{"id":3934,"date":"2017-06-05T09:00:48","date_gmt":"2017-06-05T09:00:48","guid":{"rendered":"https:\/\/endoscopiaterapeutica.net\/pt\/uncategorized\/sindrome-blue-rubber-bleb-nevus\/"},"modified":"2023-03-13T17:08:07","modified_gmt":"2023-03-13T17:08:07","slug":"sindrome-blue-rubber-bleb-nevus","status":"publish","type":"post","link":"https:\/\/endoscopiaterapeutica.net\/pt\/casosclinicos\/sindrome-blue-rubber-bleb-nevus\/","title":{"rendered":"S\u00cdNDROME BLUE RUBBER BLEB NEVUS"},"content":{"rendered":"\t\t<div data-elementor-type=\"wp-post\" data-elementor-id=\"3934\" class=\"elementor elementor-3934\">\n\t\t\t\t\t\t<section class=\"penci-section penci-disSticky penci-structure-10 elementor-section elementor-top-section elementor-element elementor-element-540ccb6e elementor-section-boxed elementor-section-height-default elementor-section-height-default\" data-id=\"540ccb6e\" data-element_type=\"section\" data-e-type=\"section\">\n\t\t\t\t\t\t<div class=\"elementor-container elementor-column-gap-default\">\n\t\t\t\t\t<div class=\"penci-ercol-100 penci-ercol-order-1 penci-sticky-ct    elementor-column elementor-col-100 elementor-top-column elementor-element elementor-element-76ef2000\" data-id=\"76ef2000\" data-element_type=\"column\" data-e-type=\"column\">\n\t\t\t<div class=\"elementor-widget-wrap elementor-element-populated\">\n\t\t\t\t\t\t<div class=\"elementor-element elementor-element-15d59e42 elementor-widget elementor-widget-text-editor\" data-id=\"15d59e42\" data-element_type=\"widget\" data-e-type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t\t\tPaciente \u00a0masculino, 16 anos, \u00a0com hist\u00f3ria de anemia ferropriva persistente secund\u00e1ria a hemorragia digestiva baixa (HDB) recorrente desde os 3 anos de idade sendo necess\u00e1rias in\u00fameras hemotransfus\u00f5es.\n\nAntecedente cir\u00fargico de laparotomia aos 6 anos com realiza\u00e7\u00e3o de enterectomia (1,5 m de jejuno) e ressec\u00e7\u00e3o de 13 les\u00f5es cujo anatomopatol\u00f3gico concluiu se tratarem de\u00a0hemangiomas cavernosos.\n\nFoi admitido para investiga\u00e7\u00e3o com relato de epis\u00f3dio de HDB h\u00e1 30 dias. Apresentava-se hipocorado, com Hb: 4,1 g\/dl e Ht: 12,5%. Estava\u00a0em uso de talidomida. No exame f\u00edsico destacava-se a presen\u00e7a de um n\u00f3dulo viol\u00e1ceo em dorso de m\u00e3o e diminuta les\u00e3o em p\u00e9 (vide fotos abaixo).\n\n<a href=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2017\/06\/blue-nevus-punho.jpg\" data-rel=\"penci-gallery-image-content\" ><img fetchpriority=\"high\" decoding=\"async\" class=\"size-medium wp-image-7831 aligncenter\" src=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2021\/08\/blue-nevus-punho.jpg\" alt=\"\" width=\"300\" height=\"226\" \/><\/a>\n\n<a href=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2017\/06\/blue-nevus-mao.jpg\" data-rel=\"penci-gallery-image-content\" ><img decoding=\"async\" class=\"size-medium wp-image-7830 aligncenter\" src=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2021\/08\/blue-nevus-mao.jpg\" alt=\"\" width=\"300\" height=\"226\" \/><\/a>\n\nRealizou tomografia de abdome identificou v\u00e1rias les\u00f5es com caracter\u00edsticas vasculolinf\u00e1ticas \u00a0nas al\u00e7as de intestino delgado e c\u00f3lon. Identificadas tamb\u00e9m les\u00f5es na adrenal esquerda, perit\u00f4nio, par\u00eanquima e hilo hep\u00e1tico.\n\nEndoscopia digestiva alta sem altera\u00e7\u00f5es.\n\nA colonoscopia (vide abaixo) evidenciou 4 les\u00f5es polipoides subpediculadas, medindo at\u00e9 25mm, bem delimitadas, levemente viol\u00e1ceas, localizadas em c\u00f3lon transverso, descendente, \u00e2ngulo espl\u00eanico e reto, por\u00e9m sem sinais de sangramento. Tais achados eram sugestivos de hemangiomas.\n\n<a href=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2017\/06\/blue-nevus-colonoscopia.jpg\" data-rel=\"penci-gallery-image-content\" ><img decoding=\"async\" class=\"size-medium wp-image-7834 aligncenter\" src=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2021\/08\/blue-nevus-colonoscopia.jpg\" alt=\"\" width=\"300\" height=\"225\" \/><\/a>\n\n<a href=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2017\/06\/blue-nevus-colonoscopia2.jpg\" data-rel=\"penci-gallery-image-content\" ><img loading=\"lazy\" decoding=\"async\" class=\"size-medium wp-image-7835 aligncenter\" src=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2017\/06\/blue-nevus-colonoscopia2-300x225.jpg\" alt=\"\" width=\"300\" height=\"225\" \/><\/a>\n\nA enteroscopia alta identificou 2 les\u00f5es em jejuno: um hemangioma de 1,5 cm e uma angioectasia, ambas tratadas com arg\u00f4nio.\n\nBaseado nos achados da colonoscopia e no passado de hemangiomas em intestino delgado foi aventada a hip\u00f3tese da S\u00edndrome do Nevo Azul (Blue Rubber Bleb Nevus \u2013 BRBN).\n\nNeste caso, por conta do tamanho das les\u00f5es col\u00f4nicas optamos por realizar o tratamento endosc\u00f3pico com inje\u00e7\u00e3o de cianoacrilato. \u00a0Esta conduta foi baseada em relatos da literatura que mostram uma boa taxa de sucesso e menor recorr\u00eancia. O procedimento foi realizado sem intercorr\u00eancias.\n\n&nbsp;\n\n&nbsp;\n\n<div style=\"position:relative;padding-top:56.20608899297424%;\"><iframe id=\"panda-1d0f9edd-fa85-47c2-8c7c-6adb6bc59de1\" src=\"https:\/\/player-vz-a75e9d45-986.tv.pandavideo.com.br\/embed\/?v=1d0f9edd-fa85-47c2-8c7c-6adb6bc59de1\" style=\"border:none;position:absolute;top:0;left:0;\" allow=\"accelerometer;gyroscope;autoplay;encrypted-media;picture-in-picture\" allowfullscreen=true width=\"100%\" height=\"100%\" fetchpriority=\"high\"><\/iframe><\/div>\n\nA colonoscopia de controle em 1 semana e ap\u00f3s cerca de 5 meses apresentaram bons resultados. Paciente n\u00e3o apresentou mais HDB e manteve hemoglobina ap\u00f3s 1 ano em torno de 15,1 g\/dl e Ht de 43%.\n\n<strong>Discuss\u00e3o<\/strong>\n\nA Blue Rubber Bleb Nevus Syndrome (SBRBN) \u00e9 uma doen\u00e7a rara caracterizada por malforma\u00e7\u00f5es vasculares (hemangiomas carvenosos) localizadas principalmente na pele e trato gastrintestinal. A maior parte dos casos s\u00e3o espor\u00e1dicos, mas h\u00e1 alguns relatos em grupos de fam\u00edlias, o que sugere ter uma base gen\u00e9tica com heran\u00e7a autoss\u00f4mica dominante.\n\nA maior morbidade desta doen\u00e7a se deve ao sangramento gastrintestinal, geralmente auto-limitado, evidenciando-se pela perda oculta nas fezes. A depender do tamanho e extens\u00e3o dos hemangiomas pode ocorrer hemat\u00eamese, melena ou enterorragia. Outras complica\u00e7\u00f5es gastrintestinais, quando as les\u00f5es s\u00e3o de grandes dimens\u00f5es, s\u00e3o: volvo, intussuscep\u00e7\u00e3o e isquemia intestinal.\n\nO diagn\u00f3stico diferencial se d\u00e1 com outras s\u00edndromes que cursam com les\u00f5es vaculares cut\u00e2neas e similares em outros \u00f3rg\u00e3os que s\u00e3o:\n<ul>\n \t<li>Redu Osler Weber (TGI e pulm\u00e3o)<\/li>\n \t<li>Klippel- Trenaunay (osso, tecidos moles e S. Nervoso)<\/li>\n \t<li>Sturge Weber (meninges)<\/li>\n \t<li>Von Hippel Lindau (SNC e retina)<\/li>\n \t<li>Maffucci (esqueleto)<\/li>\n \t<li>Cobb (Medula espinhal)<\/li>\n<\/ul>\nNos casos leves o tratamento \u00e9 conservador com suplementa\u00e7\u00e3o de ferro e\/ou transfus\u00f5es sangu\u00edneas. Em casos de HDB recorrente \u00e9 necess\u00e1rio o tratamento endosc\u00f3pico ou eventualmente o cir\u00fargico. A ecoendoscopia pode ser \u00fatil para confirmar a natureza hemangiomatosa das les\u00f5es.\n\nPara as les\u00f5es localizadas no trato gastrintestinal as modalidades terap\u00eauticas endosc\u00f3picas a depender do tamanho e da extens\u00e3o s\u00e3o:\n<ul>\n \t<li>Terapia injetora com adrenalina<\/li>\n \t<li>Terapia t\u00e9rmica com Arg\u00f4nio<\/li>\n \t<li>Ligadura el\u00e1stica<\/li>\n \t<li>Cianoacrilato<\/li>\n \t<li>Polipectomia<\/li>\n \t<li>Dissec\u00e7\u00e3o endosc\u00f3pica submucosa<\/li>\n<\/ul>\nA recorr\u00eancia das les\u00f5es \u00e9 alta. H\u00e1 possibilidade de tratamento farmacol\u00f3gico complementar com agentes antiangiog\u00eanicos ou antiproliferativos (talidomida, octreotide, corticoides, interferon gama).\n\nO progn\u00f3stico depende da extens\u00e3o do envolvimento visceral. N\u00e3o h\u00e1 rela\u00e7\u00e3o com transforma\u00e7\u00e3o maligna.\n\nApesar da raridade do caso a mensagem a ser passada \u00e9 que o tratamento endosc\u00f3pico pode contribuir para a melhora na qualidade de vida com menor necessidade de hemotransfus\u00f5es, menor custo e tempo de interna\u00e7\u00e3o de pacientes. Optamos pela terapia com cianoacrilato por ser uma op\u00e7\u00e3o segura e eficaz.\n\n&nbsp;\n\n* <strong>Caso cl\u00ednico relatado com a colabora\u00e7\u00e3o da Dra Marina Pamponet Motta.<\/strong>\n\n<strong>REFER\u00caNCIAS BIBLIOGR\u00c1FICAS<\/strong>\n\nDwivedi M, et al. Gastrointestinal endoscopy 2002; 55(7): 943-946<u><sup>1<\/sup><\/u>\n\nZurakowski J, et al. Endoscopy 2008, 40: E120-E121\n\nFerreira, M. J Port Gastroenterol 2009; 16;115-119\n\nMavrogenis, G. Endoscopy 2011, 43: E291-292\n\n&nbsp;\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t<\/div>\n\t\t","protected":false},"excerpt":{"rendered":"<p>Paciente \u00a0masculino, 16 anos, \u00a0com hist\u00f3ria de anemia ferropriva persistente secund\u00e1ria a hemorragia digestiva&hellip;<\/p>\n","protected":false},"author":142,"featured_media":3933,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"","_lmt_disable":"","footnotes":""},"categories":[150],"tags":[],"ano":[276],"tipo":[153],"volume":[263],"class_list":["post-3934","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-casosclinicos","ano-276","tipo-endoscopia-digestiva-alta","volume-volume-i"],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v27.2 (Yoast SEO v27.2) - 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