{"id":8822,"date":"2017-11-28T08:24:42","date_gmt":"2017-11-28T08:24:42","guid":{"rendered":"http:\/\/old.institutcataladelpeu.com\/?p=8822"},"modified":"2017-11-28T08:24:42","modified_gmt":"2017-11-28T08:24:42","slug":"la-sindrome-klippel-trenaunay-weber-definicio-i-caracteristiques","status":"publish","type":"post","link":"https:\/\/old.institutcataladelpeu.com\/ca\/la-sindrome-klippel-trenaunay-weber-definicio-i-caracteristiques\/","title":{"rendered":"La s\u00edndrome de Klippel – Tr\u00e9naunay – Weber. Definici\u00f3 i caracter\u00edstiques."},"content":{"rendered":"

La s\u00edndrome de Klippel – Tr\u00e9naunay – Weber \u00e9s una afectaci\u00f3 cong\u00e8nita del desenvolupament ossi, dels vasos sanguinis i dels teixits tous.<\/p>\n

Les caracter\u00edstiques d’aquesta s\u00edndrome s\u00f3n:<\/p>\n

1) L’aparici\u00f3 de taques de vi de Porto o hemangiomes plans en el naixement. Apareix en el 90% dels casos aproximadament i se situen en cames, natges i abdomen. Aquestes taques poden afectar el teixit subcutani, ossos, m\u00fasculs o \u00f2rgans com la melsa, el fetge o el c\u00f2lon.<\/p>\n

2) Augment del creixement ossi i de teixits tous. Pot apar\u00e8ixer al moment del naixement i progressa durant la inf\u00e0ncia. Creix en longitud \u00f2ssia com en grossor, majorment a causa del creixement de teixits tous.<\/p>\n

3) L’aparici\u00f3 de venes varicoses. S\u00f3n cong\u00e8nites i no es detecten fins que el nen comen\u00e7a a deambular. Es localitzen per sota del genoll o lateralment. Per sobre del genoll afecten sistemes venosos superficials i profunds i fins i tot a determinats \u00f2rgans.<\/p>\n

Altres complicacions de la s\u00edndrome de Klippel – Tr\u00e9naunay – Weber s\u00f3n les malformacions limf\u00e0tiques a nivell obstructiu o de linfedemes aix\u00ed com l’asimetria facial, alteracions de la grand\u00e0ria del cap, sindact\u00edlia, parest\u00e8sies, descalcificaci\u00f3, trombosi, \u00falceres, etc.<\/p>\n

El creixement excessiu d’ossos i teixits tous apareix en la inf\u00e0ncia i sol afectar una sola cama, (encara que tamb\u00e9 pot manifestar-se en tors i bra\u00e7os). La cama afectada per aquest creixement excessiu pot presentar dolor, dificultat per mobilitzar-se o sensaci\u00f3 de pesadesa.<\/p>\n

La s\u00edndrome de Klippel – Tr\u00e9naunay – Weber \u00e9s de causa desconeguda encara que se li relaciona amb la mutaci\u00f3 del gen PIK3CA associada al sobrecreixement.<\/p>\n

El diagn\u00f2stic ha d’avaluar-se mitjan\u00e7ant tomografia computerizada, resson\u00e0ncia magn\u00e8tica, ecografia i limfoescintigrafia. El diagn\u00f2stic diferencial ha d’incloure la displ\u00e0sia venosa, el linfedema i els tumors ossis.<\/p>\n

La s\u00edndrome de Klippel – Tr\u00e9naunay – Weber \u00e9s un proc\u00e9s benigne progressiu el tractament del qual en el 60% dels casos \u00e9s simptom\u00e0tic i conservador.<\/p>\n

La cirurgia sol indicar-se en casos de linfedema, cel\u00b7lulitis i insufici\u00e8ncia venosa cr\u00f2nica. La epifisiodesi tamb\u00e9 \u00e9s una t\u00e8cnica que frena el naixement ossi excessiu.<\/p>\n

Entre els tractaments no quir\u00fargics, es recorre a l\u2019escleroter\u00e0pia per normalitzar el flux sanguini en les venes afectades.<\/p>\n

Tamb\u00e9 s’utilitzen les peces de compressi\u00f3 com a ajuda a altres procediments de reducci\u00f3 de volum mitjan\u00e7ant l’\u00fas de dispositius pneum\u00e0tics intermitents (s’inflen i desinflen a intervals fixos).<\/p>\n

 <\/p>\n

\"La

S\u00edndrome de Klippel – Tr\u00e9naunay – Weber.<\/p><\/div>\n

 <\/p>\n

\"La

S\u00edndrome de Klippel – Tr\u00e9naunay – Weber. Observeu la major grand\u00e0ria del peu esquerre.<\/p><\/div>\n

 <\/p>\n

\"La

S\u00edndrome de Klippel – Tr\u00e9naunay – Weber. Observeu la pres\u00e8ncia de taques de vi de Porto.<\/p><\/div>\n

 <\/p>\n

\"La

S\u00edndrome de Klippel – Tr\u00e9naunay – Weber. Observeu la pres\u00e8ncia de venes varicoses.<\/p><\/div>\n

 <\/p>\n

\"La

S\u00edndrome de Klippel – Tr\u00e9naunay – Weber. Observeu la difer\u00e8ncia de grossor entre ambdues extremitats.<\/p><\/div>\n

 <\/p>\n

\"La

S\u00edndrome de Klippel – Tr\u00e9naunay – Weber. Observeu la difer\u00e8ncia de tots dos avantpeus.<\/p><\/div>\n

 <\/p>\n

\"La

S\u00edndrome de Klippel – Tr\u00e9naunay – Weber. Observeu l’asimetria de la petjada plantar de l\u2019avantpeu.<\/p><\/div>\n","protected":false},"excerpt":{"rendered":"

La s\u00edndrome de Klippel – Tr\u00e9naunay – Weber \u00e9s una afectaci\u00f3 cong\u00e8nita del desenvolupament ossi, dels vasos sanguinis i dels teixits tous. Les caracter\u00edstiques d’aquesta s\u00edndrome s\u00f3n: 1) L’aparici\u00f3 de taques de vi de Porto o hemangiomes plans en el naixement. Apareix en el 90% dels casos aproximadament i se situen en cames, natges i […]<\/p>\n","protected":false},"author":3,"featured_media":8818,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[276,279,282,346,306],"tags":[],"class_list":["post-8822","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-alteracions-dermatologiques","category-altres","category-articles","category-icp-ca","category-patologia-ca"],"yoast_head":"\nLa s\u00edndrome de Klippel - Tr\u00e9naunay - Weber. 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