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Dysphagia is a common condition in clinical practice; however, an unusual type of dysphagia due to compression of the esophagus by an abnormal right subclavian artery may be discovered in a rare subset of patients. The prognosis and treatment will depend on the severity of the symptoms and the compromise of surrounding structures. We present the case of an 18-year-old female who presented with gradually progressive dysphagia. At first, it was treated as gastroesophageal reflux disease; nonetheless, the dysphagia became severe, and after a thorough evaluation, an aberrant right subclavian artery that compressed the esophagus was discovered along with a truncus bicaroticus. She was successfully treated with surgery without any complications. On follow-ups, she's doing well.

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An aberrant right subclavian artery (ARSA) is a rare variation of normal anatomy occurring in 0.5% to 1.8% of the population. No current guidelines are available regarding ARSA management, and surgical intervention should be evaluated carefully. Moreover, symptomatic patients with a dominant left arch and aberrant ARSA require a surgical approach from the right side of the chest for ligation and division of the aberrant artery at its origin on the aorta. The ARSA can then be reimplanted onto the right common carotid artery via a supraclavicular incision. The extensive mobilization in the chest allows for easy reimplantation in the supraclavicular region and eliminates reliance on the collateral circulation. Postoperative monitoring is reliable and easy with radial pulse examinations.

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This case report describes a case of severe dysphagia lusoria secondary to an aberrant right subclavian artery causing compression of the esophagus. Our 62-year-old female patient presented with severe dysphagia and underwent right carotid-subclavian bypass with uncovered thoracic endovascular aortic repair and coil embolization of the aberrant right subclavian artery. This case is unique in that an uncovered dissection stent graft was used to avoid occluding the anatomic left subclavian artery and, therefore, avoid a left carotid-subclavian bypass. This case highlights a unique anatomic variant, its surgical repair, and the long-term improvement in the patient's quality of life.

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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.

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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.

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Background: Aberrant right subclavian artery is only rarely observed in veterinary medicine. Some animals may present postprandial regurgitation and progressive weight loss, which is considered an incidental finding unrelated to clinical alterations. Advanced imaging techniques such as thoracic CT scan, magnetic resonance imaging (MRI) and contrast angiography are used for the accurate detection of lesions, anatomical changes and specific information about vascular rings. This paper describes the clinical changes, imaging exams and therapeutic approach in a female dog with megaesophagus induced by an aberrant right subclavian artery. Case: A 2-month-old female bull terrier, weighing 1.6 kg, with a history of regurgitation immediately or a few minutes after a meal, diarrhea, polyphagia, progressive emaciation and apathy for 45 days, was treated at a University Veterinary Hospital. The dog's physical examination revealed 7% dehydration and body condition score 1 (scale 1 to 5), but no cardiac or pulmonary alterations upon auscultation. The hematological analysis and renal and hepatic serum enzymes were within the normal range for the species. In view of the presumptive clinical diagnosis of vascular anomaly, suggested by the contrast X-ray examination, a chest tomography was performed, which revealed altered aortic arch shape and contours, and a posterior aneurysm in the area of abnormal connection of the right subclavian artery. The patient was released with a prescription for conservative dietary management for megaesophagus. Within two weeks, the patient returned with a report of a good response to the prescribed therapy, absence of vomiting and diarrhea, and an increase in body weight. Surgical correction was recommended, but has not been performed so far, but conservative treatment for megaesophagus was continued. No further episodes of regurgitation were identified during the nine-month follow-up period. Discussion: In the case reported here, the right subclavian artery is considered anatomically atypical because it arises directly from the aortic arch. This vascular anomaly passes on to the right pectoral limb, dorsal to the esophagus, contracting it in its dorsal aspect. It tends to affect purebred dogs, occurring more frequently in Irish setters, German shepherds and Labrador retrievers, although it has been described in other breeds such as the bull terrier documented here and mixed breed dogs. Vascular ring anomalies may not cause clinical changes in animals and represent only incidental findings, or they may lead to gastrointestinal changes resulting from esophageal stricture, contributing to megaesophagus and clinical signs of esophageal obstruction, especially in recently weaned puppies. Such alterations were observed in this case, with the dog presenting regurgitation, immediately or a few minutes after a meal, megaesophagus, diarrhea and progressive weight loss. A CT scan was performed to confirm the type and location of the vascular anomaly and diagnostic accuracy, as recommended in the literature. Dietary therapy is one of the approaches adopted for patients presenting with regurgitation resulting from megaesophagus secondary to vascular anomalies. The dog in this report responded well to the medical therapy; nevertheless, the treatment of choice to correct the esophageal obstruction caused by this anomaly is surgical sectioning of the aberrant vessel by right intercostal thoracotomy, given that the degree of esophageal dilation and dysfunction tends to increase over time. However, at this time, the animal's owner decided to suspend the recommended surgical procedure.

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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.

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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.

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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.

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The treatment options for aberrant right subclavian artery vary depending on the presence of Kommerell's diverticulum. Because there is a tendency not to report mortalities of these rare cases in the literature, it is hard to reach a conclusion on treatments from the limited data on post-interventional results in these patients. We report our experience with a 67-year old patient with an aberrant right subclavian aneurysm with Kommerell's diverticulum, diagnosed by chance.

As opções de tratamento para artéria subclávia direita aberrante variam dependendo da presença de divertículo de Kommerell. Como há uma tendência a não relatar mortalidade nos raros casos descritos na literatura, é difícil chegar a uma conclusão sobre tratamentos tendo em vista os dados limitados sobre resultados pós-intervenção nesses pacientes. Relatamos aqui nossa experiência com um paciente de 67 anos de idade com aneurisma de artéria subclávia aberrante direita com divertículo de Kommerell diagnosticado ao acaso.

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The treatment options for aberrant right subclavian artery vary depending on the presence of Kommerell's diverticulum. Because there is a tendency not to report mortalities of these rare cases in the literature, it is hard to reach a conclusion on treatments from the limited data on post-interventional results in these patients. We report our experience with a 67-year old patient with an aberrant right subclavian aneurysm with Kommerell's diverticulum, diagnosed by chance

As opções de tratamento para artéria subclávia direita aberrante variam dependendo da presença de divertículo de Kommerell. Como há uma tendência a não relatar mortalidade nos raros casos descritos na literatura, é difícil chegar a uma conclusão sobre tratamentos tendo em vista os dados limitados sobre resultados pós-intervenção nesses pacientes. Relatamos aqui nossa experiência com um paciente de 67 anos de idade com aneurisma de artéria subclávia aberrante direita com divertículo de Kommerell diagnosticado ao acaso

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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.

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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.

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Objetivos: Dentro de las malformaciones vasculares de las arterias en el tórax encontramos: 1) doble arco aórtico; 2) arco aórtico derecho y conducto arterioso izquierdo persistente; 3) arteria subclavia derecha aberrante; 4) arteria pulmonar izquierda aberrante, y 5) arteria innominada anómala. Se revisan los pacientes con arteria subclavia derecha aberrante y su manejo. Métodos: Se estudiaron en forma retrospectiva los expedientes de 29 pacientes en edad pediátrica, de enero de 1992 a diciembre 2012, con las siguientes variables: edad de inicio de los síntomas, manifestaciones clínicas, defectos cardiovasculares, método diagnóstico y abordaje quirúrgico. Resultados: El mayor número de pacientes cursó de forma asintomática, únicamente el 31% lo hizo con síntomas durante el primer año de vida, y se llevó a cabo el diagnóstico en un 35% mediante cateterismo. Fue la persistencia del conducto arterioso la cardiopatía más frecuente, con un 13%; el síndrome de Down se encontró en un 21% de los pacientes. El tratamiento más utilizado fue la sección de la arteria subclavia aberrante para la liberación del esófago. Conclusiones: Es importante la sospecha diagnóstica en pacientes con sintomatología durante la alimentación, con trastornos de la deglución con sólidos y, en algunos casos, con disfagia o hasta con dificultad respiratoria. Un número significativo de estos pacientes no son diagnosticados oportunamente, algunos alcanzan la edad adulta sin diagnóstico. El hallazgo de esta malformación ocurre durante los estudios de imagen, cuando se evalúa la aorta o en el estudio del reflujo gastroesofágico, ya que con el trago de bario se aprecia la compresión extrínseca del esófago.

Objectives: Congenital vascular malformations of the major arteries in the chest have been classified into 5 groups: 1) double aortic arch; 2) right aortic arch with left ligament or persistent ductus arteriosus; 3) aberrant subclavian artery; 4) aberrant left pulmonary artery, and 5) anomalous innominate artery. We reviewed the patients with aberrant right subclavian artery and their treatment. Methods: We studied retrospectively the records of 29 patients with aberrant right subclavian artery in childhood, from January 1992 to December 2012, analyzing the following variables: age at onset, clinical manifestations, associated cardiovascular defects, diagnosis and surgical approach method. Results: We found that most patients have an asymptomatic course, only 31% of them course with symptoms during the first year of life, with an incidental diagnosis of 35% during catheterization or other imaging studies. Patent ductus arteriosus was the most frequently associated congenital malformation, with 13%. Down's syndrome was found in 21%. The most common treatment was surgical section of the aberrant subclavian artery to release the esophagus. Conclusions: This vascular abnormality must be suspected in those patients with dysphagia, dyspnea, chest pain during feeding or breathing difficulties. A significant number of patients are not diagnosed in time, some reach adulthood without a diagnosis. This malformation is often found in imaging studies when evaluating the aorta or in a gastroesophageal reflux study, in which the barium bolus reveals the extrinsic compression of the esophagus.

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OBJECTIVES: Congenital vascular malformations of the major arteries in the chest have been classified into 5 groups: 1) double aortic arch; 2) right aortic arch with left ligament or persistent ductus arteriosus; 3) aberrant subclavian artery; 4) aberrant left pulmonary artery, and 5) anomalous innominate artery. We reviewed the patients with aberrant right subclavian artery and their treatment. METHODS: We studied retrospectively the records of 29 patients with aberrant right subclavian artery in childhood, from January 1992 to December 2012, analyzing the following variables: age at onset, clinical manifestations, associated cardiovascular defects, diagnosis and surgical approach method. RESULTS: We found that most patients have an asymptomatic course, only 31% of them course with symptoms during the first year of life, with an incidental diagnosis of 35% during catheterization or other imaging studies. Patent ductus arteriosus was the most frequently associated congenital malformation, with 13%. Down's syndrome was found in 21%. The most common treatment was surgical section of the aberrant subclavian artery to release the esophagus. CONCLUSIONS: This vascular abnormality must be suspected in those patients with dysphagia, dyspnea, chest pain during feeding or breathing difficulties. A significant number of patients are not diagnosed in time, some reach adulthood without a diagnosis. This malformation is often found in imaging studies when evaluating the aorta or in a gastroesophageal reflux study, in which the barium bolus reveals the extrinsic compression of the esophagus.

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In one male cadaver of Colombian nationality, dissected by a group of medical students during a gross anatomy course at the Universidad del Valle in Cali, Colombia, three anatomical variations were found: an anomalous or aberrant right subclavian artery (ARSA), a non-recurrent inferior laryngeal nerve and a right thoracic duct. The aortic arch gave origin to four instead of three arteries, which, from right to left, were the right common carotid, the left common carotid, the left subclavian and the right subclavian arteries. The anatomical variation of the right subclavian artery is known also as lusoria artery, in which case the artery passes behind the esophagus and the trachea in its course towards the right side of the neck. The perimeters of the aortic arch and of the lusoria artery were measured in different sites; those of the lusoria artery gradually reduced during the course of the artery towards the right side of the neck. The non-recurrent inferior laryngeal nerve originated at a right angle from the right vagus nerve, at the level of the thyroid gland. The nerve described a horizontal course in its way towards the larynx, passing behind the right lobe of the gland, in close relation with the branches of the inferior thyroid artery. Of the three possible variations in the course of the non-recurrent inferior laryngeal nerve, the one found corresponds to the horizontal course or type II. The course of the thoracic duct in the thorax was normal, situated behind the esophagus, between the azygos vein and the thoracic aorta, but it gradually deviated towards the right side of the neck to end in the internal jugular vein. Out of the extensive databases that were consulted, only one report was found of these three simultaneous variations.

En un cadáver de nacionalidad colombiana, disecado por un grupo de estudiantes de medicina durante un curso de anatomía macroscópica en la Universidad del Valle de Cali, Colombia, tres variaciones anatómicas fueron encontradas: una arteria subclavia derecha anómala o aberrante (ARSA), un nervio laríngeo inferior no recurrente y un conducto torácico derecho. Del arco o cayado aórtico emergían cuatro en lugar de tres arterias, las cuales de derecha a izquierda eran las arterias carótida común derecha, carótida común izquierda, subclavia izquierda y subclavia derecha. Esta variación anatómica de la subclavia se conoce también como arteria lusoria, en cuyo caso la arteria pasa por detrás del esófago y de la tráquea en su curso hacia el lado derecho del cuello. Los perímetros del arco aórtico y de la arteria lusoria fueron medidos en diferentes sitios; los de la arteria lusoria fueron disminuyendo gradualmente en el curso de la arteria hacia el lado derecho del cuello. El nervio laríngeo inferior no recurrente se originaba en un ángulo recto del nervio vago derecho, a la altura de la glándula tiroidea. El nervio siguió un curso horizontal en dirección a la laringe, pasando por detrás del lobo derecho de la glándula, en estrecha relación con las ramas de la arteria tiroidea inferior. De los tres tipos de variación posibles en el curso del nervio laríngeo inferior no recurrente, el encontrado corresponde a la del curso horizontal o tipo II. El curso del conducto torácico en el tórax fue normal, situado por detrás del esófago, entre la vena ácigos y la aorta torácica, pero gradualmente se fue desviando hacia el lado derecho del cuello para desembocar en la vena yugular interna. En las extensas bases de datos consultadas solo se encontró un reporte de estas tres variaciones simultáneas.

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