{"orpha":99112,"name":"Absence of innominate vein","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Absence of brachiocephalic vein"],"definition":"A rare congenital anomaly of the great veins characterized by absence of the left brachiocephalic vein (or innominate vein), resulting in an anomalous venous vasculature. Patients are usually asymptomatic and the anomaly is typically discovered intraoperatively. An association with persistence of left superior vena cava, permanent levoatrial cardinal vein or anomaly of the inferior vena cava has been reported in some cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99112/","orphanet_url":"https://www.orpha.net/en/disease/detail/99112","names":{"fr":{"name":"Absence de veine innominée","synonyms":["Absence de veine brachiocéphalique"],"definition":"Une anomalie congénitale rare des gros vaisseaux caractérisée par l'absence de la veine gauche brachiocéphalique (ou veine innominée), conduisant à un système veineux anormal. Les patients sont habituellement asymptomatiques et l'anomalie est typiquement découverte de manière fortuite, pendant une intervention chirurgicale. Une persistance de la veine cave supérieure gauche, une veine cardinale lévo-atriale permanente ou une anomalie de la veine case inférieure ont été rapportées dans certains cas."},"de":{"name":"Fehlen der Kopf-Armvene","synonyms":["Fehlen der Vena innominata","Vena brachiocephalica, fehlende"],"definition":"Eine seltene kongenitale Anomalie der großen Venen, die durch das Fehlen der linken Kopf-Armvene (Vena brachiocephalica oder Vena innominata) gekennzeichnet ist, was zu eine Anomalie des venösen Gefäßsystems führt. Die Patienten sind in der Regel asymptomatisch und die Anomalie wird typischerweise intraoperativ entdeckt. In einigen Fällen wurde über eine Assoziation mit einer persistierenden linken Vena cava superior, einer persistierenden laevoatriale Kardinalvene oder einer Anomalie der Vena cava inferior berichtet."},"es":{"name":"Ausencia de vena innominada","definition":"Es una anomalía congénita poco frecuente de los grandes vasos caracterizada por ausencia de la vena braquiocefálica izquierda (o vena innominada), lo que resulta en una vasculatura venosa anómala. Los afectados suelen encontrarse asintomáticos y la anomalía suele detectarse intraoperatoriamente. En algunos casos se ha descrito una asociación con la vena cava superior izquierda persistente, la vena cardinal levoatrial permanente o anomalías de la vena cava inferior."},"it":{"name":"Aplasia della vena anonima","definition":"L'aplasia della vena anonima è un'anomalia congenita rara dei grandi vasi, caratterizzata dall'assenza della vena brachicefalica sinistra (o vena anonima), che esita in un'anomalia del sistema vascolare venoso. Di solito i pazienti sono asintomatici e l'anomalia viene individuata nel corso di interventi chirurgici. Sono stati documentati pazienti nei quali si associavano la persistenza della vena cava superiore sinistra o della vena cardinale levo-atriale oppure anomalie della vena cava inferiore."},"nl":{"name":"Afwezigheid van vena brachiocephalica","synonyms":["Afwezigheid van arm-hoofdader","Afwezigheid van brachiocefalische ader"],"definition":"Een zeldzame, congenitale anomalie van de grote aders, gekarakteriseerd door afwezigheid van de linker brachiocefalische ader (of vena innominata), wat resulteert in een abnormale veneuze vasculatuur. Patiënten zijn meestal asymptomatisch en de anomalie wordt doorgaans intraoperatief ontdekt. In sommige gevallen werd een associatie met persisterende linker vena cava superior, blijvende cardinale vene aan linkeratrium of anomalie van de onderste holle ader gerapporteerd."},"pt":{"name":"Ausência de veia inominada"},"pl":{"name":"Brak żyły bezimmiennej","synonyms":["Brak żyły ramienno-głowowej"]},"cs":{"name":"Chybění vena innominata","synonyms":["Chybění vena brachiocefalica"]},"uk":{"name":"Відсутність безіменної вени","synonyms":["Відсутність брахіоцефальної вени"],"definition":"A rare congenital anomaly of the great veins characterized by absence of the left brachiocephalic vein (or innominate vein), resulting in an anomalous venous vasculature. Patients are usually asymptomatic and the anomaly is typically discovered intraoperatively. An association with persistence of left superior vena cava, permanent levoatrial cardinal vein or anomaly of the inferior vena cava has been reported in some cases."},"zh":{"name":"无名静脉缺失","synonyms":["头臂静脉缺如"],"definition":"该病是一种罕见的先天性大静脉畸形，其特征是缺少左头臂静脉(或无名静脉)，导致静脉系统异常。患者一般没有症状，手术中通常会发现异常。一些病例报道与左上腔静脉持续存在、永久性左房主静脉或下腔静脉畸形有关。"},"tr":{"name":"Innominate ven yokluğu","synonyms":["Brakiyosefalik ven yokluğu"]}},"xrefs":[{"source":"ICD-10","code":"Q26.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA86.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0020443","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707656","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":95498,"name":"Congenital anomaly of superior vena cava","classifications":[147,148,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}