A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: A case report

Abstract

Intestinal lymphangiectasia (IL) is a rare disease characterized by dilated lymphatic vessles in the intestinal wall and small bowel mesentery which induce loss of protein and lymphocytes into bowel lumen. Because it most often occurs in the intestine and cannot be detected by upper gastroendoscopy or colonoscopy, and the value of common image examinations such as X-ray and computerized tomography (CT) are limited, the diagnosis of IL is difficult, usually needing the help of surgery. Capsule endoscopy is useful in diagnosing intestinal diseases, such as IL. We here report a case of IL in a female patient who was admitted for the complaint of recurrent edema accompanied with diarrhea and abdominal pain over the last twenty years, and aggravated ten days ago. She was diagnosed by M2A capsule endoscopy as a primary IL and confirmed by surgical and pathological examination.

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

“…A rare, previous case of intestinal lymphangiectasia caused by multiple myeloma involved the mesenteric lymph nodes [9] . PIL was suggested because there was no evidence of pancreatic disease, systemic lupus erythematosus, Menetrier's disease, other intestinal disorders, other disorders of intestinal lymphatics or intestinal lymphoma [10] . Ruptured lymphatic vessels resulting from inflammatory diseases of the small intestine or malrotation were reported to be mechanisms of secondary intestinal lymphangiectasis.…”

MRI in Primary Intestinal Lymphangiectasia: A Diagnostic Tool and to Delineate Localization of Involvement

“…This approach has significant shortcomings as the lymphatics maybe dilated only post prandial and not visualized when the endoscopy and biopsy were performed [3]. In addition if the region of small bowel involvement is distal to the duodenum this approach would give a falsely normal result [4][5][6][7][8][9][10][11]. This aspect assumes more significance as it's the patients who have localized involvement, benefit most from a definitive surgery wherein the involved segment would be removed and the patient would lead a normal life, with normal no dietary restrictions thereafter [13][14][15].…”

“…Given the rarity of PIL the evaluation and management strategies have not changed much over the past decade [2]. There have been reports on successful usage of computerized tomography (CT) scan and video capsule endoscopy (VCE) to diagnose and delineate the extent of intestinal involvement [4][5][6][7][8][9][10][11][12]. The significance of determining the extent of involvement lies in the good response to surgical excision of the involved intestinal segment if a localized disease is present, as medical management outcomes are modest and often frustrating [13][14][15].…”

Late onset Intestinal Lymphangiectasia -A rare cause of bipedal edema in middle age

“…Dilated lymphatics are located primarily in the small bowel resulting in severe leakage of lymph in to the gastrointestinal tract which translates into loss of proteins, immunoglobulins and lymphocytes 2,3 .This entity IJBR (2013) 04 (10) www.ssjournals.com first described by Waldmann in 1961, primarily affects children (generally diagnosed before 3 years of age) and young adults but may be diagnosed later in adulthood 4,5 . Since then, no more than two hundred cases have been reported 6 . Presentation in children is with non bloody diarrhea and odema.…”

“…Various modalities have been used to treat this condition, but with limited success -low fat diet with supplementary medium chain triglycerides, antiplasmin -tranexamic acid, octreotide, surgical resection and steroids 3,6 .…”