Imaging the Central Conducting Lymphatics: Initial Experience with Dynamic MR Lymphangiography

Krishnamurthy, Rajesh; Hernandez, Alberto D.; Kavuk, Serife; Annam, Aparna; Pimpalwar, Sheena

doi:10.1148/radiol.14131399

Cited by 109 publications

(102 citation statements)

References 7 publications

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“…Clinically differentiating CCLA from GLA is often difficult. Dynamic contrast MR lymphangiography can be used to assess for obstruction, degree of collateralization, and chylolymphatic reflux but this imaging technology, along with providers who have expertise in this technique, is limited to only a few centers in the country . Because the time period of this study occurred prior to the recognition of CCLA as a distinct diagnosis, there is the possibility of unintentional inclusion of CCLA patients in this analysis.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of systemic sirolimus in the treatment of generalized lymphatic anomaly and Gorham–Stout disease

Ricci

Hammill

Mobberley‐Schuman

et al. 2019

Pediatric Blood & Cancer

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Background Generalized lymphatic anomaly (GLA) and Gorham–Stout disease (GSD) are rare complicated lymphatic malformations that occur in multiple body sites and are associated with significant morbidity and mortality. Treatment options have been limited, and conventional medical therapies have been generally ineffective. Emerging data suggest a role for sirolimus as a treatment option for complex lymphatic anomalies. Procedure Disease response was evaluated by radiologic imaging, quality of life (QOL), and clinical status assessments in children and young adults with GLA and GSD from a multicenter systematic retrospective review of patients treated with oral sirolimus and the prospective phase 2 clinical trial assessing the efficacy and safety of sirolimus in complicated vascular anomalies (NCT00975819). Sirolimus dosing regimens and toxicities were also assessed. Results Eighteen children and young adults with GLA (n = 13) or GSD (n = 5) received oral sirolimus. Fifteen patients (83%) had improvement in one or more aspects of their disease (QOL 78%, clinical status 72%, imaging 28%). No patients with bone involvement had progression of bone disease, and the majority had symptom or functional improvement on sirolimus. Improvement of pleural and pericardial effusion(s) occurred in 72% and 50% of affected patients; no effusions worsened on treatment. Conclusions Sirolimus appears effective at stabilizing or reducing signs/symptoms of disease in patients with GLA and GSD. Functional impairment and/or QOL improved in the majority of individuals with GLA and GSD with sirolimus treatment.

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Section: Discussionmentioning

confidence: 99%

Efficacy of systemic sirolimus in the treatment of generalized lymphatic anomaly and Gorham–Stout disease

Ricci

Hammill

Mobberley‐Schuman

et al. 2019

Pediatric Blood & Cancer

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“…7–10 Only a few studies have investigated the central lymphatic channel. 11,12 However, in fact, lymphatic circulation disorders that require prompt management or surgical correction may be caused by central lymphatic vessel problems. Therefore, critical cases provide an important opportunity to advance the understanding of lymphatic disorders.…”

Section: Discussionmentioning

confidence: 99%

Anatomic and Functional Evaluation of Central Lymphatics With Noninvasive Magnetic Resonance Lymphangiography

et al. 2016

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Accurate assessment of the lymphatic system has been limited due to the lack of optimal diagnostic methods. Recently, we adopted noncontrast magnetic resonance (MR) lymphangiography to evaluate the central lymphatic channel. We aimed to investigate the feasibility and the clinical usefulness of noninvasive MR lymphangiography for determining lymphatic disease.Ten patients (age range 42–72 years) with suspected chylothorax (n = 7) or lymphangioma (n = 3) who underwent MR lymphangiography were included in this prospective study. The thoracic duct was evaluated using coronal and axial images of heavily T2-weighted sequences, and reconstructed maximum intensity projection. Two radiologists documented visualization of the thoracic duct from the level of the diaphragm to the thoracic duct outlet, and also an area of dispersion around the chyloma or direct continuity between the thoracic duct and mediastinal cystic mass.The entire thoracic duct was successfully delineated in all patients. Lymphangiographic findings played a critical role in identifying leakage sites in cases of postoperative chylothorax, and contributed to differential diagnosis and confirmation of continuity with the thoracic duct in cases of lymphangioma, and also in diagnosing Gorham disease, which is a rare disorder. In patients who underwent surgery, intraoperative findings were matched with lymphangiographic imaging findings.Nonenhanced MR lymphangiography is a safe and effective method for imaging the central lymphatic system, and can contribute to differential diagnosis and appropriate preoperative evaluation of pathologic lymphatic problems.

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“…16 This need for preintervention assessment of the lymphatic system has led to the development of MR lymphangiography techniques to classify the etiology of the chylothorax as well as the site of the leak. [6][7][8] As stated above, several disorders that originate as chylous ascites may present as chylothorax. Thus, the first step in our diagnostic algorithm for patients with nontraumatic chylothorax is to evaluate for chylous ascites (Fig.…”

Section: Managementmentioning

confidence: 96%

“…The potential for nontraumatic chylothorax to originate from a retroperitoneal leak is rationale for using diagnostic imaging with dynamic contrast enhanced magnetic resonance (MR) lymphangiography (DCMRL), which would be discussed in detail in a separate article in this edition. [6][7][8] Another group of conditions, which may present with nontraumatic chylothorax where preintervention imaging is critical to confirming the diagnosis and developing a treatment strategy, are lymphatic conduction disorders. The etiology of these conditions is not understood but they may result primarily from abnormal formation of lymphatic ducts or their valves or may occur secondarily from increased lymphatic volume or flow in the setting of heart failure or cirrhosis.…”

mentioning

confidence: 99%

Nontraumatic Chylothorax: Diagnostic Algorithm and Treatment Options

Nadolski

2016

Techniques in Vascular and Interventional Radiology

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Imaging the Central Conducting Lymphatics: Initial Experience with Dynamic MR Lymphangiography

Abstract: The dynamic MR lymphangiographic technique with intranodal injection of gadolinium-based contrast material is feasible and can provide useful information in a variety of lymphatic flow abnormalities involving the CCL.

Cited by 109 publications

References 7 publications

Efficacy of systemic sirolimus in the treatment of generalized lymphatic anomaly and Gorham–Stout disease

Efficacy of systemic sirolimus in the treatment of generalized lymphatic anomaly and Gorham–Stout disease

Anatomic and Functional Evaluation of Central Lymphatics With Noninvasive Magnetic Resonance Lymphangiography

Nontraumatic Chylothorax: Diagnostic Algorithm and Treatment Options

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