Extensive Intestinal Lymphangiectasia Results in Hemorrhagic Duodeno-Jejunitis

Abstract

Intestinal lymphangiectasia is a rare disorder in which the lymph vessels that supply the lining of the small intestine are occluded. When this occurs, malabsorption ensues from a protein-losing enteropathy. To date, only a few cases of small bowel bleeding have been linked to intestinal lymphangiectasia. We highlight a case of intestinal lymphangiectasia causing both hemorrhagic duodeno-jejunitis and refractory blood loss anemia. A 68-year-old male with a history of hypothyroidism and dyslipidemia was referred to our institution for melena. Previous upper endoscopy (EGD) revealed hemorrhagic duodenitis, and a colonoscopy was unremarkable. Patient was requiring blood transfusions every 3 days for a total of 36 units of blood. His serum albumin was 1.5 g/dL. On computed tomography angiography of the abdomen/pelvis, the celiac trunk was severely stenosed with a hook-like appearance, suggestive of median arcuate ligament syndrome (MALS). Exploratory laparotomy was done to evaluate for MALS, but there were findings of extensive lymphatic vessels. An abdominal angiogram showed good perfusion to the stomach, celiac trunk, and duodenum, and a lymphangiogram of the abdomen/pelvis ruled out lymphatic blockage. A follow-up EGD with push enteroscopy revealed congestive duodenojejunopathy with numerous lymphangiectasia which appeared to be the source of bleeding. As the patient remained transfusion-dependent, he was scheduled for small bowel resection of the involved segment assisted by intraoperative enteroscopy. Intestinal lymphangiectasia is a rare disease characterized by focal or diffuse dilated enteric lymphatics. This case portrays a potential pathologic link between protein-losing enteropathy and refractory gastrointestinal bleeding. Although there is paucity in literature discussing this entity, it is hypothesized that increased pressure within the lymphatic system can cause venous congestion, and therefore gastrointestinal bleeding. In our case, we attempted to hold enteral nutrition and added octreotide to decrease lymphatic pressure and subsequently control his bleeding. Unfortunately, although his bleeding frequency decreased, it did not stop completely. Our case is unique as it highlights a rare entity that can result in a disabling, life-incompatible outcome. It also emphasized the need to better understand the underlying pathophysiology to effectively treat this condition. Regardless, a multidisciplinary approach proved to be beneficial in management.