{"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":"megaencefalia-malformacao-capilar-polimicrogiria","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/pt\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/","title":{"rendered":"Megalencefalia-malforma\u00e7\u00e3o capilar-polimicrogiria"},"content":{"rendered":"<p><\/p><div id=\"ez-toc-container\" class=\"ez-toc-v2_0_89 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\">Conte\u00fado<\/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=\"Alternar tabela de conte\u00fado\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Alternar<\/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\/pt\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFQue-es-megalencefaliacapilar-malformacion-polimicrogiria\" >O que \u00e9 megaencefalia?malforma\u00e7\u00e3o capilar-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\/pt\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFQuien-contrae-megalencefalia-malformacion-capilar-polimicrogiria\" >Quem recebe megaencefalia-malforma\u00e7\u00e3o 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\/pt\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFQue-causa-la-megalencefalia-malformacion-capilar-polimicrogiria\" >O que causa a megaencefalia-malforma\u00e7\u00e3o 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\/pt\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFCuales-son-las-caracteristicas-clinicas-de-la-megalencefalia-malformacion-capilar-polimicrogiria\" >Quais s\u00e3o as caracter\u00edsticas cl\u00ednicas da megaencefalia-malforma\u00e7\u00e3o 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\/pt\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFComo-se-realiza-el-diagnostico-de-megalencefalia-malformacion-capilar-polimicrogiria\" >Como \u00e9 feito o diagn\u00f3stico de megaencefalia-malforma\u00e7\u00e3o 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\/pt\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#Cual-es-el-diagnostico-diferencial-para-megalencefalia-malformacion-capilar-polimicrogiria\" >Qual \u00e9 o diagn\u00f3stico diferencial para megaencefalia-malforma\u00e7\u00e3o 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\/pt\/wiki\/megalencefalia-malformacion-capilar-polimicrogiria\/#%C2%BFCual-es-el-resultado-de-la-megalencefalia-malformacion-capilar-polimicrogiria\" >Qual \u00e9 o resultado da megaencefalia-malforma\u00e7\u00e3o 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>O que \u00e9 megaencefalia?<span class=\"term\" data-term-id=\"756\">capilar<\/span> <span class=\"term\" data-term-id=\"1631\">malforma\u00e7\u00e3o<\/span>-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Megalencefalia-malforma\u00e7\u00e3o capilar-polimicrogiria (MCAP) foi descrita pela primeira vez em 1997 e \u00e9 caracterizada por:<\/p>\n<ul>\n<li>\n<span class=\"term\" data-term-id=\"1214\">Macrocefalia<\/span> (uma condi\u00e7\u00e3o em que a cabe\u00e7a \u00e9 anormalmente grande)<\/li>\n<li>\n<span class=\"term\" data-term-id=\"47\">Cut\u00e2neo<\/span> <span class=\"term\" data-term-id=\"753\">vascular<\/span> anomalias<\/li>\n<li>Polimicrogiria (uma malforma\u00e7\u00e3o cerebral indicada por v\u00e1rios pequenos giros). <\/li>\n<\/ul>\n<p>Recursos adicionais incluem v\u00e1rios graus de assimetria <span class=\"term\" data-term-id=\"1422\">som\u00e1tico<\/span> crescimento excessivo, <span class=\"term\" data-term-id=\"68\">distal<\/span> malforma\u00e7\u00f5es dos membros e hiperelasticidade. Menos de 300 casos foram relatados na literatura.<\/p>\n<p>MCAP tamb\u00e9m \u00e9 conhecido como macrocefalia-malforma\u00e7\u00e3o capilar (MCM), para refletir o grande tamanho do c\u00e9rebro, grande tamanho da cabe\u00e7a e polimicrogiria que caracterizam o <span class=\"term\" data-term-id=\"350\">s\u00edndrome<\/span>.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQuien-contrae-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>Quem recebe megaencefalia-malforma\u00e7\u00e3o capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>MCAP ocorre esporadicamente e afeta meninos e meninas igualmente sem predile\u00e7\u00f5es \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>O que causa a megaencefalia-malforma\u00e7\u00e3o capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Alguns casos de MCAP foram encontrados para ter som\u00e1tica <span class=\"term\" data-term-id=\"871\">muta\u00e7\u00f5es<\/span> em fosfatidilinositol-4, a subunidade catal\u00edtica alfa 5-bifosfato 3-quinase (<em>PIK3CA<\/em>) <span class=\"term\" data-term-id=\"774\">gene<\/span> no <span class=\"term\" data-term-id=\"1271\">cromossoma<\/span> 3q26.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCuales-son-las-caracteristicas-clinicas-de-la-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>Quais s\u00e3o as caracter\u00edsticas cl\u00ednicas da megaencefalia-malforma\u00e7\u00e3o capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Estes crit\u00e9rios principais devem estar presentes para o diagn\u00f3stico de MCAP:<\/p>\n<ul>\n<li>Macrocefalia (&gt; percentil 95) e <span class=\"term\" data-term-id=\"1759\">progressivo<\/span> megaencefalia<\/li>\n<li>\n<p>malforma\u00e7\u00e3o capilar.<\/li>\n<\/ul>\n<p>Crit\u00e9rios Menores: Participa\u00e7\u00e3o Vari\u00e1vel<\/p>\n<ul>\n<li>\n<span class=\"term\" data-term-id=\"16\">Assim\u00e9trico<\/span> supercrescimento<\/li>\n<li>Atraso no desenvolvimento<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1543\">Linha do meio<\/span> malforma\u00e7\u00e3o capilar facial<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1195\">Neonatal<\/span> <span class=\"term\" data-term-id=\"1101\">hipotonia<\/span>\n<\/li>\n<li>sindactilia<\/li>\n<li>Sali\u00eancia frontal (proemin\u00eancia arredondada dos ossos frontal e parietal do cr\u00e2nio)<\/li>\n<li>hipermobilidade articular<\/li>\n<li>Pele hiperel\u00e1stica e\/ou pastosa.<\/li>\n<li><span class=\"term\" data-term-id=\"1046\">Hidrocefalia<\/span><\/li>\n<li>Convuls\u00f5es<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-realiza-el-diagnostico-de-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>Como \u00e9 feito o diagn\u00f3stico de megaencefalia-malforma\u00e7\u00e3o capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>O diagn\u00f3stico de MCAP \u00e9 feito com base em sinais cl\u00ednicos e estudos de neuroimagem. Ambos os crit\u00e9rios maiores devem estar presentes, com crit\u00e9rios menores vari\u00e1veis.<\/p>\n<p><span class=\"term\" data-term-id=\"1747\">Base<\/span> <span class=\"term\" data-term-id=\"1410\">imagem de resson\u00e2ncia magn\u00e9tica<\/span> (<span class=\"term\" data-term-id=\"1116\">Resson\u00e2ncia magn\u00e9tica<\/span>) do c\u00e9rebro e da coluna vertebral \u00e9 recomendado no momento do 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>Qual \u00e9 ele <span class=\"term\" data-term-id=\"813\">diagn\u00f3stico diferencial<\/span> para megaencefalia-malforma\u00e7\u00e3o capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>O MCAP deve ser diferenciado de outras s\u00edndromes de supercrescimento com malforma\u00e7\u00f5es vasculares, incluindo:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"48\">Pele<\/span> marmorata telangiect\u00e1sica cong\u00eanita<\/li>\n<li>S\u00edndrome de Beckwith-Weidemann<\/li>\n<li>S\u00edndrome de Proteus<\/li>\n<li>\n<p>S\u00edndrome de cravo<span class=\"term\" data-term-id=\"1036\">cong\u00eanito<\/span>, lipomatoso, supercrescimento, malforma\u00e7\u00f5es vasculares, <span class=\"term\" data-term-id=\"846\">epid\u00e9rmico<\/span> nevo e anormalidades espinhais\/esquel\u00e9ticas e\/ou <span class=\"term\" data-term-id=\"1224\">escoliose<\/span>)<\/li>\n<li>\n<em>PTEN<\/em> <span class=\"term\" data-term-id=\"345\">hamartoma<\/span> <span class=\"term\" data-term-id=\"289\">tumor<\/span> s\u00edndrome<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-resultado-de-la-megalencefalia-malformacion-capilar-polimicrogiria\"><\/span>Qual \u00e9 o resultado da megaencefalia-malforma\u00e7\u00e3o capilar-polimicrogiria?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Os pacientes com MCAP requerem vigil\u00e2ncia m\u00e9dica cont\u00ednua, bem como fisioterapia e terapia ocupacional. A interven\u00e7\u00e3o precoce \u00e9 \u00fatil para superar a defici\u00eancia e alcan\u00e7ar marcos de desenvolvimento.<\/p>\n<p>Os pacientes t\u00eam maior risco de desenvolver meduloblastoma, meningioma, tumor de Wilms e <span class=\"term\" data-term-id=\"613\">leucemia<\/span> durante toda a sua vida. <\/p>\n<p>Al\u00e9m dos exames m\u00e9dicos anuais, todos os pacientes devem realizar uma consulta de cardiologia no momento do diagn\u00f3stico.<\/p>\n<p>As recomenda\u00e7\u00f5es de imagem de acompanhamento incluem:<\/p>\n<ul>\n<li>RM do c\u00e9rebro no momento do diagn\u00f3stico e a cada 6 meses at\u00e9 os 2 anos de idade.<\/li>\n<li>s\u00e9rie abdominal <span class=\"term\" data-term-id=\"1376\">sonografia<\/span> a cada 3-6 meses durante os primeiros 7 anos 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\/pt\/wp-json\/wp\/v2\/wiki\/16693","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/comments?post=16693"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/wiki\/16693\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/media?parent=16693"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/categories?post=16693"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/tags?post=16693"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}