Multiple abdominal telangiectases and lymphangiectases. A limited form of Osler-Weber-Rendu disease?
We describe a 23-year-old man with protein-losing enteropathy, iron deficiency anemia, and recurrent gastrointestinal bleeding. The patient eventually developed disseminated intravascular coagulation, portal vein thrombosis, and extensive small bowel infarction. The autopsy showed multiple telangiectases in the intestines, mesentery, liver, gallbladder, renal pelves, and diaphragm. In addition, lymphangiectases were found in the retroperitoneal space, intestines, and liver. These lesions appeared to have been the cause of the gastrointestinal bleeding and the protein-losing enteropathy. The case most likely represents Osler-Weber-Rendu disease without the usual manifestations in the skin and oral-nasal cavities.
Baba
R
R
Institute of Gastroenterology, Tokyo Women's Medical College, Japan.
Hashimoto
E
E
Yashiro
K
K
Nagasako
K
K
Hayashi
N
N
Nishikawa
T
T
Ludwig
J
J
D000328:Adult; D017809:Fatal Outcome; D006801:Humans; D008201:Lymphangiectasis, Intestinal / Q000150:complications* / Q000473:pathology; D008297:Male; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000473:pathology*