Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome.

Mar 2013

Xinias I, Mavroudi A, Sapountzi E, Thomaidou A, Fotoulaki M, Kalambakas A, Karypidou E, Kollios K, Pardalos G,Imvrios G.

Source

Third Pediatric Department, Papageorgiou Hospital, Thessaloniki, Greece.

Abstract

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema,lymphedema, diarrhea, ascites and other manifestations. We report two pediatric cases with PIL with extremely different outcome in a 3-year follow-up period. The first patient presented with persistent diarrhea, hypoalbuminemia and failure to thrive, while the second patient presented with an abrupt eyelid edema. Hypoproteinemia was the common laboratory finding for the two patients and upper gastrointestinal endoscopy established the diagnosis. The first patient relapsed five times during the follow-up period after the diagnosis had been made and required intravenous albumin administration and micronutrient supplementation. The second patient revealed normal gastrointestinal endoscopy 4 months after the diagnosis had been established; he followed an unrestricted diet and remained asymptomatic throughout the follow-up period. PIL can be either severe, affecting the entire small bowel, leading to lifetime disease, or sometimes affects part of the small bowel, leading to transient disorder.

KEYWORDS:

Children,,Outcome, Primary intestinal lymphangiectasia, Protein-losing enteropathy, Wireless capsule endoscopy

PubMed

A case of recurrent gastrointestinal bleeding and protein-losing gastroenteropathy.

A case of recurrent gastrointestinal bleeding and protein-losing gastroenteropathy.

Nat Clin Pract Gastroenterol Hepatol. 2007 May
Herfarth H,
Hofstadter F,
Feuerbach S,
Jurgen Schlitt H,
Scholmerich J,
Rogler G.
Department of Medicine, Division of Gastroenterology and Hepatology, University of North Carolina, Chapel Hill, NC 27599, USA. hherf@med.unc.edu

BACKGROUND: A 40-year-old male with pentalogy of Fallot (a congenital heart defect with five anatomical components) presented with recurrent gastrointestinal bleeding. He had recently recovered from a heart operation, which was performed to reconstruct the right ventricular outflow tract.

INVESTIGATIONS: Laboratory tests and absorption tests, esophagogastroduodenoscopy, capsule endoscopy, human serum albumin scintigraphy, lymphoscintigraphy, CT and abdominal lymph-node histology.

DIAGNOSIS: Intestinal lymphangiectasia with concurrent protein-losing gastroenteropathy and recurrent gastrointestinal bleeding.

MANAGEMENT: Despite a low-fat diet and surgical suturing of multiple small-bowel ulcerations the gastrointestinal bleeding continued. Serum albumin levels remained very low and severe lymphedema occurred. Unfortunately, the patient developed severe sepsis and died of multiple organ failure.

PMID: 17476211 [PubMed - in process]