{"id":7081,"date":"2024-11-21T01:30:24","date_gmt":"2024-11-21T01:30:24","guid":{"rendered":"https:\/\/prrf.org\/?page_id=7081"},"modified":"2025-10-24T22:20:50","modified_gmt":"2025-10-24T22:20:50","slug":"fevr","status":"publish","type":"page","link":"https:\/\/prrf.org\/ja\/pediatric-retinal-diseases\/fevr\/","title":{"rendered":"\u5bb6\u65cf\u6027\u6ef2\u51fa\u6027\u785d\u5b50\u4f53\u7db2\u819c\u75c7\uff08FEVR\uff09"},"content":{"rendered":"<div id=\"home\"  data-column-margin=\"90px\" data-midnight=\"dark\"  class=\"wpb_row vc_row-fluid vc_row full-width-section vc_row-o-equal-height vc_row-flex vc_row-o-content-middle  top_padding_tablet_8pct bottom_padding_tablet_8pct\"  style=\"padding-top: 0px; padding-bottom: 0px; \"><div class=\"row-bg-wrap\" data-bg-animation=\"none\" data-bg-animation-delay=\"\" data-bg-overlay=\"false\"><div class=\"inner-wrap row-bg-layer\" ><div class=\"row-bg viewport-desktop using-bg-color\"  style=\"background-color: #ffffff; \"><\/div><\/div><\/div><div class=\"row_col_wrap_12 col span_12 dark left\">\n\t<div style=\"z-index: 100; \" class=\"vc_col-sm-3\/5 vc_col-xs-12 wpb_column column_container vc_column_container col padding-2-percent no-extra-padding_tablet no-extra-padding_phone\"  data-padding-pos=\"right\" data-has-bg-color=\"false\" data-bg-color=\"\" data-bg-opacity=\"1\" data-animation=\"\" data-delay=\"0\" >\n\t\t<div class=\"vc_column-inner\" >\n\t\t\t<div class=\"wpb_wrapper\">\n\t\t\t\t<div id=\"fws_69f365c472b71\" data-midnight=\"\" data-column-margin=\"default\" class=\"wpb_row vc_row-fluid vc_row inner_row\"  style=\"\"><div class=\"row-bg-wrap\"> <div class=\"row-bg\" ><\/div> <\/div><div class=\"row_col_wrap_12_inner col span_12  left\">\n\t<div  class=\"vc_col-sm-12 wpb_column column_container vc_column_container col child_column has-animation no-extra-padding inherit_tablet inherit_phone\"   data-padding-pos=\"all\" data-has-bg-color=\"false\" data-bg-color=\"\" data-bg-opacity=\"1\" data-animation=\"fade-in-from-bottom\" data-delay=\"0\" >\n\t\t<div class=\"vc_column-inner\" >\n\t\t<div class=\"wpb_wrapper\">\n\t\t\t<div class=\"divider-wrap\" data-alignment=\"default\"><div style=\"height: 50px;\" class=\"divider\"><\/div><\/div>\n<div class=\"wpb_text_column wpb_content_element  vc_custom_1732152517322\" >\n\t<div class=\"wpb_wrapper\">\n\t\t<h1>\u5bb6\u65cf\u6027\u6ef2\u51fa\u6027\u785d\u5b50\u4f53\u7db2\u819c\u75c7\uff08FEVR\uff09<\/h1>\n\t<\/div>\n<\/div>\n\n\n\n\n<div class=\"wpb_text_column wpb_content_element\" >\n\t<div class=\"wpb_wrapper\">\n\t\t<p><strong>Introduction and History<\/strong><\/p>\n<p>Familial Exudative Vitreoretinopathy, or FEVR, is a rare genetic eye condition that affects the development of blood vessels in the retina, whereby the blood vessels at the edges of the retina don&#8217;t form properly. This can lead to a lack of blood supply and oxygen, causing the growth of new, abnormal blood vessels. These weak vessels can leak fluid (exudate) or bleed, sometimes leading to the formation of scar tissue, retinal detachment (where the retina pulls away from the back of the eye), and vision loss.<\/p>\n<p>FEVR is &#8220;familial,&#8221; meaning it can be passed down through families. However, the severity of FEVR can vary significantly, even among family members. Some people may exhibit no symptoms, while others may experience severe vision problems.<\/p>\n<p>FEVR was first identified and described as a distinct eye disease in 1969 by doctors Criswick and Schepens. They observed a pattern of abnormal retinal blood vessel development in several families, distinct from other known eye conditions.<\/p>\n<p><strong>Who Is Affected?<\/strong><\/p>\n<p>FEVR is a rare condition, and its exact frequency isn&#8217;t precisely known, partly because mild cases may go undiagnosed. It affects both males and females, though the pattern of inheritance can differ depending on the specific gene involved. FEVR can occur in people of all racial and ethnic backgrounds. Because it&#8217;s a genetic condition, individuals with a family history of FEVR are at a higher risk. The age at which FEVR is discovered can vary from early infancy to adulthood, though symptoms often begin in childhood.<\/p>\n<p><strong>Diagnosis<\/strong><\/p>\n<p>Diagnosing FEVR involves a careful eye examination by an ophthalmologist (eye doctor), often a specialist in retinal diseases. The doctor will look for characteristic signs in the retina, such as:<\/p>\n<ul>\n<li>Avascular periphery: Areas at the edge of the retina where blood vessels haven&#8217;t developed.<\/li>\n<li>Dragged vessels: Retinal blood vessels that appear stretched or pulled out of place.<\/li>\n<li>Exudates: Leaky fluid from abnormal blood vessels.<\/li>\n<li>Neovascularization: Growth of new, abnormal blood vessels.<\/li>\n<li>Retinal folds (Figure) or detachments.<\/li>\n<\/ul>\n<p>A key diagnostic test is wide-field fluorescein angiography, which highlights any abnormalities, such as missing vessels or leakage. Genetic testing can also help confirm the diagnosis by identifying mutations in genes known to cause FEVR, such as FZD4, LRP5, TSPAN12, and NDP. Examining family members can also be important.<\/p>\n<p><strong>Treatment<\/strong><\/p>\n<p>Treatment for FEVR aims to prevent the disease from worsening and to preserve vision. The specific treatment depends on how severe the condition is:<\/p>\n<ul>\n<li>Observation: Mild cases with no vision problems may only need regular monitoring.<\/li>\n<li>Laser Photocoagulation: This is a standard treatment that uses a laser to treat avascular (lacking blood vessels) areas of the retina. This can help prevent abnormal blood vessels from growing and leaking.<\/li>\n<li>Anti-VEGF Injections: Medications called anti-vascular endothelial growth factor (anti-VEGF) drugs can be injected into the eye.<sup>1.<\/sup> These drugs help to reduce the growth of abnormal blood vessels and decrease leakage.<\/li>\n<li>Surgery (Vitrectomy and\/or Scleral Buckle): If FEVR leads to significant bleeding in the vitreous (the gel-like substance filling the eye) or causes a retinal detachment, surgery may be needed. A vitrectomy removes the vitreous gel and any blood or scar tissue. Sometimes a scleral buckle, a tiny silicone band, is placed around the eye to support the retina.<\/li>\n<\/ul>\n<p><strong>Prognosis<\/strong><\/p>\n<p>The outlook for individuals with FEVR varies greatly. With early diagnosis and timely treatment, vision can often be stabilized, and severe vision loss can be prevented in many cases. However, FEVR is a lifelong condition and can sometimes progress despite treatment, especially in more severe forms. Some individuals may experience significant vision impairment. Regular follow-up appointments with an eye doctor are crucial for monitoring the condition.<\/p>\n<p><strong>Current Research<\/strong><\/p>\n<p>Researchers are working diligently to gain a deeper understanding of FEVR and develop new treatments. Current research focuses on:<\/p>\n<ul>\n<li>Genetics: Identifying more genes that cause FEVR and understanding how these genes affect retinal development. This could lead to better genetic counseling and potentially gene-based therapies in the future.<\/li>\n<li>Improved Imaging and Diagnosis: Developing more advanced ways to see and assess the retinal changes in FEVR.<\/li>\n<li>New Medications: Investigating new drugs that could more effectively stop abnormal blood vessel growth or promote normal vessel development. One such area involves drugs that mimic a naturally occurring protein called norrin, which is crucial for the formation of healthy retinal blood vessels.<\/li>\n<li>Surgical Techniques: Refining surgical methods to improve outcomes for retinal detachments and other complications.<\/li>\n<\/ul>\n<p>Continued research holds promise for even more effective ways to manage FEVR and preserve the vision of those affected.<\/p>\n<p><strong>References<\/strong><\/p>\n<ol>\n<li>Criswick, V. G., &amp; Schepens, C. L. (1969). Familial exudative vitreoretinopathy. American Journal of Ophthalmology, 68(4), 578\u2013594.<\/li>\n<li>Orphanet: Familial exudative vitreoretinopathy. (Consult an up-to-date version on www.orpha.net)<\/li>\n<li>MedlinePlus Genetics: Familial exudative vitreoretinopathy. (Consult an up-to-date version on medlineplus.gov\/genetics)<\/li>\n<li>Drenser Ranchod TM, Ho LY, Drenser KA, Capone A Jr, Trese MT. Clinical presentation of familial exudative vitreoretinopathy. Ophthalmology. 2011 Oct;118(10):2070-5.<\/li>\n<li>Cicerone AP, Dailey W, Sun M, Santos A, Jeong D, Jones L, Koustas K, Drekh M, Schmitz K, Haque N, Felisky JA, Guzman AE, Mellert K, Trese MT, Capone A, Drenser KA, Mitton KP. A Survey of Multigenic Protein-Altering Variant Frequency in Familial Exudative Vitreo-Retinopathy (FEVR) Patients by Targeted Sequencing of Seven FEVR-Linked Genes. Genes (Basel). 2022 Mar 11;13(3):495.<\/li>\n<\/ol>\n\t<\/div>\n<\/div>\n\n\n\n\n\t\t<\/div> \n\t<\/div>\n\t<\/div> \n<\/div><\/div><div id=\"fws_69f365c4730a1\" data-midnight=\"\" data-column-margin=\"default\" class=\"wpb_row vc_row-fluid vc_row inner_row  bottom_padding_tablet_40px bottom_padding_phone_40px\"  style=\"padding-bottom: 0px; \"><div class=\"row-bg-wrap\"> <div class=\"row-bg\" ><\/div> <\/div><div class=\"row_col_wrap_12_inner col span_12  left\">\n\t<div  class=\"vc_col-sm-12 wpb_column column_container vc_column_container col child_column has-animation no-extra-padding inherit_tablet inherit_phone\"   data-padding-pos=\"all\" data-has-bg-color=\"false\" data-bg-color=\"\" data-bg-opacity=\"1\" data-animation=\"fade-in-from-bottom\" data-delay=\"0\" >\n\t\t<div class=\"vc_column-inner\" >\n\t\t<div class=\"wpb_wrapper\">\n\t\t\t<div class=\"nectar-cta  alignment_tablet_default alignment_phone_default display_tablet_inherit display_phone_inherit\" data-color=\"accent-color\" data-using-bg=\"true\" data-style=\"arrow-animation\" data-display=\"block\" data-alignment=\"left\" data-text-color=\"custom\" ><h6 style=\"color: #ffffff;\"><span class=\"link_wrap\" style=\"padding-top: 15px; padding-right: 35px; padding-bottom: 15px; padding-left: 35px;\"><a  class=\"link_text\" role=\"button\" href=\"\/ja\/pediatric-retinal-diseases\/\"><span class=\"text\">Other Diseases<\/span><svg class=\"next-arrow\" aria-hidden=\"true\" width=\"20px\" height=\"25px\" viewbox=\"0 0 50 80\" xml:space=\"preserve\">\n  <polyline stroke=\"#ffffff\" stroke-width=\"9\" fill=\"none\" stroke-linecap=\"round\" stroke-linejoin=\"round\" points=\"0, 0 45, 40 0, 80\"\/>\n  <\/svg>  <span class=\"line\"  style=\"background-color: #ffffff;\"><\/span> <\/a><\/span><\/h6><\/div>\n\t\t<\/div> \n\t<\/div>\n\t<\/div> \n<\/div><\/div><div class=\"divider-wrap\" data-alignment=\"default\"><div style=\"height: 40px;\" class=\"divider\"><\/div><\/div>\n\t\t\t<\/div> \n\t\t<\/div>\n\t<\/div> \n\n\t<div  class=\"vc_col-sm-2\/5 vc_col-xs-12 wpb_column column_container vc_column_container col no-extra-padding inherit_tablet inherit_phone\"  data-padding-pos=\"all\" data-has-bg-color=\"false\" data-bg-color=\"\" data-bg-opacity=\"1\" data-animation=\"\" data-delay=\"0\" >\n\t\t<div class=\"vc_column-inner\" >\n\t\t\t<div class=\"wpb_wrapper\">\n\t\t\t\t<div id=\"fws_69f365c473444\" data-midnight=\"\" data-column-margin=\"default\" class=\"wpb_row vc_row-fluid vc_row inner_row  min_width_desktop_43vw min_width_tablet_50vw min_width_phone_50vw\"  style=\"\"><div class=\"row-bg-wrap\"> <div class=\"row-bg\" ><\/div> <\/div><div class=\"row_col_wrap_12_inner col span_12  left\">\n\t<div  class=\"vc_col-sm-12 wpb_column column_container vc_column_container col child_column no-extra-padding inherit_tablet inherit_phone\"   data-padding-pos=\"all\" data-has-bg-color=\"false\" data-bg-color=\"\" data-bg-opacity=\"1\" data-animation=\"\" data-delay=\"0\" >\n\t\t<div class=\"vc_column-inner\" >\n\t\t<div class=\"wpb_wrapper\">\n\t\t\t<div class=\"img-with-aniamtion-wrap\" data-max-width=\"100%\" data-max-width-mobile=\"default\" data-shadow=\"none\" data-animation=\"ro-reveal-from-right\" >\n      <div class=\"inner\">\n        <div class=\"hover-wrap\"> \n          <div class=\"hover-wrap-inner\">\n            <img loading=\"lazy\" decoding=\"async\" class=\"img-with-animation disease-detail-img skip-lazy\" data-delay=\"0\" height=\"468\" width=\"624\" data-animation=\"ro-reveal-from-right\" src=\"https:\/\/prrf.org\/wp-content\/uploads\/2025\/06\/FEVR-Eye-Image.jpg\" alt=\"Image of eye: The retina is drawn into a fold by scar tissue.\" srcset=\"https:\/\/prrf.org\/wp-content\/uploads\/2025\/06\/FEVR-Eye-Image.jpg 624w, https:\/\/prrf.org\/wp-content\/uploads\/2025\/06\/FEVR-Eye-Image-300x225.jpg 300w, https:\/\/prrf.org\/wp-content\/uploads\/2025\/06\/FEVR-Eye-Image-16x12.jpg 16w\" sizes=\"auto, (max-width: 624px) 100vw, 624px\" \/>\n          <\/div>\n        <\/div>\n      <\/div>\n    <\/div>\n<div class=\"wpb_text_column wpb_content_element\" style=\" max-width: 500px; display: inline-block;\">\n\t<div class=\"wpb_wrapper\">\n\t\t<p><em>The retina is drawn into a fold by scar tissue.<\/em><\/p>\n\t<\/div>\n<\/div>\n\n\n\n\n\t\t<\/div> \n\t<\/div>\n\t<\/div> \n<\/div><\/div>\n\t\t\t<\/div> \n\t\t<\/div>\n\t<\/div> \n<\/div><\/div>","protected":false},"excerpt":{"rendered":"Familial Exudative Vitreoretinopathy (FEVR) Introduction and History Familial Exudative Vitreoretinopathy, or FEVR, is a rare genetic eye condition that affects the development of blood vessels...","protected":false},"author":2,"featured_media":0,"parent":6486,"menu_order":34,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_coblocks_attr":"","_coblocks_dimensions":"","_coblocks_responsive_height":"","_coblocks_accordion_ie_support":"","om_disable_all_campaigns":false,"footnotes":""},"class_list":["post-7081","page","type-page","status-publish"],"_links":{"self":[{"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/pages\/7081","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/comments?post=7081"}],"version-history":[{"count":12,"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/pages\/7081\/revisions"}],"predecessor-version":[{"id":8530,"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/pages\/7081\/revisions\/8530"}],"up":[{"embeddable":true,"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/pages\/6486"}],"wp:attachment":[{"href":"https:\/\/prrf.org\/ja\/wp-json\/wp\/v2\/media?parent=7081"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}