Search

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]

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]

The purpose of this study was to assess the safety and indications for cardiac magnetic resonance (CMR) with myocardial perfusion imaging (MPI) in a cohort of children and young adults. A retrospective review of 178 children and young adults who underwent CMR with MPI was performed. Studies were categorized based on study protocols as MPI with resting perfusion only, adenosine stress MPI, exercise-induced stress MPI, and MPI for cardiac mass diagnosis. Relevant clinical history, exam indications, and adverse reactions following gadolinium-based contrast agent and adenosine administration were recorded. Studies were reviewed for the presence of myocardial perfusion defects, wall motion abnormalities, and delayed myocardial enhancement. The most common indications from MPI were congenital heart disease (CHD), Kawasaki disease, anomalous coronary artery, or myocardial mass characterization. Of these, 51% were protocoled with adenosine stress, 23% without stress, 6% with exercise stress, and 20% for cardiac mass evaluation. Excluding patients for myocardial mass evaluation, MPI defects were present in 16% (14 with adenosine stress, 1 with exercise stress, 8 on resting studies only). For cardiac mass evaluation, a mass was confirmed in 58%. No adverse reactions occurred with intravenous administration of a gadolinium-based contrast agent. Three self-limited adverse reactions, 2 patients with chest pain, and 1 patient with bradycardia, occurred following adenosine administration. MPI is a safe modality for the evaluation of pediatric and young adults with minimal adverse events. The most common indications for MPI were for the evaluation of CHD, Kawasaki disease, anomalous coronary artery, or myocardial mass characterization. [ABSTRACT FROM AUTHOR]

Although subpleural cysts are known to be associated with Down syndrome, their etiology and prevalence remains unknown. To determine the prevalence of subpleural cysts in children with Down syndrome and the association with prematurity, congenital heart disease (CHD), extracorporeal membrane oxygenation (ECMO), and chronic ventilator support. A review of the CT examinations of 25 children with Down syndrome was performed to determine the presence, location, and distribution of cysts along with associated abnormalities. Charts were reviewed and coexistent diagnoses and past treatments were recorded. The prevalence of subpleural cysts was 36% with no significant association with CHD, ECMO, or chronic ventilator support. An association was found in the two children with a history of prematurity. The cysts were most commonly found in the anteromedial portion of the lung. Subpleural cysts are common in Down syndrome and should not be confused with another pathological process. An association with prematurity was found, but the low number of children in this study makes the connection uncertain. The etiology remains unclear, but it has been hypothesized that the cysts are associated with lung hypoplasia. [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) is a routinely used imaging modality for the diagnosis and treatment planning of many health conditions in children and adults. Yet, its use has been limited in many institutions for patients with cardiac implantable electronic devices (CIEDs) due to safety concerns. Current evidence relates primarily to devices with transvenous leads. However, patients with complex cardiac anatomy and palliative surgery procedures often require epicardial pacemakers. To date, very few studies have addressed MRI safety considerations with non-conditional CIEDs or abandoned epicardial leads in infants, and to our knowledge, this is the first report that shows Fontan palliation patients who underwent Dynamic Contrast enhanced MR Lymphangiography (DCMRL) with these types of devices. We present our institutional experience with five cases where a DCMRL was safely performed in three children and two adults with Fontan palliation to evaluate their lymphatic anatomy and guide interventional procedures. Regarding our brief experience, we concluded that DCMRL may be considered in post-Fontan patients with non-conditional CIEDs, including epicardial leads, seeking the best diagnostic and treatment options available. Institutional protocols must be revised in advance to perform this technique in a controlled setting. • Patients with MR non-conditional cardiac implantable electronic devices (CIEDs) are still restricted from the use of MRI due to safety concerns. • Fontan patients have a complex anatomy that usually comes with abnormal lymphatic drainage. • The recent utilization of Dynamic Contrast enhanced MR Lymphangiography (DCMRL) in Fontan patients has improved their prognosis by allowing the visualization and treatment of their lymphatic leakages. • DCMRL may be considered in post-Fontan patients with non-conditional CIEDs, including epicardial leads, seeking the best diagnostic and treatment options available. [ABSTRACT FROM AUTHOR]

Introduction: In patients with congenital heart disease (CHD), transplant-free survival after a diagnosis of plastic bronchitis (PB) is poor (~ 70% at 2 years). Percutaneous lymphatic intervention (LI) can decrease cast production, but little is known about intermediate-term survival. Methods: This is a single center, retrospective review of LI in subjects with CHD and PB. Intermediate-term outcomes and quality of life (QOL) data were obtained by chart review and telephone survey. Results: From 8/2013 to 2/2018, 70 subjects underwent initial LI (e.g. thoracic duct embolization). Of those, 11 (16%) had repeat LI (Table 1). There were 3 deaths; 2 after the index procedure (aspiration event; multisystem organ failure) and 1 after repeat LI (pulmonary hemorrhage). Transplant-free survival was 89% at 19 months and 82% at 2 years (Figure 1). Sufficient follow-up data were available for 63 subjects (90%). At median follow-up of 19.0 months (IQR 8.2-30.4), 81% were cast-free. After LI, daily or weekly cast production decreased from 37% and 44% to 5% and 6%, respectively (p < 0.0001); respiratory support also decreased (p = 0.0016). There were 5 (7%) new diagnoses of protein losing enteropathy (PLE) in subjects without PLE history and 4 have since resolved. Survey results were available for 52 subjects (74%). Most parents reported improvement in their child's health (83%), QOL (87%), and physical activity (60%). Conclusion: At intermediate follow-up, percutaneous LI for PB resulted in (1) improved transplant-free survival (82% at 2 years after LI), (2) significant decrease in cast production (81% cast-free), and (3) improved QOL in 87%. Thus, LI is an effective treatment for PB in CHD patients. [ABSTRACT FROM AUTHOR]

Introduction: Percutaneous lymphatic interventions (LI) target the pulmonary lymphatic abnormalities seen in patients with plastic bronchitis (PB). We sought to characterize short-term outcomes after LI in patients with congenital heart disease (CHD) and PB. Methods: This is a single center, retrospective review of LI in subjects with CHD and PB. Medical records were abstracted for short-term outcomes and complication data up to 2 weeks after discharge. Results: From 8/2013 to 2/2018, 70 subjects underwent LI at a median age of 8.8 years (IQR 5.4-12.0). The majority (86%) had Fontan circulation. History of chylothorax or protein losing enteropathy was present in 44% and 17% of subjects, respectively. By imaging, there was abnormal lymphatic perfusion to both lungs in 52 subjects (84%). Prior to LI, central venous pressure was a median of 15 mmHg (IQR 13-17). LI involved complete embolization of the thoracic duct in 43 (61%), selective embolization of lymphatic channels in 23 (33%), and other techniques in 4 (6%). A majority of the subjects (93%) were extubated on the day of the procedure (Table 1). The median intensive care and hospital lengths of stay were 2 days (IQR 1-4) and 6 days (IQR 4-8), respectively. Repeat LI prior to discharge was rare (1%). Common complications included transient abdominal pain (77%), any hypotension (60%), and systemic inflammatory response syndrome (21%). Major adverse events (defined as stroke, cardiac arrest, or death) occurred in 4 subjects (6%). In the two fatalities, one had acute cardiac arrest after an aspiration event and one had multi-organ system failure. Conclusions: Percutaneous LI was well tolerated in a majority of subjects with CHD and PB, which demonstrates both the feasibility and safety of this procedure. While major adverse events were uncommon, they included embolic stroke, cardiac arrest, and death. Future studies will focus on assessing patient and procedural risk factors associated with adverse short-term outcomes. [ABSTRACT FROM AUTHOR]