Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: a case report

Journal of Medical Case Reports

Qi-kui Chen¹  Li-na Zhao¹  Xiao-yu Qiao²  Tao Yu¹  Yu Lai¹ 

[1] Department of Gastroenterology, Sun Yat-sen Memorial Hospital, Sun Yat-Sen University, 107 Yan Jiang Xi Road, Guangzhou, Guangdong 510120, People’s Republic of China;Department of Emergency, the Third Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong, People’s Republic of China

关键词: Medium-chain triglyceride diet;    Intestinal lymphangiectasia;    Hypoproteinemia;    Edema;    Diarrhea;   

Others  :  1195134
DOI  :  10.1186/1752-1947-7-19

 received in 2012-09-22, accepted in 2012-11-22,  发布年份 2013

【 摘 要 】

Introduction

Primary intestinal lymphangiectasia is a disorder characterized by exudative enteropathy resulting from morphologic abnormalities of the intestinal lymphatics. Intestinal lymphangiectasia can be primary or secondary, so the diagnosis of primary intestinal lymphangiectasia must first exclude the possibility of secondary intestinal lymphangiectasia. A double-balloon enteroscopy and biopsy, as well as the pathology can be used to confirm the diagnosis of intestinal lymphangiectasia. A polymeric diet containing medium-chain triglycerides and total parenteral nutrition may be a useful therapy.

Case presentation

A 17-year-old girl of Mongoloid ethnicity was admitted to our hospital with a history of diarrhea and edema. She was diagnosed with protein-losing enteropathy caused by intestinal lymphangiectasia. This was confirmed by a double-balloon enteroscopy and multi-dot biopsy. After treatment with total parenteral nutrition in hospital, which was followed by a low-fat and medium-chain triglyceride diet at home, she was totally relieved of her symptoms.

Conclusion

Intestinal lymphangiectasia can be diagnosed with a double-balloon enteroscopy and multi-dot biopsy, as well as the pathology of small intestinal tissue showing edema of the submucosa and lymphangiectasia. Because intestinal lymphangiectasia can be primary or secondary, the diagnosis of primary intestinal lymphangiectasia must first exclude the possibility of secondary intestinal lymphangiectasia. A positive clinical response to the special diet therapy, namely a low-fat and medium-chain triglyceride diet, can further confirm the diagnosis of primary intestinal lymphangiectasia.

【 授权许可】

   
2013 Lai et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

【 参考文献 】

• [1]Vignes S, Bellanger J: Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis 2008, 3:5. BioMed Central Full Text
• [2]Mistilis SP, Stephen DD, Skyring AP: Intestinal lymphangiectasia. Mechanism of enteric loss of plasma-protein and fat. Lancet 1965, 1:77-81.
• [3]Jeffries GH, Chapman A, Sleisenger MH: Low-fat diet in intestinal lymphangiectasia. Its effects on albumin metabolism. N Engl J Med 1965, 270:761-766.
• [4]Kuroiwa G, Takayama T, Sato Y, Takahashi Y, Fujita T, Nobuoka A, Kukitsu T, Kato J, Sakamaki S, Niitsu Y: Primary intestinal lymphangiectasia successfully treated with octreotide. J Gastroenterol 2001, 36:129-132.
• [5]Toskes P: Gastrointestinal diseases: malabsorption. In Cecil Textbook of Medicine. 18th edition. Edited by Wyngaarden J, Smith L. Philadelphia: WB Saunders; 1988:732-745.
• [6]Aoyagi K, Iida M, Yao T, Matsui T, Okada M, Oh K, Fujishima M: Characteristic endoscopic features of intestinal lymphangiectasia: correlation with histological findings. Hepatogastroenterology 1997, 44:133-138.
• [7]Aoyagi K, Iida M, Yao T, Matsui T, Okada M, Fujishima M: Intestinal lymphangiectasia: value of double-contrast radiographic study. Clin Radiol 1994, 49:814-819.
• [8]Aoyagi K, Iida M, Matsumoto T, Sakisaka S: Enteral nutrition as a primary therapy for intestinal lymphangiectasia: value of elemental diet and polymeric diet compared with total parenteral nutrition. Dig Dis Sci 2005, 50:1467-1470.
• [9]Rust C, Pratschke E, Hartl W, Kessler M, Weibecke B, Sauerbruch T, Paumgartner G, Beuers U: Fibrotic entrapment of the small bowel in congenital intestinal lymphangiectasia. Am J Gastroenterol 1998, 93:1980-1983.
• [10]Mine K, Matsubayashi S, Nakai Y, Nakagawa T: Intestinal lymphangiectasia markedly improved with antiplasmin therapy. Gastroenterology 1989, 96:1596-1599.
• [11]Bac DJ, Van Hagen PM, Postema PT, ten Bokum AM, Zondervan PE, van Blankenstein M: Octreotide for protein-losing enteropathy with intestinal lymphangiectasia. Lancet 1995, 24:1639.
• [12]Ballinger AB, Farthing MJ: Octreotide in the treatment of intestinal lymphangiectasia. Eur J Gastroenterol Hepatol 1998, 10:699-702.
• [13]Rubin DC: Small intestine: anatomy and structural anomalies: Lymphangiectasia. In Textbook of Gastroenterology. 3rd edition. Edited by Yamada T. Philadelphia: Lippincott Williams &Wilkins; 1999:1578-1579.
• [14]Goldberg RI, Calleja GA: Protein-losing gastroenteropathies. In Boccus Gastroenterology. 5th edition. Edited by Haubrich WS, Schaffner F, Berk JE. Philadelphia: WB Saunders; 1995:1072-1086.
• [15]Alfano V, Tritto G, Alfonsi L, Cella A, Pasanisi F, Contaldo F: Stable reversal of pathologic signs of primitive intestinal lymphangiectasia with a hypolipidic, MCT-enriched diet. Nutrition 2000, 16:303-304.