Objectives and Study: Protein losing enteropathy (PLE) is a rare complication following paediatric liver transplantation (LTx), mostly related to venous outflow obstruction of the liver. Here, we discuss diagnosing a thus far unknown cause of PLE following paediatric LTx and its surgical treatment. Method: Case-study Results: A boy received an LTx (segments 2 and 3, postmortal heart-beating donor, Roux-en-Y hepaticojejunostomy) at the age of 7 months because of ornithine transcarbamylase deficiency. At the age of 18 months, he developed generalized edema and ascites. PLE was diagnosed, as faecal alpha-1 antitrypsin levels were markedly increased. Clinically, he required suppletion of albumin intravenously every two weeks, for which a venous access port was implanted. Routine diagnostic work-up for PLE was negative. No infectious cause of PLE was found. Endoscopy (gastroduodeno-/ colonoscopy, capsule endoscopy) showed no mucosal injuries and no signs of lymphangiectasia. PET/CT scan showed no signs of post-transplant lymphoproliferative disorder. Vascular origin, most notably venous outflow obstruction, is a known cause of PLE following LTx. Therefore catheterisation was performed, which showed no signs of venous outflow obstruction (normal central venous pressure of 9 mm Hg in the inferior vena cava and normal pressure in the hepatic vein of 9 mmHg), and only a slightly increased wedge pressure in the hepatic vein (19 mmHg). A liver biopsy taken during the same procedure showed no relevant pathology. To find the localization of protein loss, an albumin scan (technetium-99m labeled albumin) was performed, which confirmed intestinal albumin loss. Of note, early recordings (dynamic 0-30 min and early static SPECT-CT recordings at 30, 60, 90 minutes after tracer injection instead of after 120 minutes) were done, to detect where albumin entered the intestine. The affected area on the albumin scan was where the Roux loop was expected. With regard to the albumin loss in the Roux loop, local bacterial overgrowth or local lymphangiectasia, possibly due to (venous) congestion, were considered. Treatment with Metronidazole did not improve albumin loss, rendering local bacterial overgrowth an unlikely cause of the PLE. Therefore a surgical revision of the Roux loop was performed. During the procedure, no torsion of the Roux loop was observed. The explanted loop macroscopically showed a small abnormal area with a thin hyperaemic mucosa (Figure 1A). Histological analysis showed focal lymphangiectasia (Figure 1B), which was considered the site of protein loss. Following surgical revision, PLE disappeared and serum albumin levels remained stable, up to now (6 months post-revision, Figure 1C). We suspect that the local PLE in the Roux loop arose from local congestion of lymphatic outflow. Conclusion: Here, we report diagnosing a thus far unknown cause of PLE following LTx in a child. The Roux loop was found to be the site of albumin loss using early SPECT-CT recordings during an albumin scan. Notably, this was in the absence of relevant portal hypertension. Surgical revision of the Roux loop has stopped the PLE up to now, 6 months post-revision (Figure presented).
|Status||Published - 1-apr-2018|