Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature

Huber, Rudolf M.; Semmler, Georg; Mayr, Alexander; Offner, Felix; Datz, Christian

doi:10.3748/wjg.v26.i48.7707

Cited by 16 publications

(34 citation statements)

References 60 publications

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“…PIL often develops in childhood or youth, and most cases are diagnosed before the age of 3 years. In recent years, an increasing number of cases have been reported in adults[ 2 , 13 - 17 ]. In this study, we found that the age at onset differed among the four types of endoscopy.…”

Section: Discussionmentioning

confidence: 99%

Endoscopic classification and pathological features of primary intestinal lymphangiectasia

Meng¹,

Liu²,

Xue³

et al. 2022

WJG

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BACKGROUND The appearance of the intestinal mucosa during endoscopy varies among patients with primary intestinal lymphangiectasia (PIL). AIM To classify the endoscopic features of the intestinal mucosa in PIL under endoscopy, combine the patients’ imaging and pathological characteristics of the patients, and explain their causes. METHODS We retrospectively analyzed the endoscopic images of 123 patients with PIL who were treated at the hospital between January 1, 2007 and December 31, 2018. We compared and analyzed all endoscopic images, classified them into four types according to the endoscopic features of the intestinal mucosa, and analyzed the post-lymphographic computed tomography (PLCT) and pathological characteristics of each type. RESULTS According to the endoscopic features of PIL in 123 patients observed during endoscopy, they were classified into four types: nodular-type, granular-type, vesicular-type, and edematous-type. PLCT showed diffuse thickening of the small intestinal wall, and no contrast agent was seen in the small intestinal wall and mesentery in the patients with nodular and granular types. Contrast agent was scattered in the small intestinal wall and mesentery in the patients with vesicular and edematous types. Analysis of the small intestinal mucosal pathology revealed that nodular-type and granular-type lymphangiectasia involved the small intestine mucosa in four layers, whereas ectasia of the vesicular- and edematous-type lymphatic vessels largely involved the lamina propria mucosae, submucosae, and muscular layers. CONCLUSION Endoscopic classification, combined with the patients’ clinical manifestations and pathological examination results, is significant and very useful to clinicians when scoping patients with suspected PIL.

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Section: Discussionmentioning

confidence: 99%

Endoscopic classification and pathological features of primary intestinal lymphangiectasia

Meng¹,

Liu²,

Xue³

et al. 2022

WJG

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Section: Lymphatic Intestinal Lymphangiectasiamentioning

confidence: 99%

“…Primary intestinal lymphangiectasia (PIL) is a rare disorder of unknown etiology resulting in protein-losing enteropathy [149,150]. Although usually developing in early childhood, cases have also been described in adults [149]. The disease is caused by a diffuse or localized dilatation and/or rupture of intestinal lymphatic vessels in the mucosa, submucosa, or subserosa due to high pressure in lymphatic vessels.…”

Section: Lymphatic Intestinal Lymphangiectasiamentioning

confidence: 99%

Small and Large Intestine (I): Malabsorption of Nutrients

2021

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Numerous disorders can alter the physiological mechanisms that guarantee proper digestion and absorption of nutrients (macro- and micronutrients), leading to a wide variety of symptoms and nutritional consequences. Malabsorption can be caused by many diseases of the small intestine, as well as by diseases of the pancreas, liver, biliary tract, and stomach. This article provides an overview of pathophysiologic mechanisms that lead to symptoms or complications of maldigestion (defined as the defective intraluminal hydrolysis of nutrients) or malabsorption (defined as defective mucosal absorption), as well as its clinical consequences, including both gastrointestinal symptoms and extraintestinal manifestations and/or laboratory abnormalities. The normal uptake of nutrients, vitamins, and minerals by the gastrointestinal tract (GI) requires several steps, each of which can be compromised in disease. This article will first describe the mechanisms that lead to poor assimilation of nutrients, and secondly discuss the symptoms and nutritional consequences of each specific disorder. The clinician must be aware that many malabsorptive disorders are manifested by subtle disorders, even without gastrointestinal symptoms (for example, anemia, osteoporosis, or infertility in celiac disease), so the index of suspicion must be high to recognize the underlying diseases in time.

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“…Intestinal lymphangiectasia (IL) is a rare disease with unknown prevalence and is characterized by dilated lymphatic vessels resulting in the leakage of lymphatic fluids into the intestinal lumen [ 1 ]. Primary IL has an early onset with undetermined etiology, whereas secondary IL is associated with preexisting conditions, including myxedema heart, systemic lupus erythematosus, radiotherapy/chemotherapy, Waldenstrom’s macroglobulinemia, primary peritoneal carcinoma, diffuse large B-cell lymphoma, and primary hypoparathyroidism [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…Clinical presentations often include diarrhea/vomiting, edema, third cavity fluid accumulation, and recurrent infections. While hypoalbuminemia, hypogammaglobulinemia, lymphopenia, and hypocalcemia are well-known laboratory findings for IL, little is known about the magnesium levels in IL patients [ 1 ]. Absorption of magnesium in the small intestine plays an important part in its homeostasis, along with skeletal storage and renal excretion.…”

Section: Introductionmentioning

confidence: 99%

Hypomagnesemia in intestinal lymphangiectasia: a case report and review of the literature

et al. 2022

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Background Intestinal lymphangiectasia (IL) is a rare disease characterized by dilation of lymphatic vessels and leakage of lymphatic fluids into the intestinal lumen, causing depletion of lymphocytes, protein, lipids, fat-soluble vitamins, and electrolytes. Hypomagnesemia can occur in IL patients but is seldom discussed. Case presentation A 30-year-old Tibetan woman who had chronic diarrhea, edema, tetany, and tingling was diagnosed with IL. Prominent hypomagnesemia was noticed. She was treated with a medium-chain triglyceride (MCT) diet and nutrient supplementation with satisfactory results. We also present a systematic review of hypomagnesemia in IL cases from the published literature. Conclusions Hypomagnesemia may be an overlooked complication of IL, thus monitoring serum magnesium concentrations in IL patients is crucial.

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Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature

Cited by 16 publications

References 60 publications

Endoscopic classification and pathological features of primary intestinal lymphangiectasia

Endoscopic classification and pathological features of primary intestinal lymphangiectasia

Small and Large Intestine (I): Malabsorption of Nutrients

Hypomagnesemia in intestinal lymphangiectasia: a case report and review of the literature

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