Clinical Trial: Imaging of Lymphatic Anomalies

Study Status: Enrolling by invitation
Recruit Status: Enrolling by invitation
Study Type: Interventional

Official Title: Dynamic Contrast Enhanced MR Lymphangiogram Imaging of Lymphatic Anomalies (LA)

Brief Summary: Lymphatic Anomalies (LA) is characterized by proliferation of lymphatic tissue causing deterioration of pulmonary function. Understanding changes in lymphatic anatomy in these patients is hindered by the difficulty of imaging the lymphatic system. Dynamic Contrast Enhanced MR Lymphangiogram (DCMRL) may be useful in investigating pathological changes in the lymphatic system.

Detailed Summary:

Lymphatic anomalies (LA) are a spectrum of rare diseases classified into lymphatic tumors and malformations. Complicated LA cases in the past have been called lymphangiomatosis because of varied disseminated involvement including soft tissue and viscera such as the spleen, liver and bone. Pleural effusions and pericardial effusions are often associated with these lesions. These are now classified into different phenotypes such as Generalized Lymphatic Anomaly (GLA), Gorham's Stout Disease (GSD) and Kaposiform Lymphangiomatosis (KLA). These complicated phenotypes can cause massive osteolysis causing a morbidity and mortality from infection or paralysis or worsening pulmonary function and effusions (GSD, GLA, KLA).

The major cause of mortality and morbidity in these patients is the deterioration of pulmonary function by chronic chylous effusions and progressive interstitial lung disease. Unfortunately, little is known about biomarkers, risk stratification or the pathophysiology of this progression. The understanding of changes in patients' lymphatic anatomy with LA is hindered by the difficulty of imaging the lymphatic system. Dynamic Contrast Enhanced MR Lymphangiogram (DCMRL) is a technique that has recently been developed, allowing dynamic MR imaging of the lymphatic system by injecting gadolinium contrast agent in the groin lymph nodes.

This technique has been previously used to identify pathological lymphatic perfusion of the lung parenchyma in patients with plastic bronchitis and neonatal chylothorax. Based on these imaging findings, a treatment algorithm has been designed and used to successfully treat the majority of those patients with these conditions.


Sponsor: Children's Hospital of Philadelphia

Current Primary Outcome: Describe lymphatic anatomy of participants with lymphatic anomalies (LA) [ Time Frame: 2 Days ]

The primary endpoint will be the description of lymphatic anatomy assessing if participants have a single or branched Thoracic Duct.


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Development of a classification system for lymphatic flow [ Time Frame: 2 days ]
    Development of a classification system based on retrograde or anterograde flow of lymphatic fluid.
  • Description of Lymphatic malformations in participants with lymphatic anomalies [ Time Frame: 2 days ]
    Assessing if lymphatic malformation are present or absent in participants with lymphatic anomalies.


Original Secondary Outcome: Same as current

Information By: Children's Hospital of Philadelphia

Dates:
Date Received: April 11, 2016
Date Started: April 2016
Date Completion: October 2017
Last Updated: May 4, 2017
Last Verified: May 2017