Intestinal lymphangiectasia presenting with duodeno-jejunal polyposis: enteroscopic findings

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A 55-year-old woman with a pelvic fracture was referred by the orthopedic department at our hospital for severe hypoproteinemia. The patient had been diagnosed as having primary intestinal lymphangiectasia at the age of 25. Under capsule endoscopy, numerous filiform-like polyps with whitish villi were observed from the distal duodenum to the jejunum ([Fig. 1 a]) with exudation of a chylous substance from the mucosal surface ([Fig. 1 b]).

Fig. 1 Capsule endoscopic findings. a Numerous filiform-like polyps seen in the jejunum. b Chylous exudates covering the jejunal mucosa.

These became obscure as the capsule advanced, with diminutive whitish spots or mosaic mucosal patterns as the only findings in the middle and the distal part of the small bowel. Oral double-balloon endoscopy (DBE) also identified numerous filiform-like polyps as well as diminutive whitish spots in the distal duodenum and in the jejunum ([Fig. 2]).

Fig. 2 Double-balloon endoscopy (DBE) findings: numerous filiform-like polyps along with diminutive whitish spots.

Histologic examination of the biopsy specimens taken from the whitish mucosa showed obviously dilated lymphatics in the lamina propria mucosa ([Fig. 3]), compatible with the diagnosis of intestinal lymphangiectasia.

Fig. 3 Histologic section of the whitish mucosa showing obviously dilated lymphatic vessels in the lamina propria mucosa.

In intestinal lymphangiectasia, dilated lymphatic vessels in the intestinal wall are usually seen as diminutive whitish spots or whitish reticular mucosal pattern [1]. However, unusual endoscopic findings, similar to our case, have been recently reported in patients with markedly dilated lymphatic vessels in the submucosa [2]. It thus seems possible that the observed multiple polyposis-like pattern is an endoscopic feature of an advanced stage of intestinal lymphangiectasia.

Small intestinal polyps are found in various intestinal pathologies, such as familial adenomatous polyposis [3], Cronkhite-Canada syndrome [4], and multiple lymphomatous polyposis [5]. Our endoscopic findings suggest that intestinal lymphangiectasia should be considered among the differential diagnoses in patients with small intestinal polyps found by capsule endoscopy or DBE. On such occasions, alterations in the surrounding mucosa characterized by whitish villi and chylous exudates could be indicative of intestinal lymphangiectasia.

Competing interests: None

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The page number has been corrected to E281 – E281.

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