1/15. Isolated fetal ascites caused by primary lymphangiectasia: a case report.We present a case of isolated fetal ascites diagnosed by ultrasonography at 29 weeks' gestation. cordocentesis revealed a normal karyotype and negative viral titers. Postnatally, the diagnosis of primary lymphangiectasia was made by intestinal biopsy. To our knowledge this is the first description of prenatal manifestation of primary lymphangiectasia.- - - - - - - - - - ranking = 1keywords = ascites (Clic here for more details about this article) |
2/15. Successful resection of localized intestinal lymphangiectasia post-Fontan: role of (99m)technetium-dextran scintigraphy.Intestinal lymphangiectasia is a well-recognized complication of the fontan procedure, occurring in up to 24% of patients. Because of the loss of chylous fluid into the gut lumen, protein-losing enteropathy results as well as lymphopenia and hypogammaglobulinaemia. In some cases, dilated lymphatics in the intestinal serosa or mesentery also rupture, causing chylous ascites. Standard medical and cardiac surgical interventions are generally ineffective and the condition is frequently lethal. We report a case of intractable and life-threatening chylous ascites and chylothorax in a 14-year-old girl, associated with intestinal lymphangiectasia and protein-losing enteropathy after a fontan procedure for tricuspid atresia. The condition was refractory to all standard medical therapies, including dietary modifications, diuretics, corticosteroid therapy, albumin infusions, octreotide, heparin, bowel rest, and parenteral nutrition. Cardiac surgery to optimize her hemodynamic status was also ineffective and large volume pleural and ascitic fluid losses continued. Having exhausted all other therapeutic modalities, (99m)technetium-dextran scintigraphy was performed to assess the extent of intestinal protein loss and the potential for surgical intervention. Scintigraphy suggested localized protein loss from the proximal jejunum and subsequent segmental resection was effective. Postoperatively, ascites and pleural effusions resolved, and there was no evidence of short bowel syndrome. growth has accelerated and the patient has entered puberty. There is mild persistent intestinal protein loss requiring diuretic therapy. Ascites or pleural effusions are absent, and the patient remains well >2 years after surgery. Intestinal lymphangiectasia post-Fontan procedures has traditionally been ascribed to hemodynamic factors such as raised systemic venous pressure, which would predispose to a generalized intestinal lesion. However, in this case, scintigraphy demonstrated a localized, surgically correctible lesion. To our knowledge, this is the first reported case of the use of (99m)technetium-dextran scintigraphy for this indication and of successful partial small bowel resection in such a case.- - - - - - - - - - ranking = 0.6keywords = ascites (Clic here for more details about this article) |
3/15. Acquired intestinal lymphangiectasia successfully treated with a low-fat and medium-chain triacylglycerol-enriched diet in a patient with liver transplantation.Intestinal lymphangiectasia is defined as a dilatation of small bowel lymphatic capillaries and a loss of lymph into the bowel lumen. Clinically it is characterized by hypoproteinaemia and oedema. We present here a case of protein-losing enteropathy due to intestinal lymphangiectasia after liver transplantation in a 57-year-old man who was transplanted for hepatitis c virus. Four years after liver transplantation, the patient developed hypoalbuminaemia and ascites associated with recurrence of cirrhosis. The sudden fall in serum albumin led us to look for a cause of reduction other than or in addition to cirrhosis. Duodenal biopsies showed tall villi with dilated lymphatic vessels and widening of the villi caused by oedema, demonstrating intestinal lymphangiectasia. In this case a low-fat diet supplemented with medium-chain triacylglycerols achieved an early clinical improvement with increased serum albumin levels and ascites disappearance. Intestinal lymphangiectasia should be suspected in liver-transplanted patients developing hypoproteinaemia and hypoalbuminaemia after the recurrence of cirrhosis.- - - - - - - - - - ranking = 0.4keywords = ascites (Clic here for more details about this article) |
4/15. Intestinal lymphangiectasia: a forgotten cause of chronic diarrhea.Intestinal lymphangiectasia is a rare autosomal dominant disorder or acquired condition that leads to lymph obstruction, poor chyle transport and concomitant problems. We describe the cases of two women with chronic diarrhea in whom the common signs of lymphagiectasia-hypoalbuminemia, lymphopenia and distal edema- were found. One of them also had pleural effusion and chylous ascites. The diagnosis was performed by intestinal biopsy. We herein review the histopathologic, radiographic and endoscopic features of this disorder and case reports in Mexican population.- - - - - - - - - - ranking = 0.2keywords = ascites (Clic here for more details about this article) |
5/15. Intestinal lymphangiectasia associated with angiofollicular lymph node hyperplasia (Castleman's disease).A patient presenting with predominantly gastrointestinal symptoms and a history of myocardial infarction was found to have ascites, hepatosplenomegaly, para-aortic lymphadenopathy, thrombocytosis, and a paraproteinaemia. A jejunal biopsy specimen showed lymphangiectasia and histology of the spleen and lymph nodes showed angiofollicular hyperplasia or Castleman's disease of the hyaline vascular type. This association has not previously been described and, moreover, systemic symptoms are unusual in this variant of Castleman's disease.- - - - - - - - - - ranking = 0.2keywords = ascites (Clic here for more details about this article) |
6/15. Sonography of intestinal lymphangiectasia.Real-time ultrasonography was used to evaluate three pediatric patients with biopsy-proven intestinal lymphangiectasia. The sonographic findings were ascites, diffuse bowel wall thickening, mesenteric edema, dilated mesenteric lymphatics, and thickened walls of the gallbladder and urinary bladder. Recognition of the sonographic pattern of intestinal lymphangiectasia is useful since ultrasonography is often the first imaging examination performed in children with abdominal disorders.- - - - - - - - - - ranking = 0.2keywords = ascites (Clic here for more details about this article) |
7/15. Primary intestinal lymphangiectasia: clinical and CT findings.We present two patients with biopsy proven primary intestinal lymphangiectasia in whom CT demonstrated diffuse nodular thickening of small bowel without adenopathy or hepatosplenomegaly. One patient had extensive ascites. Although the CT findings are not specific to lymphangiectasia, they may allow one to suggest the diagnosis in the patient with protein losing enteropathy and help distinguish it from inflammatory bowel disease, lymphoma, celiac disease, or whipple disease.- - - - - - - - - - ranking = 0.2keywords = ascites (Clic here for more details about this article) |
8/15. chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome.In 1964 Samman and White described 13 patients with lymphoedema of the lower extremities associated with an unusual dystrophy of the finger and toe nails: this they termed the 'yellow-nail' syndrome. Affected nails were thickened, excessively curved along both axes, very slow growing and of yellowish-grey hue; cuticle and lunula were usually absent and onycholysis was frequently evident. Lower limb lymphangiography in most individuals revealed hypoplasia, or aplasia of the lymphatics, similar to that occurring in primary lymphoedema: other patients also developed pleural effusions of high protein content or ascites suggestive of a more generalised disorder of the lymphatic system. Here we describe a patient in whom the classical 'yellow-nail' syndrome was associated with intestinal and chylous ascites.- - - - - - - - - - ranking = 1.2keywords = ascites (Clic here for more details about this article) |
9/15. A simple screening method for detecting gastrointestinal protein loss in intestinal lymphangiectasia.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.- - - - - - - - - - ranking = 0.2keywords = ascites (Clic here for more details about this article) |
10/15. Experience with peritoneo-venous shunting for congenital chylous ascites in infants and children.The management of chylous ascites presenting in association with primary lymphedema of the limbs and possibly lymphatic malformation in the lungs is difficult when the increasing abdominal distension causes respiratory distress. laparotomy may be useful in traumatic chylous ascites or in intestinal lymphangiectasia localized to a segment of the bowel. It would seem that when conservative management such as diuretics, diet, and repeated abdominal paracentesis do not improve the respiratory distress, a peritoneo-venous shunt is logical. This report of two patients with severe chylous ascites and generalized lymphatic malformations causing or accentuating respiratory distress, stresses the possibility that peritoneo-venous shunts may not have the same long term function as seen in cirrhotic ascites. Long-term follow-up on the use of these shunts specifically for chylous ascites is not available.- - - - - - - - - - ranking = 1.8keywords = ascites (Clic here for more details about this article) |
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