primäre intestinale Lymphangiektasie

Primäre
intestinale Lymphangiektasie in der Computertomografie: Multiple Darmwandverdickungen, retikuläre Durchsetzung des mesenterialen Fettgewebes als Korrelat für die Lymphangiektasien.
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Individual
approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature. Nodal lymphangiography and therapeutic embolization is performed using diluted glue in Patient 3
bmcpediatr.biomedcentral.com • CC-by-4.0
A case of
primary intestinal lymphangiectasia with non-Hodgkin lymphoma. Plain computed tomography shows ascites and diffuse thickening of multiple small bowel loops (a, b). Endoscopic views of the ileocecal valve (c) and terminal ileum (d)
bmcgastroenterol.biomedcentral.com • CC-by-4.0
Primary
intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: a case report. A double-balloon enteroscopy and pathologic investigation were performed before and after 10-week treatment. A and B: The double-balloon enteroscopy revealed lymphangiectasia-like changes in the small intestinal mucosa. C: The pathologic analysis of small intestinal tissue before treatment showed chronic inflammation, edema in the submucosa, and lymphangiectasia (hematoxylin and eosin stain×200). D and E: The lymphangiectasia-like changes in the small intestinal mucosa vanished after diet therapy. F: The pathologic results after treatment were improved (hematoxylin and eosin stain×200).
jmedicalcasereports.biomedcentral.com • CC-by-2.0
Individual
approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature. Diagnostic flow and individual therapeutic strategy of primary intestinal lymphangiectasia
bmcpediatr.biomedcentral.com • CC-by-4.0
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Intestinal lymphangiectasia is a condition characterized by dilated intestinal lacteals causing loss of lymph into the lumen of the small intestine. This can result in hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia and lymphopenia.

Epidemiology

It may present in either children or adults.

Pathology

The condition may be localized or diffuse. It can occur from a number of etiologies, which include:

  • primary/congenital form: primary idiopathic intestinal lymphangiectasia / Waldmann disease
  • secondary form: resulting from a tumor, fibrosis, or another cause of lymphatic obstruction.

The definitive diagnosis is made by endoscopic evaluation and biopsy of the duodenum and/or jejunum or alternatively from surgically resected small intestine.

Biochemical tests
  • fecal alpha-1-antitrypsin is often raised

Radiographic features

General

Non-specific and may show bowel wall thickening to varying degrees.

Complications

  • malignant transformation into lymphoma: may occur in around 5% of cases .

Treatment and prognosis

Treatment has usually comprised of a low fat diet along with medium-chain triglyceride oral supplements.

History and etymology

It was first described by TA Waldmann in 1961 .

Differential diagnosis

As a broad differential of bowel thickening, consider forms of enteritis.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu primäre intestinale Lymphangiektasie:
Hemihyperplasie
 
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