Abstract
IntroductionPrimary 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 presentationA 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.ConclusionIntestinal 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.
Highlights
Primary intestinal lymphangiectasia is a disorder characterized by exudative enteropathy resulting from morphologic abnormalities of the intestinal lymphatics
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
Summary
We presented a case in which PIL was suspected which involved edema associated with protein loss. The pathologic analysis of small intestinal tissue showing edema in the submucosa and lymphangiectasia confirmed the diagnosis of IL. The diagnosis of PIL was established after excluding the secondary pathogeny. A positive clinical response to the special diet therapy of low-fat and MCTs further confirmed the diagnosis of PIL. Authors’ contributions YL and TY wrote the paper; XYQ and LNZ confirmed the pathologic diagnosis; TY performed the double-balloon enteroscopy; QKC and TY interpreted the patient data regarding PIL and cured the patient. All authors read and approved the final manuscript. Author details 1Department of Gastroenterology, Sun Yat-sen Memorial Hospital, Sun YatSen University, 107 Yan Jiang Xi Road, Guangzhou, Guangdong 510120, People’s Republic of China. Received: 22 September 2012 Accepted: 22 November 2012 Published: 14 January 2013
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