[Exudative enteropathy in Klippel-Trenaunay syndrome]

Abstract

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.