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    <IdentifierDoi>10.3205/oc000160</IdentifierDoi>
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    <ArticleType>Case Report</ArticleType>
    <TitleGroup>
      <Title language="en">Bevacizumab as a treatment option for choroidal neovascularisation due to large optic nerve drusen in a 14-year-old girl</Title>
    </TitleGroup>
    <CreatorList>
      <Creator>
        <PersonNames>
          <Lastname>Steinhorst</Lastname>
          <LastnameHeading>Steinhorst</LastnameHeading>
          <Firstname>Nils Alexander</Firstname>
          <Initials>NA</Initials>
          <AcademicTitleSuffix>MD</AcademicTitleSuffix>
        </PersonNames>
        <Address>Department of Ophthalmology, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246 Hamburg, Germany, Phone: &#43;49 40 741054417<Affiliation>Department of Ophthalmology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany</Affiliation></Address>
        <Email>n.steinhorst&#64;uke.de</Email>
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      <Creator>
        <PersonNames>
          <Lastname>Spitzer</Lastname>
          <LastnameHeading>Spitzer</LastnameHeading>
          <Firstname>Martin</Firstname>
          <Initials>M</Initials>
          <AcademicTitleSuffix>PhD, MD</AcademicTitleSuffix>
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        <Address>
          <Affiliation>Department of Ophthalmology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany</Affiliation>
        </Address>
        <Creatorrole corresponding="no" presenting="no">author</Creatorrole>
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      <Creator>
        <PersonNames>
          <Lastname>Skevas</Lastname>
          <LastnameHeading>Skevas</LastnameHeading>
          <Firstname>Christos</Firstname>
          <Initials>C</Initials>
          <AcademicTitleSuffix>PhD, MD</AcademicTitleSuffix>
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        <Address>
          <Affiliation>Department of Ophthalmology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany</Affiliation>
        </Address>
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    <PublisherList>
      <Publisher>
        <Corporation>
          <Corporatename>German Medical Science GMS Publishing House</Corporatename>
        </Corporation>
        <Address>D&#252;sseldorf</Address>
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    <SubjectGroup>
      <SubjectheadingDDB>610</SubjectheadingDDB>
      <Keyword language="en">juvenile</Keyword>
      <Keyword language="en">optic disc drusen</Keyword>
      <Keyword language="en">choroidal neovascularisation</Keyword>
      <Keyword language="en">cystoid edema</Keyword>
      <Keyword language="en">bevacizumab</Keyword>
    </SubjectGroup>
    <DatePublishedList>
      
    <DatePublished>20200806</DatePublished></DatePublishedList>
    <Language>engl</Language>
    <License license-type="open-access" xlink:href="http://creativecommons.org/licenses/by/4.0/">
      <AltText language="en">This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License.</AltText>
      <AltText language="de">Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung).</AltText>
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    <SourceGroup>
      <Journal>
        <ISSN>2193-1496</ISSN>
        <Volume>10</Volume>
        <JournalTitle>GMS Ophthalmology Cases</JournalTitle>
        <JournalTitleAbbr>GMS Ophthalmol Cases</JournalTitleAbbr>
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    <ArticleNo>33</ArticleNo>
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    <Abstract language="en" linked="yes"><Pgraph><Mark1>Purpose:</Mark1> To report the effects of a single intravitreous injection of bevacizumab for the treatment of secondary choroideal neovascularisation due to large optic disc drusen.</Pgraph><Pgraph><Mark1>Methods:</Mark1> A 14-year-old female patient with painless loss of vision i<TextGroup><PlainText>n one e</PlainText></TextGroup>ye presented with unusually large optic disc drusen and j<TextGroup><PlainText>uxtapapillar</PlainText></TextGroup>y choroidal neovascularisation with subretinal hemorrhag<TextGroup><PlainText>e. S</PlainText></TextGroup>he was treated with a single intravitreous injection of bevacizumab.</Pgraph><Pgraph><Mark1>Results:</Mark1> Visual acuity increased from 20&#47;100 to 20&#47;25 within 4 weeks after injection and remained at this level during the 12-month follow-up period.</Pgraph><Pgraph><Mark1>Conclusions:</Mark1> Bevacizumab is a possible primary treatment option for secondary choroidal neovascularisation due to large optic disc drusen in children as an alternative to other more invasive or complex procedures.</Pgraph></Abstract>
    <TextBlock linked="yes" name="Introduction">
      <MainHeadline>Introduction</MainHeadline><Pgraph>Optic disc drusen (ODD) are remnants of mucopolysa<TextGroup><PlainText>ccharides f</PlainText></TextGroup>rom degenerated ganglion cells which calcify and accumulate within the optic nerve head over time <TextLink reference="1"></TextLink>. The incidence of ODD in children is reported to b<TextGroup><PlainText>e 0</PlainText></TextGroup>.4&#37;. They seem to become visible around the age o<TextGroup><PlainText>f 1</PlainText></TextGroup>2 <TextLink reference="2"></TextLink>.</Pgraph><Pgraph>With increasing size, they raise the optic disc, blur out the edges, and lead to abnormal vascular branching and formation of cilioretinal vessels, causing hemorrhages <TextLink reference="3"></TextLink>. This destruction of the parapapillary anatomy can cause visual field defects and the formation of choroidal neovascular membranes (CNVM) that lead to decreased visual acuity (VA) <TextLink reference="4"></TextLink>.</Pgraph><Pgraph>Because of the rare occurrence of CNVM associated with ODD, different therapeutic approaches have been repo<TextGroup><PlainText>rted o</PlainText></TextGroup>nly via small case studies. Next to surgical rem<TextGroup><PlainText>oval </PlainText></TextGroup><TextLink reference="5"></TextLink> and photodynamic therapy <TextLink reference="6"></TextLink> or laser coagulation <TextLink reference="7"></TextLink>, intravitreous injection of anti-VEGF agents seems to be an option with long-term satisfying results <TextLink reference="8"></TextLink>, <TextLink reference="9"></TextLink>, <TextLink reference="10"></TextLink>, <TextLink reference="11"></TextLink>.</Pgraph></TextBlock>
    <TextBlock linked="yes" name="Patient and methods">
      <MainHeadline>Patient and methods</MainHeadline><Pgraph>A 14-year-old female patient presented at our clinic with painless loss of vision in her left eye, slowly progressing for over one week. VA had dropped to 20&#47;100 on the affected side, whilst retaining 20&#47;20 vision on the other eye. Intraocular pressure was within physiological limits. Further examination showed a blurry and prominent optic disc with an adjacent decent subretinal hemorrhage (Figure 1 <ImgLink imgNo="1" imgType="figure"/>). The contralateral optic disc also displayed a slight blurriness, but there was no sign of subretinal hemorrhage. Optical coherence tomography (OCT) showed the formation of a juxtapapillary cystoid macula edema (CME) with subretinal scarring. Corresponding to this, the intravenous fluorescein angiography (IVFA) displayed leakage at this location throughout all phases, followed by pooling. Additionally, ultrasound was carried out, which exposed unusual large and deeply located ODD on both eyes. These were however significantly larger and closer to the optic disc&#8217;s surface on the affected left eye.</Pgraph><Pgraph>After discussing the potential treatment options and risks with the child&#8217;s parents, a single intravitreous injection of 1.25 mg bevacizumab was given under general anesthesia.</Pgraph></TextBlock>
    <TextBlock linked="yes" name="Results">
      <MainHeadline>Results</MainHeadline><Pgraph>VA increased to 20&#47;25 over a period of 4 weeks. The cystoid edema as well as the subretinal hemorrhage resolved, leaving a small subretinal scar and a mild dissociation of the pigment epithelium (Figure 2 <ImgLink imgNo="2" imgType="figure"/>). The patient and her parents were instructed to perform self-tests using the Amsler grid and to report to the clinic, should any deterioration occur. Further appointments were scheduled regularly at an interval of 4 weeks for 6 months overall, then followed by examinations every 12 weeks. During this phase, the VA fluctuated between 20&#47;25 and 20&#47;20. There was no recurrence of CME or subretinal bleeding during the follow-up period.</Pgraph></TextBlock>
    <TextBlock linked="yes" name="Discussion">
      <MainHeadline>Discussion</MainHeadline><Pgraph>In the last decade, intravitreal injections of anti-VEGF agents have proven to be an effective therapy for a variety of retinal and especially macula diseases, for example neovascular age-related degeneration and diabetic macular edema. Disease-adapted guidelines were promoted and published for the initial therapy as well as for the long-term intervention <TextLink reference="11"></TextLink>, <TextLink reference="12"></TextLink>, <TextLink reference="13"></TextLink>. Because of the rare incidence of CNVM secondary to ODD, especially in children, there are no established or widely accepted treatment regimens until today.</Pgraph><Pgraph>In our case, the patient showed a complete remission after only one injection. The options of further injections at a fixed interval, or of close observation and treatment only if required were discussed with the patient and her parents. The decision was made towards short-interval follow-up examinations. Compared to the results of anti-VEGF therapy that have been published in other case series, the juvenile secondary CNVM in our case respo<TextGroup><PlainText>nded b</PlainText></TextGroup>etter to this form of treatment. Until today, no further injections were necessary, confirming our choice of treatment plan.</Pgraph></TextBlock>
    <TextBlock linked="yes" name="Notes">
      <MainHeadline>Notes</MainHeadline><SubHeadline>Competing interests</SubHeadline><Pgraph>The authors declare that they have no competing interests.</Pgraph></TextBlock>
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          <Caption><Pgraph><Mark1>Figure 1: A) Funduscopy revealed a prominent and blurry optic disc with subretinal hemorrhage spreading towards the edge of the macula region; B) CNVM made visible through mid-phase IVFA; C) OCT scan with ODD and parapapillary cystoid edema, subretinal scarring; D) Shallow but prominent ODD are revealed through hyperreflectivity in low-gain mode using ultrasound.</Mark1></Pgraph></Caption>
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          <Caption><Pgraph><Mark1>Figure 2: Fundus image and OCT scan A) prior to treatment and B) 12 months after injection. The subretinal hemorrhage and cystoid edema resolved, leaving behind a dissociation of pigment epithelium and a shallow subretinal scar.</Mark1></Pgraph></Caption>
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