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Klinische Wochenschrift

, Volume 66, Issue 11, pp 502–507 | Cite as

Exsudative enteropathie beim klippel-trenaunay-syndrom

  • M. Cooreman
  • H. Lübke
  • M. Wienbeck
  • G. Strohmeyer
Kasuistiken

The klippel-trénaunay-syndrome associated with a protein-loosing enteropathy

Summary

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 exsudative enteropathy.

In spite of a regular intravenous protein substitution for many years, this patient had develloped 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 of51Cr-labelled albumin excretion in the stool for two days after the intravenous injection of 3.07 MBq51Cr 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.

Key words

Klippel-Trénaunay-Syndrome Lymph flow obstruction Protein-loosing enteropathy 

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Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • M. Cooreman
    • 1
  • H. Lübke
    • 1
  • M. Wienbeck
    • 1
  • G. Strohmeyer
    • 1
  1. 1.Medizinische Klinik und Poliklinik, Abteilung GastroenterologieUniversität DüsseldorfGermany

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