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PRIMARY INTESTINAL LYMPHANGECTASIA: DIAGNOSIS BY VIDEO CAPSULE ENDOSCOPY; A CASE REPORT
M Al Hatlani, E Al Tamimi, AMGA Sant’Anna, R Issenman
Division of Pediatric Gastroenterology and Nutrition, Department of Pediatric, McMaster Children’s Hospital, McMaster University, Hamilton, Canada
INTRODUCTION: Intestinal lymphangectasia (IL) is a congenital, acquired or inherited disorder of the lymphatic vessels associated with protein-losing enteropathy. Primary lymphangiectasia is less well understood. Endoscopic examination and duodenal biopsy are the standard criteria for the diagnosis; the use of video capsule endoscopy (VCE) in pediatric patients has been documented in only a few cases.
Case scenario: We describe an 8 year old girl who had abdominal pain and diarrhea for 8 months associated with lower limb edema. Pertinent laboratory finding were: WBC: 4.8×109, lymphocytes: 0.4×109, albumin: 22g/l, IgA: 0. 4g/l, IgG: 2.0g/l, TSH: 6.8mu/l, freeT3: 5.14(n), antiendomysial antibodies were negative, normal renal function test and electrolytes; urine analysis was negative for proteinuria. 72 hour fecal fat collection yielded a fat content of 7.6 g/day, fecal alpha-1-antitrypsine was elevated: 299 mL/dl, small bowel follow through showed mild to moderate thickening of mucosal folds in the proximal and mid jejunum. UGI endoscopic biopsies were normal, colonic biopsies showed non-specific inflammation. Low fat, high MCT diet was initiated, and supplemented with parenteral nutrition. Video capsule endoscopy was done 10 days later. White opaque spots on jejunal mucosa indicated dilated lacteal vessels. The serum albumin level increased to 35g/l, diarrhea resolved, and lower limb edema improved after three weeks of dietary treatment and parental nutrition.
CONCLUSION: Video capsule endoscopy provided images of the entire small bowel, and confirmed the diagnosis of intestinal lymphangectasia in the absence of the ability to obtain mid gut specimens.