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Rev Med Interne. 2018 Jul;39(7):580-585. doi: 10.1016/j.revmed.2017.07.009. Epub 2017 Sep 1.

[Primary intestinal lymphangiectasia (Waldmann's disease)].

[Article in French]

Author information

1
Unité de lymphologie, Centre de référence des maladies vasculaires rares (lymphœdèmes primaires), hôpital Cognacq-Jay, 15, rue Eugène-Millon, 75015 Paris, France. Electronic address: stephane.vignes@cognacq-jay.fr.
2
Service de gastro-entérologie et nutrition, hôpital Saint-Antoine, AP-HP, 184, rue du Faubourg Saint-Antoine, 75571 Paris cedex 12, France.

Abstract

Primary intestinal lymphangiectasia (PIL), Waldmann's disease, is a rare disorder of unknown etiology characterized by dilated intestinal lacteals leading to lymph leakage into the small-bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. The main symptom is bilateral lower limb edema. Edema may be moderate to severe including pleural effusion, pericarditis or ascites. Protein-losing enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance and diagnosis by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of biopsies. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Several B-cell lymphomas of the gastrointestinal tract or with extra-intestinal localizations were reported in PIL patients. A long-term strictly low-fat diet associated with medium-chain triglyceride and liposoluble vitamin supplementation is the cornerstone of PIL medical management. Octreotide, a somatostatin analog, have been proposed with an inconsistent efficacy in association with diet. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. A prolonged clinical and biological follow-up is recommended.

KEYWORDS:

Lymphangiectasies intestinales primitives; Maladie de Waldmann; Primary intestinal lymphangiectasia; Waldmann's disease

PMID:
28867533
DOI:
10.1016/j.revmed.2017.07.009
[Indexed for MEDLINE]

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