Intestinal lymphangiectasia: a case report.
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A ten month old female Turkish child with chylous ascites, diarrhea, steatorrhea, peripheral edema and hypoproteinemia was investigated for protein losing enteropathy which probably dated from the first weeks of life. Gastrointestinal protein loss appeared to be due to abnormalities of the intestinal lymphatics. In order to detect a localized lymphoenteric fistula, lymphangiography was tried but failed due to hypoplasia of peripheral lymphatics. However, three hours after intradermal injection of Patent Blue, the dye appeared in the stools of the patient, suggesting intestinal protein loss via a lympho-enteric fistula. This observation may provide the basis for a diagnostic test for gastro-intestinal protein loss in patients with intestinal lymphangiectasia.
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We report a man aged 68 years old with pneumothorax and chronic bilateral pleural effusion in association with a history of yellow nails. The diagnosis of yellow nail syndrome based on yellow nails, lymphedema, chronic pleural effusion and intestinal lymphangiectasia.
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A 47-year-old man with primary intestinal lymphangiectasia developed chylous reflux into the skin of the right flank. The clinical appearance resembled lymphangioma circumscriptum with multiple dome-shaped vesicles filled with milky-white fluid which discharged on to surrounding skin. Backflow of chyle into the skin of the lower limb, perineum or external genitalia has been described as a rare finding in patients with primary lymphoedema but we believe this to be the first case involving the abdomen and the first case describing chylous reflux into the skin in a patient with intestinal lymphangiectasia.