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Results for “Lymphangiectasis, Intestinal”

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PubMed1999

Bilateral groin adenolymphoceles: an unusual presentation of chylous reflux.

We report an unusual presentation of a young man with bilateral groin lymph nodal adenolymphoceles and right leg lymphedema as a manifestation of intestinal lymphangiectasia. Chylous reflux was supported by conventional and isotopic lymphography as well as by a total lipid test showing delayed triglyceride absorption 24 hours after ingestion of 60 gm of butter. After excision of groin masses in conjunction with dietary control (short-medium chain triglycerides), manual massage, pneumatic compression, and long-term wearing of a low stretch elastic garments he remains well.

Adult↗

PubMed1981

Synopsis of endoscopic and other morphological findings in intestinal lymphangiectasia.

Intestinal lymphangiectasia may present as a primary congenital disorder as well as a secondary form due to numerous underlying diseases. Endoscopically, whitish swollen tips of the villi are fairly characteristic; histologically, dilatation of the lymphatics can be demonstrated. By means of electron microscopy dilated intercellular spaces with aggregation of protein precipitations as well as ectatic lymph vessels with abnormal structure are found. Possible relations to the pathomechanism are discussed.

Duodenum↗

PubMed1986

Endoscopic diagnosis of primary intestinal lymphangiectasia using a high-fat meal.

The case of a 17-year-old woman is described, in whom a suspected diagnosis of primary intestinal lymphangiectasia was confirmed endoscopically and histologically only after a high-fat meal had been given the night before the procedure. A characteristic endoscopic finding was the appearance of tiny white dots scattered in the duodenum and jejunum, which on histological examination proved to be dilated lymph vessels. Treatment with low-fat diet and MCT-supplementation was successful.

Adolescent↗

PubMedDate not supplied

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.

Adult↗

PubMed1996

Protein-losing enteropathy due to intestinal lymphangiectasia accompanied by mesenteric lymph node fibrosis.

An autopsy case of a 74-year-old man suffering from edema in the legs and diagnosed as protein-losing enteropathy due to intestinal lymphangiectasia is presented. He underwent a radical operation and radiotherapy for esophageal carcinoma at age 65 years and the onset of protein-losing enteropathy was at age 70. Postmortem examination disclosed widespread abnormalities of the duodenal and small intestinal lymphatics and mesenteric lymph node fibrosis. The mucosal lymphatic capillaries were markedly dilated. In the submucosal and serosal layers, the lymphatic vessels showed marked dilatation in some areas and fibrous occlusion with very thick muscle layers in others. All these lymphatic changes were presumed to be attributable to a reactive process secondary to lymph congestion, which it is suggested, was caused by the mesenteric lymph node fibrosis.

Aged↗

PubMed1986

Dietary management of intestinal lymphangiectasia complicated by short gut syndrome.

The dietary management of a child with intestinal lymphangiectasia complicated by short gut syndrome following surgery at the age of 3 weeks is described. Diets containing amino acids and peptides were not tolerated due to high osmolality. Satisfactory nutrition was achieved by using a feed with low osmolality and containing whole whey protein, maltodextrin, medium-chain triglycerides (MCT) and a small amount of long-chain triglycerides (LCT) to supply essential fatty acids (RD231).

Female↗

PubMed1989

Autosomal recessive intestinal lymphangiectasia and lymphedema, with facial anomalies and mental retardation.

We report on two male and two female relatives with intestinal lymphangiectasia; severe lymphedema of limbs, genitalia, and face; facial anomalies; seizures; mild growth retardation; and moderate mental retardation. Main facial anomalies are a flat face, flat nasal bridge, hypertelorism, small mouth, tooth anomalies, and ear defects. Their parents are consanguineous. This disorder probably is an hitherto undescribed autosomal recessive syndrome.

Abnormalities, Multiple↗

PubMed1976

Intestinal lymphangiectasia: the variability of presentation. A study of five cases.

Five patients with intestinal lymphangiectasia are described. They all had evidence of protein-loss from the gut but this was of a variable degree. In four patients the diagnosis was first suggested by the appearances of jejunal biopsy. The factors influencing presentation are discussed as are the possible co-existence of other gastronitestinal disorders with this congenital abnormality. It seems likely that the condition is commoner than hitherto believed.

Adolescent↗

PubMed2005

Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia.

Primary intestinal lymphangiectasia (PIL), so-called Waldmann's disease, is an uncommon condition, characterized by dilated intestinal submucosal and subserosal lymphatics of the gastrointestinal tract. Protein-losing enteropathy is the most common manifestation of this supposed congenital disease. Since the initial description in 1961, 11 cases of lymphoma have been reported suggesting that PIL predisposes to lymphoma. Here, we report the first case of primary nodal location lymphoma during PIL with recovery of the protein-losing enteropathy after its treatment by radiochemotherapy.

Adult↗

PubMedDate not supplied

[Necrolytic migratory erythema in Waldmann's disease].

BACKGROUND: We report a case of necrolytic migratory erythema in a patient with Waldmann's disease. PATIENTS AND METHODS: A 55-year-old male patient with a history of Waldmann's disease was hospitalized for a rash on the trunk and limbs comprising annular polycyclic lesions with peripheral scaling evocative of necrolytic migratory erythema. High-protein and fatty-acid-supplemented parenteral feeding led to rapid improvement of the patient's cutaneous lesions. DISCUSSION: Waldmann's disease is characterized by intestinal lymphatic abnormalities leading to exudative intestinal disease causing protein loss in the bowel lumen and deficient fatty acid absorption. The pathogenesis of necrolytic migratory erythema is not fully understood. Increased serum glucagon does not appear to be the only mechanism involved. The occurrence of necrolytic migratory erythema in a patient with Waldmann's disease supports the current physiopathological hypothesis of the role of decreased plasma protein and amino acid levels in necrolytic migratory erythema.

Dietary Fats↗

PubMed1990

[Radiologic aspects of a case of Waldmann's disease].

The Waldmann's disease or intestinal lymphangiectasia is a rare disease. We observed a case which occurred in a 14 years old boy. The disease was discovered in the screening of an intermittent diarrhea. The diagnosis was suspected on biological signs of protein-losing enteropathy and the presence of lymphangiectasias seen during endoscopy. The lymphography was conclusive showing the abnormal opacification of the mesenteric region. The other examinations were also important to assert the idiopathic origin of the disease.

Adolescent↗

PubMed1996

A case of the yellow nail syndrome associated with massive chylous ascites, pleural and pericardial effusions.

A 26-year-old male patient with a history of chronic peripheral lymphedema, yellowish coloured slow growing nails and pleural effusions since early childhood is described. After 23 years he developed a chylous ascites and scintigraphy with technetium-99m labeled albumin clearly demonstrated a diffuse protein loss involving the whole jejunum and ileum. Subsequent jejunal and duodenal biopsies showed the typical histological findings of intestinal lymphangiectasia thereby confirming a diffuse intestinal lymphatic damage. In addition to the gastrointestional symptoms the patient developed a pericardial effusion diagnosed by echocardiographic imaging. Dietary treatment with middle chained triglycerides and intravenous human albumin supplementation was followed by the reduction of the ascites and improvement of the peripheral lymphedema. To our knowledge this is the first description of the yellow nail syndrome associated with a diffuse lymphangiectasia involving the whole small bowel.

Adult↗

PubMed1975

Intestinal lymphangiectasia. Long-term results with MCT diet.

The clinical course of 6 children with primary intestinal lymphangiectuasia who have been treated with low fat medium chain triglyceride-supplemented diets for between 3 and 8 years (4 for longer than 5 years) is described. Though laboratory findings indicate continuing chyle leak, evidence for long-term benefit from dietary treatment is provided by symptomatic relief while on the diet, clinical relapse upon relaxation of t,e regimen, and improvement in growth rates. In most patients the underlying lymphatic defect, and thus the need for dietary treatment, appears to be permanent.

Body Height↗

PubMed1985

Aplasia cutis congenita and intestinal lymphangiectasia. An unusual association.

Aplasia cutis congenita (ACC) is a rare skin defect usually localized to the vertex. It has been reported in association with other disorders involving mainly ectodermal and mesodermal structures. We discovered the association of ACC and intestinal lymphangiectasia (IL) in a patient and probably in his brother. At birth, the propositus had ACC of the vertex and edema, which persisted for six months. At 3 years of age he presented with generalized edema and was found to have hypoproteinemia and lymphopenia. Radioisotope studies and a small-intestinal biopsy confirmed the diagnosis of IL. On a fat-free, medium-chain triglyceride-containing diet, clinical and laboratory findings returned to normal. A sibling born one year after his brother's presentation had nonpitting limb edema and extensive ACC of the vertex with an underlying bony defect. He died in shock at 2 months of age, after sudden profuse bleeding from the sagittal sinus. The association between ACC and IL is another example of combined anomalies with both ectodermal and mesodermal involvement that is most probably not coincidental.

Child, Preschool↗

PubMed1984

Intestinal lymphangiectasia in the Lundehund. Scanning electron microscopy of intestinal mucosa.

Intestinal lymphangiectasia of obscure etiology is frequent among Norwegian Lundehunds ("puffin-dog"). A study of three Lundehunds with typical symptoms, including wasting, diarrhea and ascites, revealed segmental distention of lymphatics resembling primary lymphangiectasia in man. Atrophy, fusion and balloon-like swelling of villi with occasional rupture of lacteals were seen. Subepithelial fluid accumulation caused bulging of the epithelium. Microvilli showed partial or complete atrophy. The protein loss in the Lundehund enteropathy may be due to rupture of lacteals, increased paracellular permeability, caused by enhanced hydrostatic tissue pressure, and decreased absorption through a malformed brush border.

Animals↗

PubMedDate not supplied

[Exudative enteropathy in congenital lymphedema-lymphangiectasia syndrome].

BACKGROUND: Congenital peripheral elephantiasiformic alterations are very rare in paediatric patients. In a patient with lymphangiectasia-lymphedema syndrome we demonstrate over a 8-year follow-up that not only cosmetic and social indications for surgical treatments but also internal care become important during the course. PATIENT: We report on a boy with congenital lymphedemas of the extremities and the genital region, which were several times surgically treated. The patient became symptomatic firstly with tetanic cramps caused by malabsorption syndrome due to intestinal lymphangiectasia at the age of 6 years. Synopsis of clinical and laboratory findings and the patient's course are pointing to a mild Hennekam syndrome with still unknown aetiology. RESULTS: The boy developed adequately with permanent oral substitution of electrolytes and vitamins, protein-rich diet, supplementation of medium-chain fatty acids and compressing bandages. Infusions of human albumin to correct persistent hypalbuminemia as well as cytostatic treatment with cyclophosphamide as a formal trial were ineffective and are not advisable, therefore.

Child↗

PubMed1982

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.

Body Weight↗

PubMed2002

Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy.

Lymphangiectasia is a congenital or acquired disorder characterized by abnormal, dilated lymphatics with a variable age of presentation. We describe a case of lymphangiectasia with intestinal and pulmonary involvement in an adolescent female, who presented with many of the classic features including chylous pleural effusions, lymphopenia, hypogammaglobinemia, and a protein-losing enteropathy. She also presented with recurrent lower gastrointestinal bleeding, which is infrequently described. The patient did not improve with bowel rest and a low-fat medium-chain triglyceride diet and had little improvement with octreotide acetate therapy. However, she had a clinical response to antiplasmin therapy, trans-4-aminothylcyclohexamine carboxylic acid (tranexamic acid) in terms of serum albumin and gastrointestinal bleeding. She continues to have exacerbations of her condition, as well as persistent lymphopenia and chronic pleural effusions.

Adolescent↗