Generalized lymphangiectasia is a rare congenital disorder characterized by dilated lymphatic vessels with a fatal prognosis, especially in cases with thoracic involvement. We describe the use of propranolol in the therapy of generalized lymphangiectasia in a preterm infant with hydrops fetalis. Propranolol was well tolerated and effective within the first months. It remains to be shown whether...

A patient is described who presented with pleural effusions, asymmetrical oedema of the face and limbs, and numerous warts of the hands and feet. He was shown to have vitamin B12 malabsorption, steatorrhoea, and a protein-losing enteropathy. Lymphography showed asymmetrical lymphatic hypoplasia. Jejunal biopsy showed intestinal lymphangiectasia. The hypoproteinaemia responded well to a low-fat,...

We report an unusual presentation of a young man with bilateral groin lymph nodal adenolymphoceles and right leg lymphedema as a manifestation of intestinal lymphangiectasia. Chylous reflux was supported by conventional and isotopic lymphography as well as by a total lipid test showing delayed triglyceride absorption 24 hours after ingestion of 60 gm of butter. After excision of groin masses in...

To the Editor: I read with interest the article on “Congenital chylous ascites and lymphedema in Down’s syndrome” published in Nov. 2012 issue [1]. However I would like to make some comments on the management of the given case. Association of Down syndrome with primary chylous ascites has been well documented in literature with previous published case reports [2, 3]. Though authors mention inve...

The clinical course of 6 children with primary intestinal lymphangiectuasia who have been treated with low fat medium chain triglyceride-supplemented diets for between 3 and 8 years (4 for longer than 5 years) is described. Though laboratory findings indicate continuing chyle leak, evidence for long-term benefit from dietary treatment is provided by symptomatic relief while on the diet, clinica...

The Yellow Nail Syndrome is a rare clinical entity, first described in 1967 by P.D. Samman and W.F. White. The triad slow-growing dystrophic yellow nails, lymphedema and chronic respiratory disorders is the typical manifestation of the disease but some variations have been described as well as associations with chylothorax, chylous ascites, intestinal lymphangiectasia, thyroid abnormalities, ma...

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 ...

Lymphatic malformations are serious but poorly understood conditions that present therapeutic challenges. The goal of this study was to compare strategies for inducing regression of abnormal lymphatics and explore underlying mechanisms. CCSP-rtTA/tetO-VEGF-C mice, in which doxycycline regulates VEGF-C expression in the airway epithelium, were used as a model of pulmonary lymphangiectasia. After...