Imaging of Lymphatic Anomalies

NCT02744027 | Completed

• 1 other identifier: 15-012331
• Study Type: interventional
• Target: 10
• Locations: 1 country
• Sites: 1

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.

Conditions

Gorham Disease; Lymphatic Diseases; Lymphangiomatosis

Keywords

Generalized Lymphatic Anomaly; Kaposiform Lymphangiomatosis; Dynamic Contrast Enhanced Magnetic Resonance Lymphangiogram; Magnetic Resonance Imaging

Outcome Measures

Primary Outcomes (1)

• Describe lymphatic anatomy of participants with lymphatic anomalies (LA)

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

  2 Days

Secondary Outcomes (2)

• Development of a classification system for lymphatic flow

  2 days

• Description of Lymphatic malformations in participants with lymphatic anomalies

  2 days

Study Arms (1)

Dynamic Contrast Enhanced Magnetic Contrast Imaging

OTHER

Dynamic Contrast Enhanced Magnetic Resonance (MR) Lymphangiogram and heavy T2 Magnetic Resonance imaging data will be evaluated for abnormal lymphatic perfusion of the lung parenchyma. Abdominal and thoracic lymphatic malformations will be characterized by location, number, size, relationship to other organs and perfusion patterns in order to create a basis of imaging classification of lymphatic abnormalities (LA). Subjects will undergo both Dynamic Contrast Enhanced Magnetic Resonance Lymphangiogram (DCMRL) and Heavy Weighted T2 Imaging.

Other: Dynamic Contrast Enhanced Magnetic Resonance Lymphangiography (DCMRL); Other: Heavy Weighted T2 Imaging (T2W); Other: Lymph Node Access

Interventions

Dynamic Contrast Enhanced Magnetic Resonance Lymphangiography (DCMRL) OTHER

Following completion of the T2 lymphatic mapping, contrast agent administered will be calculated based on weight. Investigators will use dosage of 0.1cc/kg of contrast which will be injected by hand simultaneously into each lymphatic node (LN) at a rate of 0.5-1 cc per min. One minute after the injection, scanning will be initiated using high spatial and temporal resolution magnetic resonance angiography (syngo time-resolved angiography with stochastic trajectories \[TWIST\]) sequence. The sequence parameters will be adjusted with a time delay such that a complete volume will be acquired approximately every 20-60 seconds. This will be followed by additional scans with a high-resolution navigator gated 3-dimensional flash inversion recovery (IR) sequence. In all subjects the scan area covered the neck, chest, and abdomen as caudal as feasible.

Dynamic Contrast Enhanced Magnetic Contrast Imaging

Heavy Weighted T2 Imaging (T2W) OTHER

Magnetic Resonance imaging (MRI) will be performed and T2W MRI lymphatic mapping will be performed using a respiratory navigated and cardiac gated 3 dimensional turbo spin echo sequence with pre-set parameters. Scan time varies from 2-5 min depending upon the size of the subject.

Dynamic Contrast Enhanced Magnetic Contrast Imaging

Lymph Node Access OTHER

The procedure will be performed under sedation and anesthesia if participants are aged 16 or under. The procedure will be performed with local anesthesia for participants aged over 16 years old. Using ultrasound guidance two inguinal lymph nodes (one each side) will be directly accessed with 25-gauge spinal needles attached to a short connector tubing. A shallow angle for puncture will be used to create a long subcutaneous tract to assist in stabilizing the needle. A small amount of an oil-based contrast or water soluble contrast will be injected under fluoroscopy guidance to confirm the correct position of the needles inside the lymph nodes. After stabilizing the needle, the subjects will be transferred into the MRI suite.

Dynamic Contrast Enhanced Magnetic Contrast Imaging

Eligibility Criteria

• Age: 2 Years - 80 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• Subjects diagnosed with Lymphatic Anomalies (GLA, KL, Gorham disease) with radiological and or pathological confirmation of lung involvement will be considered for the study.
• Girls \> 11 y.o. with a negative urine pregnancy test and contraception use.
• Registration in the International Lymphangiomatosis and Gorham's Disease Alliance (LGDA) Patient Registry or referral by a physician.

You may not qualify if:

• Subjects with contraindications to contrast enhanced MRI: allergy to gadolinium imaging agents, impaired renal function (GFR\<30) and presence of paramagnetic objects.
• Claustrophobia or contraindications to sedation/anesthesia
• Uncorrectable coagulopathy (bleeding disorders).
• Pregnant or lactating females.

Sponsors & Collaborators

• Children's Hospital of Philadelphia: lead
• University of Pennsylvania: collaborator

Study Sites (1)

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

Location

Related Publications (1)

• Itkin M, Rabinowitz DA, Nadolski G, Stafler P, Mascarenhas L, Adams D. Abnormal Pulmonary Lymphatic Flow in Patients With Lymphatic Anomalies and Respiratory Compromise. Chest. 2020 Aug;158(2):681-691. doi: 10.1016/j.chest.2020.02.058. Epub 2020 Mar 24.

  PMID: 32220591 DERIVED

MeSH Terms

Conditions

Osteolysis, Essential; Lymphatic Diseases

Condition Hierarchy (Ancestors)

Bone Diseases, Developmental; Bone Diseases; Musculoskeletal Diseases; Osteolysis; Bone Resorption; Hemic and Lymphatic Diseases

Study Officials

• Yoav Dori, MD, PhD

  Children's Hospital of Philadelphia

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: DIAGNOSTIC
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: April 11, 2016
• First Posted: April 20, 2016
• Study Start: April 1, 2016
• Primary Completion: December 1, 2017
• Study Completion: December 1, 2017
• Last Updated: March 21, 2018

Record last verified: 2018-03

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)