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We report the case of a child with 22q11 microdeletion who presented with abdominal lymphatic dysplasia resulting in exsudative enteropathy. This primitive and localized lymphatic malformation is consistent with the vascular theory in the velocardiofacial syndrome.
Authors analysed in an infant with primary intestinal lymphangiectasia the number of intraepithelial lymphocytes and the distribution of T lymphocyte subpopulations in the jejunal mucosa with immunohistochemical methods. It was established that the number of intraepithelial lymphocytes and of the cells belonging to the various T lymphocytes markedly decreased in the patient compared to controls, however the decrease of the CD4 cells was less than that of CD8 cells, thus the CD4/CD8 ratio increased. Authors conclude that the increase of CD4/CD8 ratio in the jejunal mucosa may explain the absence of frequent gut infections in intestinal lymphangiectasia.
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Protein-losing enteropathy secondary to intestinal lymphangiectasia was diagnosed in a 1 1/2 -year-old female Doberman Pinscher. Poor growth rate, intermittent diarrhea, ascites, edema, hypoproteinemia, grossly dilated intestinal lymphatics, and impaired fat absorption characterized the disease. There was histopathologic evidence of dilatation of lacteals and excessive chromium (51Cr)-labeled human albumin in the feces following its intravenous administration. Sixteen weeks of a special diet (high in carbohydrates and medium-chain triglycerides; low in long-chain triglycerides) led to remission of clinical signs. Serum albumin increased over its initial value of 1.09 to 1.7 g/dl, while serum globulin increased from 1.71 to 1.9 g/dl. Following 9 months of dietary therapy, serum albumin increased to 2.1 g/dl and serum globulin increased to 2.6 g/dl.
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A 43-year old patient came to our clinic with chronic diarrhea. Determination of the faecal alpha 1-antitrypsin-clearance led to the diagnosis of exsudative enteropathy. Blood counts showed pronounced lymphocytopenia. Histopathological findings from intestinal and colorectal biopsies showed diffuse lymphangiectasis. Following exclusion of secondary types, our diagnosis was primary intestinal lymphangiectasis. Additional distinctive morphological and anamnestic features strongly suggested presence of Noonan's syndrome. Characteristic manifestations of Noonan's syndrome include changes in the lymphatic vessels in accordance with primary lymphangiectasis. Frequently, these changes are localized in the lungs. To date, only rare cases of intestinal lymphangiectasia in Noonan's syndrome have been reported. Treatment consisted of a protein-rich diet, with reduced fat content enriched by middle-chain fatty acids, as well as twice-daily injections of 200 micrograms octreotide. With this therapy, the symptoms improved.
Congenital intestinal lymphangiectasia is a rare protein-losing enteropathy that is characterized by diarrhea and peripheral edema. This report presents a 37-yr-old woman who had suffered from recurrent diarrhea and peripheral edema since her early childhood and who was admitted for severe attacks of abdominal pain. A diagnosis of intestinal lymphangiectasia was made endoscopically, histologically, and radiographically. Laparotomy revealed complete fibrotic entrapment of the small bowel, which caused partial mechanical bowel obstruction. Surgical decortication led to recovery. To the best of our knowledge, this is the first report on fibrotic entrapment of the small bowel in a patient with long lasting intestinal lymphangiectasia.
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.
A 47-yr-old man with hepatitis B virus associated liver cirrhosis was admitted to our hospital with diarrhea and generalized edema and diagnosed as protein-losing enteropathy due to intestinal lymphangiectasia by intestinal biopsy and 99m Tc albumin scan. During hospitalization, he received subcutaneous octreotide therapy. After 2 weeks of octreotide therapy, follow-up albumin scan showed no albumin leakage, and the serum albumin level was sustained. We speculate that liver cirrhosis can be a cause of intestinal lymphangiectasia and administration of octreotide should be considered for patients with intestinal lymphangiectasia whose clinical and bio-chemical abnormalities do not respond to a low-fat diet.
The two cases reported here show intestinal obstruction may occur by coagulation of lymph around the loops. They permit us to better understand the physiopathology of blockage of the lymph vessels whether congenital as in the first case or traumatic as in the second case. Traumatic rupture of the cisterns chyli is exceptional. The histological lesions observed on clamped biopsies show clearly the pathology of protein-losing enteropathy.
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The case of a 60 year old man in whom diarrhea, steatorrhea and protein losing enteropathy developed one year after the diagnosis of Waldenström's macroglobulinemia is reported. Histological study of multilevel peroral biopsies of the small bowel mucosa showed the following features: a) lymphangiectasia, b) intralymphatic and interstitial deposits composed of lipids and immunoglobulin M of the kappa type (as demonstrated by specific histoimmunofluorescence), c) a mild lymphoplasmocytoid infiltrate, corresponding to the location of Waldenström's macroglobulinemia. The patient died six months later, in spite of improvement of his digestive tract functional abnormalities by a low-fat diet. Sixteen cases of small intestinal involvement have been reported during the course of Waldenström's macroglobulinemia, of which only one showed features similar to those found in our case. Lymphangiectasia appear to be due to mesenteric lymph node involvement by the tumoral process, and/or to increased blood viscosity; in turn the lymphangiectasia seem to be responsible for steatorrhea and plasma protein losses, since in our case both lipids and the immunoglobulin M-kappa were shown to cross the villous epithelium by immunohistochemistry.
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The case of a woman with SLE and protein-losing enteropathy secondary to intestinal lymphangiectasia is described. Corticosteroid therapy improved all clinical symptoms, laboratorial findings and intestinal histological alterations.
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