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Klippel-Trenaunay-Syndrom

Hemiparese und Atemnot bei einer 15-jährigen Betroffenen

Klippel-Trenaunay syndrome

Hemiparesis and dyspnoea in a 15-year-old girl

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Zusammenfassung

Das Klippel-Trenaunay-Syndrom ist eine seltene, angeborene Angiodysplasie. Sie zeigt die typische Trias aus vaskulärer Malformation, Knochen- und Weichteilhypertrophie und venöser Varikosis. Eine seltene, aber potenziell lebensbedrohliche Komplikation ist das Auftreten thrombembolischer Ereignisse. Wir berichten von einem 15-jährigen Mädchen mit plötzlicher Bewusstseinstrübung, Halbseitensymptomatik und anhaltender Luftnot bei angeborener Angiodysplasie am rechten Bein. Als Ursache der Beschwerden fanden sich multiple Lungenembolien sowie ein ischämischer Schlaganfall aufgrund einer paradoxen Embolie bei offenem Foramen ovale. Ausgangspunkt der Embolien waren vermutlich Thrombosen im Bereich der Angiodysplasie. Anhand dieses Fallberichts werden die aufgetretenen diagnostischen Schwierigkeiten diskutiert und ein Überblick über die verfügbare Literatur zu der seltenen Kombination aus Klippel-Trenaunay-Syndrom, Lungenembolie und paradoxer Embolie im Kindes- und Jugendalter gegeben.

Abstract

Klippel-Trenaunay syndrome is a rare, congenital angiodysplasia characterized by the clinical triad of vascular malformation, unilateral soft tissue and bone hypertrophy and venous varicosis. Thromboembolic events are rare but potentially life-threatening. We report a case of a 15-year-old girl with sudden loss of consciousness, hemiparesis and dyspnoea. We diagnosed pulmonary embolism and ischaemic stroke due to paradoxical brain embolism through a patent foramen ovale. Obviously, a venous malformation of the lower extremity was the source of these thromboembolic events. We discuss the diagnostic challenges of this case and review the literature regarding the rare combination of Klippel-Trenaunay syndrome, pulmonary embolism and paradoxical brain embolism among paediatric patients. To our knowledge, we describe the third case of stroke associated with Klippel-Trenaunay syndrome.

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Authors and Affiliations

  1. Klinik für Kinder- und Jugendmedizin, Kliniken Ludwigsburg-Bietigheim gGmbH, Posilipostraße 4, 71640, Ludwigsburg, Deutschland

    F. Kaßberger, R. Knorr, U. Stahl, B. Weiß, M. Schroth, K. Braun & M. Walka

  2. Universitätsklinik für Kinder- und Jugendmedizin, Universität Tübingen, Tübingen, Deutschland

    F. Kaßberger

  3. Zentrum für angeborene Herzfehler, Olgahospital Stuttgart, Stuttgart, Deutschland

    F. Uhlemann

Authors
  1. F. Kaßberger
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  2. R. Knorr
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  3. U. Stahl
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  4. B. Weiß
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  5. M. Schroth
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  6. K. Braun
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  7. F. Uhlemann
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  8. M. Walka
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Correspondence to F. Kaßberger.

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Kaßberger, F., Knorr, R., Stahl, U. et al. Klippel-Trenaunay-Syndrom. Monatsschr Kinderheilkd 159, 140–144 (2011). https://doi.org/10.1007/s00112-010-2337-7

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  • DOI: https://doi.org/10.1007/s00112-010-2337-7

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