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A Case of Congenital Intestinal Lymphangiectasia

Journal of the Korean Pediatric Society 1998;41(9):1293-1298.
Published online September 15, 1998.
A Case of Congenital Intestinal Lymphangiectasia
Pan Joo Lim1, Jae Ock Park1, Dong Hwan Lee1, Sang Mann Shin1, Dong Won Ki2
1Department of Pediatrics, College of Medicine, Soonchunhyang University, Seoul, Korea
2Department of Pathology, College of Medicine, Soonchunhyang University, Seoul, Korea
선천성 장림프관확장증 1례
임판주1, 박재옥1, 이동환1, 신상만1, 김동원2
1순천향대학교 의과대학 소아과학교실
2순천향대학교 의과대학 병리학교실
Pan Joo Lim, Email: 1
Intestinal lymphangiectasia is a primary or secondary disorder of lymphatics which is associated with protein-losing enteropathy, hypoalbuminemia, edema, abdominal distension and growth failure. The patients with this disease lose albumin, immunoglobulins, and lymphocytes through the bowel. We have experienced one case of intestinal lymphangiectasia in 8 month-old boy, whose chief complaint was abdominal distension and watery diarrhea. He had hypoalbuminemia(1.7gm/dl). The diagnosis of intestinal lymphangiectasia was confirmed by endoscopic jejunal biopsy. Pretibial pitting edema improved after medium chain triglyceride feeding. A brief review with related literatures is also presented.
Key Words: Intestinal lymphangiectasia, Jejunal biopsy

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