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Case Reports
. 2015 Jun 21;21(23):7320-5.
doi: 10.3748/wjg.v21.i23.7320.

How to treat an extensive form of primary intestinal lymphangiectasia?

Rosana Troskot  1 , Dragan Jurčić  1 , Ante Bilić  1 , Marija Gomerčić Palčić  1 , Stanko Težak  1 , Ivana Brajković  1
Affiliations
Case Reports

How to treat an extensive form of primary intestinal lymphangiectasia?

Rosana Troskot et al. World J Gastroenterol. .

Abstract

We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia, which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa. Although numerous therapeutic strategies are available, only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.

Keywords: Diet; Malabsorption; Octreotide; Primary intestinal lymphangiectasia; Seizure; Treatment.

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Figures

Figure 1
Figure 1
X-ray examination of the small intestine. X-ray examination of the small intestine revealing thickened folds as well as a dilated lumen of the jejunum and ileum.
Figure 2
Figure 2
Double balloon enteroscopy. Double balloon enteroscopy demonstrating edematous distal duodenum and jejunal mucosa scattered with white spots. A-C: duodenum; D-F: Jejunum.
Figure 3
Figure 3
Histopathological findings. Histopathological findings: dilated lymphatic vessels in the tips of whitish jejunal villi (hematoxylin-eosin stain, magnification × 40).
Figure 4
Figure 4
Technetium 99m human serum albumin scintigraphy. Scintigraphy showing Technetium 99m labeled human serum albumin extravasation throughout small intestine after 6 h of injection (A and B) and presence in the large intestine after 24 h of injection (C and D).
See this image and copyright information in PMC

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