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Seltene Ursache einer Rektumblutung: Das Klippel-Trénaunay-Weber-Syndrom

A rare case of rectal bleeding: the Klippel-Trénaunay-Weber syndrome

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coloproctology Aims and scope

Zusammenfassung

Das Klippel-Trénaunay-Weber-Syndrom, charakterisiert durch Knochen- und Weichteilhypertrophie, variköse Venenektasien und Naevus flammeus, kann in bis zu 12,5% der Fälle mit einer Hämangiomatose des distalen Kolons verbunden sein. Aus diesen Gefäßmalformationen kann es bei bis zu 30% zu teils lebensbedrohlichen Blutungen kommen.

Therapeutisch versucht man zunächst, durch endoskopische Blutstillungsverfahren eine Reduzierung der Blutungsneigung zu erreichen. Als darauf folgendes chirurgisches Verfahren wird hier erstmalig eine transanale „Sperroperation” durch ein zirkuläres Klammergerät, ähnlich der Hämorrhoidenresektion nach Longo, angewandt, um die mechanisch am stärksten beanspruchte Region bei dieser Erkrankung im distalen Rektum zu resezieren. Durch diesen Eingriff konnte eine transabdominelle Rektumresektion mit ihren intra-und postoperativen Risiken vermieden werden.

Abstract

Klippel-Trénaunay-Weber syndrome is characterized by bony and soft tissue hypertrophy, varicose veins and naevus flammeus. Up to 12.5% of the cases are associated with hemangiomas of the distal colon. From these vascular malformations lifethreatening bleeding can occur in up to 30% of the patients.

The first treatment is an endoscopic one to diminish the possibility of bleeding. After this treatment, we carried out a transanal “blocking operation”, similar to the hemorrhoidectomy of Longo, to resect the mechanically most stressed region of this disease. With this operation we avoided a transabdominal rectal resection with all its intra- and postoperative complications.

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Authors and Affiliations

  1. Chirurgie II/Koloprotologie, Raphaelsklinik Münster, Akademisches Lehrkrankenhaus der Westfälischen Wilhelms-Universtät München, München, Deutschland

    Gerd W. Kolbert, Jens Michel & Franz Raulf

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  1. Gerd W. Kolbert
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  2. Jens Michel
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  3. Franz Raulf
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Correspondence to Gerd W. Kolbert.

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Kolbert, G.W., Michel, J. & Raulf, F. Seltene Ursache einer Rektumblutung: Das Klippel-Trénaunay-Weber-Syndrom. Coloproctol 21, 174–177 (1999). https://doi.org/10.1007/BF03044498

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  • DOI: https://doi.org/10.1007/BF03044498

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