RESUMO
Percutaneous transhepatic lymphatic embolization (PTLE) and peroral esophagogastroduodenoscopy (EGD) duodenal mucosal radiofrequency ablation (RFA) were performed to manage protein-losing enteropathy (PLE) in patients with congenital heart disease (CHD). Five procedures were performed in 4 patients (M/F = 3/1, median age: 49 years [range 31-71 years]). Transhepatic lymphangiography demonstrated abnormal peri-duodenal lymphatic channels. After methylene blue injection through transhepatic access, subsequent EGD evaluation showed methylene blue extravasation at various sites in the duodenal mucosa. Endoscopic RFA of the leakage sites followed by PTLE using 3:1 ethiodized oil to n-butyl cyanoacrylate glue resulted in improved symptoms and serum albumin (pre-procedure: 2.6 g/dL ± 0.2; post-procedure: 3.5 g/dL ± 0.4, p=0.004) over a median follow-up of 16 months (range 5-20). Transhepatic lymphangiography and methylene blue injection with EGD evaluation of the duodenal mucosa can help diagnose PLE. Combined PTLE and EGD-RFA can be considered to treat patients with PLE.