A Truncus Bicaroticus and an Aberrant Right Subclavian Artery. A Rare Case and Literature Review / Un Tronco Bicarótico y una Arteria Subclavia Derecha Aberrante. Un Caso Raro y Revisión de la Literatura

SUMMARY: An aberrant right subclavian artery described by David Bayford, is rare and one of the aortic arch anomalies. It gives usually incidental findings. We present the case of a 57-year-old woman who was admitted to a neurology outpatient clinic due to a headache and no obvious pathology was detected during a physical examination. In consequence of the brain and chest CT angiography, incidental anomalies of the aortic arch branches were found and asymptomatic aberrant right subclavian artery and bicarotid trunk was diagnosed. The presence of this anomaly is becoming increasingly important in avoiding vascular injuries and cerebral complications in patients undergoing endovascular intervention on the aorta aortic arch surgery, thyroidectomy, or cervicothoracic surgery. So, detailed knowledge of variations of the subclavian artery and bicarotid trunk is paramount for radiologists and surgeons operating on the region of the anterior neck.

Una arteria subclavia derecha aberrante descrita por David Bayford, es rara y es una de las anomalías del arco aórtico. Además, suele ser hallazgos accidentales. Presentamos el caso de una mujer de 57 años que ingresó a una consulta de neurología por dolor de cabeza, sin embargo, no se le detectó patología evidente al examen físico. En el angio-TC de cerebro y tórax, se encontraron anomalías incidentales de las ramas del arco aórtico y se diagnosticó arteria subclavia derecha aberrante asintomática y tronco bicarotídeo. Determinar la presencia de esta anomalía es cada vez más importante, para así evitar lesiones vasculares y complicaciones cerebrales en pacientes sometidos a intervención endovascular de la aorta, cirugía del arco aórtico, tiroidectomía o cirugía cervicotorácica. Por lo tanto, el conocimiento detallado de las variaciones de la arteria subclavia y el tronco bicarotídeo es primordial para los radiólogos y cirujanos que operan en la región anterior del cuello.

Aberrant right subclavian artery: a series of case reports and discussion of morphology and its clinical and surgical implications / Arteria subclavia derecha aberrante, una serie de informes de casos y discusión de la morfología y sus implicaciones clínicas y quirúrgicas

SUMMARY: Aberrant right subclavian artery (arteria lusoria) is a rare embryological abnormality but the most common among aortic arch vascular anomalies. It represents an anatomical variant of right subclavian artery originating as the last branch of aortic arch, passing then retroesophageal to the normal position. It is usually asymptomatic and is found mostly incidentally during imaging examinations. Symptoms are produced when the aberrant artery compresses the nearby structures and the most frequent symptoms are dysphagia and dyspnea. It may be associated with other vascular or heart abnormalities. We are presenting a series of case reports which presented an aberrant right subclavian artery alone or associated with other vascular abnormalities, diagnosed incidentally in adult patients while performing CT examinations for other reasons. Through a literature review we aim to discuss the clinical implications of this vascular anomaly, to point out the importance of being aware of it especially in patients with dysphagia or dyspnea or in patients who undergo operations in the thorax and neck or vascular surgery and endovascular procedures involving the aortic arch and its branches.

RESUMEN: La arteria subclavia derecha aberrante (Arteria lusoria) es una anomalía embriológica rara, pero la más común entre las anomalías vasculares del arco aórtico. Representa una variante anatómica de la arteria subclavia derecha que se origina como la última rama del arco aórtico, pasando luego retroesofágicamente a la posición normal. Por lo general, esta anomalía es asintomática y se encuentra principalmente de manera incidental durante los exámenes de imagen. Los síntomas se producen cuando la arteria aberrante comprime las estructuras cercanas y los síntomas más frecuentes son la disfagia y la disnea. Puede estar asociado con otras anomalías vasculares o cardíacas. Presentamos una serie de informes de casos en los que se presentó una arteria subclavia derecha aberrante única o asociada a otras anomalías vasculares, diagnosticada incidentalmente en pacientes adultos durante la realización de TC por otros motivos. A través de una revisión bibliográfica pretendemos discutir las implicaciones clínicas de esta anomalía vascular, señalar la importancia de conocerla especialmente en pacientes con disfagia o disnea o en pacientes sometidos a operaciones de tórax y cuello o cirugía vascular y procedimientos endovasculares. involucrando el arco aórtico y sus ramas.

Endovascular Treatment in the Subclavian Steal Syndrome: Series of 29 Patients

Subclavian steal syndrome is a group of symptoms resulting fromretrograde flow in the vertebral artery, "stealing" blood from the posterior intracranial circulation and other territories, caused by stenosis or occlusion of the subclavian artery proximal to the origin of the same vertebral artery, or even of the brachiocephalic trunk. Most of the time, it is an incidental finding in patients with other conditions or cerebrovascular risk factors. We report a series of 29 patients with an angiographic diagnosis, in which 7 received treatment (all endovascular), all with symptoms directly related to this condition. Advanced age, systemic arterial hypertension, diabetes mellitus, smoking and stroke were comorbidities frequently related. Six patients improved completely after the procedure and one remained with vertigo.

Prenatal Diagnosis of Aberrant Right Subclavian Artery: Association with Genetic Abnormalities / Diagnóstico pré-natal de artéria subclávia direita anômala: associação com anormalidades genéticas

Abstract Objective The objective of the present study was to determine the frequency of malformations and chromosomal abnormalities in a population of fetuses with an aberrant right subclavian artery (ARSA). Methods This is a 6-year retrospective study of fetuses with a prenatal diagnosis of ARSA conducted during the period between September 2013 and June 2019 at a fetal medicine unit. Data were collected from ultrasound, fetal echocardiograms, genetic studies, and neonatal records. Results An ARSA was diagnosed in 22 fetuses. An ARSA was an isolated finding in 18 out of 22 cases (82%). Associated abnormal sonographic findings were found in 4 cases. All cases underwent invasive testing. In 1 of the cases, a chromosomal abnormality was detected (mos 45,X [13]/46,X,e(X) (p22.1q22.1)). No cases of congenital heart disease were found in any of these fetuses. There were two cases in which the postnatal evaluation revealed amalformation: one case of hypospadias and 1 case of cleft palate. Conclusion The presence of an isolated ARSA is benign and is not associated with chromosomal abnormalities. The finding of ARSA, however, warrants a detailed fetal ultrasound in order to exclude major fetal abnormalities and other soft markers.

Resumo Objetivo O objetivo do presente estudo foi determinar a frequência demalformaçães e anomalias cromossômicas em uma população de fetos com artéria subclávia direita aberrante (ARSA). Métodos Este é um estudo retrospectivo de 6 anos de fetos com diagnóstico prénatal de ARSA realizado durante o período de setembro de 2013 a junho de 2019 em uma unidade de medicina fetal. Os dados foram coletados de ultrassom, ecocardiograma fetal, estudos genéticos e registros neonatais. Resultados Um ARSA foi diagnosticado em 22 fetos. Um ARSA foi um achado isolado em 18 dos 22 casos (82%). Achados ultrassonográficos anormais associados foram encontrados em 4 casos. Todos os casos foram submetidos a testes invasivos. Em um dos casos, foi detectada uma anormalidade cromossômica (mos 45, X [13] / 46, X, e (X) (p22.1q22.1)). Nenhum caso de doença cardíaca congênita foi encontrado em qualquer um desses fetos. Houve dois casos em que a avaliação pós-natal revelou a malformação: um caso de hipospádia e 1 caso de fenda palatina. Conclusão A presença de ARSA isolado é benigna e não está associada a anormalidades cromossômicas. O achado de ARSA, no entanto, justifica uma ultrassonografia fetal detalhada para excluir anormalidades fetais importantes e outros marcadores leves.

Aberrant right subclavian artery in a dog - case report / [Ectopia de artéria subclávia direita em cão - relato de caso]

The brachiocephalic trunk and the left subclavian artery originate from the aortic arch, and both supply blood to the head, neck, and thoracic limbs. Anatomical variations, such as an aberrant right subclavian artery, are congenital conditions rarely observed in dogs, Thus, the objective of the present report was to describe a case of aberrant right subclavian artery in a 9-year-old Dalmatian. However, this anomaly was a finding in which the patient was asymptomatic during its 9 years of life and only at this age did he exhibit signs including sialorrhea, vomiting, hyporexia, and noisy deglutition. Blood count, biochemical profile, and thoracic radiography led to a diagnosis of megaesophagus and aspiration pneumonia. Despite the recommended treatment, the patient did not respond well; as such, the owner elected to euthanize the animal. On necropsy, the right subclavian artery originated directly from the aortic arch, followed a route from left to right dorsally to the esophagus, and then formed an impression of the vascular path over the muscular wall of the esophagus. The esophagus, in turn, exhibited a flaccid wall and dilation in the caudal portion to the vascular path made by the ectopic position of the right subclavian artery.(AU)

O tronco braquiocefálico e a artéria subclávia esquerda emergem do arco aórtico e são responsáveis por fazerem o suprimento sanguíneo para cabeça, pescoço e membros torácicos. Variações anatômicas, como a ectopia da artéria subclávia direita, são alterações congênitas raramente encontradas em cães, cujas alterações do sistema digestivo acontecem em pacientes recém-desmamados e não em adultos. Assim, o objetivo deste relato é descrever um caso de ectopia da artéria subclávia direita em uma cadela, Dálmata, de nove anos de idade. No entanto, essa anomalia foi um achado do qual o paciente foi assintomático durante os nove anos de vida e somente com essa idade apresentou sinais como sialorreia, vômito, hiporexia e deglutição ruidosa. O hemograma e os perfis bioquímicos, associados à radiografia torácica, levaram a um diagnóstico de megaesôfago e pneumonia aspirativa. Mesmo seguindo o tratamento recomendado, houve piora clínica do quadro e o animal foi submetido à eutanásia. À macroscopia, a artéria subclávia direita originava-se direto do arco aórtico, fazia um percurso da esquerda para a direita dorsalmente ao esôfago e, então, formava uma impressão do trajeto vascular sobre a parede muscular do esôfago. O esôfago, por sua vez, apresentava parede flácida e dilatação na porção caudal ao trajeto vascular feito pela posição ectópica da artéria subclávia direita.(AU)

Hybrid treatment of complex aortic arch anomaly with saccular aneurysm

Abstract Aortic arch anomalies are not clinically important unless they cause compression symptoms due to aneurysmatic dilatation. Aortic anomalies need to be treated when they cause complex thoracic aortic diseases, and the treatment approach has evolved over time from open surgical methods, which have high mortality and morbidity rates, to hybrid methods. A case of a 68-year-old male patient with complex aortic arch anomaly treated with hybrid arch repair is reported in this study. Aortic branches were common carotid trunk and aberrant right subclavian artery with a saccular aneurysm.

Arteria subclavia derecha retroesofágica: reporte de caso / Right retroesophageal subclavian artery: case report

RESUMEN: Durante el desarrollo embrionario suelen ocurrir anomalías a nivel del arco aórtico primordial. Una de estas es la arteria subclavia retroesofágica derecha (ASDR) la cual es un defecto frecuente de los arcos aórticos embriológicos y se debe a la interrupción del cuarto arco aórtico (AO) derecho entre las depresiones para la arteria carótida común y la arteria subclavia. Las variaciones anatómicas y morfológicas del AO y sus ramas son significativas para los procedimientos diagnósticos y quirúrgicos en el tórax y el cuello. Reportar una ASDR encontrada durante disección, correlacionando esta con sus implicancias clínicas. De un cadáver de sexo femenino, de edad y causa de muerte desconocida, fijado con formaldehído al 10 % y aguada destilada. Se realizó disección del corazón y los grandes vasos, en especial el AO, desarrollado según la técnica convencional, para remover el órgano luego de disecar el pericardio y disecar la arteria aorta ascendente y el AO, preservando plenamente su configuración externa y sus ramas colaterales, donde se encontró la ASDR. La ASDR presentó un diámetro en su origen de 12,13 mm. La longitud existente entre la ASDR y la Arteria carótida común Derecha (ACCD) fue de 43,84 mm. El diámetro del esófago a nivel cefálico y caudal de la ASDR alcanzó valores de 17,59 mm y 13,82 mm respectivamente. Por su parte los diámetros de la tráquea a nivel cefálico y caudal a la ASDR, fueron respectivamente 22,12 mm y 13,30 mm. El conocimiento de esta variante anatómica resulta de gran interés a la hora de interpretar y orientar el diagnóstico de las posibles causas de una disfagia asociada a una arteria subclavia retroesofágica y fundamenta aún más la importancia del estudio mediante disección.

SUMMARY: During embryonic development, anomalies usually occur at the primordial aortic arch (AA) level. One of these is the right retroesophageal subclavian artery (RSA). The anatomical and morphological variations of AA and its branches are significant for diagnostic and surgical procedures in the thorax and neck. The objective of the study was to report an RSA found during the dissection, correlating this with the corresponding clinical implications. A human female cadaver of unknown age and cause of death was used and conserved in 10 % formaldehyde and distilled wash. The dissection of the heart and large vessels was performed. There was particular emphasis one the AA, and development according to conventional techniques, removing the organ after dissecting the pericardium and dissecting the ascending aorta and the AA. External function and its collateral branches were fully preserved, where the RSA was located. The RSA presented a diameter at its origin of 12.13 mm. The length between the RSA and the common right carotid artery (CRCA) was 43.84 mm. The diameter of the esophagus at the cephalic and caudal level of the RSA has values of 17.59 mm and 13.82 mm respectively. Furthermore, the diameters of the trachea at cephalic and caudal level to the RSA, respectively, were 22.12 mm and 13.30 mm. Knowledge of this anatomical variant is of great interest when interpreting and guiding the diagnosis of potential causes of a dysphagia associated with a retroesophageal subclavian artery, and is even more important during dissection studies.

Arteria subclavia aberrante izquierda, hallazgo infrecuente / Aberrant left subclavian artery, an uncommon variation

Objetivo: La arteria subclavia aberrante (ASA) o arteria lusoria, es una de las malformaciones más comunes del arco aórtico; en especial su variante derecha. Por otro lado, la arteria subclavia aberrante izquierda es más rara. Presentamos un caso infrecuente de un paciente con síndrome deleción 22q11.2 y ASA izquierda con síntomas en extremidad superior izquierda. La ASA izquierda es una condición muy poco frecuente y hay escasa información sobre su tratamiento quirúrgico. La mayoría de las veces, el ASA es asintomática; especialmente en adultos. Si presenta clínica, sus síntomas son disfagia, tos, disnea, claudicación de la extremidad superior. La presencia de sintomatología es una indicación quirúrgica. El tratamiento quirúrgico de una ASA es discutido. Éste va a depender de la anatomía, comorbilidades y experiencia del cirujano.

Objective: The Aberrant Right Subclavian Artery (ARSA) or Lusoria Artery is one of the most common aortic arch malformations. Aberrant Left Subclavian Artery (ALSA) on the other hand, is a much rarer condition. We present an uncommon case of ALSA in a patient with 22q11.2 deletion syndrome with upper limb symptoms and review the treatment options. ALSA is an exceedingly rare condition and information on its surgical treatment is scarce. In most cases the presence of an ALSA is asymptomatic, especially in adults. Some of the most typical symptoms are dysphagia (dysphagia lusoria), cough, dyspnea, claudication of the upper limb extremity. When symptomatic, the patient has indication of surgery. The treatment of an ALSA is still debatable and it depends on the anatomy, comorbidities and surgeon´s experience.

Endovascular Treatment in the Subclavian Steal Syndrome

When the proximal occlusion or stenosis of the subclavian or of the brachiocephalic artery may require distal arterial filling through reversal flow from the vertebral artery, causing clinically significant blood supply reduction to the brainstem, it is called subclavian steal syndrome (SSS). We report a 54-year-old male patient who presented with multiple episodes of syncopes and vascular claudication due to right SSS. He underwent an angioplasty, evolving with complete improvement of the symptoms. We review the clinical presentation, the diagnosticmethods, and the treatment options of the disease.

Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular / Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring

Resumen: El arco aórtico derecho puede estar asociado a subclavia izquierda aberrante, en algunos casos esta se origina de una dilatación aneurismática que se conoce como divertículo de Kommerell. Se presentan 2 casos de anillo vascular formado por un arco aórtico derecho, subclavia izquierda anómala con divertículo de Kommerell y persistencia del conducto arterioso izquierdo con una revisión de la literatura acerca del desarrollo embriológico y los métodos de imagen que ayudan al diagnóstico de esta rara anomalía vascular.

Abstract: The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

Clinical discussion of the arteria lusoria: a case report / Discussão clínica a respeito da artéria lusória: um relato de caso

Abstract The right subclavian artery may originate from the left portion of the aortic arch. This aberrant vessel is known as the arteria lusoria. Its course to its usual site runs behind the esophagus, which may cause a disease known as dysphagia lusoria, responsible for symptoms of discomfort. This artery is often associated with other anomalies, such as the non-recurrent laryngeal nerve and the bicarotid trunk, and with diseases such as aneurysms, congenital heart defects, and even genetic syndromes. During routine dissection of a male cadaver fixed in 10% formalin solution, an arteria lusoria was found. This article reports the variation and discusses its embryological, clinical and surgical aspects.

Resumo Em alguns casos, a artéria subclávia direita pode se originar da porção esquerda do arco aórtico. Esse vaso aberrante é conhecido como artéria lusória. Para chegar em sua região, essa artéria passa posteriormente ao esôfago, e pode ser, portanto, causa de uma doença conhecida como disfagia lusória, desencadeando sintomas desconfortantes. A artéria lusória está frequentemente associada com outras anomalias, como o nervo laríngeo não recorrente e o tronco bicarotídeo, assim como pode estar em associação com aneurismas, defeitos cardíacos congênitos e até síndromes genéticas. Durante dissecação cadavérica de rotina, foi observada a presença dessa artéria em um cadáver do sexo masculino fixado em uma solução de formalina a 10%. O objetivo deste trabalho é relatar a variação conhecida como artéria lusória e trazer destaque para seus aspectos embriológicos, clínicos e cirúrgicos.

Hallazgo de arteria lusoria en el estudio de otitis media a repetición: a propósito de una observación pediátrica y revista breve de la literatura / Arteria Lusoria revealed by a accute media otitis: about a pediatric observation and brieve review of literatura

Arteria Lusoria (AL) o arteria subclavia aberrante derecha es una malformación vascular rara. Es la más común de las malformaciones del arco aórtico. Fue descrita por primera vez por Hunalud en 1735. La etiología no está clarificada hasta hoy en día. AL puede formar parte de un cuadro sindrómico (síndrome de Down, síndrome de Edwards) o de una malformación cardiaca compleja. Es casi siempre asintomática, pero algunas veces puede revelarse por signos respiratorios o disfagia. Se elige habitualmente el tratamiento conservador. Les presentamos el caso de un niño de 2 años que presentó una AL revelada atipicamente por otitis media a repetición.

Arteria Lusoria (AL) or aberrant right subclavian artery is a rare congenital aortic arch malformation. Hunauld described it for the first time in 1735.The cause is until now no totally clarified. Usually it is associated with chromosomal disorders such as Down's syndrome, Edwards's syndrome or appears in childhood with complexes cardiovascular abnormalities. Patients are frequently asymptomatic, but it may be revealed by respiratories symptoms or by a dysphagia. The treatment is generally conservative. We expose a case report about a child of 2 years old with AL diagnosed after several episodes of purulent otitis.

Aberrant right subclavian artery-esophageal fistula: massive upper gastrointestinal hemorrhage secondary to prolonged intubation / Fístula de artéria subclávia direita anômala com esôfago - hemorragia digestiva alta maciça secundária a intubação gástrica prolongada

ABSTRACT Aberrant right subclavian artery-esophageal fistula is a rare but potentially fatal complication. It may be associated with procedures, such as tracheostomy and tracheal or esophageal intubation, and yields massive upper gastrointestinal bleeding difficult to identify and to control. A high index of suspicion is essential for early diagnosis and better prognosis. We report a rare case of a patient who survived after emergent surgical procedure for massive upper gastrointestinal bleeding secondary to aberrant right subclavian artery-esophageal fistula after prolonged intubation.

RESUMO A fístula de artéria subclávia direita anômala com o esôfago é uma complicação rara, mas potencialmente fatal. Pode estar associada a procedimentos como traqueostomia e intubação traqueal ou esofágica e originar hemorragia digestiva alta maciça, de difícil identificação e controle. Um elevado índice de suspeição é essencial para o diagnóstico precoce e a melhoria do prognóstico. Relatamos caso raro de doente que sobreviveu após intervenção cirúrgica emergente por hemorragia digestiva alta maciça secundária a fístula de artéria subclávia direita anômala com esôfago, após intubação gástrica prolongada.

Aberrant Origin of Vertebral Artery and its Clinical Implications

Abstract Aberrant origin of vertebral artery is rare. The anatomical features and clinical significance of this lesion remain to be clarified. A comprehensive collection of the pertinent literature resulted in a cohort of 1286 cases involving 955 patients and 331 cadavers. There were more left than right and more unilateral than bilateral aberrant vertebral arteries. Patients with aberrant origin of vertebral artery were often asymptomatic and in only 5.5% of the patients their symptoms were probably related to the aberrant origin of vertebral artery. The acquired cardiovascular lesions were present in 9.5% of the patients, 20.9% of which were vertebral artery-associated lesions. Eight (0.8%) patients had a vertebral artery dissection. Logistic regression analysis showed significant regressions between bovine trunk and left vertebral artery (P=0.000), between the dual origins of vertebral artery and cerebral infarct/thrombus (P=0.041), between associated alternative congenital vascular variants and cervical/aortic dissection/atherosclerosis (P=0.008). Multiple logistic regression demonstrated that side of the aberrant origin of vertebral artery (left vertebral artery) (P=0.014), arch branch pattern (direct arch origin) (P=0.019), presence of the common trunk (P=0.019), associated acquired vascular disorder (P=0.034) and the patients who warranted management (P=0.000) were significant risk predictors for neurological sequelea. The patients with neurological symptoms and those for neck and chest operations/ interventions should be carefully screened for the possibility of an aberrant origin of vertebral artery. The results from the cadaver metrology study are very helpful in the design of the aortic stent. The arch branch pattern has to be taken into consideration before any maneuver in the local region so as to avoid unexpected events in relation to aberrant vertebral artery.

Síndrome del desfiladero torácico neurogénico causado por la arteria escapular descendente: comunicación de un caso / Neurogenic thoracic gland syndrome caused by the descending descending artery: case report

El síndrome del desfiladero torácico es una entidad clínica compleja que abarca varias situaciones donde hay compresión del paquete vásculo-nervioso que suministra las extremidades superiores en el canal cervicotorácico. El síndrome se puede dividir en neurogénico y vascular de acuerdo con la estructura comprometida, siendo la primera la más frecuente. Entre las causas descritas están las costillas cervicales, anomalías de la primera costilla, músculos anómalos o hipertrofiados, traumatismos y tumores de la región. Describimos el caso de una paciente de 47 años que presenta un cuadro de cervicobraquialgia asociado con parestesias y paresia en la mano izquierda compatible con síndrome del desfiladero torácico en que la causa de la compresión era la arteria escapular descendente. Fue realizada una revisión de la literatura siendo encontrado sólo un caso similar.

The thoracic outlet syndrome is a complex clinical entity that encompasses several situations where there is compression of the neurovascular bundle that supplies the upper limbs in the cervicothoracic channel. It can be divided in neurogenic and vascular according to the structure committed, the first being the most frequently found. Among the causes described there are cervical ribs, anomalies of the first rib, anomalous or hypertrophied muscles, trauma and tumors of the region. We report the case of a 47 year old patient who presents with a clinical picture of cervicobrachialgia associated with paresthesia and paresis on the left hand compatible with thoracic outlet syndrome in which the cause of compression was the descending scapular artery.We conducted a literature review and found only one similar case.

Type I persistent proatlantal artery associated with fusiform subclavian artery aneurysm: Report of one case / Arteria proatlántica persistente tipo I asociada a un aneurisma fusiforme de la arteria subclavia: Presentación de un caso

We report a 61 years old male presenting with a right cerebral infarction, along with a type I persistent left proatlantal artery (PA), which is a form of primitive carotid-basilar anastomosis. The patient had an absence of the ipsilateral vertebral artery (VA) and hypoplasia of the contralateral VA, while the basilar artery was supplied by the PA. Other vascular anomalies present were a fusiform aneurysm of the right subclavian artery, and an A1 segment aplasia of the hypoplastic anterior right cerebral artery, which originated from the anterior communicating artery. To our knowledge these anomalies were not described previously.

Avaliação da artéria subclávia direita na ecografia do primeiro trimestre / Right subclavian artery evaluation during first trimester ultrasound scan

OBJETIVO: Avaliar a artéria subclávia direita durante a ecografia do primeiro trimestre, descrever a técnica para a sua avaliação e, em caso de identificação de artéria subclávia direita aberrante (ARSA), determinar sua associação com alterações cromossómicas e/ou malformações cardíacas e sua orientação. MÉTODOS: Estudo prospectivo que consistiu na avaliação da artéria subclávia direita no primeiro trimestre (comprimento crânio caudal entre 45 e 84 milímetros), em todas as gestações únicas, consecutivas, por um único examinador, ecógrafo Voluson E8 (GE Healthcare, Zipf, Áustria) com sonda transabdominal RAB 4-8-D, 2 a 8 MHz, por um período não superior a 2 minutos, numa população geral de baixo risco. Com a ajuda do power/color Doppler, a artéria subclávia direita foi classificada como normal ou aberrante. Foi utilizada análise de regressão estatística (IBM SPSS Statistics for Windows, versão 20.0) para estudar o grau de associação entre a falha na avaliação/classificação da artéria subclávia direita e o comprimento crânio-caudal fetal e o índice de massa corporal materno. RESULTADOS: A mediana da idade materna foi de 30 anos (variando entre 17 e 43 anos) e a mediana do tempo de gestação no momento da avaliação da artéria subclávia direita foi de 12 semanas de gestação (variando entre 11 e 13 semanas de gestação). A avaliação da artéria subclávia direita foi possível em 138/176 (78,4%) dos casos. ARSA foi diagnosticada em um único caso (0,7%). Esse feto com ARSA também apresentou um foco hiperecogênico no ventrículo cardíaco esquerdo. Foi realizada ecocardiografia fetal às 16 semanas de gestação, que confirmou o diagnóstico de ARSA e foco hiperecogênico. A amniocentese revelou cariótipo normal, 46, XX. CONCLUSÃO: É possível fazer o diagnóstico de ARSA na ecografia do primeiro trimestre. O nosso único caso de ARSA apresentou um cariótipo normal sem malformações cardíacas associadas. .

PURPOSE: To determine the feasibility of evaluation of the right subclavian artery during the first trimester ultrasound scan, as well as to describe the technique for its evaluation and, in case of aberrant right subclavian artery (ARSA) identification, to determine its association with chromosomal abnormalities and/or cardiac malformations and its management. METHODS: A prospective study for evaluation of the right subclavian artery during the first trimester ultrasound scan (crown-to-rump length between 45 and 84 mm), in all consecutive single pregnancies, by a single examiner, using a Voluson E8 system (GE Healthcare, Zipf, Austria) with a 2 to 8 MHz RAB 4-8-D transabdominal probe, within a short period of time (less than 2 minutes), in a general low risk population. Color and/or power Doppler flow mapping was used to classify the right subclavian artery as normal or aberrant. Regression analysis with the IBM SPSS Statistics software for Windows, version 20.0 was used to determine the significance of the association between failure to examine/classify the right subclavian artery and both fetal crown-rump length and maternal body mass index. RESULTS : Median maternal age was 30 years (range: 17-43 years) and median gestational age at the time of evaluation of the right subclavian artery was 12 weeks (range: 11-13 weeks). The evaluation of the right subclavian artery was successful in 138/176 (78.4%) of the cases. ARSA was diagnosed in a single case (0.7%). This fetus with ARSA also presented a hyperechogenic focus on the left cardiac ventricle. Fetal echocardiography at 16 weeks of gestation was performed and confirmed ARSA and the hyperechogenic focus. Amniocentesis revealed a normal 46, XX karyotype. CONCLUSION: ARSA can be identified during a routine first trimester ultrasound scan. Our single ARSA case had a normal karyotype and no associated cardiac malformations. .

Double incomplete aortic arch and Kommerell's Diverticulum as a cause of chronic cough / Doble arco aórtico incompleto y divertículo de Kommerell como causa de tos crónica

Vascular rings which can cause symptoms related the trachea and esophagus compression occur in less than 1% of all cardiovascular malformations. Double incomplete aortic arch with right-sided aorta and aberrant left subclavian artery is the rarest one, and its present in 0.04-0.1% of autopsy series. A case of this malformation with a Kommerell's Diverticulum is presented. This diverticulum has risk of severe complications such as dissection and/or rupture.

Los anillos vasculares pueden causar síntomas relacionados a compresión de tráquea y esófago y ocurren en menos del 1% de todas las malformaciones cardiovasculares. El doble arco aórtico incompleto con arco aórtico a la derecha y arteria subclavia izquierda aberrante es la forma más rara y se presenta en el 0.04 a 0.1% de las series de autopsia. Se presenta un caso de esta malformación con un divertículo de Kommerell. El divertículo tiene riesgo de complicaciones severas como disección y/o ruptura.

Malformación arterio venosa cervical compleja sintomática / Complex arteriovenous malformation of the neck

Background: Arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system and forming arterial, venous and capillary nests. Case report: We report a female consulting for the first time at the age of 15 for an AVM located in the left posterior region of the neck. The patient was operated at that time, performing an extensive excision of the lesion and covering the defect with a skin flap. The lesion relapsed three years later. Vascular imaging revealed the malformation with afferent arteries from the subclavian, vertebral and left occipital arteries and a great posterior cervical nest that drained through a great anomalous vein to the subclavian vein. A surgical ligation of all the anomalous branches of the subclavian vein was performed, to perform a local excision in a second intervention. The patient got pregnant, delaying the intervention and had a normal delivery at the age of 19. At 21 years of age, she consulted again due to a great growth of her AVM, with repeated bleeding episodes, requiring transfusions. The patient was treated with embolization of the nest and the afferent arteries. Finally at 23 years of age, the lesion was excised again and the defect was covered with a skin flap. The patient had a good postoperative evolution.

Introducción: Las Malformaciones Arterio-Venosas (MAV) son alteraciones estructurales congénitas del desarrollo del sistema vascular en que se observan comunicaciones anómalas arterio-venosas conformando un "nido" arterio-venoso-capilar. Caso Clínico: Mujer que consulta a los 15 años de edad por una MAV en la región cervical posterior izquierda desde su nacimiento. Se efectúa una resección amplia de la lesión hasta el plano aponeurótico cubriendo el defecto con un colgajo de rotación cutáneo-adiposo. Evoluciona bien, pero recidiva luego de tres años. Un estudio vascular a los 18 años de edad evidencia la MAV con arterias aferentes desde la arteria subclavia, vertebral y occipital izquierdas, un gran "nido" cervical posterior que drena por una gran vena anómala hacia la vena subclavia izquierda. Se efectúa una ligadura quirúrgica de todas las gruesas ramas anómalas de la arteria subclavia, para efectuar una nueva resección local en un segundo tiempo. Sin embargo, esto se pospone pues la paciente se embaraza y tiene un parto normal a los 19 años. Vuelve a consultar a los 21 años por un gran crecimiento de su MAV con hemorragias a repetición, algunas muy profusas que obligan a transfundirla. Una AngioRNM confirma la MAV con un gran nido cervical posterior con nuevas aferencias. Se efectúa embolizaciones directas locales con espuma de polidocanol y micropartículas, y finalmente con alcohol. Una vez reducido el nido se emboliza además las principales aferencias por vía endovascular y se efectúa a los 23 años una amplia resección de la MAV cubriendo el defecto con un colgajo miocutáneo de dorsal ancho izquierdo. Ha evolucionado bien hasta ahora. Conclusión: El diagnóstico y tratamiento de una MAV compleja representa un gran desafío al equipo médico tratante que acompaña y atiende al paciente en el transcurso del tiempo, y a veces por el resto de la vida.