Sedoanalgesia con ketodex para la intubación selectiva en la resección de un aneurisma gigante de la arteria subclavia. Reporte de caso / Sedoanalgesia with ketodex for selective intubation in the resection of a giant aneurysm of the subclavian artery associated. Case report

Resumen: El aneurisma gigante de la arteria subclavia asociado a un síndrome de vena cava superior se presenta de forma poco frecuente. La complejidad del abordaje dependerá del tamaño, forma y disposición del aneurisma, particularmente cuando el colapso de la vía aérea es potencial por un efecto compresivo de la masa mediastinal durante la aplicación del relajante neuromuscular. Presentamos el caso de un hombre de 57 años con disnea en decúbito dorsal que incrementa con el decúbito lateral izquierdo de cinco años de evolución. El estudio radiológico reporta masa mediastinal gigante compatible con aneurisma de la arteria subclavia derecha que causa compresión y desplazamiento de la vía aérea, particularmente en tráquea a nivel de la carina. El paciente es programado para resección del aneurisma y requiere de intubación selectiva para el aislamiento del pulmón derecho durante la cirugía, que fue realizada con la técnica de paciente despierto usando sedoanalgesia con ketodex. El objetivo del caso es compartir la experiencia con el uso de ketodex como una alternativa para la tolerancia de procedimientos que requieren de la cooperación del paciente, en donde el propofol y el relajante neuromuscular pueden ser un problema para la permeabilidad y el abordaje de la vía aérea.

Abstract: A giant subclavian artery aneurysm associated with superior vena cava syndrome occurs infrequently. Complexity of the approach will depend on the size, shape, and position of the aneurysm, particularly when a potential collapse of the airway is expected due to the compressive effect of the mediastinal mass after using neuromuscular relaxant for airway approach. We present a case in a 57-year-old male with shortness of breath in dorsal that increases with left lateral decubitus during five years of evolution. Radiological studies reported giant mediastinal mass compatible with an aneurysm of the right subclavian artery, which produces critical compression and airway displacement, particularly in trachea at level of the carina. The patient is scheduled for resection of the aneurysm and requires selective intubation for isolation of the right lung during surgery which was performed with the awake patient technique using sedoanalgesia with ketodex. The objective of the case is to share the experience using ketodex as an alternative for the tolerance of procedures that requires the cooperation of the patient where propofol and neuromuscular relaxant may be a problem for airway permeability and approach.

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.

Artéria subclávia direita aberrante: relato de caso e revisão de literatura / Aberrant right subclavian artery: case report and literature review

Resumo A artéria subclávia direita aberrante, também conhecida como artéria lusória, é a anomalia do arco aórtico mais comum, ocorrendo entre 0,5 e 1% da população. Possui prevalência em mulheres e normalmente está associada a outras variações anatômicas, como o nervo laríngeo não recorrente, presente em 86,7% dos casos. Em sua maioria, a artéria subclávia direita aberrante não apresenta sintomas. Descrevemos essa alteração em uma paciente de 82 anos, hipertensa e assintomática, que havia sido submetida a uma angiotomografia toracoabdominal para a avaliação de uma dissecção crônica tipo III (DeBakey) associada à dilatação de aorta descendente. No achado, a artéria subclávia direita aberrante apresentava percurso retroesofágico associado a um divertículo de Kommerell. Devido à raridade, realizamos revisão bibliográfica integrativa das bases de dados MEDLINE, UpToDate, LILACS, SciELO e Portal CAPES dos últimos 6 anos e discutimos as alterações anatômicas mais frequentes, a sintomatologia e as condutas terapêuticas adotadas.

Abstract The aberrant right subclavian artery, also known as the arteria lusoria, is the most common aortic arch anomaly, occurring in 0.5 to 1% of the population. There is a higher prevalence in women and it is usually associated with other anatomical variations, such as the non-recurrent laryngeal nerve, present in 86.7% of cases. In the majority of cases, the aberrant right subclavian artery causes no symptoms. We describe this anomaly in an 82-year-old, hypertensive, and asymptomatic patient who had undergone a thoracoabdominal angiography to investigate a chronic DeBakey type III aortic dissection with dilation of the descending aorta. The aberrant right subclavian artery followed a retroesophageal course and was associated with a Kommerell diverticulum. In view of its rarity, we conducted an integrative bibliographic review of literature from the last 6 years indexed on the Medline, UpToDate, Lilacs, Scielo, and Portal Capes databases and discuss the most frequent anatomical changes, symptomatology, and therapeutic management adopted.

Bilateral variation in the branching pattern of the subclavian artery: an unusual finding with clinical implications / Variação bilateral no padrão de ramificação da artéria subclávia: um achado incomum com implicações clínicas

Abstract The subclavian artery is a significant branch of the aortic arch. We present a rare case of a bilateral variation in the branching pattern of the subclavian artery, observed in an adult male cadaver aged 70 years. On both the sides of the neck, all the branches of the subclavian artery took their origin from its first part. There was a rare occurrence of a cervicodorsoscapular trunk, which gave rise to superficial cervical, suprascapular, and dorsal scapular arteries. The same branching pattern was observed on the left side of the neck, with the presence of another cervicodorsoscapular trunk. Thyrocervical trunk and transverse cervical artery were both absent from the cervical region bilaterally. The inferior thyroid artery was a direct branch from the subclavian artery. Knowledge regarding variations of the subclavian artery is very important as lateral cervical region arteries are important for flap harvesting in plastic and reconstruction surgery. Preoperative radiologic evaluation of pedicles might help in choosing the optimal flap design, prevent ischemic complications, and help to improve overall treatment outcomes.

Resumo A artéria subclávia é um ramo significativo do arco da aorta. Apresentamos um caso raro de variação bilateral do padrão de ramificação da artéria subclávia, observada em um cadáver adulto do sexo masculino de 70 anos. Em ambos os lados do pescoço, todos os ramos da artéria subclávia originavam-se de sua primeira parte. Houve rara ocorrência de tronco escapular cervical dorsal, que deu origem às artérias cervical superficial, supraescapular e escapular dorsal. O mesmo padrão de ramificação foi observado no lado esquerdo do pescoço, com a presença de tronco escapular cervical dorsal. O tronco tireocervical e a artéria cervical transversa estavam ausentes em ambas as regiões cervicais direita e esquerda. A artéria tireóidea inferior consistia em um ramo direto da artéria subclávia. O conhecimento das variações da artéria subclávia é fundamental, pois as artérias da região cervical lateral são importantes para a obtenção de retalhos em cirurgias plásticas e reconstrutivas. A avaliação radiológica pré-operatória dos pedículos pode ajudar na escolha do desenho ideal do retalho, prevenir complicações isquêmicas e ajudar a melhorar o resultado geral do tratamento.

Tetralogia de Fallot Associada a Artéria Subclávia Direita Aberrante. Implicações Clínicas / Tetralogy of Fallot Associated with Aberrant Right Subclavian Artery. Clinical Implications

Resumo Desde a primeira descrição da tetralogia de Fallot (ToF) em 1671 por Niels Stensen e em 1888 por Étienne-Louis Arthur Fallot, vários trabalhos relataram essa anomalia juntamente com suas variantes e anomalias cardiovasculares concomitantes. A artéria subclávia direita aberrante (ASDA) é a anomalia do arco aórtico mais comum. Diferentemente da artéria subclávia esquerda aberrante, a ocorrência de ASDA em pacientes com ToF só foi relatada casuisticamente. Apresentamos dois pacientes de ToF com ASDA. É importante notar que o conhecimento da coexistência das duas anomalias tem pontos muito práticos durante correções endovasculares ou cirúrgicas de defeitos cardíacos congênitos (inclusive ToF).

Abstract Since the first description of Tetralogy of Fallot (ToF) in 1671 by Niels Stensen and in 1888 by Étienne-Louis Arthur Fallot, numerous papers have reported on this anomaly, along with its variants and concomitant cardiovascular anomalies. Aberrant right subclavian artery (ARSA) is the most common anomaly of the aortic arch. Different from the left aberrant subclavian artery, occurrence of ARSA in ToF-patients has only casuistically been reported so far. The present study reports on two ToF-patients with ARSA. It is important to note that knowledge of the coexistence of both anomalies has highly practical points during surgical or endovascular corrections of congenital heart defects (including ToF).

Coarctation of the aorta in a young female treated with left subclavian artery to descending thoracic aorta bypass: a case report / Coarctação de aorta em jovem tratada com artéria subclávia esquerda para bypass de aorta torácica descendente: relato de caso

Abstract Coarctation of the aorta is a rare congenital abnormality, with an incidence of 6-8% of all congenital heart problems. It is usually diagnosed in childhood during routine clinical examination and adults mostly present with hypertension. Various investigations like transthoracic echocardiography, computed tomography, and magnetic resonance angiography can help with diagnosis. Prognosis depends on age at presentation and the severity of coarctation. Treatment options available are open and endovascular repair. Extra-anatomical bypass is the preferred option in cases with unfavorable anatomy. Long term follow up is required post repair due to risk of restenosis and aneurysm formation. Here is a case in which a young female presented with hypertension, was diagnosed with coarctation of the aorta, and was treated a left subclavian artery to descending thoracic aorta bypass. Her postoperative course was uneventful and she had improvement in hypertension.

Resumo A coarctação da aorta é uma anomalia congênita rara, com incidência de 6-8% entre todos os problemas cardíacos congênitos. É geralmente diagnosticada na infância durante o exame clínico de rotina, e os adultos normalmente apresentam hipertensão. Diversas investigações, como ecocardiografia transtorácica, tomografia computadorizada e angiorressonância magnética, podem auxiliar no diagnóstico. O prognóstico depende da idade na apresentação e da gravidade da coarctação. As opções de tratamento disponíveis são o reparo aberto e o reparo endovascular. O bypass extra-anatômico é a opção preferida em caso de anatomia desfavorável. O acompanhamento de longo prazo é necessário após o reparo devido ao risco de reestenose e formação de aneurisma. Descrevemos o caso de uma jovem do sexo feminino que apresentou hipertensão, a qual foi diagnosticada como coarctação da aorta. Foi realizada derivação da artéria subclávia esquerda para a aorta torácica descendente. O pós-operatório transcorreu sem intercorrências, e a paciente apresentou melhora da hipertensão.

Oculo-auriculo-vertebral spectrum associated with aberrant subclavian artery in an infant with recurrent respiratory distress / Espectro oculoauriculovertebral associado à artéria subclávia aberrante em lactente com desconforto respiratório recorrente

ABSTRACT Objective: To describe an infant with craniofacial microsomia and recurrent respiratory distress associated with aberrant right subclavian artery in order to review its most frequent congenital anomalies and alert the pediatrician to its rarer and more severe complications. Case description: This case report involves an 18-month-old male infant, only son of non-consanguineous parents. At birth, the child presented craniofacial dysmorphisms (facial asymmetry, maxillary and mandibular hypoplasia, macrostomia, grade 3 microtia, and accessory preauricular tag) restricted to the right side of the face. Additional tests showed asymmetric hypoplasia of facial structures and thoracic hemivertebrae. No cytogenetic or cytogenomic abnormalities were identified. The patient progressed to several episodes of respiratory distress, stridor, and nausea, even after undergoing gastrostomy and tracheostomy in the neonatal period. Investigation guided by respiratory symptoms identified compression of the esophagus and trachea by an aberrant right subclavian artery. After surgical correction of this anomaly, the infant has not presented respiratory symptoms and remains under multidisciplinary follow-up, seeking rehabilitation. Comments: Craniofacial microsomia presents a wide phenotypic variability compared to both craniofacial and extracraniofacial malformations. The latter, similarly to the aberrant right subclavian artery, is rarer and associated with morbidity and mortality. The main contribution of this case report was the identification of a rare anomaly, integrating a set of malformations of a relatively common condition, responsible for a very frequent complaint in pediatric care.

RESUMO Objetivo: Descrever lactente com microssomia craniofacial e desconforto respiratório recorrente associado à artéria subclávia direita aberrante, com o intuito de revisar as anomalias congênitas mais frequentes e alertar o pediatra sobre suas complicações mais raras e de maior gravidade. Descrição do caso: Lactente do sexo masculino, 18 meses de idade e filho único de casal não consanguíneo. Ao nascimento, foram observadas dismorfias craniofaciais (assimetria facial, hipoplasia maxilar e mandibular, macrostomia, microtia grau 3 e apêndice na linha trago-oral) restritas ao lado direito da face. Os exames complementares evidenciaram hipoplasia assimétrica da face e hemivértebras torácicas. Não foram identificadas anormalidades citogenéticas ou citogenômicas. O paciente evoluiu com diversos episódios de desconforto respiratório, estridor e náuseas, mesmo tendo realizado gastrostomia e traqueostomia no período neonatal. A investigação direcionada para os sintomas respiratórios identificou a compressão de esôfago e traqueia por uma artéria subclávia direita aberrante. Após a correção cirúrgica dessa anomalia, o lactente não tem apresentado sintomas respiratórios e mantém seguimento multidisciplinar buscando reabilitação. Comentários: A microssomia craniofacial apresenta grande heterogeneidade fenotípica em relação às malformações tanto craniofaciais como extracraniofaciais. Estas últimas, como a artéria subclávia direita aberrante, são mais raras e associadas à morbimortalidade. A principal contribuição deste relato de caso foi o reconhecimento de uma anomalia rara, integrando um conjunto de malformações de uma condição relativamente comum e sendo responsável por uma queixa muito frequente no atendimento pediátrico.

Evaluación morfológica del músculo subclavio en una muestra de material de autopsia: inferencias clínicas / Morphological evaluation of the subclavian muscle in a sample of autopsy material: clinical inferences

RESUMEN: El músculo subclavio (MS) guarda estrecha relación con las estructuras neurovasculares (ENV) en el tercio medio de la región clavicular, situación de suma importancia para la realización de procedimientos invasivos de esta región. Pocos estudios han determinado la distancia desde el MS hacia la vena subclavia (VS), arteria subclavia (AS) y plexo braquial (PB). El propósito de este estudio fue valuar la expresión morfológica del MS y su relación con las ENV en una muestra de especímenes cadavéricos frescos. Estudio descriptivo en el que se realizó disección anatómica sobre la región clavicular de 30 especímenes de cadáveres humanos frescos no reclamados del Instituto Nacional de Medicina Legal y Ciencias Forenses, Colombia. Se realizó la caracterización cualitativa y cuantitativa del MS y se determinó la relación del MS con las ENV en la región clavicular. El MS presentó una longitud de 102,4±10,3 mm, con un espesor anteroposterior de 8,8±1,9 mm y superoinferior de 7,0±1,9 mm. El MS se caracterizó así: tipo I en 12 (40 %) especímenes, tipo II en 18 especímenes, tipo IIa: 10 especímenes. No encontramos tipos III y IV. La VS, AS y PB se relacionaron con el MS en el tercio medial y medio de su longitud a una distancia desde el margen superior del MSde 9,4±2,2 mm, 11,3±2,1 mm y 12,0±1,5 mm respectivamente. Los MS se relacionaron con las ENV de la región clavicular en una zona comprendida entre el 36,2±3,6 % y 89,4±4,8 % de su longitud total. Los hallazgos cualitativos y morfométricos de este estudio enriquecen los conceptos anatómicos subclaviculares y son de utilidad para el manejo quirúrgico de fracturas de clavícula.

SUMMARY: The subclavian muscle (MS) is closely related to the neurovascular structures (VNS) in the middle third of the clavicular region, and is critical when performing invasive procedures in this region. Few studies have determined the distance from the MS to the subclavian vein (VS), subclavian artery (AS) and brachial plexus (PB). The purpose of this study was to evaluate the morphological expression of MS and its relationship with VNS in a sample of fresh cadaveric specimens. The study involved the anatomical dissection in the clavicular region of 30 specimens of fresh unclaimed human cadavers from the National Institute of Forensic Medicine and Sciences, Colombia. The qualitative and quantitative characterization of the MS was carried out and the relationship of the MS with the VNS in the clavicular region was determined. The MS presented a length of 102.4 ± 10.3 mm, with an anteroposterior thickness of 8.8 ± 1.9 mm and a superoinferior thickness of 7.0 ± 1.9 mm. The DM was characterized as follows: type I in 12 (40 %) specimens, type II in 18 specimens, type IIa: 10 specimens. We did not find types III and IV. The SV, AS and PB were related to the MS in the medial and middle third of its length at a distance from the upper edge of the MS of 9.4 ± 2.2 mm, 11.3 ± 2.1 mm and 12.0 ± 1,5 mm respectively. The MS were related to the VNS of the clavicular region in an area between 36.2 ± 3.6 % and 89.4 ± 4.8 % of its total length. The qualitative and morphometric findings of this study enrich the subclavicular anatomical concepts and are useful for the surgical management of clavicle fractures.

Arteria subclavia derecha aberrante: a propósito de un caso pediátrico / Aberrant right subclavia artery: purpose of a pediatric case

Los anillos vasculares son anomalías anatómicas que ocurren durante el desarrollo embrionario del arco aórtico, sus ramas y de la arteria pulmonar. Estas estructuras vasculares pueden conducir a grados variables de sintomatología respiratoria y/o digestiva debido a la formación de un anillo completo o parcial que comprime la tráquea y/o el esófago. La arteria subclavia derecha aberrante es la anomalía más común del arco aórtico, con una incidencia reportada entre 0.5 a 2.5%. Generalmente es asintomática, sin embargo, la disfagia es el síntoma más frecuente porque en la mayoría de los casos transcurre detrás del esófago. El diagnóstico puede ser incidental en casos asintomáticos o como resultado del estudio de síntomas digestivos y/o respiratorios persistentes o recurrentes. Los pacientes sintomáticos requieren intervención quirúrgica. Describimos el caso de una paciente de 3 meses de edad con arteria subclavia derecha aberrante. El esofagograma mostró compresión extrínseca del esófago, y la angiotomografía computada confirmó el diagnóstico. La corrección quirúrgica se realizó exitosamente.

Vascular rings are anatomical abnormalities that occur during embryonic development of the aortic arch, its branches, and the pulmonary artery. These vascular structures can lead to variable degrees of respiratory and/or digestive symptoms by forming a complete or partial ring compressing the trachea and/or the esophagus. The aberrant right subclavian artery is the most common aortic arch anomaly, with reported incidence between 0.5 to 2.5%. It is generally asymptomatic; however, dysphagia is the most recognized symptom because in majority of the cases it crosses behind the esophagus. The diagnosis can be incidental when they are asymptomatic or as a result of the study of persistent or recurrent digestive and/or respiratory symptoms. Symptomatic patients require surgical intervention. We describe the case of a 3-month-old female patient with aberrant right subclavian artery. The barium esophagram showed extrinsic compression of the esophagus, and the computed tomography angiography confirmed the diagnosis. Surgical correction was successfully performed.

Reparación endovascular de las lesiones traumáticas de la arteria subclavia / Endovascular repair of traumatic subclavian artery injuries

Resumen Introducción: La reparación convencional de la arteria subclavia es desafiante, con una morbilidad del 24% y mortalidad del 5% al 25%; las técnicas endovasculares permiten la reparación arterial subclavia desde un acceso distal, reduciendo la elevada morbimortalidad asociada. Objetivo: Evaluar los resultados a largo plazo del tratamiento endovascular de las lesiones traumáticas de la arteria subclavia. Materiales y Método: Estudio descriptivo, observacional (revisión de serie de casos unicéntrica) de pacientes sometidos a la reparación endovascular de la arteria subclavia, debido a lesiones traumáticas, utilizando dos técnicas (stent balón expandible y oclusión endovascular con balón) durante un período de 12 años (2007-2019) en el Hospital Dr. Eduardo Pereira de Valparaíso, Chile. Resultados: Se realizaron 15 procedimientos consecutivos, electivos (86,67%), urgencias (13,33%), sexo masculino (66,67%), femenino (33,33%), edad promedio de 55,8 años (rango 26-69), abordaje utilizado: arteria femoral común (93,33%) y arteria braquial (6,67%), etiología de las lesiones: iatrogenia (66,67%) y trauma (33,33%), reparación mediante stent balón expandible (66,67%), oclusión endovascular con balón (33,33%), tasa de éxito técnico (100%), tasa de permeabilidad primaria a 1, 5, 10 años del 100%, 93,33% y 86,66% respectivamente, seguimiento medio (61,4 meses), estancia hospitalaria promedio (3,3 días), tiempo quirúrgico promedio (75 min), no hubo morbilidad cardiovascular, neurológica central o mortalidad relacionada al procedimiento. Discusión: La técnica endovascular elimina la necesidad de disección quirúrgica, disminuyendo el riesgo de lesión de estructuras adyacentes, especialmente en pacientes politraumatizados. Conclusión: En pacientes adecuadamente seleccionados, la técnica endovascular representa una excelente estrategia terapéutica de reparación de las lesiones subclavias.

Introduction: Conventional subclavian artery repair is challenging, with 24% morbidity and 5% to 25% mortality. Endovascular techniques allow subclavian repair from a distal artery, reducing the associated high morbidity and mortality. Aim: To evaluate the long-term results of endovascular treatment of traumatic lesions of the subclavian artery. Materials and Method: Descriptive, observational study (single-center case series review) of patients undergoing endovascular repair of the subclavian artery due to traumatic injuries, using two techniques (expandable balloon stent and endovascular balloon occlusion), during a period of 12 years (2007-2019), at the Dr. Eduardo Pereira Hospital in Valparaíso, Chile. Results: 15 consecutive procedures were performed, elective (86.67%), emergencies (13.33%), male sex (66.67%), female (33.33%), average age of 55.8 years (range 26-69), approach used: common femoral artery (93.33%) and brachial artery (6.67%), etiology of the lesions: iatrogenesis (66.67%) and trauma (33.33%), repair by expandable balloon stent (66.67%), balloon occlusion (33.33%), technical success rate (100%), primary patency rate at 1, 5, 10 years of 100%, 93.33% and 86.66% respectively, mean follow-up (61.4 months), average hospital stay (3.3 days), average surgical time (75 min), there was no cardiovascular, central neurological morbidity or mortality related to the procedure. Discussion: Endovascular techniques eliminate the need for surgical dissection, reducing the risk of injury to adjacent structures, especially in multiple trauma patients. Conclusion: In properly selected patients, the endovascular technique represents an excellent therapeutic strategy for the repair of subclavian artery lesions.

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.

Damage control surgery for thoracic outlet vascular injuries: the new resuscitative median sternotomy plus REBOA / Cirugía de control de daños para lesiones del opérculo torácico: nuevo abordaje mediante esternotomía mediana y REBOA

Abstract Thoracic vascular trauma is associated with high mortality and is the second most common cause of death in patients with trauma following head injuries. Less than 25% of patients with a thoracic vascular injury arrive alive to the hospital and more than 50% die within the first 24 hours. Thoracic trauma with the involvement of the great vessels is a surgical challenge due to the complex and restricted anatomy of these structures and its association with adjacent organ damage. This article aims to delineate the experience obtained in the surgical management of thoracic vascular injuries via the creation of a practical algorithm that includes basic principles of damage control surgery. We have been able to show that the early application of a resuscitative median sternotomy together with a zone 1 resuscitative endovascular balloon occlusion of the aorta (REBOA) in hemodynamically unstable patients with thoracic outlet vascular injuries improves survival by providing rapid stabilization of central aortic pressure and serving as a bridge to hemorrhage control. Damage control surgery principles should also be implemented when indicated, followed by definitive repair once the correction of the lethal diamond has been achieved. To this end, we have developed a six-step management algorithm that illustrates the surgical care of patients with thoracic outlet vascular injuries according to the American Association of the Surgery of Trauma (AAST) classification.

Resumen El trauma vascular torácico está asociado con una alta mortalidad y es la segunda causa más común de muerte en pacientes con trauma después del trauma craneoencefálico. Se estima que menos del 25% de los pacientes con una lesión vascular torácica alcanzan a llegar con vida para recibir atención hospitalaria y más del 50% fallecen en las primeras 24 horas. El trauma torácico penetrante con compromiso de los grandes vasos es un problema quirúrgico dado a su severidad y la asociación con lesiones a órganos adyacentes. El objetivo de este artículo es presentar la experiencia en el manejo quirúrgico de las lesiones del opérculo torácico con la creación de un algoritmo de manejo quirúrgico en seis pasos prácticos de seguir basados en la clasificación de la AAST. que incluye los principios básicos del control de daños. La esternotomía mediana de resucitación junto con la colocación de un balón de resucitación de oclusión aortica (Resuscitative Endovascular Balloon Occlusion of the Aorta - REBOA) en zona 1 permiten un control primario de la hemorragia y mejoran la sobrevida de los pacientes con trauma del opérculo torácico e inestabilidad hemodinámica.

Síndrome del Desfiladero Torácico en una niña. Caso clínico / Thoracic outlet syndrome in a girl. Clinical case

Introducción: El Síndrome del Desfiladero Torácico lo conforma una serie de síntomas y signos causados por la compresión de las estructuras neurovasculares en su salida por el desfiladero torácico a nivel supraclavicular.2 Dependiendo de la estructura afectada se habla de Síndrome del Desfiladero Torácico Neurológico, cuando la compresión es neurológica, SDTA cuando es arterial y SDTV cuando la compresión es venosa.3La presentación en la infancia es excepcional y la aparición con déficits motores se presenta en uno entre un millón de casos.1-6Los síntomas de dolor, debilidad y parestesias en la mano son orientativos y obligan a descartar esta entidad, así como signos clínicos de atrofia de musculatura son indicativos de la cronicidad.Los estudios preoperatorios como la electromiografía, RMN y angiografía asociada a RMN y pruebas dinámicas contribuyen al diagnóstico de esta entidad.7 La resección de la primera costilla y la escalenotomía es el procedimiento quirúrgico habitual en estos casos.8Presentamos el caso de una niña de 8 años que inicia sintomatología coincidiendo con la toma de biopsia a nivel supraclavicular en estudio de tumoración.

Introduction: The Thoracic Outlet Syndrome (TOS) conforms series of symptoms and signs caused by a compression of the neurovascular structures in the output thoracic pass at supraclavicluar level2. Depending on the affected structure, the syndrome can be Nerve Thoracic Outlet Syndrome (NTOS), Arterial Thoracic Outlet Syndrome (ATOS) or Venous Thoracic Outlet Syndrome (VTOS).3 The presentation in childhood is exceptional and the appearance with motor deficits occurs in one in a million cases.1The main clinical signs of TOS in adults include ip-silateral upper limb pain and discomfort, weakness, cold intolerance, and numbness of the hand. During physical examination, the muscles of the ipsilateral limb are relatively weak, and anesthesia, or pinprick sensation without pain is present on the inner sur- face of the hand and forearm. Thenar and hypothenar muscle atrophy may also be seen.In contrast, in children and teenagers, TOS usually presents as neck discomfort, upper limb numbness, weakness, and sensory loss.9We present the case of an 8-years-old girl started symptomatology coinciding with a biopsy taken for supraclavicular tumor at this level.

Tratamento endovascular com endoprótese aórtica para aneurisma de artéria subclávia secundário à fístula arteriovenosa axilo-axilar traumática tardia / Endovascular treatment with aortic endoprostheses for subclavian artery aneurysm secondary to late traumatic axillary-axillary arteriovenous fistula

Resumo As fístulas arteriovenosas (FAVs) traumáticas envolvendo os vasos axilares e subclávios são incomuns e correspondem de 5 a 10% de todos os traumas arteriais. A anatomia complexa dessa região torna desafiador o tratamento desse segmento. Neste desafio terapêutico, descrevemos o caso de um homem de 73 anos, encaminhado por edema progressivo e úlcera no membro superior direito, com história pregressa de ferimento por arma de fogo na região infraclavicular direita há cerca de 50 anos. Foi realizada angiotomografia e identificou-se FAV axilo-axilar associada à tortuosidade e dilatação aneurismática de artéria subclávia a jusante. O paciente foi submetido à intervenção endovascular com endoprótese cônica (monoilíaca) 26 × 14 × 90 mm Braile® na artéria subclávia aneurismática, posterior à saída da artéria vertebral direita, e endoprótese monoilíaca 16 × 16 × 95 mm Excluder® com sobreposição na primeira prótese, apresentando resultado satisfatório. Portanto, descreve-se a possibilidade de utilização de endoprótese aórtica em situação incomum e de exceção, com sucesso.

Abstract Traumatic arteriovenous fistulas (AVFs) involving the axillary and subclavian vessels are uncommon and account for 5 to 10% of all arterial traumas. The complex anatomy of this region makes treatment of this segment challenging. In this therapeutic challenge, we describe the case of a 73-year-old man, referred for progressive edema and ulceration involving the right upper limb and with a history of gunshot wound to the right infraclavicular region about 50 years previously. Angiotomography was performed and an axillary-axillary AVF was found, associated with tortuosity and aneurysmatic dilation of the subclavian artery downstream. He underwent endovascular intervention and a conical (monoiliac) 26 × 14 × 90 mm Braile® endoprosthesis was used in the aneurysmatic subclavian artery, posterior to the exit of the right vertebral artery and a 16 × 16 × 95mm Excluder® monoiliac endoprosthesis was placed overlapping the first prosthesis, showing a satisfactory result. Therefore, the possibility of successfully using aortic endoprostheses in an unusual and exceptional situation is described.

Tratamento cirúrgico da forma arterial da síndrome do desfiladeiro torácico associado à costela cervical / Surgical treatment of cervical rib-associated arterial thoracic outlet syndrome

Resumo A forma arterial da síndrome do desfiladeiro torácico é rara e está associada a uma anomalia anatômica, geralmente uma costela cervical. Suas manifestações são muito variadas. Este artigo tem como proposta relatar dois casos de apresentações clínicas distintas: microembolização e aneurisma. Em ambos, uma costela cervical estava presente. O diagnóstico foi realizado através da história, do exame físico, das manobras posturais e das radiografias. A angiotomografia computadorizada proporcionou o detalhe anatômico necessário para o planejamento operatório. O tratamento cirúrgico foi realizado pela abordagem supraclavicular, com sucesso em ambos casos.

Abstract The arterial form of thoracic outlet syndrome is rare and is associated with anatomic anomalies, generally a cervical rib. It has a varied range of manifestations. The aim of this article is to describe two cases with different clinical presentations: microembolization and aneurysm. A cervical rib was present in both cases. Diagnosis was made on the basis of history, physical examination, postural maneuvers, and X-rays. Computed tomography angiography provided the anatomic detail necessary to plan surgery. Surgical treatment was performed via supraclavicular access, successfully, in both cases.

Tratamento de oclusão arterial em membro superior causada por costela cervical / Treatment of upper limb arterial occlusion caused by a cervical rib

Resumo A síndrome da costela cervical ocorre quando o triângulo intercostoescalênico é ocupado por uma costela cervical, deslocando o plexo braquial e a artéria subclávia anteriormente, o que pode gerar dor e espasmo muscular. O objetivo deste estudo é discutir sobre o diagnóstico da síndrome da costela cervical e as possibilidades de tratamento. Este desafio terapêutico descreve a condução clínica e cirúrgica de uma paciente de 37 anos com obstrução arterial em membro superior causada por costela cervical.

Abstract The cervical rib syndrome occurs when the interscalene triangle is occupied by a cervical rib, displacing the brachial plexus and the subclavian artery forward, which can cause pain and muscle spasms. The objective of this study is to discuss diagnosis of the cervical rib syndrome and treatment possibilities. This therapeutic challenge describes clinical and surgical management of a 37-year-old female patient with upper limb arterial occlusion caused by a cervical rib.

Arteria subclavia derecha aberrante aislada. Reporte de un caso / Isolated aberrant right subclavian artery. A case report

Resumen La arteria subclavia derecha aberrante (ARSA) es la anomalía del arco aórtico más común. La probabilidad de hallar un ARSA durante un estudio ecográfico es de alrededor de 1%. En aquellos fetos con ARSA, la probabilidad de tener otros hallazgos cardiacos y/o extracardiacos o una anomalía cromosómica es elevada. La prevalencia de la relación del ARSA con el síndrome de Down es de aproximadamente 20%, por lo que este marcador puede contribuir al asesoramiento del síndrome de Down en el segundo trimestre y probablemente en el primer trimestre. La recomendación ante el hallazgo de ARSA es realizar un estudio detallado de la anatomía fetal en busca de otros marcadores de aneuploidías y realizar ecocardiografía fetal. La realización de estudios invasivos quedará limitado a aquellas situaciones en donde además del ARSA se encuentren otros marcadores u otras condiciones que aumenten el riesgo de síndrome de Down. Sin embargo, ante el hallazgo de ARSA aislado, el incremento en el riesgo es igual a cero, pudiendo considerarse una variante de la normalidad.

Abstract An aberrant right subclavian artery (ARSA) is the most common branch abnormality of the aortic arch. It can be identified by ultrasound scan in 1% of cases. The probability of association with cardiac and/or extracardiac anomalies, as well as chromosomal abnormality, is high. Prevalence of ARSA with Down syndrome is approximately 20%, and this marker may contribute to counseling on Down syndrome during the second trimester and maybe in the first trimester. When ARSA is found, recommendations include a detailed study of the fetal anatomy for other markers of aneuploidy and to obtain a fetal echocardiogram. Invasive studies will be limited to those situations where, in addition to ARSA, other markers or other conditions that increase the risk of Down syndrome are found. However, the finding of an isolated ARSA increases the risk to zero and is considered a normal variant.

Controle de danos para lesão de artéria subclávia / Damage control for subclavian artery injury

Resumo Os traumas penetrantes de vasos subclávios atingem mortalidade de até 60% em um cenário pré-hospitalar. A mortalidade no intraoperatório varia de 5-30%. Apresenta-se um caso de estratégia de controle de danos para um paciente com lesão na origem da artéria subclávia esquerda, através de ligadura, sem necessidade de outra intervenção, mantendo a viabilidade do membro superior esquerdo por meio de circulação colateral. Os autores fazem uma revisão sobre vias de acesso e estratégias de tratamento com ênfase em controle de danos para lesões de vasos subclávios.

Abstract Mortality from penetrating traumas involving the subclavian vessels can be as high as 60% in pre-hospital settings. Operating room mortality is in the range of 5-30%. This paper presents a case in which a strategy for damage control was employed for a patient with an injury to the origin of the left subclavian artery, using subclavian ligation, with no need for any other intervention, and maintaining viability of the left upper limb via collateral circulation. The authors also review surgical approaches and treatment strategies with a focus on damage control in subclavian vessel injuries.

Vasos subclavios: ¿Qué tan cerca se encuentran de la clavícula? / Subclavian vessels: how close are they to the clavicle?

INTRODUCCIÓN: la lesión de los vasos subclavios durante la cirugía de clavícula es una situación rara, de suceder podría resultar incluso mortal; conocer su ubicación es indispensable para minimizar ese riesgo.OBJETIVO: determinar la ubicación y la distancia de la AS y VS respecto a la clavícula. Secundariamente, identificar las características particulares que influencien la ubicación y la distancia. MATERIALES Y MÉTODO: estudio retrospectivo, AngioTAC de tórax y cuello entre 2012 y 2017; se midió la longitud de la clavícula, distancia y dirección de los vasos subclavios en cada tercio de la clavícula, como también la angulación entre una horizontal y el centro de los vasos subclavios. Resultados: 39 AngioTC, 78 hombros. Distancia AS/clavícula tercio proximal, medio y distal 32,8mm (20,3-46,3), 15,4mm (6,8-28,0) y 62,7mm (37,0-115,4) respectivamente. La distancia VS/clavícula tercio proximal, medio y distal fue: 7,4mm (1,0-19,2), 16,2mm (6,7-34,7) y 67,1mm (29,7-117,0) respectivamente. La ubicación de AS y VS con respecto a la clavícula es posterosuperior en el tercio proximal, posteroinferior en el tercio medio e inferior en el tercio distal. CONCLUSIÓN: En el tercio proximal la vena puede estar solo a 1mm de la clavícula y la arteria a 6mm en dirección antero-posterior, resultando esa zona la más peligrosa. En el tercio medio la distancia es mayor, pudiendo estar arteria y vena a solo 6mm, la dirección de brocado más peligrosa es antero-inferior con una inclinación promedio de 45° caudal. El tercio distal es el más seguro, los vasos están al menos a 30mm de distancia hacia caudal. Nivel de evidencia III.

BACKGROUND: injury to the subclavian vessels during clavicle surgery is a rare situation, if it happens it could even be fatal; knowing their location is essential to minimize that risk. OBJECTIVE: determine location and distance of the AS and VS with respect to the clavicle. Secondarily identify particular characteristics that influence location and distance. MATERIAL AND METHODS: retrospective study, AngioTAC of thorax and neck between 2012 and 2017; it was measured the length of the clavicle, distance and direction of the subclavian vessels in each third of the clavicle and angulation between a horizontal and the center of the subclavian vessels were measured. Results: 39 AngioTC, 78 shoulders. AS / clavicle third proximal, middle and distal distance 32.8mm (20.3-46.3), 15.4mm (6.8-28.0) and 62.7mm (37.0-115.4) respectively. Distance VS / clavicle third proximal, middle and distal was: 7.4mm (1.0-19.2), 16.2mm (6.7-34.7) and 67.1mm (29.7-117.0) respectively. The location of AS and VS with respect to the clavicle is posterosuperior in the proximal third, posteroinferior in the middle third and inferior in the distal third. CONCLUSION: In the proximal third the vein can be only 1mm from the clavicle and the artery to 6mm in the anterior-posterior direction, this zone is the most dangerous. In the middle third the distance is greater, artery and vein can be only to 6mm, the most dangerous drilling direction is antero-inferior with an average inclination of 45° caudal. The distal third is the safest, the vessels are at least 30mm away from the vessels. Level of evidence III.

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.