Generalized Lymphangiectasia in a Patient Presenting With Overt Obscure Gastrointestinal Bleeding: 1094

Maen Kamal,Jason Mader,Rezwan Ahmed, Muralikrishna Mukkamala, Teresa Limjoco,Yaser Rayyan

AMERICAN JOURNAL OF GASTROENTEROLOGY(2014)

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摘要
Introduction: Intestinal lymphangiectasias (IL) are disorders characterized by dilated intestinal lacteals. Often reported in children, and less commonly in adults, its presentation is subtle, causing a slow loss of lymph into the gastrointestinal tract. In this case, we describe a rare presentation of overt obscure gastrointestinal bleeding (OOGIB) caused by generalized lymphangiectasia (GL). A 61-year-old white male presented to the hospital with a 1-week history of fatigue, worsening dyspnea with moderate exertion, and melena. His past medical history was significant for CAD, iron deficiency anemia (IDA), and HIV with a last reported undetectable viral load treated with efavirenz, emtricitabine, and tenofovir. His vital signs were stable and complete blood count was consistent with IDA. His Hgb and HCT on presentation were 6.4gm/dL and 21.5%. The patient was transfused and had improvement to his anemia. Further work-up revealed serum iron of 33 uL/dL, TIBC of 395 ug/dL, iron saturation of 8.4%, transferrin of 370mg/dL, and serum ferritin of 5.6 ng/mL. His stool was positive for occult blood and serum folate, and B12 levels were within normal range. The patient underwent upper and lower endoscopy that showed a duodenal diverticulum, mild diverticulosis in the ascending and transverse colon, and streaks of blood without an identified source. The patient’s vital signs and hemoglobin were closely monitored post-procedure, and he subsequently required a second blood transfusion. Capsule endoscopy (CE) was then performed and showed an actively bleeding polypoid mass in the jejunum, limiting visualization to the cecum. A single-balloon enteroscopy (SBE) was done next and identified an actively bleeding tumor at the distal jejunum. The patient underwent exploratory laparotomy and had resection of his jejunal mass as well as a Meckel’s diverticulectomy. With the exception of an ileus, the patient did well post-operatively and tissue pathology of the mass confirmed GL. IL may be associated with hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia, and lymphopenia. The common clinical features of IL, which our patient did not exhibit, include bilateral lower limb edema, pleural effusion, and chylous ascites. Patients may have steatorrhea with increased loss of fecal fat and protein, while others may present simply with IDA. Most lymphangiectasias detected in adults appear to have little to no clinical features of malabsorption. Our case demonstrates an unusual presentation of OOGIB secondary to IL, which, to our knowledge, has not been reported. It is important to promptly identify this condition, and we would like to emphasize the role of CE and SBE in securing the diagnosis.
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Lymphatic Malformations
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