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PubMed1986

[Primary intestinal lymphangiectasis with antenatal manifestation].

Antenatal ascites diagnosed by ultrasound examination was punctured and shunted in utero. Radiologic, endoscopic and histologic data led to diagnosis of primitive intestinal lymphangiectasia during the second year of life. Antenatal revelation of Waldmann's disease is extremely rare in pediatric literature.

Ascites↗
PubMedDate not supplied

[Intestinal lymphangiectasis: late complication of transportation of the great vessels corrected by the Mustard technic. A clinical case].

The Great Arteries Transposition corrected by Mustard's repair is often complicated with inferior vena cava obstruction with consequent intestinal lymphangiectasia and protein-losing enteropathy. We report a case of a 9 years old boy who underwent Mustard's repair and presented diarrhoea, abdominal pain, hypoproteinemia and ascites. The fecal elimination of 51Cr-labeled albumin was remarkable increased and the intestinal biopsy showed a noteworthy lymphangiectasia due to an hemodynamic overload. Therefore subjects with Great Arteries Transposition corrected by Mustard's repair should be valued in the post-surgical follow-up by 51Cr-labeled albumin fecal elimination and by intestinal biopsy, already sensitive in asymptomatic phase.

Child↗
PubMed1990

[Intestinal lymphangiectasis secondary to cicatricial fibrosis of mesenteric nodes: a nosologic entity?].

Exsudative enteropathy was suspected in a 27-year-old man with lower limb edema, hypoprotidemia and hypoalbuminemia. Gastrointestinal mucosa, kidney, liver, and heart were normal. Laparoscopy showed diffuse small intestine lymphangiectasia. This diagnosis was confirmed by the microscopic examination of several biopsies obtained at laparotomy. Pathological examination of peritoneal, lymph nodes, and liver biopsies showed fibrous thickening of the peritoneum and fibrosis of the lymph nodes. Our patient has been followed for 16 years. Substantial improvement of clinical symptoms was obtained by following a special salt-free diet containing short-chain triglycerides. However biochemical abnormalities have persisted. Exsudative enteropathy due to intestinal lymphangiectasia may be observed in heart and liver diseases as well as in malignant affections of mesenteric lymph nodes. If these conditions are excluded, intestinal lymphangiectasia may be considered as a primitive lymph vessel malformation. The discovery of primitive intestinal lymphangiectasia in an adult cannot be attributed to congenital abnormalities alone. Fibrosis encountered in some cases suggests that an inflammatory process of unknown origin may trigger the onset of intestinal lymphangiectasia.

Adult↗