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Results for “Lymphangiectasis, Intestinal”

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PubMed1980

Primary intestinal lymphangiectasia. Comparison between endoscopic and radiological findings.

Two children with primary intestinal lymphangiectasia (PIL) are presented, both of whom had been prescribed an MCT supplemented diet low in long chain fatty acids. The hypoproteinemic edema improved in one patient, but persisted in the other. Following several years of dietary therapy, both subjects were re-evaluated, and a duodenoscopy and radiography of the digestive tract were performed. The duodenoscopic procedure evidenced two types of lymphangiectatic plaques on the surface of the duodenal mucosa: one form had a diameter of less than 1 mm, while the other exceeded 3 mm. The smaller lesions were seen in the child with the more favorable clinical course, whereas both types were observed in the other patient. Radiological examination disclosed the typical anomalies of intestinal lymphangiectasia only in the subject who had not responded to the dietary regimen. These results suggest that endoscopy affords a more precise assessment of the anatomic injury and could play an important role in formulating an accurate clinical diagnosis.

Child↗
PubMed1997

Expansion of the phenotype in Hennekam syndrome: a case with new manifestations.

We report on a female with lymphedema, facial anomalies, intestinal lymphangiectasia, and moderate mental retardation consistent with the diagnosis of Hennekam syndrome. In addition, she had a number of other anomalies not previously described in this autosomal recessive disorder, including a congenital heart defect, atretic ear canals, vesicoureteral reflux, and rectal prolapse.

Abnormalities, Multiple↗
PubMed1976

Congenital lymphangiectasia and atopy.

Intestinal lymphangiectasia, a disease characterized by excessive intestinal protein loss, asymmetrical peripheral edema, ascites, immunologic deficiencies, lymphocytopenia, hypoalbuminemia, imparied lymphocyte transformation, gastrointestinal symptoms and retarded growth, is the result of abnormal, distorted and obstructed lymph channels, causing rupture of intestinal lacteals from back-flow of lymph, with leakage of nutrient-laden lymph into the lumen of the bowel. A case of congenital intestinal lymphagiectasia is described, with the additional problems of allergic asthma, rhinitis, eczema and lactase deficiency. This patient, an 11-year-old child, was greatly benefited by proper allergy management (elimination diet, hyposensitization) plus restriction of fats and supplementing the diet with medium-chain triglycerides (MCT).

Asthma↗
PubMed1995

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.

Adult↗
PubMed1975

Constrictive pericarditis, intestinal lymphangiectasia, and reversible immunologic deficiency.

A patient with constrictive pericarditis, secondary intestinal lymphangiectasia, and protein-losing enteropathy was demonstrated to have the characteristic immunologic deficiency associated with intestinal lymphangiectasia: hypogammaglobulinemia, lymphocytopenia, cutaneous anergy, impaired allograft rejection. and diminished in vitro lymphocyte proliferative responses. Following surgical correction of the cardiac abnormality, the intestinal lymphangiectasia and protein-losing gastroenteropathy resolved and was accompanied by a slow but progressive return of normal immune function. This documented reversal of the immunologic deficiency in intestinal lymphangiectasia supports the concept that the immune defect in this syndrome is due to the excessive loss of lymphocytes and immunoglobulins into the gastrointestinal tract.

Adolescent↗
PubMed1986

Intestinal lymphangiectasia in an adolescent. A case study of edema.

We report a patient who presented with bilateral leg swelling, in whom a diagnosis of intestinal lymphangiectasia was made by small bowel biopsy. Failure to consider the differential diagnosis of edema and lymphedema in an adolescent could cause a considerable delay in diagnosis. We present a brief review and approach to the edematous patient.

Adolescent↗
PubMed1981

Intestinal telangiectasis in Turner's syndrome.

A 37-year old female with Turner's syndrome, iron deficiency anemia and intermittent gastrointestinal hemorrhage is described. Gastrointestinal endoscopy with biopsies, revealed telangiectasia in the duodenal bulb, the cecum and the ascending colon. Endoscopy should be performed in patients with Turner's syndrome and anemia even if there are not signs of active gastrointestinal bleeding.

Adult↗
PubMed1995

Chyloabdomen in a neonatal foal.

A 12-hour-old female standardbred foal developed signs of abdominal pain, tachycardia, tachypnoea and fever associated with chylous ascites. Small intestinal obstruction was due to segmental, mid-jejunal lymphangiectasia. Post mortem examination revealed a lack of communication between afferent and efferent lymphatic vessels in the mesenteric lymphocentre, a defect which was suspected to be congenital.

Abdominal Pain↗
PubMed2005

Aplasia cutis congenita, skull defect, brain heterotopia, and intestinal lymphangiectasia.

We describe a female infant with a previously unreported combination of manifestations characterized by aplasia cutis, skull defect, brain heterotopia, mild congenital lymphedema, and intestinal lymphangiectasia. The association of intestinal lymphangiectasia and aplasia cutis, and the association of intestinal lymphangiectasia with brain heterotopia in the lymphedema-lymphangiectasia-mental retardation syndrome have been described in single reports. In one family, the association of cortical dysplasia and congenital lymphedema have been related to mutations in the RELN gene.

Abnormalities, Multiple↗
PubMed2003

[Early-onset of primary intestinal lymphangiectasia. A case report and diet treatment].

Primary intestinal lymphangiectasia is a rare disorder, characterized by hypoproteinemia due to obstruction of the intestinal lymphatic vessels and loss of lymph fluid in the gastrointestinal tract. The case of a 3-month old patient with protein-losing enteropathy due to a primitive intestinal lymphangiectasia diagnosed with duodenal histology is reported. The adapted formula was replaced by a formula enriched with medium-chain triglycerides (MCT) and the patient presented a clinical and biochemical improvement. The importance of an early diagnosis and the efficacy of treatment with MCT is stressed.

Female↗
PubMed1999

Lymphoscintigraphic manifestations of Hennekam syndrome--a case report.

Hennekam syndrome is a rare, recently described genetic disorder in which facial anomalies and mental retardation accompany congenital lymphedema and intestinal lymphangiectasia. Several other somatic abnormalities have variously been described, as have milder degrees of lymphatic dysfunction. The authors herein describe a case of Hennekam syndrome in which the diagnostic difficulties were partially overcome by the judicious use of radionuclide scintigraphy to verify the lymphedematous component of the patient's presentation.

Adolescent↗
PubMed1985

Reduced intestinal protein loss in Crohn's disease after lymphovenous anastomosis.

In a 27-year-old patient suffering from Crohn's disease covering nearly the entire small intestine, the major problem was a marked protein-losing enteropathy which required long-term intravenous replacement. Accidentally, we disclosed a dilated thoracic duct with incompetent valves and stenosis at the junction into the subclavian vein in this patient. To reduce the intestinal protein loss, an anastomosis was performed between the thoracic duct and the internal jugular vein. Subsequently the intestinal protein loss dropped from 40 to 26% and replacement of proteins could be reduced. The patient was observed for 4 years postoperatively and the therapeutic effect continued. Besides this therapeutic aspect, the present case gives cause to consider the possible role of the lymphatics in the pathogenesis of Crohn's disease.

Adult↗
PubMed1986

Pathological findings of lymphangiectasia of the large intestine in a patient with protein-losing enteropathy.

Lymphangiectasia of the large intestine associated with protein-losing enteropathy is reported. A 33-yr-old man suffered from diarrhea, sometimes mixed with blood. Colonoscopic study revealed reddish and edematous mucosa with multiple flat elevated lesions and giant folds in the localized segment of the rectosigmoid. An intestinal clearance test of alpha 1-antitrypsin revealed the association of protein-losing enteropathy. Operation was performed successfully, resulting in a marked improvement of symptoms and laboratory data, especially serum total protein, albumin, IgG, and Leu-2a-positive cells (suppressor/cytotoxic T cells). Giant folds consisted of the submucosal edema and hyperplasia of the epithelial glands with cystic dilatation of glands, and flat elevated lesions consisted of mucosal and submucosal edema associated with intestinal lymphangiectasia, adipose tissue, and blood capillaries. The population of the Leu-2a-positive cells in the lamina propria and intraepithelial layer was decreased and that of the Leu-3a-positive cells (helper/inducer T cells) in the lamina propria was increased.

Adult↗
PubMed2004

A 55-year-old man with hypogammaglobulinemia, lymphopenia, and unrelenting cutaneous warts.

A 55-year-old white man with a history of hypertension, fibromyalgia, and colonic polyps presented with unrelenting plantar warts on his hands and feet for the past 4 years. He was otherwise healthy and without a history of recurrent infections. Physical examination was unremarkable except for extensive warts on his hands and feet. Pertinent laboratory findings included hypoalbuminemia, hypogammaglobulinemia, and lymphopenia most severely affecting CD4(+) T cells. Testing for HIV infection was negative. This clinical and laboratory presentation suggested a combined humoral and cellular immunodeficiency syndrome that could be best explained by loss of lymphocytes, immunoglobulins, and other serum proteins. Additional immunologic testing revealed a marked reduction in peripheral blood naive (CD4(+)CD45RA(+)) T cells. A 24-hour stool collection showed a markedly elevated alpha(1)-antitrypsin level. These findings were most consistent with the diagnosis of intestinal lymphangiectasia, a type of protein-losing enteropathy associated with hypoalbuminemia, hypogammaglobulinemia, and lymphopenia, characterized by a preferential loss of naive CD4(+) T cells into the gastrointestinal tract. This case illustrates the importance of considering intestinal loss of immunoglobulins and lymphocytes in the differential diagnosis of the adult patient who presents with laboratory evidence of a combined humoral and cellular immunodeficiency. It also underscores the diagnostic utility of the clinical immunology laboratory and how flow cytometry, in particular, can contribute to an understanding of pathogenic mechanisms.

Agammaglobulinemia↗
PubMedDate not supplied

[Conception and pregnancy without complications in parenteral home nutrition].

A case is reported of conception and successful completion of a pregnancy in a 31 year old woman maintained on home parenteral nutrition while suffering from a primary distension of the intestinal lymphatic vessels and intestinal loss of protein. A healthy, 3400 g female infant was born near term by cesarean section. This case confirms the few so far published experiences showing that conception and maintenance of the pregnancy under total parenteral nutrition needs no or just a slight adaptation of the regimen.

Adult↗
PubMed2000

Two brothers with Hennekam syndrome and cerebral abnormalities.

We report two brothers with mental retardation, lymphoedema of the limbs and facial anomalies. Hennekam et al. (Am J Med Genet 34:593-600; 1989) described four patients with identical signs and intestinal lymphangiectasia. To confirm the diagnosis of Hennekam syndrome we undertook a duodenal biopsy from the older brother which revealed intestinal lymphangiectasia. So far only one patient with Hennekam syndrome and cerebral abnormalities has been described. This patient presented with pachygyria in the parietal area. Cerebral MRI in our two cases revealed small subcortical hyperintensities in both patients and a large cystic lesion in the younger patient probably representing an old media infarction.

Abnormalities, Multiple↗
PubMed2003

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.

Adolescent↗