Primary Intestinal Lymphangiectasia: Is It Always Bad Two Cases with Different Outcome

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.

An unusual case of chylous ascites: A case report

“…Some patients have a short period of fever initially, while others present with acute ileus and some develop the symptoms gradually [2,4,5,[8][9][10][11][12][13][14][15] .…”

“…IL was first described in 1961 by Waldmann et al and since then about 200 cases have been reported [1,[4][5][6][7] . We present a patient with primary IL as a rare cause of intractable, but potential curable ascites.…”

Severe Refractory Anemia in Primary Intestinal Lymphangiectasia. A Case Report

“…Primary intestinal lymphangiectasia is usually diagnosed in childhood, but adult cases have also been reported. Worldwide, few over 200 cases have been described [3]. The diagnosis is confirmed by the endoscopic appearance of the small bowel lymphangiectasia and the corresponding histology on the biopsies.…”

“…The histopathology report of the enterobiopsy specimens confirmed the diagnosis of PIL (Figs. 3,4). Immunohistochemistry was negative for lymphoma.…”

“…The need for nutritional therapy is permanent, because on withdrawal the exudative enteropathy reappears [2]. In refractory forms of PIL, there are reports of successful treatment with antiplasmin agents (tranexamic acid), somatostatin analogues (octreotide), corticosteroids, segmental bowel resection, peritoneovenous shunt and intestinal transplant [2,3].…”