{"id":16693,"date":"2020-04-29T06:56:05","date_gmt":"2020-04-29T09:56:05","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/"},"modified":"2020-05-03T10:30:03","modified_gmt":"2020-05-03T13:30:03","slug":"megalencefalia-malformacion-capilar-polimicrogiria","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/fr\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/","title":{"rendered":"M\u00e9galenc\u00e9phalie-malformation capillaire-polymicrogyrie"},"content":{"rendered":"<p><\/p><div id=\"ez-toc-container\" class=\"ez-toc-v2_0_88 counter-hierarchy ez-toc-counter ez-toc-grey ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Contenu<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Basculer la table des mati\u00e8res\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Basculer<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewbox=\"0 0 24 24\" version=\"1.2\" baseprofile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 eztoc-toggle-hide-by-default' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/doctorhoogstra.com\/fr\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFQue-es-megalencefaliacapilar-malformacion-polimicrogiria\" >\u00bfQu\u00e9 es megalencefalia?capilar malformaci\u00f3n-polimicrogiria?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/doctorhoogstra.com\/fr\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFQuien-contrae-megalencefalia-malformacion-capilar-polimicrogiria\" >\u00bfQui\u00e9n contrae megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/doctorhoogstra.com\/fr\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFQue-causa-la-megalencefalia-malformacion-capilar-polimicrogiria\" >\u00bfQu\u00e9 causa la megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/doctorhoogstra.com\/fr\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFCuales-son-las-caracteristicas-clinicas-de-la-megalencefalia-malformacion-capilar-polimicrogiria\" >\u00bfCu\u00e1les son las caracter\u00edsticas cl\u00ednicas de la megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/doctorhoogstra.com\/fr\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFComo-se-realiza-el-diagnostico-de-megalencefalia-malformacion-capilar-polimicrogiria\" >\u00bfC\u00f3mo se realiza el diagn\u00f3stico de megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/doctorhoogstra.com\/fr\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#Cual-es-el-diagnostico-diferencial-para-megalencefalia-malformacion-capilar-polimicrogiria\" >Cu\u00e1l es el diagn\u00f3stico diferencial para megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/doctorhoogstra.com\/fr\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFCual-es-el-resultado-de-la-megalencefalia-malformacion-capilar-polimicrogiria\" >\u00bfCu\u00e1l es el resultado de la megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-es-megalencefaliacapilar-malformacion-polimicrogiria\"><\/span>\u00bfQu\u00e9 es megalencefalia?<span class=\"term\" data-term-id=\"756\">capillaire<\/span> <span class=\"term\" data-term-id=\"1631\">malformation<\/span>-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Megalencefalia-malformaci\u00f3n capilar-polimicrogiria (MCAP) se describi\u00f3 por primera vez en 1997 y se caracteriza por:<\/p>\n<ul>\n<li>\n<span class=\"term\" data-term-id=\"1214\">Macroc\u00e9phalie<\/span> (una condici\u00f3n donde la cabeza es anormalmente grande)<\/li>\n<li>\n<span class=\"term\" data-term-id=\"47\">Cutan\u00e9<\/span> <span class=\"term\" data-term-id=\"753\">vasculaire<\/span> anomalies<\/li>\n<li>Polimicrogiria (una malformaci\u00f3n cerebral indicada por varias convoluciones cerebrales peque\u00f1as). <\/li>\n<\/ul>\n<p>Las caracter\u00edsticas adicionales incluyen diversos grados de asim\u00e9trico <span class=\"term\" data-term-id=\"1422\">somatique<\/span> crecimiento excesivo, <span class=\"term\" data-term-id=\"68\">distal<\/span> malformaciones de las extremidades e hiperelasticidad. Se han reportado menos de 300 casos en la literatura.<\/p>\n<p>MCAP tambi\u00e9n se conoce como malformaci\u00f3n macrocefalia-capilar (MCM), para reflejar el tama\u00f1o del cerebro grande, el tama\u00f1o de la cabeza grande y la polimicrogiria que caracterizan la <span class=\"term\" data-term-id=\"350\">syndrome<\/span>.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQuien-contrae-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>\u00bfQui\u00e9n contrae megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>El MCAP ocurre espor\u00e1dicamente y afecta a ni\u00f1os y ni\u00f1as por igual sin predilecciones \u00e9tnicas.<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-causa-la-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>\u00bfQu\u00e9 causa la megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Se ha encontrado que algunos casos de MCAP tienen som\u00e1tica <span class=\"term\" data-term-id=\"871\">mutations<\/span> en la fosfatidilinositol-4, la subunidad catal\u00edtica alfa 5-bisfosfato 3-quinasa (<em>PIK3CA<\/em>) <span class=\"term\" data-term-id=\"774\">g\u00e8ne<\/span> dans <span class=\"term\" data-term-id=\"1271\">chromosome<\/span> 3q26.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCuales-son-las-caracteristicas-clinicas-de-la-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>\u00bfCu\u00e1les son las caracter\u00edsticas cl\u00ednicas de la megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Estos criterios principales deben estar presentes para que se diagnostique MCAP:<\/p>\n<ul>\n<li>Macrocefalia (&gt; percentil 95) y <span class=\"term\" data-term-id=\"1759\">progressive<\/span> megalencefalia<\/li>\n<li>\n<p>Malformaci\u00f3n capilar.<\/li>\n<\/ul>\n<p>Criterios menores: participaci\u00f3n variable<\/p>\n<ul>\n<li>\n<span class=\"term\" data-term-id=\"16\">Asym\u00e9trique<\/span> crecimiento excesivo<\/li>\n<li>Retard de d\u00e9veloppement<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1543\">Ligne m\u00e9diane<\/span> malformaci\u00f3n capilar facial<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1195\">N\u00e9onatal<\/span> <span class=\"term\" data-term-id=\"1101\">hypotonie<\/span>\n<\/li>\n<li>Sindactilia<\/li>\n<li>Protuberancia frontal (prominencia redondeada de los huesos frontal y parietal del cr\u00e1neo)<\/li>\n<li>Hipermovilidad articular<\/li>\n<li>Piel hiperel\u00e1stica y \/ o pastosa.<\/li>\n<li><span class=\"term\" data-term-id=\"1046\">Hydroc\u00e9phalie<\/span><\/li>\n<li>Saisies<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-realiza-el-diagnostico-de-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>\u00bfC\u00f3mo se realiza el diagn\u00f3stico de megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>El diagn\u00f3stico de MCAP se realiza con base en signos cl\u00ednicos y estudios de neuroimagen. Ambos criterios principales deben estar presentes, con criterios menores variables.<\/p>\n<p><span class=\"term\" data-term-id=\"1747\">Base<\/span> <span class=\"term\" data-term-id=\"1410\">image de r\u00e9sonance magn\u00e9tique<\/span> (<span class=\"term\" data-term-id=\"1116\">R\u00e9sonance magn\u00e9tique<\/span>) del cerebro y la columna vertebral se recomienda al momento del diagn\u00f3stico.<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"Cual-es-el-diagnostico-diferencial-para-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>Quel est le <span class=\"term\" data-term-id=\"813\">diagnostic diff\u00e9rentiel<\/span> para megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>MCAP debe distinguirse de otros s\u00edndromes de sobrecrecimiento con malformaciones vasculares que incluyen:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"48\">Peau<\/span> marmorata telangiectatica cong\u00e9nitale<\/li>\n<li>S\u00edndrome de Beckwith Weidemann<\/li>\n<li>Syndrome de Proteus<\/li>\n<li>\n<p>Syndrome des clous de girofle<span class=\"term\" data-term-id=\"1036\">cong\u00e9nital<\/span>, lipomatoso, sobrecrecimiento, malformaciones vasculares, <span class=\"term\" data-term-id=\"846\">\u00e9pidermique<\/span> nevus y anomal\u00edas espinales \/ esquel\u00e9ticas y \/ o <span class=\"term\" data-term-id=\"1224\">scoliose<\/span>)<\/li>\n<li>\n<em>PTEN<\/em> <span class=\"term\" data-term-id=\"345\">hamartoma<\/span> <span class=\"term\" data-term-id=\"289\">tumeur<\/span> syndrome<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-resultado-de-la-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>\u00bfCu\u00e1l es el resultado de la megalencefalia-malformaci\u00f3n capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Los pacientes con MCAP requieren vigilancia m\u00e9dica continua, as\u00ed como terapia f\u00edsica y ocupacional. La intervenci\u00f3n temprana es \u00fatil para superar la discapacidad y lograr hitos en el desarrollo.<\/p>\n<p>Los pacientes tienen un mayor riesgo de desarrollar meduloblastoma, meningioma, tumor de Wilms y <span class=\"term\" data-term-id=\"613\">leuc\u00e9mie<\/span> a lo largo de su vida. <\/p>\n<p>Adem\u00e1s de los ex\u00e1menes m\u00e9dicos anuales, todos los pacientes deben tener una consulta de cardiolog\u00eda al momento del diagn\u00f3stico.<\/p>\n<p>Las recomendaciones de im\u00e1genes de seguimiento incluyen:<\/p>\n<ul>\n<li>IRM del cerebro al momento del diagn\u00f3stico y cada 6 meses hasta la edad de 2 a\u00f1os.<\/li>\n<li>Abdominal serie <span class=\"term\" data-term-id=\"1376\">sonograf\u00eda<\/span> cada 3\u20136 meses durante los primeros 7 a\u00f1os de vida. <\/li>\n<\/ul>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>\u00bfQu\u00e9 es megalencefalia?capilar malformaci\u00f3n-polimicrogiria? Megalencefalia-malformaci\u00f3n capilar-polimicrogiria (MCAP) se describi\u00f3 por primera vez en 1997 y se caracteriza por: Macrocefalia (una condici\u00f3n donde la cabeza es anormalmente grande) Cut\u00e1neo vascular anomal\u00edas&#8230;<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[],"class_list":["post-16693","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/wiki\/16693","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/comments?post=16693"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/wiki\/16693\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/media?parent=16693"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/categories?post=16693"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/fr\/wp-json\/wp\/v2\/tags?post=16693"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}