[Exudative enteropathy in Klippel-Trenaunay syndrome].
The triad of the Klippel-Trénaunay Syndrome consists of varicose veins, "port-wine" haemangioma of the skin and bone and soft-tissue hypertrophy with a different extension. Often an obstruction of lymphatic vessels and lymphoedema accompany the syndrome. We observed for the first time a patient with an impressive Klippel-Trénaunay-Syndrome in combination with a symptomatic exudative enteropathy. In spite of a regular intravenous protein substitution for many years, this patient had developed a monstrous elephantiasis of the lower extremities. A lymphography demonstrated a blockade of the lymph flow at the height of the middle paraaortic lymph nodes. The cysterna chyli and the Ductus thoracicus were not visualised. The measurement of 51Cr-labelled albumin excretion in the stool for two days after the intravenous injection of 3.07 MBq 51Cr showed an excretion of 17.9% of the total dose, which means an elevated gastrointestinal protein-loss. However, intestinal lymphangiectasia was not seen on histologic examination of bioptic material of duodenal and jejunal mucosa. These results show that the Klippel-Trénaunay Syndrome may be accompanied by a protein-loosing enteropathy due to obstruction of the gastrointestinal lymph flow. As the intestinal lymphangiectasia may occur locally, it is not always demonstrable directly on pathologic examination of biopsies.
Cooreman
M
M
Medizinische Klinik und Poliklinik, Abteilung Gastroenterologie, Universität Düsseldorf.
Lübke
H
H
Wienbeck
M
M
Strohmeyer
G
G
D000328:Adult; D000798:Angiomatosis / Q000473:pathology*; D004386:Duodenum / Q000473:pathology; D004604:Elephantiasis / Q000473:pathology; D005260:Female; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D007583:Jejunum / Q000473:pathology; D007715:Klippel-Trenaunay-Weber Syndrome / Q000473:pathology*; D008208:Lymphatic System / Q000002:abnormalities* / Q000473:pathology; D008220:Lymphography; D011504:Protein-Losing Enteropathies / Q000473:pathology*
Exsudative Enteropathie beim Klippel-Trenaunay-Syndrom.