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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" article-type="case-report" dtd-version="1.1" specific-use="sps-1.8" xml:lang="en">
  <front>
    <journal-meta>
      <journal-id journal-id-type="publisher-id">ijcs</journal-id>
      <journal-title-group>
        <journal-title>International Journal of Cardiovascular Sciences</journal-title>
        <abbrev-journal-title abbrev-type="publisher">Int. J. Cardiovasc. Sci.</abbrev-journal-title>
      </journal-title-group>
      <issn pub-type="ppub">2359-4802</issn>
      <issn pub-type="epub">2359-5647</issn>
      <publisher>
        <publisher-name>Sociedade Brasileira de Cardiologia</publisher-name>
      </publisher>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v3">zFkv6HMjWw8nP3NKnCQBQjN</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S2359-56472018000500548</article-id>
      <article-id pub-id-type="doi">10.5935/2359-4802.20180053</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Case Reports</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Persistent Primitive Hypoglossal Artery Associated with Brain Stem Ischemia in an Elderly Patient</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Souto</surname>
            <given-names>Rafael Mansur</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
          <xref ref-type="aff" rid="aff2">2</xref>
          <xref ref-type="corresp" rid="c1"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Santos</surname>
            <given-names>Alair Augusto Sarmet Moreira Damas dos</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Nacif</surname>
            <given-names>Marcelo Souto</given-names>
          </name>
          <xref ref-type="aff" rid="aff1">1</xref>
          <xref ref-type="aff" rid="aff2">2</xref>
        </contrib>
      </contrib-group>
      <aff id="aff1">
        <institution content-type="orgname">Complexo Hospitalar de Niter&#243;i</institution>
        <institution content-type="orgdiv1">Centro de Imagem</institution>
        <addr-line>
          <city>Niter&#243;i</city>
          <state>RJ</state>
        </addr-line>
        <country country="BR">Brazil</country>
        <institution content-type="original">Centro de Imagem, Complexo Hospitalar de Niter&#243;i, Niter&#243;i, RJ - Brazil</institution>
      </aff>
      <aff id="aff2">
        <institution content-type="orgname">Universidade Federal Fluminense</institution>
        <institution content-type="orgdiv1">Programa de P&#243;s-gradua&#231;&#227;o em Ci&#234;ncias Cardiovasculares</institution>
        <addr-line>
          <city>Niter&#243;i</city>
          <state>RJ</state>
        </addr-line>
        <country country="BR">Brazil</country>
        <institution content-type="original">Programa de P&#243;s-gradua&#231;&#227;o em Ci&#234;ncias Cardiovasculares, Universidade Federal Fluminense, Niter&#243;i, RJ - Brazil</institution>
        <institution content-type="normalized">Universidade Federal Fluminense</institution>
      </aff>
      <author-notes>
        <corresp id="c1"><bold>Mailing Address: Rafael Mansur Souto</bold>. Rua Farme de Amoedo, n 149, Postal Code: 22420-020, Rio de Janeiro, RJ - Brazil. E-mail: <email>rafaelmansur1250@hotmail.com</email></corresp>
        <fn fn-type="con">
          <p>
            <bold>Author contributions</bold>
          </p>
          <p>Conception and design of the research: Souto RM, Santos AASMD, Nacif MS. Acquisition of data: Souto RM, Nacif MS. Analysis and interpretation of data: Souto RM, Nacif MS. Writing of the manuscript: Souto RM, Nacif MS. Critical revision of the manuscript for intellectual content: Souto RM, Santos AASMD, Nacif MS.</p>
        </fn>
        <fn fn-type="conflict">
          <p>
            <bold>Potential Conflict of Interest</bold>
          </p>
          <p>No potential conflict of interest relevant to this article was reported.</p>
        </fn>
      </author-notes>
      <pub-date pub-type="epub-ppub">
        <season>Sep-Oct</season>
        <year>2018</year>
      </pub-date>
      <volume>31</volume>
      <issue>5</issue>
      <fpage>548</fpage>
      <lpage>550</lpage>
      <history>
        <date date-type="received">
          <day>18</day>
          <month>08</month>
          <year>2017</year>
        </date>
        <date date-type="rev-recd">
          <day>05</day>
          <month>02</month>
          <year>2018</year>
        </date>
        <date date-type="accepted">
          <day>27</day>
          <month>03</month>
          <year>2018</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="https://creativecommons.org/licenses/by/4.0/" xml:lang="en">
          <license-p>This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
        </license>
      </permissions>
      <kwd-group xml:lang="en">
        <title>Keywords</title>
        <kwd>Cerebrovascular Disease, persistent primitive hypoglossal artery</kwd>
        <kwd>primitive arteries, carotid-basilar anastomoses, Arteriosclerosis</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <body>
    <sec sec-type="intro">
      <title>Introduction</title>
      <p>The primitive hypoglossal artery (PHA) crosses the hypoglossal canal, being one of the four vessels that acts as anastomosis between the primitive dorsal aorta and longitudinal neural arteries. The PHA typically retracts 40 days after pregnancy but may persist in some situations.<xref ref-type="bibr" rid="B1">1</xref>
			</p>
      <p>Direct anastomoses between the basilar and carotid arteries are rare.<xref ref-type="bibr" rid="B1">1</xref><sup>-</sup><xref ref-type="bibr" rid="B3">3</xref> These vessels retreat during the fortieth day of fetal development, when the emergence of the posterior communicating arteries occurs unicating vessels are the trigeminal, the primitive hypoglossal, the proatlantal and the ophthalmic vessels.<xref ref-type="bibr" rid="B1">1</xref><sup>,</sup><xref ref-type="bibr" rid="B2">2</xref><sup>,</sup><xref ref-type="bibr" rid="B4">4</xref> The persistence of the primitive hypoglossal artery has an incidence of 0.01% to 0.03%, being the second most frequent among the four vessels.<xref ref-type="bibr" rid="B4">4</xref><sup>-</sup><xref ref-type="bibr" rid="B6">6</xref>
			</p>
      <p>Persistent PHA (PPHA) occurs when it emerges at the level of C1 to C3 and enters the posterior fossa, crossing the hypoglossal canal, and ending at the basilar artery.<xref ref-type="bibr" rid="B4">4</xref> The posterior communicating artery is hypoplastic or absent. The diagnosis may be attained through angiotomography and angiography assessments.<xref ref-type="bibr" rid="B1">1</xref>
			</p>
    </sec>
    <sec sec-type="cases">
      <title>Case report</title>
      <p>An 80-year-old man, a patient with systemic arterial hypertension and diabetes mellitus, had malaise with lipothymia for two days, and therefore sought medical assistance. After he showed no improvement, he returned to the emergency unit a few days later, with persistent symptoms of dysarthria and apathy, as well as right hemiparesis. During his clinical evolution, he showed worsening of the condition and homolateral dysmetria.</p>
      <p>Initially, the patient was diagnosed with left-sided cerebrovascular accident (CVA) in the brainstem, with diffusion restriction disclosed by a skull MRI (<xref ref-type="fig" rid="f1">Figure 1A</xref>). Subsequently, the carotid and vertebral Doppler showed the following findings: calcified plaques in both carotid bulbs with approximately 50% of bilateral obstruction and preserved flow in both vertebral arteries.</p>
      <p>
        <fig id="f1">
          <label>Figure 1</label>
          <caption>
            <title>(A) Magnetic resonance showing diffusion restriction in the brain stem on the left. (B) Angiotomography with MIP reconstruction and bone subtraction, demonstrating the emergence of the hypoglossal artery and the left internal carotid artery. (C) Comparative image between the two carotid arteries and the emergence of the primitive hypoglossal on the left.</title>
          </caption>
          <graphic xlink:href="2359-5647-ijcs-31-05-0548-80053-gf01.jpg"/></fig>
      </p>
      <p>In the angiotomography of the carotid and vertebral arteries, PHA persistence was shown combined with the basilar artery (<xref ref-type="fig" rid="f1">Figures 1B</xref> and <xref ref-type="fig" rid="f1">1C</xref>), as well as atherosclerotic arterial disease with slight carotid bulb lumen reduction on the right and moderate on the left.</p>
      <p>In most cases, PPHA occurs only as an incidental finding at an examination indicated for other reasons. It is important, however, to be aware that when a carotid endarterectomy is intended for some other reason, there are some risks in the presence of such condition. Among these are the high carotid exposure and the challenges of maintaining cerebral perfusion.<xref ref-type="bibr" rid="B1">1</xref>
			</p>
      <p>In our case, the patient developed brainstem CVA, with subsequent hemorrhagic transformation. At the angiotomography, the patient showed the presence of mixed plaque, affecting the distal end of the left common carotid, bulb and ostium of the internal carotid artery, generating moderate luminal reduction of the internal carotid on this side. He also showed signs of contralateral carotid bulb atherosclerotic disease. This fact is described in the literature,<xref ref-type="bibr" rid="B6">6</xref> according to which patients with persistent hypoglossal artery have high risks of atherosclerotic disease and cerebrovascular accidents, as well as subarachnoid hemorrhage and aneurysms.</p>
      <p>PPHA is often identified in imaging tests; however, in the presence of atherosclerotic disease, it may be of clinical importance due to the increased chance of developing cerebral infarction.<xref ref-type="bibr" rid="B6">6</xref><sup>,</sup><xref ref-type="bibr" rid="B7">7</xref> Associated with this condition, the passage of emboli from the internal carotid artery to the vertebrobasilar system becomes possible.<xref ref-type="bibr" rid="B8">8</xref>
			</p>
      <p>Our patient did not receive dual antiplatelet therapy due to the hemorrhagic transformation of the CVA and was submitted to an expectant conduct, as recommended by the literature.<xref ref-type="bibr" rid="B9">9</xref> The drainage of bulky hematomas is essential for the maintenance of life, but more discrete hemorrhagic events can be followed without major interventions,<xref ref-type="bibr" rid="B9">9</xref> as in this case.</p>
    </sec>
    <sec sec-type="conclusions">
      <title>Conclusion</title>
      <p>The above case report showed the clinical importance of the persistence of primitive arteries, more specifically the primitive hypoglossal artery, in a context of cerebrovascular accident and its consequences.</p>
    </sec>
  </body>
  <back>
    <fn-group>
      <fn fn-type="financial-disclosure">
        <p>
          <bold>Sources of Funding</bold>
        </p>
        <p>There were no external funding sources for this study.</p>
      </fn>
      <fn fn-type="other">
        <p>Study Association</p>
        <p>This article is part of the thesis of master submitted by Rafael Mansur Souto, from Universidade Federal Fluminense (UFF).</p>
      </fn>
    </fn-group>
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