Lymphangiectasia of the small intestine: description and pathophysiology of the roentgenographic signs

Olmsted, William W.; Madewell, John E.

doi:10.1007/bf02256372

Cited by 20 publications

(4 citation statements)

References 11 publications

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“…Many cases, however, have been reported to be congenital in origin and have most likely been caused by failure in the proper formation of lymphatic vessels, although IL can be seen as a consequence of acquired obstruction of the lymphatic system [17]. In this study, primary IL was classified into either the white or non-white type, as was secondary IL.…”

Section: Discussionmentioning

confidence: 99%

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Ohmiya

Nakamura²,

Yamamura³

et al. 2014

Digestion

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Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy. Results: Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal E₁-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response. i 2014 S. Karger AG, Basel

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Section: Discussionmentioning

confidence: 99%

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Ohmiya

Nakamura²,

Yamamura³

et al. 2014

Digestion

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“…Histopathological examination of duodenumjejunum and ileum biopsies confirm the presence of lacteal juice dilated mucosa (from moderate to severe) and submucosal lymphatic vessels with polyclonal normal plasma cells 8 .CT and MRI are helpful in rulling out many secondary forms of intestinal lymphangiectasia. CT may show dilatation of small bowel, thickening and hypervascularity of the mucosal folds with MRI confirming the hyperintensity on T1 images suggestive of protein rich fluid 9,10 .…”

Section: Discussionmentioning

confidence: 90%

Late onset Intestinal Lymphangiectasia -A rare cause of bipedal edema in middle age

Singh

Chand

Verma

et al. 2013

Int Jour of Biomed Res

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“…PIL is considered as congenital in origin, most likely been caused by failure in the proper formation of lymphatic vessels during infants or earlier, and mutation or dysfunction of some genes or transcriptional factors, such as vascular endothelial growth factor-C, VEGFR3, prosperorelated homeoboxtranscriptional factor, forkhead transcriptional factor and SOX18, have been reported to be related to lymphedematous diseases and lymphangiectasia [ 20 , 21 , 22 ]. IL can also be a consequence of acquired obstruction of the lymphatic system or age-related lymphatic changes [ 23 ]. IL also may be diagnosed in adults, even in elderly patients [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

Small intestinal mucosal abnormalities using video capsule endoscopy in Intestinal lymphangiectasia

Lin

Liu

et al. 2023

Preprint

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Objectives To disclose the video capsule endoscopy (VCE) characteristics of small intestinal mucosal abnormalities in patients with intestinal lymphangiectasia (IL), and to investigate the relationship between clinical and VCE characteristics. Methods Consecutive patients with IL underwent the VCE were enrolled into the retrospective study. The cases were classified into white villi group and non-white villi group according to mucosal abnormalities detected by VCE. Clinical and endoscopic characteristics were investigated and analyzed. Results A total of 98 patients with IL with a median onset age of 26.3 ± 19.2 years were included. VCE disclosed the following small intestinal lesions: (i) white villi type (57/98, 58.2%), i.e.: white-tipped or granular villi, white nodular villi or plaques; (ii) non-white villi type (41/98, 41.8%), i.e.: diffused low and round villi; (iii) complications (46/98, 46.9%), i.e.: bleeding, ulcers, protruding or vesicular-shaped lesions, stenosis and lymphatic leakage. 58.2% (57) and 41.8% (41) of cases were classified into white villi and non-white villi group respectively. The percentage of chylothorax in white villi group was significantly lower than non-white villi group (12/57 vs. 19/41, p = 0.008). In VCE, there were no significant differences in involved segments and total detected rate of complications between white villi and non-white villi groups (p > 0.05), while the detected rate of lymphatic leakage in white villi group was significantly higher than non-white villi group (31.6% vs. 12.2%, p = 0.026). Conclusions Our study identified the entire small intestinal mucosal abnormalities of IL by VCE, especially in endoscopic complications. IL has specific VCE abnormalities in addition to classical endoscopic findings.

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Lymphangiectasia of the small intestine: description and pathophysiology of the roentgenographic signs

Cited by 20 publications

References 11 publications

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Late onset Intestinal Lymphangiectasia -A rare cause of bipedal edema in middle age

Small intestinal mucosal abnormalities using video capsule endoscopy in Intestinal lymphangiectasia

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