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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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Animais , Feminino , Cães , Artéria Subclávia/anatomia & histologia , Artéria Subclávia/anormalidades , Artéria Subclávia/diagnóstico por imagem , Acalasia Esofágica/veterinária , Espectroscopia de Ressonância Magnética , Tomografia Computadorizada por Raios X/veterináriaRESUMO
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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Humanos , Masculino , Feminino , Gravidez , Adolescente , Adulto , Adulto Jovem , Artéria Subclávia/anormalidades , Anormalidades Congênitas/diagnóstico , Ultrassonografia Pré-Natal , Aberrações Cromossômicas , Anormalidades Cardiovasculares/genética , Anormalidades Cardiovasculares/diagnóstico por imagem , Artéria Subclávia/diagnóstico por imagem , Ecocardiografia , Testes Genéticos , Estudos RetrospectivosRESUMO
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