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

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


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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  1. 1.
    Baskerville PA, Ackroyd JS, Browse NL (1985) The etiology of the Klippel-Trenaunay syndrome. Ann Surg 202:624Google Scholar
  2. 2.
    Baskerville PA, Ackroyd JS, Lea Thomas M, Browse NL (1985) The Klippel-Trenaunay syndrome: clinical, radiological and haemodynamic features and management. Br J Surg S 75:232Google Scholar
  3. 3.
    Bourde C (1974) Classification des syndroms de Klippel-Trenaunay et de Parkes-Weber d'apres les donnees angiographiques. Ann Radiol (Paris) 160Google Scholar
  4. 4.
    Brooksaler F (1966) The angioosteohypertrophy syndrome. Klippel-Trenaunay-Weber syndrome. Am J Child 112:161Google Scholar
  5. 5.
    Granger DN, Cook BH, Taylor AE (1976) Structural locus of transmucosal albumin efflux in canine ileum. A fluorescent study. Gastroenterology 71:1023Google Scholar
  6. 6.
    Harper PS (1971) Sturge-Weber syndrome with Klippel-Trenaunay-Weber syndrome (abstr). Birth Defects 7:314Google Scholar
  7. 7.
    Jeffries GH, Holman HR, Sleisenger MH (1962) Plasma proteins and the gastrointestinal tract. N Engl J Med 266:652Google Scholar
  8. 8.
    Klippel M, Trenaunay P (1900) Du naevis variqueux osteohypertrophique. Arch Gen Med (Paris) 185:641Google Scholar
  9. 9.
    Koblet HP, Vesin H, Diggelmann S, Barandun S (1965) Physiology and pathophysiology of plasma protein metabolism. Huber, BernGoogle Scholar
  10. 10.
    Lal S, Narayanan PS (1970) Klippel-Trenaunay syndrome involving the upper extremity. Indian J Med Sci 24:432Google Scholar
  11. 11.
    Lewis-Barr D, Legros J (1946) Les hypertrophies partielles avec angiome et leurs rapport avec les phacomatoses. Cofin Neurol (Basel) 7:245Google Scholar
  12. 12.
    Lindenauer SM (1965) The Klippel-Trenaunay syndrome: varicosity, hypertrophy and hemangioma with no ateriovenous fistula. Ann Surg 162:303Google Scholar
  13. 13.
    Martini GA, Dölle W, Petersen F, Treske U, Strohmeyer G (1963) Die exsudative Gastroenteropathie, ein polyätiologisches Syndrom. Internist 4:197Google Scholar
  14. 14.
    Martini GA, Strohmeyer G, Bünger P (1960) Exsudative Enteropathie („essentielle Hypoproteinämie“) mit rezidivierender Gelbsucht und Verkalkungen im Bauchraum. Dtsch med Wochenschr 85:586Google Scholar
  15. 15.
    Martorell F, Monserat J (1962) Aplasia of the iliac vein and Klippel-Trenaunay syndrome. Angiologia 14:72Google Scholar
  16. 16.
    McGuigan JE, Purkerson ML, Trudeau WL, Peterson ML (1968) Studies of the immunologic defects associated with intestinal lymphangiectasia. Ann Intern Med 68:398Google Scholar
  17. 17.
    Murray M (1969) Structural changes in bovine ostertagiasis assosiated with increased permeability of the bowel wall to macromolecules. Gastroenterology 56:763Google Scholar
  18. 18.
    Owens DW, Garcia E, Pierce RR, Castrow FF (1973) Klippel-Trenaunay-Weber syndrome with pulmonary vein varicosity. Arch Dermatol 108:111Google Scholar
  19. 19.
    Parkes-Weber FF (1907) Angiome formation in connection with hypertrophy of limbs and hemihypertrophy. Br J Dermat 19:231Google Scholar
  20. 20.
    Parkes-Weber FF (1918) Hemangiectatic hypertrophy of limbs — congenital phlebacteriectasis and so-called congenital variceal veins. Br J Child Dis 15:13Google Scholar
  21. 21.
    Pomerantz M, Waldmann ThA (1963) Systemic lymphatic abnormalities assosiated with gastrointestinal protein loss secondary to intestinal lymphangiecasis. Gastroenterology 45:703Google Scholar
  22. 22.
    Sciubba JJ, Brown AM (1977) Oral-facial manifestations of Klippel-Trenaunay-Weber syndrome. Report of two cases. Oral Surg 43:227Google Scholar
  23. 23.
    Servelle M (1984) Klippel and Trenaunays syndrome: 768 operated cases. Ann Surg 201:365Google Scholar
  24. 24.
    Servelle M (1945) La veinographie, va-t-elle nous permettre des demembrer le syndrome de Klippel et Trenaunay et l'hemangioectasie hypertrophique de Parkes-Weber. Presse Med 24:353Google Scholar
  25. 25.
    Servelle M, Albeaux-Fernet M, Labord S, Chaobot J, Rougeulle J (1957) Lesions de vaisseaux lymphatiques dans les Malformations congenitales des veins profundes. Presse Med 65:531Google Scholar
  26. 26.
    Servelle M, Babillot J (1980) Les malformations des veines profondes dans le syndrome de Klippel et Trenaunay. Phlebologie 33:31Google Scholar
  27. 27.
    Strober W, Wochner RD, Carbone PP, Waldmann TA (1967) Intestinal lymphangiectasia: A protein-losing enteropathy with hypogammaglobulinemia, lymphocytopenia and impaired homograft rejection. J Clin Invest 46:1643Google Scholar
  28. 28.
    Troost BT, Savino PJ, Lozito JC (1975) Tuberous sclerosis and Klippel-Trenaunay-Weber syndrome. Assosiation of two complete phakomatoses in a single individual. J Neurol Neurosurg Psychiatry 38:500Google Scholar
  29. 29.
    Waldmann ThA, Steinfeld JL, Dutcher F, Davidson JD, Gordon RS (1961) Role of gastrointestinal system in “idiopathic” hypoproteinemia. Gastroenterology 41:197Google Scholar
  30. 30.
    You CK, Rees J, Gillis DA, Steeves J (1983) Klippel-Trenaunay syndrome: a review. Canadian J Surg 26:399Google Scholar

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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