ARRS 2022 Abstracts

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2082. Etiology and Characterization of Thoracic Duct Obstruction in Patients with Lymphatic Flow Disorders
Authors * Denotes Presenting Author
  1. Abhay Srinivasan *; Children's Hospital of Philadelphia; Perelman School of Medicine at the University of Pennsylvania
  2. Christopher Smith; Children's Hospital of Philadelphia; Perelman School of Medicine at the University of Pennsylvania
  3. Fernando Escobar; Children's Hospital of Philadelphia; Perelman School of Medicine at the University of Pennsylvania
  4. Yoav Dori; Children's Hospital of Philadelphia; Perelman School of Medicine at the University of Pennsylvania
  5. Ganesh Krishnamurthy; Children's Hospital of Philadelphia; Perelman School of Medicine at the University of Pennsylvania
Objective:
We seek to characterize thoracic duct (TD) obstruction found in patients with lymphatic flow disorders, with focus on etiology and analysis of pressure gradients (PGs) measured between the thoracic central venous system and the thoracic duct.

Materials and Methods:
We conducted a retrospective review of patients with lymphatic flow disorders who underwent direct lymphangiography and/or lymphatic embolization. Patient demographic, clinical, direct lymphangiography, and intra-procedural data were analyzed.

Results:
The study included 11 patients with median age of 10.2 years (range 1.9 - 25 y) and Female:Male ratio of 6:5. Clinical presentation included six (55%) patients with chylothorax and five (45%) patients with protein-losing enteropathy; 8/11 (72%) patients had congenital heart disease. Etiology of TD obstruction was TD outlet stenosis in five(45%) patients, TD outlet obstruction in two (18%) patients, compression of the TD by the aorta in two (18%) patients, lymphatic malformation compressing the duct in one (9%) patient, and attempted TD ligation leading to TD stenosis in one (9%) patient. TD pressures were measured in 10/11 (91%) patients, with a median TD pressure of 15.5 mm Hg (range 6 - 34 mm Hg) and a median PG between the duct and the venous system of 6 mm Hg (2 - 17 mm Hg). Nine patients underwent lymphatic intervention, with eight patients undergoing procedures to specifically alleviate the TD obstruction, including balloon dilation in 7/8 (88%) and mass drainage with sclerotherapy in 1/8 (12%). In these patients, pre-procedure median PG was 6 mm Hg (range 2 - 17 mm Hg) with post-procedure median PG of 0.5 mm Hg (range 0 - 5 mm Hg) (p=0.007). Two of 11 (18%) patients did not undergo intervention and had no change in symptoms. In 7/9 (78%) of the patients who underwent an intervention, there was complete resolution of symptoms, with transient improvement in symptoms in 1/9 patient (11%), and no change in 1/9 (11%) patient.

Conclusion:
We describe our experience with characterization of TD obstruction in patients with lymphatic flow disorders. Obstruction of the TD may be a significant contributor to the pathophysiology of these disorders. Thorough assessment of TD flow dynamics is important in the management of these patients, and PG measurement may be a useful technique in this evaluation.