Intestinal Lymphangiectasia Workup

Updated: Jan 19, 2018
  • Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Burt Cagir, MD, FACS  more...
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Workup

Laboratory Studies

Serum protein levels

The most common laboratory finding in intestinal lymphangiectasia is hypoproteinemia. Hypoalbuminemia is most prominent, and lymphocytopenia and hypogammaglobulinemia (eg, immunoglobulin A [IgA], immunoglobulin G [IgG], immunoglobulin M [IgM]) are also prominent. Cholesterol levels are not usually elevated.

Alpha1-antitrypsin levels

In random dry stools, levels of alpha1-antitrypsin has been used to indirectly measure protein leakage in the gastrointestinal (GI) tract. Alpha1-antitrypsin is negligibly broken down by intestinal proteases and, thus, is excreted intact in the stool. However, although measurement of stool alpha1-antitrypsin may serve as a good screening examination for protein loss, several studies have shown poor correlation between the value of alpha1-antitrypsin in the stool and its clearance measurement. In part, this is because of increased degradation of alpha1-antitrypsin in different milieus. For example, the breakdown of alpha1-antitrypsin is higher in environments where the pH level is less than 3, as in the stomach or small bowel in hyperacidity states.

The most specific test for protein loss in the GI tract is direct measurement of alpha1-antitrypsin clearance from plasma. Values greater than 24 mL/day in patients without diarrhea (diarrhea increases alpha1-antitrypsin clearance) and over 56 mL/day in those with diarrhea indicate protein loss in the GI tract. GI bleeding has also been shown to increase alpha1-antitrypsin clearance as a result of whole blood loss.

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Imaging Studies

Double-contrast radiographs of the small bowel may be helpful, because they may show thickened folds due to intestinal edema from hypoproteinemia, nodular protrusions, and absence of mucosal ulcerations.

Ultrasonography and computed tomography (CT) scanning are also useful in identifying dilated intestinal loops, regular and diffuse thickening of the intestinal walls, plical hypertrophy, and mesenteric edema. CT scans may help show circumferential thickening of the small bowel wall with low attenuation (<30 H).

Multidetector CT (MDCT) scanning after direct lympangiography appears to provide an accurate evaluation and diagnosis of primary intestinal lymphangiectasia. In a retrospective study of 55 affected patients, all of whom underwent MDCT after direct lymphangiography, investigators noted that MDCT identified intra-intestinal, extra-intestinal, and lymphatic vessel abnormalities, including different degrees of intestinal dilatation, small bowel wall thickening, ascites, mesenteric edema, mesenteric nodules, lumbar trunk and intestinal trunk reflux. [9]

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Procedures

Endoscopy

Repeatedly, the role of endoscopy has been proven useful. Small bowel enteroscopy not only helps detect mucosal changes suggestive of the disease but also allows acquisition of histologic samples to establish a diagnosis. [10, 11, 12]

White villi and/or spots (dilated lacteals), white nodules, and submucosal elevations are observed. Xanthomatous plaques are often visualized.

Capsule endoscopy

Capsule endoscopy has also been used to help identify the characteristic changes of intestinal lymphangiectasia not reachable with standard endoscopy.

Jejunal biopsy

Jejunal biopsy establishes a definitive diagnosis and shows dilation of mucosal and submucosal lymphatic channels. To increase the diagnostic yield, large biopsy forceps should be used when available. In addition, because of the patchy involvement of the small bowel, obtaining multiple biopsy samples from different areas is recommended.

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Histologic Findings

Intestinal biopsy results reveal the characteristic dilatation of the lymph vessels of the mucosa and submucosa without any evidence for inflammation.

Intestinal villi of normal height with dilated lym Intestinal villi of normal height with dilated lymphatics as usually seen on histology of villi in intestinal lymphagiectasia.
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