Large Intestinal Lymphangiectasia Polyp, a benign condition with clinical consequences

Introduction
Intestinal lymphangiectasia is a relatively rare condition of gastrointestinal tract that is seen as pathological dilation of lymph, blood vessels and main cause of protein losing enteropathy.[1] It is classified into two groups: primary type, which has no particular causes, and secondary type, which is associated with marked inflammation. It is characterized by hypoproteinemia, oedema and lymphocytopenia.[2]

A 60 years old Hispanic female was referred for colon cancer screening. She had never had a colonoscopy. She was in good health and gave no history of change in her bowel habits, abdominal pain, or rectal bleeding. On colonoscopy she was found to have a large unusual looking polyp as shown in Figure-1. Biopsies of the polyp showed that it consisted of lymphangiectasia, and telangiectasia as shown in Figure-2.

Discussion
The Primary intestinal Lymphangiectasia occurs in children and adults due to congenital deformity of bowel lymphatic system and the secondary one is more frequently in adults, the patient may be asymptomatic or manifest an elevated lymphatic pressure as in lymphoma, systemic lupus erythematosus, inflammatory bowel disease, malignancies, constructive pericarditis and cardiac disease.[2,3] Intestinal Lymphangiectasia was first described in 1961 by Waldman and characterized by dilation of intestinal mucosa, submucosal and subserosal intestinal lymphatics leading to obstruction, rupture and leak of lymph fluid in the bowel lumen that cause lymphopenia, hypogammaglobulinemia and albuminemia.[3] The diagnosis can be made by clinical manifestations including fatigue, chronic diarrhea, abdominal pain, nausea, bilateral lower limb oedema, ascites and sometimes with recurrent gastrointestinal bleeding, then the definitive diagnosis requires endoscopy and biopsy.[4] The endoscopic findings are scattered white plaques covering the mucosa but it may be normal and in such cases video capsule endoscopy and double balloon enteroscopy assisted biopsy to make the diagnosis.[3] The diagnosis of intestinal lymphangiectasia is established by histology of the grossly dilated lymphatic’s seen in lamina propria of the small bowel (Duodenum, Jejunum and Ileum), the villi is distorted and enlarged.[5] There are some syndromes linked with Intestinal Lymphangiectasia like Turner, Von Ricklinghaeson, Noonan, Hennkam and yellow nail.[3] The immunological findings of intestinal lymphangiectasia are low CD4 T cells, skin allergy, reduced immunoglobulin levels (IgA, IgG, IgM), lymphocytopenia and graft rejection.[4] There had been few similar cases reported regarding asymptomatic Intestinal Lymphangiectasia, and it was shown by dilatation of intestinal lymphatics leading to obstruction, rupture and leak of lymph into the bowel lumen. It can be confused or misdiagnosed with secondary malignancies like lymphoma, systemic lupus erythematosus, and patients treated with chemotherapy and radiotherapy.[7]

Conclusion
Intestinal Lymphangiectasia, so far, is usually a benign condition with clinical consequences. Sometimes, it’s presented with symptoms and very rare discovered by screening (asymptomatic). However, this does not mean to overlook this disorder, it is very important to exclude other serious conditions especially lymphoma and secondary malignancies.