Purpose or Case Report: Protein-losing enteropathy (PLE) a highly morbid disease characterized by loss of serum proteins into the bowel. The etiology of idiopathic PLE has been poorly understood and no therapy has been available. We present a case series of 3 patients with idiopathic PLE successfully diagnosed using a combination of Dynamic Contrast Enhanced MR (DCMRL) hepatic, mesenteric, lymphangiography and treated with retrograde endoscopic glue embolization.
Methods & Materials: 3 patients (average 14.3 yrs, F:M/1:2) with idiopathic PLE underwent DCMRL, hepatic and mesenteric lymphangiography, followed by the transendoscopic lymphatic embolization. Patient demographics, pre-procedure albumin, symptoms, imaging findings, procedural details, and follow up information were collected.
Results: DCMRL demonstrated absence of the thoracic duct in one patient and ectatic thoracic duct in two patients as well as leakage of the contrast into intestinal lumen. This leakage was confirmed with injection of methylene blue dye into the mesenteric and liver lymphatics, with endoscopy. Retrograde endoscopic embolization of the leaking sites was performed via 25G endoscopic needle, twice in two patients and once in one patient. All three patient's serum albumin normalized with resolution of the symptom. There were no complications. Mean follow up time was 23.1 months (38.6, 28.3, 2.3 months respectively).
Conclusions: In this study we demonstrated the obstruction of the thoracic duct with reflux of the lymph into the duodenal lumen as a cause of PLE. Retrograde endoscopic embolization of the leaking ducts in duodenum resulted in symptomatic and laboratory resolution of PLE.