Purpose or Case Report: Protein- losing enteropathy (PLE) is a serious complication that can occur after the Fontan operation. Increased systemic venous pressure can cause altered mesenteric circulation, lymphangiectasis, and enteric protein loss. PLE can cause significant morbidity with hypoalbuminemia, coagulation abnormalities and hypogammaglobulinemia. Dynamic contrast- enhanced (DCE) MR lymphangiography (MRL) is a minimally invasive technique that has emerged as a valuable tool for imaging lymphatic flow and lymphatic disorders. DCE MRL can aid in the localization of lymphatic leak in the intestinal lumen. The purpose of this presentation is to review the technique of DCE MRL for evaluation of protein- losing enteropathy along with the imaging findings.
We present a 12 year old female with congenital heart disease including single ventricle, heterotaxy with dextrocardia. She had a bilateral bidirectional Glenn procedure complicated by prolonged chylous effusion requiring thoracic duct ligation and pleurodesis. She underwent non-fenestrated extracardiac Fontan which was complicated by protein-losing enteropathy. Lymphatic embolization was done however she developed recurrent PLE. DCE MRL demonstrated leakage of contrast in the duodenum and proximal jejunum. She underwent thoracic duct decompression procedure which resulted in significantly improved symptoms.
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Conclusions: Assessment of lymphatic transit and identification of site of lymphatic leak in cases of protein-losing enteropathy by DCE MRL provides useful information for treatment planning and localized intervention.