• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.5
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• pp.413-422
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• 2021

Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy. Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. After exclusion of secondary intestinal lymphangiectasia, magnetic resonance lymphangiography is a promising tool for the assessment of abnormal lymphatic lesions in primary intestinal lymphangiectasia. Determining the extent of the lesion provides direction for treatment options. Focal short-segment intestinal lymphangiectasia can be treated via intestinal resection or radiologic embolization after dietary therapy failure. Diffuse intestinal lymphangiectasia and extensive lymphangiectasia should be treated with several drugs with a full understanding of their mechanisms.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.921-925
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• 2003

Intestinal lymphangiectasia, one of the protein-losing gastroenteropathies, is an uncommon disease characterized by dilated intestinal lymphatics, enteric protein loss, edema, hypoalbuminemia, and lympocytopenia. Small bowel biopsy and CT have been used to confirm the diagnosis of intestinal lymphangiectasia. Small bowel biopsy shows collections of abnormal dilated lacteals in submucosa with distortion of villi and CT findings have been described as diffuse nodular thickening of the small bowel and as linear hypodense streaking densities in the small bowel caused by dilated lymphatic channels. Demonstration of increased enteric protein loss using $^{51}Cr-$, $^{131}I-$ or $^{99m}Tc-labeled$ albumin, timed measurement of fecal excretion of radioactivity or by measuring fecal clearance of alpha 1-antitrypsin can also help the diagnosis. We experienced a rare case of intestinal lymphangiectasia in an eight year old boy who presented with facial edema, abdominal distension and intermittent diarrhea. We report a patient with intestinal lymphangiectasia, in whom abdominal CT, $^{99m}Tc-labeled$ albumin scintitigraphy, and stool alpha 1-antitrypsin measurement played key roles in determining the diagnosis. A brief review of literature was made.

• Journal of Veterinary Clinics
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• v.23 no.2
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• pp.175-179
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• 2006

A 7-year-old, 3.16 kg intact male Yorkshire terrier had the history of abdominal distension, diarrhea, and weight loss. On the basis of history takings, physical examination, laboratory tests, radiography, ultrasonography, exploratory laparotomy, and histopathological examination, the dog was diagnosed definitely as intestinal lymphangiectasia. In this case, signs and results are consistent with those of other reports, but some clinicopathological findings such as hypocalcemia and hypocholesterolemia are not. This means that the findings were not completely in accord with the typical ones of intestinal lymphangiectasia in this dog. Prednisolone was prescribed to treat. However, it was not effective sufficiently. Thus, azathioprine was added to the regimen used in the first trial, and it was quite efficient in inducing remission in intestinal lymphangiectasia. The clinical signs were improved to the combined therapy. This case report demonstrates that the combined therapy for intestinal lymphangiectasia can be used as an alternative to only glucocorticoid therapy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.253-259
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• 2004

Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.93-98
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• 1999

We experienced a case of primary intestinal lymphangiectasia in a 20-month-old boy, which was confirmed gastrofiberscopically and histologically only after a high-fat meal before the procedure. A characteristic endoscopic finding was the appearance of tiny white dots scattered in the duodenal mucosa, which were proved dilated lymph vessels in the lamina propria on histological examination. Treatment with low-fat diet and medium chain triglycerides supplementation was satisfactory.

• Journal of Veterinary Clinics
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• v.28 no.2
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• pp.262-266
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• 2011

A 4-year-old intact male, Maltese dog weighing 2.2 kg was presented for evaluation of tachypnea and hyperthermia. On initial evaluation, the dog was dyspneic, but alert and responsive. Muffled heart sound was noted with auscultation of the right hemithorax. The radiographic findings were consolidation of the right middle lung lobe, pleural effusion, and abdominal fluid accumulation. Thoracic computed tomography confirmed a contrast-enhancing mass within the right middle lung lobe. Surgical exploration of the thoracic cavity was performed using a right fifth intercostal thoracotomy. Examination of the right lung lobe revealed $360^{\circ}C$ torsion of the right middle lung lobe at the level of the hilus, in a clockwise direction. Lung lobectomy was performed using a thoracoabdominal stapling device with 2.5-mm vascular staples. Full-thickness intestinal biopsy by exploratory laparotomy was taken from the jejunum for histological assessment. Histopathologic examination of the full-thickness intestinal biopsy revealed distended lacteal located within the submucosa of the jejunum. Intestinal lymphangiectasia was resolved with prednisone and low fat diets. Subsequent communication with the owner revealed that the patient was in good health 6 months post-discharge.

• Journal of Veterinary Science
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• v.24 no.2
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• pp.25.1-25.6
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• 2023

An eight-year-old Maltese dog presented with diarrhea and anorexia. Ultrasonography revealed marked focal wall thickening with loss of layering in the distal ileum. Contrast-enhanced computed tomography (CT) revealed a preserved wall layer with hypoattenuating middle wall thickening. In some segments of the lesion, small nodules protruding toward the mesentery from the outer layer were observed. Histopathology revealed focal lipogranulomatous lymphangitis (FLL) with lymphangiectasia. This is the first report to describe the CT features of FLL in a dog. CT features of preserved wall layers with hypoattenuating middle wall thickening and small nodules can assist in diagnosing FLL in dogs.

• Proceedings of the Korean Society of Veterinary Clinics Conference
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• 2007.05a
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• pp.142-142
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• 2007

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.6
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• pp.594-600
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• 2019

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, postdouble switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ${\leq}0.9g/dL$) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.923-927
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• 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.