<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1646-706X</journal-id>
<journal-title><![CDATA[Angiologia e Cirurgia Vascular]]></journal-title>
<abbrev-journal-title><![CDATA[Angiol Cir Vasc]]></abbrev-journal-title>
<issn>1646-706X</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Angiologia e Cirurgia Vascular]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1646-706X2021000400325</article-id>
<article-id pub-id-type="doi">10.48750/acv.461</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Different clinical presentations of a rare venous malformation-inferior vena cava agenesis]]></article-title>
<article-title xml:lang="pt"><![CDATA[Espetro de apresentação clínica de uma malformação venosa rara: agenesia da veia cava inferior]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Machado]]></surname>
<given-names><![CDATA[Marta]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Coelho]]></surname>
<given-names><![CDATA[Nuno]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Maximiano]]></surname>
<given-names><![CDATA[Pedro]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Semião]]></surname>
<given-names><![CDATA[Carolina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Peixoto]]></surname>
<given-names><![CDATA[João]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Fernandes]]></surname>
<given-names><![CDATA[Luís]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Brandão]]></surname>
<given-names><![CDATA[Pedro]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Canedo]]></surname>
<given-names><![CDATA[Alexandra]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro Hospitalar Vila Nova de Gaia / Espinho Serviço de Angiologia e Cirurgia Vascular ]]></institution>
<addr-line><![CDATA[Porto ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>30</day>
<month>12</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>30</day>
<month>12</month>
<year>2021</year>
</pub-date>
<volume>17</volume>
<numero>4</numero>
<fpage>325</fpage>
<lpage>329</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S1646-706X2021000400325&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S1646-706X2021000400325&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S1646-706X2021000400325&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Introduction:  Inferior vena cava agenesis is one of the most uncommon anomalies of this vessel, with an estimated prevalence of 0.0005-1% in the general population. However, around 5% of the patients younger than 30 years with a diagnosis of deep vein thrombosis have this anomaly.  Methods and material:  Report of two clinical cases of inferior vena cava agenesis with different clinical presentations. Clinical case 1: A 40-year-old man was admitted with a 3 days history of unilateral lower limb swelling and pain, gradually progressing to the inability to walk. On physical examination he had swelling, bruising and tenderness involving leg and thigh and prominent engorged superficial abdominal collateral veins. Venous Doppler Ultrasound showed left deep venous thrombosis extending from popliteal vein to common iliac vein. A computed tomography angiogram showed agenesis of the infrarenal vena cava and patent renal veins draining in to the azygous system and hemiazygous systems. The patient as discharged with rivaroxaban and compression stockings. At 2 months follow up he was asymptomatic and still anticoagulated. Clinical case 2: A 35 year- old woman, with a previous history of recurrent lower limb varicose veins surgery and left internal malleolar ulcer at 30 years presented at medical department 3 years later with complains of ulcer recurrence. A computed tomography angiogram revealed an absent infrahepatic vena cava, prominently dilated azygos and hemiazygos veins with enlarged retroperitoneal collaterals. The patient initiated dressing care with oxide zinc and oral rivaroxaban.  Discussion/conclusion:  The majority of cases remain asymptomatic. However, when symptomatic, the majority present as proximal DVT involving the iliac and femoral veins. CT or MRI should be the imagiological methods used to diagnose this anomaly. No clear consensus has been reached on therapeutic strategy, other than long-term anticoagulation and elastic stockings.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo  Introdução:  A agenesia da veia cava inferior é uma das anomalias mais raras deste vaso, com uma prevalência estimada de 0,0005-1% na população geral. Porém, cerca de 5% dos pacientes com menos de 30 anos com uma trombose venosa profunda apresentam esta anomalia.  Métodos e material:  Relato de dois casos clínicos de agenesia da veia cava inferior com diferentes apresentações clínicas. Caso clínico 1: Um homem de 40 anos recorreu à urgência com queixas de edema e dor no membro inferior esquerdo, progredindo gradualmente para incapacidade de marcha. Ao exame físico foi encontrado edema e dor a palpação da perna e coxa, bem como veias colaterais abdominais superficiais proeminentes. O ecodoppler venoso mostrou trombose venosa profunda esquerda estendendo-se da veia poplítea até a veia ilíaca comum. O angio-TC mostrou agenesia da veia cava infrarrenal e veias renais a drenar para os sistemas ázigos e hemiázigos. O paciente teve alta com rivaroxabano e meias elásticas. Aos 2 meses mantinha-se hipocoagulado e estava assintomático. Caso clínico 2: Uma mulher de 35 anos, com história prévia de cirurgia de varizes de membros inferiores recorrentes e úlcera do maléolo interno esquerdo, apresentou-se na consulta com recorrência da úlcera. O angio-TC revelou ausência de veia cava infra-hepática, veias ázigos e hemiázigos dilatadas com colaterais retroperitoneais dilatadas. A paciente iniciou cuidados de penso com óxido de zinco e rivaroxabano oral.  Discussão/conclusão:  A maioria dos pacientes com agenesia da veia cava inferior permanece assintomática. No entanto, quando sintomática, a maioria apresenta-se com uma trombose venosa profunda proximal envolvendo as veias ilíaca e femoral. A TC ou RM devem ser os métodos imagiológicos usados para diagnosticar esta anomalia. Nenhum consenso claro foi alcançado sobre a estratégia terapêutica, além da anticoagulação de longo prazo e uso de meias elásticas.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Inferior Vena Cava Agenesis]]></kwd>
<kwd lng="en"><![CDATA[Deep Vein Thrombosis]]></kwd>
<kwd lng="en"><![CDATA[Vascular anomalies]]></kwd>
<kwd lng="pt"><![CDATA[Agenesia da Veia Cava Inferior]]></kwd>
<kwd lng="pt"><![CDATA[Trombose venosa profunda]]></kwd>
<kwd lng="pt"><![CDATA[Anomalias vasculares]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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