Contraste/farmacologia , Linfografia/métodos , Imageamento por Ressonância Magnética , Adolescente , Ascite , Criança , Pré-Escolar , Feminino , Gadolínio/farmacologia , Humanos , Processamento de Imagem Assistida por Computador , Lactente , Linfonodos , Doenças Linfáticas , Sistema Linfático , Vasos Linfáticos , Masculino , Estudos Retrospectivos
13.
Paediatr Respir Rev ; 36: 2-7, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31884062

RESUMO

Pulmonary lymphatic flow disorders involve the abnormal lymphatic flow via lymphatic channels to the lungs and pleural space. Plastic bronchitis and chylothorax are the main complications of this abnormal lymphatic perfusion, which has been termed pulmonary lymphatic perfusion syndrome (PLPS). Following lymphatic access, dynamic contrast MR lymphangiography is the imaging modality of choice to diagnose these disorders. Management includes medical therapy, percutaneous interventions under fluoroscopy, and surgical interventions.


Assuntos
Bronquite/diagnóstico por imagem , Quilotórax/diagnóstico por imagem , Vasos Linfáticos/diagnóstico por imagem , Linfografia , Imageamento por Ressonância Magnética , Adolescente , Bronquite/terapia , Criança , Pré-Escolar , Quilotórax/terapia , Meios de Contraste , Dietoterapia , Suplementos Nutricionais , Gerenciamento Clínico , Embolização Terapêutica , Humanos , Lactente , Pneumopatias/diagnóstico por imagem , Pneumopatias/terapia , Anormalidades Linfáticas/diagnóstico por imagem , Anormalidades Linfáticas/terapia , Doenças Linfáticas/diagnóstico por imagem , Doenças Linfáticas/terapia , Vasos Linfáticos/anormalidades , Vasos Linfáticos/cirurgia , Microcirurgia
14.
Pediatr Radiol ; 50(13): 1872-1880, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33252755

RESUMO

Lymphangiectasias are lymphatic malformations characterized by the abnormal dilation and morphology of the lymphatic channels. The classification and treatment of these disorders can be challenging given the limited amount of literature available in children. Various imaging modalities are used to confirm suspected diagnosis, plan the most appropriate treatment, and estimate a prognosis. Prenatal evaluation is performed using both prenatal US imaging and fetal MRI. These modalities are paramount for appropriate parental counseling and planning of perinatal care. During the neonatal period, chest US imaging is a useful modality to evaluate pulmonary lymphangiectasia because other modalities such as conventional radiography and CT display nonspecific findings. Finally, the recent breakthroughs in lymphatic imaging with MRI have allowed us to better classify lymphatic disorders. Dynamic contrast-enhanced lymphangiography, conventional lymphangiography and percutaneous lymphatic procedures offer static and dynamic evaluation of the central conducting lymphatics in children, with excellent spatial resolution and the possibility to provide treatment. The purpose of this review is to discuss the normal and abnormal development of the fetal lymphatic system and how to best depict it by imaging during the prenatal and postnatal life.


Assuntos
Linfangiectasia , Doenças Linfáticas , Vasos Linfáticos , Criança , Feminino , Humanos , Recém-Nascido , Linfangiectasia/diagnóstico por imagem , Linfografia , Imageamento por Ressonância Magnética , Gravidez , Diagnóstico Pré-Natal
15.
Pediatr Radiol ; 50(8): 1148-1155, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32390060

RESUMO

BACKGROUND: Clotting factor replacement forms the pillar of treatment for children with hemophilia. Most children can be treated using peripheral venipuncture, but very young children and children with poor venous access might require a central venous catheter. Short-term and long-term complications of implantable venous access device placement (also known as port placement) can result in important morbidity and mortality in children with hemophilia. OBJECTIVE: The purpose of this study is to describe our experience with port placement in children and adolescents with severe hemophilia (<1% of the Factors VIII or IX). MATERIALS AND METHODS: We performed a retrospective review over a 10-year period to identify port placement in pediatric patients with severe hemophilia. We reviewed demographic and procedural information, access frequency, mechanical complications, and central-line-associated bloodstream infections (CLABSI). Eighteen males were included, with median age at insertion of 3.9 years (0.7-22.7 years). Fifteen of the 18 patients had hemophilia Type A and 3/18 had Type B. Thirteen had high neutralizing inhibitor titers. RESULTS: Technical success in port placement was achieved in 26/27 (96.3%) patients, with 1 port failure caused by venous occlusion from prior catheter placement. Port catheter size ranged from 5 French (Fr) to 7.5 Fr. All were single-lumen and placed via right (76.9%) or left (23.1%) internal jugular vein; 59.3% were placed during general anesthesia, and all had factor replacement prophylaxis. A peripherally inserted central catheter (PICC) was placed concurrently in 69.2% of the cases; per hospital policy, the port was only accessed 15 days post-placement to reduce the risk of site hematoma. Two patients were lost to follow-up. The total catheter days was 15,893. Ports were removed in 14/24 cases, most commonly because of CLABSI (7/24; 29.2%) and transition to peripheral infusion (3/24; 12.5%). Bleeding was the most common complication in the first 30 days after placement. There were nine CLABSI events (0.57 per 1,000 catheter days), all in patients with high neutralizing inhibitor titers. A higher frequency of port access (more or equal to daily vs. less than daily) correlated with higher infection rates (P=0.02). Median time from port insertion to first infection was 348 days (range 167-1,055 days). There were four fibrin-sheath-related catheter occlusions (0.25 per 1,000 catheter days): three catheters were salvaged with intra-catheter tissue plasminogen activator (tPA) instillation resulting in a salvage of an additional 1,214 catheter days, and one catheter was removed after tPA failure (0.06 per 1,000 catheter days). CONCLUSION: Port maintenance in boys with severe hemophilia is challenging given the need for long-term frequent device access that is associated with catheter-related infections. The rate of bleeding or infection did not differ in patients whether the device was accessed immediately or 15 days post placement. With appropriate pre- and post-procedural factor replacement, immediate and early term severe complications are not common.


Assuntos
Fatores de Coagulação Sanguínea/administração & dosagem , Cateterismo Venoso Central , Cateteres de Demora , Hemofilia A/terapia , Adolescente , Infecções Relacionadas a Cateter/etiologia , Cateterismo Venoso Central/efeitos adversos , Cateteres de Demora/efeitos adversos , Criança , Humanos , Masculino , Estudos Retrospectivos
17.
J Vasc Interv Radiol ; 29(4): 492-496, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29352697

RESUMO

PURPOSE: To evaluate technical success and the incidences of, and risk factors for, mechanical and infectious complications of venous port placement in infants. MATERIALS AND METHODS: This was a retrospective single-institution cohort study of port placement in infants (age < 1 y) from January 2006 through June 2016 (mean age, 7.5 mo ± 3.3; mean weight, 8.1 kg ± 1.9). Age, weight, sex, side of placement, tip position, and indication for placement (chemotherapy vs other) were recorded. Total catheter-days (CDs), mechanical complications, and central catheter-associated bloodstream infections (CCABSIs) were identified. RESULTS: During the study years, 64 ports were placed in 64 infants, with a technical success rate of 100%. The mean catheter life was 321 days (total range, 4-1,917 d; interquartile range [IQR], 107-421 d). There were 13 CCABSI events (0.63 per 1,000 CDs); of these, 8 (12.5% among 64 patients) required port removal for infection. There was an increase in CCABSIs in patients with left-sided port placement (relative risk [RR], 3.22; 95% confidence interval [CI], 1.02-10.14; P = .05). There were 8 mechanical complications of the port reservoir or catheter (0.39 per 1,000 CDs). Of these, 2 (3.1%) required removal. Patients in the lowest weight quartile were at an increased risk of mechanical complications (RR, 4.37; 95% CI, 1.09-17.48; P = .04). CONCLUSIONS: Venous ports can be placed with a high rate of technical success in infants. Left-sided ports and low weight are associated with increased infectious and mechanical complications, respectively.


Assuntos
Infecções Relacionadas a Cateter/epidemiologia , Cateterismo Venoso Central/métodos , Cateteres de Demora , Cateterismo Venoso Central/efeitos adversos , Cateteres de Demora/efeitos adversos , Remoção de Dispositivo , Feminino , Humanos , Lactente , Masculino , Radiografia Intervencionista , Estudos Retrospectivos , Resultado do Tratamento
18.
Pediatr Radiol ; 48(2): 270-278, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29128920

RESUMO

Magnetic resonance imaging (MRI) often provides better visualization of bone marrow abnormalities than computed tomography (CT) or fluoroscopy, but bone biopsies are usually performed using conventional CT or, more recently, C-arm CT guidance. Biopsies of bone lesions solely visible on MRI are often challenging to localize and require the operator to review the MRI on a separate console to correlate with MRI anatomical landmarks during the biopsy. The MR overlay technique facilitates such biopsies in the angiographic suite by allowing the pre-procedural 3-D MRI to be overlaid on intraprocedural 2-D fluoroscopy. This study describes our initial experience with the MR overlay technique in the angiography suite during pediatric percutaneous extremity bone biopsies of lesions visible on MRI but not on CT or fluoroscopy and demonstrates its utility in relevant clinical cases.


Assuntos
Doenças Ósseas/diagnóstico por imagem , Angiografia por Ressonância Magnética/métodos , Pontos de Referência Anatômicos , Biópsia por Agulha , Doenças Ósseas/patologia , Criança , Feminino , Fluoroscopia , Humanos , Interpretação de Imagem Assistida por Computador , Imageamento Tridimensional , Masculino , Estudos Prospectivos , Doses de Radiação , Radiografia Intervencionista , Tomografia Computadorizada por Raios X
19.
Pediatr Radiol ; 47(13): 1817-1824, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28983677

RESUMO

BACKGROUND: Reduced-dose C-arm computed tomography (CT) uses flat-panel detectors to acquire real-time 3-D images in the interventional radiology suite to assist with anatomical localization and procedure planning. OBJECTIVE: To describe dose-reduction techniques for C-arm CT at a pediatric institution and to provide guidance for implementation. MATERIALS AND METHODS: We conducted a 5-year retrospective study on procedures using an institution-specific reduced-dose protocol: 5 or 8 s Dyna Rotation, 248/396 projection images/acquisition and 0.1-0.17 µGy/projection dose at the detector with 0.3/0.6/0.9-mm copper (Cu) filtration. We categorized cases by procedure type and average patient age and calculated C-arm CT and total dose area product (DAP). RESULTS: Two hundred twenty-two C-arm CT-guided procedures were performed with a dose-reduction protocol. The most common procedures were temporomandibular and sacroiliac joint injections (48.6%) and sclerotherapy (34.2%). C-arm CT was utilized in cases of difficult percutaneous access in less common applications such as cecostomy and gastrostomy placement, foreign body retrieval and thoracentesis. C-arm CT accounted for between 9.9% and 80.7% of the total procedural DAP. CONCLUSION: Dose-reducing techniques can preserve image quality for intervention while reducing radiation exposure to the child. This technology has multiple applications within pediatric interventional radiology and can be considered as an adjunctive imaging tool in a variety of procedures, particularly when percutaneous access is challenging despite routine fluoroscopic or ultrasound guidance.


Assuntos
Doses de Radiação , Proteção Radiológica/métodos , Radiografia Intervencionista/métodos , Tomografia Computadorizada por Raios X/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Imageamento Tridimensional , Masculino , Exposição à Radiação , Estudos Retrospectivos
20.
Pediatr Radiol ; 47(8): 963-973, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28474255

RESUMO

BACKGROUND: Navigational software provides real-time fluoroscopic needle guidance for percutaneous procedures in the Interventional Radiology (IR) suite. OBJECTIVE: We describe our experience with navigational software for pediatric percutaneous bone biopsies in the IR suite and compare technical success, diagnostic accuracy, radiation dose and procedure time with that of CT-guided biopsies. MATERIALS AND METHODS: Pediatric bone biopsies performed using navigational software (Syngo iGuide, Siemens Healthcare) from 2011 to 2016 were prospectively included and anatomically matched CT-guided bone biopsies from 2008 to 2016 were retrospectively reviewed with institutional review board approval. C-arm CT protocols used for navigational software-assisted cases included institution-developed low-dose (0.1/0.17 µGy/projection), regular-dose (0.36 µGy/projection), or a combination of low-dose/regular-dose protocols. Estimated effective radiation dose and procedure times were compared between software-assisted and CT-guided biopsies. RESULTS: Twenty-six patients (15 male; mean age: 10 years) underwent software-assisted biopsies (15 pelvic, 7 lumbar and 4 lower extremity) and 33 patients (13 male; mean age: 9 years) underwent CT-guided biopsies (22 pelvic, 7 lumbar and 4 lower extremity). Both modality biopsies resulted in a 100% technical success rate. Twenty-five of 26 (96%) software-assisted and 29/33 (88%) CT-guided biopsies were diagnostic. Overall, the effective radiation dose was significantly lower in software-assisted than CT-guided cases (3.0±3.4 vs. 6.6±7.7 mSv, P=0.02). The effective dose difference was most dramatic in software-assisted cases using low-dose C-arm CT (1.2±1.8 vs. 6.6±7.7 mSv, P=0.001) or combined low-dose/regular-dose C-arm CT (1.9±2.4 vs. 6.6±7.7 mSv, P=0.04), whereas effective dose was comparable in software-assisted cases using regular-dose C-arm CT (6.0±3.5 vs. 6.6±7.7 mSv, P=0.7). Mean procedure time was significantly lower for software-assisted cases (91±54 vs. 141±68 min, P=0.005). CONCLUSION: In our experience, navigational software technology in the IR suite is a promising alternative to CT guidance for pediatric bone biopsies providing comparable technical success and diagnostic accuracy with lower radiation dose and procedure time, in addition to providing real-time fluoroscopic needle guidance.


Assuntos
Doenças Ósseas/patologia , Biópsia Guiada por Imagem/métodos , Software , Tomografia Computadorizada por Raios X/métodos , Biópsia por Agulha , Criança , Feminino , Fluoroscopia , Humanos , Masculino , Estudos Prospectivos , Doses de Radiação , Interpretação de Imagem Radiográfica Assistida por Computador , Radiologia Intervencionista , Estudos Retrospectivos
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