[Intestinal lymphangiectasis (report of a case)].
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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.
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.
Intestinal lymphangiectasia may be more protean in clinical manifestations and less rare than earlier suspected. A recent report points out that there are two types of the disorder, one congenital and the other acquired and transitory. A case is reported which fulfills the current clinical, laboratory, radiological and histological criteria for the diagnosis of the disease, and represents the first report in Scandinavia of transient intestinal lymphangiectasis with rapid and complete recovery within a few months after initiation of MCT diet.
In a 16 years old girl we found the syndrome of exsudative enteropathy with intestinal lymphangiectasy which differs from previous reported cases by several malformations of the lymphatic system. Besides changes in the intestinal lymphatics there was a retroperitoneal lymphangiodysplasia, a reduction and hypoplasia of the lymph vessels in the legs and cysts in the spleen. By substitution of albumin followed by a diet with middle chained triglycerids (MCT), serum protein was stablised but a relapse could not be prevented. Surgical treatment was not possible because the changes were too extensive. The value of diagnostic measures for differential diagnosis of the intestinal lymphangiectasy is presented.
Trying to find out the clinical elements that define the precise indication ofr a jejunal biopsy, 28 children with several pathological conditions were studied. They were 12 cases of kwashiorkor, 11 cases with chronic diarrhea, two cases of chronic pancreatitis, 2 cases with ferropenic anemia resistant to the oral treatment with iron and one case of chilous ascitis. It is concluded that only in those cases in which the biopsy is the precise medium for diagnosis is where it would be indicated, such as intestinal lymphangiectasis or in those cases with signs or evidence of malabsorption without diarrhea. The chronic diarrhea per se does not seem to be a formal indication for biopsy.
Nonimmune hydrops fetalis has been reported to be associated with congenital malformations. We describe two newborns with Noonan's syndrome who presented with nonimmune hydrops fetalis that was most likely secondary to a generalized lymphatic vessel dysplasia. Other manifestations of lymphatic abnormalities in Noonan's syndrome, such as pedal edema and pulmonary and intestinal lymphangiectasis, have been observed in children. Nonimmune hydrops represents one end of the spectrum of abnormalities seen in this syndrome.
Intestinal lymphangiectasia with lipogranulomatous lymphangitis was diagnosed at necropsy in a 6.5-year-old Maltese dog that had a history of bouts of vomiting, abdominal distention, and diarrhea. The condition was attributed to trauma to the pleural and peritoneal cavities received from bite wounds inflicted one year previously.
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Intestinal lymphangiectasia caused severe diarrhea and generalized edema in a 40-year-old man. The diagnosis was established by clinical, laboratory, and duodenal biopsy findings. The abnormalities detected on computed tomography (CT) and scintigraphy using 99mTc human serum albumin are herein described and pertinent literature is briefly reviewed.
Waldmann in 1961 redefined primary protein-losing gastroenteropathy and renamed the condition "lymphangiectasia". This abnormality, usually seen in infancy, is characterized by enlargement of folds and signs of hypersecretion in the small bowel. Enlargement of folds occurs secondary to edema of the valvulvae conniventes and lymphatic dilatation. Hypersecretion may be the result of rupture of dilated lymphatics or transudation of protein across an intact capillary epithelium. The clinical, roentgenographic, and pathologic findings are described. The pathophysiology of this condition is discussed.
Intestinal lymphangiectasia is found in a wide variety of pathologic conditions. Functional lymphangiectasia has not been well characterized. We report 20 patients followed for 9 to 55 months (mean 30 months) after incidental detection at endoscopy of lymphangiectasia. Our study indicates that functional lymphangiectasia is not pathologic and does not warrant repeat endoscopy in the absence of other clinical indications.
Tiny millet-like prominent villi with dilated lymphatics may be part of a food-induced functional lymphangiectasia. Peroral olive oil load causes diffuse transient lymphangiectasia, the gross aspect, histological findings and electron-microscopy of which is described in detail.
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Two out of 74 children with coeliac disease demonstrated severe intestinal protein loss. In both children a serial small bowel biopsy specimen showed intestinal lymphangiectasia to be also present. Intestinal lymphangiectasia is another disorder that may be associated with coeliac disease.
Bipedal lymphography was performed in 5 cases of Behçet's disease. In 4 of 5 cases, histologic examination of intestinal biopsy specimens showed lymphatic dilatation of the small intestine which were considered to be a characteristic finding of intestinal lymphangiectasia, a protein-losing enteropathy, but no patient with Behçet's disease showed reduced concentration of serum total protein as well as abnormal value of 131I-PVP test. Lymphograms showed an increase in number of iliopelvic and lumbar lymphatic vessels as a major finding, but they failed to demonstrate a hypoplasia or aplasia of lymphatic system or obstruction of thoracic duct. It is conceivable that the lymphatic dilatation of the small intestine in Behçet's disease may be related to increased flow of lymph due to excessive vascular hyperpermeability and may not be related to a block of lymphatic system which has been considered to be a cause of enteric protein loss in intestinal lymphangiectasia. The authors wish to emphasize that the dilatation of lymphatic vessel of the small intestine is not a sufficient finding to indicate the presence of protein-losing enteropathy.
Enlargement of the valvulae conniventes is an integral part of the pattern diagnosis of primary small bowel disease causing malabsorption. The pathophysiology underlying enlargement of the fold and the most typical diseases with prominent folds leading to malabsorption are discussed. Differential diagnosis is based on enlargement of the fold and on secondary signs.