| A Case of Intestinal Lymphangiectasia |
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Yae Kyung Suh1, Kyung Hee Park1, Chul Ho Jang1, Bum Soo Park1, Jeong Kee Seo1, Sung Hae Park2, Je Geun Chi2, Kyung Mo Yeon3, Kwi Won Park4 |
1Department of Pediatrics, Seoul National Universty College of Medicine, Seoul, Korea
2Department of Pathology, Seoul National Universty College of Medicine, Seoul, Korea
3Department of Radiology, Seoul National Universty College of Medicine, Seoul, Korea
4Department of Pediatric Surgery, Seoul National Universty College of Medicine, Seoul, Korea |
| 장림프관확장증 1례 |
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서예경1, 박경희1, 장철호1, 박범수1, 서정기1, 박성혜2, 지제근2, 연경모3, 박귀원4 |
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 병리학교실
3서울대학교 의과대학 방사선학교실
4서울대학교 의과대학 소아외과학교실 |
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| Abstract |
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Intestinal lymphangiectasia is a primary or secondary disorder of the gastrointestinal tract, which is associated with lymphatic dysfuction and protein-losing enteropathy. It's clinical manifestations vary widely, but the main symptoms are abdominal distention, edema, abdominal pain and growth failure.
We described a 15-yr-old boy, who suffered from above symptoms since third year of his age and diagnosed by duodenal endoscopy which showed numerous dilated lymphatics in the propria associated with a mild chronic inflammation. The additional findings were lymphopenia, increased α1-antitrypsin clearance and lymphatic flow obstruction on lymphangiography at thoractic duct-left subclavian vein junction area. The exploratory laparotomy was done and the entire small bowel was severely adhered and encapsulated with fibrinoid membrane, so adhesiolysis was impossible. The medium chain triglyceride was given for his medical treatment and generalized edema with abdominal distension was improved. This is the first biopsy proven case of intestinal lymphangiectasia in Korea. So we report this case with a review of related literatures. |
| Key Words:
Intestinal lymphangiectasia, Protein-losing enteropathy |
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