Liver Lymphatic Embolization as a Treatment of PLE

Study Questions:

What is the role of lymphangiographic imaging and liver lymphatic embolization as a treatment for protein-losing enteropathy (PLE)?

Methods:

The investigators conducted a single-center, retrospective review of imaging and interventions used in eight consecutive patients with liver lymphatic embolization and congenital heart disease (CHD) with elevated central venous pressure (CVP) complicated by PLE.

Results:

Liver lymphangiography was performed in eight patients (five males, three females; median age 21 years), seven of whom demonstrated leakage of liver lymph into the duodenum through abnormal hepatoduodenal lymphatic communications. This was confirmed by duodenoscopy with simultaneous injection of isosulfan blue dye into the liver lymphatics in six of seven patients. Liver lymphatic embolization with ethiodized oil in two patients resulted in a temporary increase in albumin blood level and symptom improvement in one patient, but was complicated by duodenal bleeding in both patients. Of the remaining six patients, liver lymphatic embolization with n-butyl cyanoacrylate glue resulted in sustained improvement of the serum albumin level and symptoms in three patients, temporary improvement in two patients, and no change in one patient with median follow-up of 135 days (range 84-1,005 days).

Conclusions:

The authors concluded that liver lymph leakage is a cause of PLE in patients with CHD and elevated CVP, and lymphatic embolization led to improved albumin levels and relief of symptoms.

Perspective:

This study reports that the mechanism of albumin loss in CHD patients with elevated CVP and PLE is leakage of this albumin-rich lymph into the duodenal lumen, predominantly through focal hepatoduodenal lymphatic channels. Glue embolization of hepatoduodenal connections to prevent leakage of the hepatic lymph into duodenum resulted in a rapid increase in serum albumin levels and improvement in symptoms in a majority of the patients. Additional studies are indicated to determine the long-term outcome and potential for sustained efficacy of liver lymphatic duct embolization procedures, as well as optimization of techniques for closure of abnormal hepatoduodenal connections that will provide longer-term resolution of PLE.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Dyslipidemia, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Congenital Heart Disease, CHD & Pediatrics and Arrhythmias, CHD & Pediatrics and Imaging, CHD & Pediatrics and Interventions, CHD & Pediatrics and Prevention, Lipid Metabolism, Interventions and Imaging, Interventions and Structural Heart Disease, Angiography, Nuclear Imaging

Keywords: Angiography, Central Venous Pressure, Duodenoscopy, Duodenum, Embolization, Therapeutic, Ethiodized Oil, Heart Defects, Congenital, Lymphatic Vessels, Lymphography, Protein-Losing Enteropathies, Primary Prevention, Serum Albumin


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