" /> Klippel-Trénaunay syndrome - CISMeF





Preferred Label : Klippel-Trénaunay syndrome;

ICD-11 definition : Klippel-Trenaunay syndrome (KTS) (OMIM 149000) is characterized by limb hypertrophy, capillary malformations and slow flow vascular malformations of the affected limb. It differs from Parkes-Weber syndrome in which limb hypertrophy and capillary malformations are associated with fast flow arteriovenous malformations and, in some cases, with mutations in the RASA1 gene .;

ICD-11 synonym : Klippel-Trenaunay-Weber syndrome;

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Klippel-Trenaunay syndrome (KTS) (OMIM 149000) is characterized by limb hypertrophy, capillary malformations and slow flow vascular malformations of the affected limb. It differs from Parkes-Weber syndrome in which limb hypertrophy and capillary malformations are associated with fast flow arteriovenous malformations and, in some cases, with mutations in the RASA1 gene .

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29/07/2025


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