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A 9.52 kg, 9-year-old, spayed female beagle was presented with the chief complaint of abdominal distention of 1 week's duration. A presumptive diagnosis of canine intestinal lymphangectasia was arrived at by exclusion of other causes for the patient's ascites. The patient was successfully treated with dietary modification and immunosuppressive therapy.
Surgical management of a case of intestinal lymphangectasis with unusual features is reported. The view that the picture was one of early segmentary enteritis was supported by some aspects of the course and various anatomopathological features.
A 23-year old male patient had eight distinct episodes of hemolytic uremic syndrome (HUS) between 8.5 and 15 years of age, five of them accompanied by hypocomplementemia. In the further course, severe hypertension, renal insufficiency as well as protein-losing enteropathy due to intestinal lymphangiectasia developed, whilst hypocomplementemia persisted. The association of recurrent HUS with hypocomplementemia and intestinal lymphangiectasia may represent a new association within a subgroup of the atypical HUS.
Multiple cases with various types of pediatric malabsorption syndromes were evaluated. The clinical manifestations, laboratory findings, pathophysiology, and histopathological descriptions of each patient were analyzed in an effort to clear the pathogenesis of the malabsorption syndromes and the treatments were undertaken. The cases studied, included one patient with cystic fibrosis, two with lactose intolerance with lactosuria (Durand type), one with primary intestinal lymphangiectasia, two with familial hypobetalipoproteinemia, one with Hartnup disease, one with congenital chroride diarrhea, one with acrodermatitis enteropathica, one with intestinal nodular lymphoid hyperplasia (NLH), five with intractable diarrhea of early infancy and four with glycogenosis type Ia. Each case description and outcome is described below: 1. A 15-year-old Japanese boy with cystic fibrosis presented with severe symptoms, including pancreatic insufficiency, bronchiectasis, pneumothorax and hemoptysis. His prognosis was poor. Analysis of the CFTR genes of this patient revealed a homozygous large deletion from intron 16 to 17b. 2. In the sibling case of Durand type lactose intolerance, the subjects'disaccaridase activity of the small bowel, including lactase, were within normal limits. The results of per oral and per intraduodenal lactose tolerance tests confirmed lactosuria in both. These observations suggested, not only an abnormal gastric condition, but also duodenal and intestinal mucosal abnormal permeability of lactose. 3. In the case of primary intestinal lymphangiectasia, the subject had a lymphedematous right arm and hand, a grossly coarsened mucosal pattern of the upper gastrointestinal tract (identified via radiologic examination) and the presence of lymphangiectasia (confirmed via duodenal mucosal biopsy). The major laboratory findings were hypoalbuminemia, decreased immunoglobulin levels and lymphopenia resulting from loss of lymph fluid and protein into the gastro-intestinal tract. 4. In two cases of heterozygous familial hypobetalipoproteinemia, serum total cholesterol and betalipoprotein levels were very low. The subjects presented with symptoms and signs of acanthocytosis and fat malabsorption. Further, one subject had neurological abnormalities such as mental retardation and severe convulsions. Treatment with MCT formula diet corrected the lipid malabsorption. 5. A 5-year-old girl presented with pellagra-like rashes, mental retardation and cerebellar ataxia. An oral tryptophan (Trp) and dipeptide (Trp-Phe) loading test were conducted and the renal clearance of amino acids was also evaluated in this patient and in controls. Following the oral Trp loading test, plasma levels of Trp indicated a lower peak in the case, reaching a maximum at 60 minutes. On the other hand, the oral dipeptide (Trp-Phe) loading test in the Hartnup patient showed the peak Trp plasma level was the same as the control subjects. The renal clearance of neutral amino acids in this case increased to levels 5 to 35 times normal. 6. In the case of congenital chloride diarrhea, the subject had secondary lactose intolerance, dehydration, hyponatremia, hypokalemia, hypochloremia, hyperreninemia and metabolic alkalosis. The chloride content of her fecal fluid was very high. The concentrations were 89-103 mEq/l. In contrast, her urine was chloride-free. The subject's growth and development improved after treatment with lactose free formura and oral replacement of the fecal loses of water, NaCl and KCl. Unfortunately, the patient died of a small bowel intussusception. The kidney histopathological finding was juxtaglomerular hyperplasia by a necropsy. 7. In the case of acrodermatitis enteropathica, the subject had characteristic skin lesions, low serum zinc levels and ALPase activity. An oral ZnSO4 loading test and intestinal mucosal histology by a peroral biopsy were conducted. The serum zinc peak level was 2 hours after the oral ZnSO4 loading test. Infant formula alone could not maintain normal serum zinc ranges. Light microscopic studies of the intestinal villous architecture showed a normal pattern. However, ultrastructual examination of several epithelial cells revealed numerous intracellular vesicles. After zinc therapy, these changes were decreased. The lesions were postulated as the secondary result of zinc deficiency. 8. A 12-year-old girl presented with hypogammaglobulinemia, recurrent infections, chronic diarrhea and intestinal NLH. A barium meal and follow-through examination showed multiple nodules throughout the stomach and intestine. The nodules, all uniform in size, were 2 mm diameter. The barium enema did not show NLH in the colon. Mucosal biopsy of the stomach and jejunum revealed the typical histology of NLH in the lamina propria. Also, achlorhydria was present in this patient and her serum gastrin levels were very high; 315-775 pg/ml. 9. In 4 cases of intractable diarrhea in early infancy (by Avery G B), a jejunal biopsy showed shortening villi and nonspecific enterocolitis. Some patients were found with only low lactase or low lactase and sucrase levels. An electron microscope analysis of the small bowel in 2 cases showed alterations: increased pinocytosis in microvillus membranes and lysosomes by endocytosis of undigested macromolecular substances. I postulated that the stated evidence was causative of this clinical profile. 10. I frequently observed diarrhea as a clinical manifestation in glycogenosis type Ia and lipid malabsorption in one case. The light and electron photomicrographs showed intestinal absorption cells with the glycogen deposits in the inferior devision of nuclei.
We report on an Italian patient affected by severe lymphedema of lower limbs, genitalia and face, intestinal lymphangiectasia, seizures, and moderate mental retardation. He has a flat face, flat nasal bridge, and hypertelorism. We propose that he presents with a severe form of Hennekam syndrome.
This is a report of 3 Chinese children with intestinal lymphangiectasia in Malaysia. Two children responded to a low fat diet and medium-chain triglyceride supplement. The third child has recurrent chylous ascites. None of the children has recurrent infections despite low CD4+ cells and low levels of IgG and IgA. Intestinal lymphangiectasia is a rare congenital disorder of the mesenteric lymphatic that leads to the obstruction of the lymphatics of the intestine and protein losing enteropathy. Restriction of dietary fat intake will usually result in remission. Recurrent chylous ascites is a problem and management can be difficult.
It report three cases of Primary Intestinal Lymphangiectasia that presented the common symptoms of diarrhea, oedema and hipoalbuminaemia in which we could observed the clinics variations that can to present this entity. It demonstrated the importance of the intestinal biopsy to get a correct diagnosis just as the essencial of use of the medium chain triglycerides with the restriction of the common fast, to recuperate these patients.
Intestinal lymphangiectasia is a disorder of the lymphatics of the gastrointestinal system that usually manifests itself with protein-losing enteropathy, malabsorption, and ascites. We report an adult patient with this disorder with an unusual presentation simulating an acute abdomen.
Primary lymphoedema associated with chylous reflux is a very rare clinical entity. We report a 3-year-old girl with unilateral lymphoedema, xanthomatosis and vaginal lymphorrhoea. Biopsy also revealed intestinal lymphangiectasia. This paper also presents a brief review of the literature and draws attention to the significance of the xanthomatous eruption in the diagnosis of a chylous reflux.
Disorders of the intestinal lymphatic transport system are rare and typically associated with protein losing enteropathy (PLE). Hypoproteinemia caused by intestinal lymphangiectasia is often associated with lymphedema of the legs and occasionally with chyluria, chylometrorrhea and chylous ascites. This article examines the varied presentations of lymphangiectasia syndromes including its pathophysiology. Diagnosis is based on signs and symptoms, specific laboratory findings, and confirmed by contrast small bowel series, lymphography and best of all laparoscopy. We describe 12 patients with PLE secondary to primary intestinal lymphangiectasia (1980-1991). Treatment was non-operative (dietary) in 8 patients and surgical in 4 including segmental resections of the jejunum in two, lymphatic-mesenteric venous anastomosis in one, and peritoneal-venous (LeVeen) shunt in one with overall satisfactory results.
We report on a patient with protein-losing enteropathy due to primitive intestinal lymphangiectasia with an early reversal of clinical and biochemical signs and a stable late reversal of pathologic signs after treatment with a hypolipidic diet enriched with medium-chain triacylglycerols.
Two cases with roentgenologic findings of extravasation of contrast medium into the stomach and colon following lymphangiography are presented. One is clinically diagnosed as primary intestinal lymphangiectasia; the other as retroperitoneal spread from uterine cancer. The significance of lymphangiography in gastrointestinal or retroperitoneal disorders is discussed.
A 3-yr-old intact male dingo (Canis familiaris dingo) presented with a 3-mo history of diarrhea. The diarrhea did not resolve with antibiotics or intestinal protectants. Fecal examination for parasites, fecal cultures, physical examination, and radiographs were unremarkable. Enteroscopic duodenal biopsies showed dilated lacteals without inflammation. Results of serum folate, cobalamin, and trypsin-like immunoreactivity were normal. Low serum total protein and albumin combined with increased fecal levels of alpha-1 protease inhibitor suggested the diagnosis of lymphangiectasia. Full-thickness intestinal biopsies of the duodenum, jejunum, and ileum revealed dilated mucosal and submucosal lacteals without associated inflammation, confirming the diagnosis of primary lymphangiectasia. Currently, the dingo is being maintained with nutritional management.
We report a male patient with increased birth weight and growth, cutis marmorata, macrocephaly, large hands and feet, thick subcutaneous tissues, postaxial polydactyly, linear skin hyperpigmentation following the lines of Blaschko, and intestinal lymphangiectasia. Although the findings resemble the recently defined macrocephaly cutis marmorata syndrome, some findings suggest that this might be a new disorder. Differential diagnosis are discussed, with a review of the literature.
This case illustrates the difficulties which occasionally are encountered in diagnosing and treating small intestinal diseases. The delay in diagnosis was due to the fact that symptoms started in 1950 but the disease was not described until 11 years later. Once the diagnosis was established, there was an unexplained failure of response to appropriate therapy.
Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. Endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. Histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables. These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.
A 22-year-old female with protein losing enteropathy due to congenital intestinal lymphangiectasia who developed non-Hodgkin's lymphoma of the bone, is described. Complete remission of the lymphomatous process and disappearance of the gastrointestinal symptoms were achieved following treatment with combination chemotherapy and local irradiation.