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This report demonstrates the feasibility and safety of using contrast-enhanced ultrasound (CEUS) to confirm intranodal needle position in children requiring dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL). A total of 7 patients were evaluated using CEUS after nodal puncture on a detachable magenetic resonance table, with 2 nodes cannulated in each patient, resulting in a combined evaluation of 14 nodes. The nodal cannulation success rate using CEUS was 85.7% (12/14 nodes). DCMRL was performed successfully in all patients with good contrast opacification of the central conducting lymphatics. Out of the 14 nodes, 2 nodes in the same patient demonstrated persistent extravasation on CEUS despite needle readjustment, requiring conversion to fluoroscopic lymphangiography. No adverse event related to the injection technique or CEUS was reported. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

This brief report demonstrates the diagnostic utility of cone-beam CT lymphangiography (CBCTL) with intranodal injection of water-soluble iodinated contrast agent for assessing lymphatic disorders in two infants who were contraindicated for MRI and oil-based contrast agent. Both infants had dextro-transposition of the great arteries (d-TGA) and presented with high-output chylothoraces that were recalcitrant to conservative medical therapy. Both infants were diagnosed with central lymphatic flow disorder based on the CBCTL findings. [ABSTRACT FROM AUTHOR]

Congenital heart disease can lead to various lymphatic complications including traumatic leaks, lymphatic overproduction, conduction abnormalities or lymphedema. Advancements in the imaging of central lymphatics and guided interventions have improved outcomes in these children. Dynamic contrast-enhanced magnetic resonance (MR) lymphangiography allows for the assessment of abnormal lymphatic drainage. This technique is preferred for evaluating lymphatic conditions such as plastic bronchitis, chylothorax, chyloptysis, chylopericardium, protein-losing enteropathy and chylous ascites, among other lymphatic disorders. In this review, we discuss lymphatic abnormalities encountered on MRI in children with congenital heart disease. We also briefly review treatment options. [ABSTRACT FROM AUTHOR]

This letter is a response to a previous article on using saline to confirm needle position in dynamic contrast-enhanced MR lymphangiography (DCMRL). The authors agree that saline can be used for this purpose, but argue that contrast-enhanced ultrasound (CEUS) has additional advantages, such as visualizing efferent lymphatic flow and detecting lympho-venous anastomoses. CEUS can also prevent venous contamination by gadolinium-based contrast agent (GBCA) and improve the workflow logistics of DCMRL in children. While there is a lack of large-scale studies comparing CEUS and saline, the authors believe that CEUS offers unique benefits that cannot be achieved with saline alone. [Extracted from the article]

This document is a reply to an article on the feasibility of using contrast-enhanced ultrasound (CEUS) to confirm intranodal needle position for dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) in children. The authors discuss the importance of stable nodal needle position for successful DCMRL examinations and the need for confirmation of adequate needle position prior to the actual MR examination. They mention previous techniques for needle position validation, such as injection of water-soluble radiographic contrast-agent under fluoroscopy, and the use of CEUS in adults. The authors argue that while CEUS may be helpful in some cases, needle position verification by saline solution alone yields a high success rate without the need for additional off-label ultrasound contrast-agent administration in children. They emphasize the need for further studies with larger patient cohorts to reach more meaningful conclusions. [Extracted from the article]

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. [ABSTRACT FROM AUTHOR]

Ultrasound contrast agent (UCA) use in radiology is expanding beyond traditional applications such as evaluation of liver lesions, vesicoureteral reflux and echocardiography. Among emerging techniques, 3-D and 4-D contrast-enhanced ultrasound (CEUS) imaging have demonstrated potential in enhancing the accuracy of voiding urosonography and are ready for wider clinical adoption. US contrast-based lymphatic imaging has been implemented for guiding needle placement in MR lymphangiography in children. In adults, intraoperative CEUS imaging has improved diagnosis and assisted surgical management in tumor resection, and its translation to pediatric brain tumor surgery is imminent. Because of growing interest in precision medicine, targeted US molecular imaging is a topic of active preclinical research and early stage clinical translation. Finally, an exciting new development in the application of UCA is in the field of localized drug delivery and release, with a particular emphasis on treating aggressive brain tumors. Under the appropriate acoustic settings, UCA can reversibly open the blood–brain barrier, allowing drug delivery into the brain. The aim of this article is to review the emerging CEUS applications and provide evidence regarding the feasibility of these applications for clinical implementation. [ABSTRACT FROM AUTHOR]

Chyluria is characterized by chyle in the urinary tract and often presents as milky-white urine. We present a case of chyluria from a lymphatic malformation in a 13-year-old boy diagnosed using dynamic intranodal contrast-enhanced magnetic resonance (MR) lymphangiography. This report demonstrates the utility of intranodal lymphangiography and interstitial lymphatic embolization to treat a pediatric patient presenting with persistent chyluria. Glue migration into the urinary collecting system is a potential complication of this procedure that can be mitigated by adjusting the n-butyl cyanoacrylate dilution with Lipiodol. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Children with Noonan syndrome are known to have increased risk for lymphatic disorders, the extent and nature of which are poorly understood.Objective: Our objective was to describe the imaging findings of the central lymphatic abnormalities in children with Noonan syndrome who underwent central lymphatic imaging.Materials and Methods: We conducted a single-center retrospective review of all children with a confirmed history of Noonan syndrome who presented for lymphatic imaging over a 5-year period. Imaging evaluation was performed on unenhanced T2-weighted (T2-W) imaging, dynamic-contrast MR lymphangiography or conventional lymphangiography. Two readers evaluated the imaging in consensus for the distribution of fluid on T2-W imaging and for lymphatic flow of intranodal contrast agent and thoracic duct abnormalities on dynamic-contrast MR lymphangiography and conventional lymphangiography. We performed a chart review for clinical history and outcomes.Results: We identified a total of 10 children, all but one of whom had congenital heart disease. Presenting symptoms included chylothorax (n=9) and ascites (n=1). Nine had T2-W imaging, seven had dynamic-contrast MR lymphangiography, and seven had conventional lymphangiography. All with T2-W imaging had pleural effusions. On both dynamic-contrast MR lymphangiography and conventional lymphangiography, perfusion to the lung was seen (n=6), with intercostal flow also seen on dynamic-contrast MR lymphangiography (n=6). The thoracic duct was not present in three children and the central thoracic duct was not present in three. A double thoracic duct was seen in two children.Conclusion: Children with Noonan syndrome and clinical evidence of lymphatic dysfunction have central lymphatic abnormalities characterized by retrograde intercostal flow, pulmonary lymphatic perfusion, and thoracic duct abnormalities. [ABSTRACT FROM AUTHOR]

Background: Interventional radiology treatment of chylothorax is well described in adults, with high technical and clinical success that decreases patient morbidity and mortality. However there is limited experience in children.Objective: To report the technical and clinical success of lymphangiography, thoracic duct embolization and thoracic duct disruption in the pediatric population.Materials and Methods: We studied 11 pediatric patients (7 boys, 4 girls; median weight 6.0 kg) who underwent lymphangiography and thoracic duct embolization from November 2015 to May 2017. All 11 (100%) children presented with chylothorax, with 1 (9%) having concomitant chylous ascites and 1 (9%) having concomitant chylopericardium. Ten (91%) children had traumatic chylothorax and one (9%) had congenital chylothorax. We recorded technical success, clinical success and complications.Results: Twelve procedures were completed in 11 children. Bilateral intranodal lymphangiography was technically successful in all (100%) patients. Central lymphatics were visualized in eight (67%) procedures. Access to central lymphatics was attempted in eight procedures and successful in five (63%). In three (37%) of the eight procedures, disruption was performed when the central lymphatics could not be accessed. Clinical success was achieved in 7/11 (64%) children. Three minor complications were reported. No major complications were encountered.Conclusion: Lymphangiography, thoracic duct embolization and thoracic duct disruption are successful interventional strategies in children with chylothorax and should be considered as viable treatment options at any age. [ABSTRACT FROM AUTHOR]

Background: Lymphatic malformation is a common benign mass in children and adults and is representative of a derangement in lymphangiogenesis. These lesions have high recurrence rates and significant morbidity associated with surgery. Several sclerotherapy regimens have been developed clinically to treat lymphatic malformations; however, an animal model has not been developed that is adequate to test the efficacy of image-guided therapeutic interventions.Objective: To develop an animal model suitable for evaluation of percutaneous treatments of lymphatic malformations.Materials and Methods: Male Harlan Sprague-Dawley rats (n = 9) received two US-guided injections of Incomplete Freund's Adjuvant (IFA) over a 2-week period. All nine rats were injected twice into the peritoneum (IP); a subgroup (n = 3) received additional injections into the neck. Three animals that received IP injections of saline were used as controls. The injection sites were monitored for the development of lesions by high-resolution ultrasonography at 2-week intervals for 100 days. High-resolution (4.7 Tesla) magnetic resonance imaging was then performed on two animals noted to have developed masses. The rats were sacrificed and histologic examination of the identified lesions was performed, including immunohistochemical staining for vascular (CD31) and lymphatic (Flt-4 and Prox-1) endothelium.Results: All animals injected with IFA developed cystic lesions. The three animals injected at dual sites were noted to have both microcystic and macrocystic malformations in the neck and microcystic plaque-like lesions in the peritoneum. The macrocystic malformations (> or =5 mm) in the neck were detected by ultrasonography and grossly later during necropsy. Histopathologic analysis revealed the cystic spaces to be lined by lymphatic endothelium supported by a connective tissue stroma. Control animals did not exhibit detectable lesions with either ultrasonography or necropsy.Conclusion: This model represents a promising tool for translational development of image-guided interventions for lymphatic malformations. It may also serve as a model for the study of lymphangiogenesis and the development of anti-lymphangiogenic therapies. [ABSTRACT FROM AUTHOR]