[Spontaneous chylothorax in pulmonary lymphangiectasis].
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The Great Arteries Transposition corrected by Mustard's repair is often complicated with inferior vena cava obstruction with consequent intestinal lymphangiectasia and protein-losing enteropathy. We report a case of a 9 years old boy who underwent Mustard's repair and presented diarrhoea, abdominal pain, hypoproteinemia and ascites. The fecal elimination of 51Cr-labeled albumin was remarkable increased and the intestinal biopsy showed a noteworthy lymphangiectasia due to an hemodynamic overload. Therefore subjects with Great Arteries Transposition corrected by Mustard's repair should be valued in the post-surgical follow-up by 51Cr-labeled albumin fecal elimination and by intestinal biopsy, already sensitive in asymptomatic phase.
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The morphology of the intestinal mucosa was reviewed using light microscopy and transmission electron microscopy from a two year-old male patient with protein losing primary intestinal lyphangiectasis. Diagnostic studies ruled out recognized forms of secondary intestinal lymphangiectasia (e.g cardiac, hepatic, renal diseases or lymphoreticular cancer). Furthermore, certain features distinguish this patient from the secondary type: decreased immunoglobulin and albumin levels, lymphocytopenia, chylous ascitis and delated lymphatics on small-bowel biopsy.
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A literature review of intestinal lymphangiectasia in the dog is presented. A Yorkshire terrier with acquired lymphangiectasia as the result of enteritis is described. The single clinical symptom was marked ascites. Diagnosis was confirmed by histology, demonstrating dilated lymph vessels in the intestinal villi of the duodenum.
Primary intestinal lymphangiectasia (PIL), first described in 1961, is a rare disease of childhood. Oedema, hypoproteinaemia and diarrhoea are characteristic symptoms. Bioptic demonstration of dilated lymphatic capillary vessels in intestinal villi and increased intestinal protein loss are diagnostic. Two patients successfully treated with a low fat diet, containing medium chain triglycerides (MCT) are reported.
Although rarely, several conditions may cause malabsorption through lymphatic obstruction. Primary lymphangiectasia, a genetically determined disease characterized by diarrhoea, steatorrhoea and protein-losing enteropathy, is one of these conditions. The Authors report their experience in three cases of small bowel lymphangiectasia occurring in adults and discuss diagnostic and therapeutic problems of the disease.
INTRODUCTION: Lymphangiectasia are different from lymphangioma because they arise following damage to the deeper lymphatic vessels. No clinical or histological features are known to distinguish lymphangioma from lymphangiectasia. CASE REPORT: We report a case of vulvar lymphangiectasia occurring 14 years after therapy of squamous carcinoma of the cervix (surgery and post-operative radiotherapy). Our treatment was cryosurgery. DISCUSSION: We analysed the 12 case reports of vulvar lymphangiectasia in the literature, emphasizing misleading clinical aspect of the warty lesions and the risk of repeated infection justifying an appropriate treatment. We propose cryosurgery which provides real benefits with a minimal trauma.