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.

Double-lumen Aortic Arch: Persistence of the Fifth Aortic Arch?

Abstract Double-lumen aortic arch is a rare congenital anomaly related to persistence of the fifth aortic arch. It may be found alone or in association with other anatomical changes of the heart. We report a case of double-lumen aortic arch associated with coarctation of the aorta and patent ductus arteriosus in a child with a congenital malformation known as the VACTERL association (vertebral defects, imperforate anus, cardiopathy, tracheoesophageal fistula, renal abnormalities and limb anomalies).

Secuestro pulmonar: a propósito de un caso / Pulmonary sequestration: a case report

Resumen Introducción El secuestro pulmonar es un segmento anormal de tejido pulmonar sin comunicación con el árbol traqueobronquial e irrigado por una arteria aberrante de origen sistémico. El secuestro pulmonar puede ser intralobar o extralobar. Caso Clínico Presentamos el caso de un neonato con dificultad respiratoria debido a secuestro pulmonar. El tratamiento quirúrgico consiste en la resección del segmento pulmonar con la ligadura y corte de su arteria. Se describe el manejo realizado y la revisión de la literatura indexada.

Introduction Pulmonary sequestration is an abnormal segment of lung tissue without communication with the tracheobronchial tree and irrigated by an aberrant artery of systemic origin. Can be intralobar or extralobar. Case report We present the case of a neonate with respiratory distress due to pulmonary sequestration. Surgical treatment consists in the resection of the lung segment with the ligature and cut of its artery. Is describe the management carried out and the review of the indexed literature.

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.

Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study / Mortalidade para Cardiopatias Congênitas e Fatores de Risco Associados em Recém-Nascidos. Um Estudo de Coorte

Abstract Background: Congenital heart diseases are the most common type of congenital defects, and account for more deaths in the first year of life than any other condition, when infectious etiologies are ruled out. Objectives: To evaluate survival, and to identify risk factors in deaths in newborns with critical and/or complex congenital heart disease in the neonatal period. Methods: A cohort study, nested to a randomized case-control, was performed, considering the Confidence Interval of 95% (95% CI) and significance level of 5%, paired by gender of the newborn and maternal age. Case-finding, interviews, medical record analysis, clinical evaluation of pulse oximetry (heart test) and Doppler echocardiogram were performed, as well as survival analysis, and identification of death-related risk factors. Results: The risk factors found were newborns younger than 37 weeks (Relative Risk - RR: 2.89; 95% CI [1.49-5.56]; p = 0.0015), weight of less than 2,500 grams (RR: 2.33 [; 95% CI 1.26-4.29]; p = 0.0068), occurrence of twinning (RR: 11.96 [95% CI 1.43-99.85]; p = 0.022) and presence of comorbidity (RR: 2.27 [95% CI 1.58-3.26]; p < 0.0001). The incidence rate of mortality from congenital heart disease was 81 cases per 100,000 live births. The lethality attributed to critical congenital heart diseases was 64.7%, with proportional mortality of 12.0%. The survival rate at 28 days of life decreased by almost 70% in newborns with congenital heart disease. The main cause of death was cardiogenic shock. Conclusion: Preterm infants with low birth weight and comorbidities presented a higher risk of mortality related to congenital heart diseases. This cohort was extinguished very quickly, signaling the need for greater investment in assistance technology in populations with this profile.

Resumo Fundamento: As cardiopatias congênitas configuram o tipo mais comum de defeitos congênitos, sendo responsáveis por mais mortes no primeiro ano de vida do que em qualquer outra condição, quando etiologias infecciosas são excluídas. Objetivo: Avaliar a sobrevida e identificar os fatores de risco nos óbitos em recém-nascidos com cardiopatia congênita crítica e/ou complexa no período neonatal. Métodos: Realizou-se um estudo de coorte, aninhado a um caso-controle aleatorizado, considerando Intervalo de Confiança de 95% (IC95%) e nível de significância de 5%, pareado por sexo do recém-nascido e idade materna. Foram feitas buscas ativas de casos, entrevistas, análise de prontuário, avaliação clínica da oximetria de pulso (teste do coraçãozinho) e do ecoDopplercardiograma, bem como análise de sobrevida e identificação dos fatores de risco relacionados ao óbito. Resultados: Os fatores de risco encontrados foram recém-nascidos com menos de 37 semanas (Risco Relativo − RR: 2,89; IC95% 1,49-5,56; p = 0,0015), peso inferior a 2.500 g (RR: 2,33; IC95% 1,26-4,29; p = 0,0068), ocorrência de gemelaridade (RR: 11,96; IC95% 1,43-99,85; p = 0,022) e presença de comorbidade (RR: 2,27; IC95% 1,58-3,26; p < 0,0001). A taxa de incidência de mortalidade por cardiopatias congênitas foi de 81 casos por 100 mil nascidos vivos. A letalidade atribuída às cardiopatias congênitas críticas foi de 64,7%, com mortalidade proporcional de 12,0%. A taxa de sobrevida aos 28 dias de vida diminuiu em quase 70% nos recém-nascidos com cardiopatias congênitas. A principal causa de óbito foi o choque cardiogênico. Conclusão: Recém-nascidos prematuros, com baixo peso e presença de comorbidades apresentaram maior risco de mortalidade relacionada às cardiopatias congênitas. Esta coorte se extinguiu muito rapidamente, sinalizando para a necessidade de maior investimento em tecnologia assistencial em populações com este perfil.

Lobectomy with ecmo support in an infant who developed pulmonary interstitial emphysema following repair of hypoplastic aortic arch

Abstract Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Agenesia de artéria carótida interna / Agenesis of the internal carotid artery

A agenesia de carótida interna é uma anomalia rara. Na maioria dos casos, é assintomática devido às anastomoses que podem estar presentes, mas pode estar associada a complicações, principalmente quando evidenciada a presença de outras alterações anatômicas ou doença aterosclerótica grave. Relatamos o caso de uma paciente feminina de 63 anos, hipertensa e diabética, com história de cirurgia prévia para clipagem de aneurisma cerebral. Na investigação através de eco-Doppler e angiotomografia de carótidas e vertebrais, foi evidenciada agenesia unilateral da artéria carótida interna esquerda. Este relato objetiva chamar atenção para a importância de se suspeitar de malformações vasculares durante a investigação de quadros neurológicos. A agenesia de carótida interna tem uma importante associação com aneurismas intracerebrais, e é possível poupar o paciente de graves complicações quando estes são identificados a tempo

Agenesis of the internal carotid artery is a rare anomaly. It is usually asymptomatic because of the presence of anastomoses, but it can be associated with complications, especially when there is evidence of other anatomical abnormalities or severe atherosclerotic disease. We report the case of a 63-year-old female patient with hypertension and diabetes and a history of intracranial aneurysm clipping. Doppler ultrasonography and computed tomography angiography of the carotid and vertebral arteries showed unilateral agenesis of the left internal carotid artery. Thisreport aims to highlight the importance of suspecting vascular malformations during investigation of neurological conditions. Internal carotid agenesis has a significant association with intracranial aneurysms and their early detection can spare the patient serious complications

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.

Tracheal compression by aberrant brachiocephalic trunk accompanying variant origin of the vertebral artery / Compresión traqueal por un tronco braquiocefálico aberrante acompañado de una variación en el origen de la arteria vertebral

The aorta is the main arterial trunk in the human body, however, its variation was extremely variable. During an educational dissection, aberrant branching pattern of aortic arch was found in a Korean cadaver. The brachiocephalic trunk (innominate artery) originated from the aortic arch at the left side of the trachea. It crossed the trachea and divided into the right common carotid and subclavian arteries. The left vertebral artery originated from the aortic arch between the origins of the left common carotid artery and the left subclavian artery, then the left vertebral artery coursed upward to the transverse foramen of the C7. The author describes this previously novel case report with aberrant brachiocephalic trunk and left vertebral arteries and discusses the clinical implications of such a variant.

La aorta es el tronco arterial principal del cuerpo humano, sin embargo, su variación es extremadamente variable. Durante una disección de rutina de un cadáver coreano, se encontró un patrón de ramificación aberrante del arco aórtico. El tronco braquiocefálico se originaba del arco aórtico en el lado izquierdo de la tráquea. Cruzaba la tráquea y se dividía en las arterias carótida común y subclavia derecha. La arteria vertebral izquierda se originó a partir del arco aórtico entre los orígenes de la arteria carótida común izquierda y la arteria subclavia izquierda, luego la arteria vertebral izquierda ascendió hacia el foramen transversal de la séptima vértebra cervical. Se describe un caso de tronco braquiocefálico aberrante y la correspondiente arteria vertebral izquierda y se discuten las implicaciones clínicas de tal variante.

Evaluation of pulmonary sequestration with multidetector computed tomography angiography in a select cohort of patients: A retrospective study

OBJECTIVES: This study aimed to evaluate the role of multidetector computed tomography angiography in diagnosing patients with pulmonary sequestration. METHODS: We retrospectively analyzed the computed tomography studies and clinical materials of 43 patients who had undergone preoperative multidetector computed tomography angiography in our hospital and had pathologically proven pulmonary sequestration. Each examination of pulmonary sequestration was reviewed for type, location, parenchymal changes, arterial supply and venous drainage on two-dimensional and three-dimensional computed tomography images. RESULTS: Multidetector computed tomography successfully detected all pulmonary sequestrations in the 43 patients (100%). This included 40 patients (93.0%) with intralobar sequestration and 3 patients (7.0%) with extralobar sequestration. The locations of pulmonary sequestration were left lower lobe (28 cases, 70% of intralobar sequestrations), right lower lobe (12 cases, 30% of intralobar sequestrations) and costodiaphragmatic sulcus (3 cases). Cases of sequestered lung presented as mass lesions (37.2%), cystic lesions (32.6%), pneumonic lesions (16.3%), cavitary lesions (9.3%) and bronchiectasis (4.6%). The angioarchitecture of pulmonary sequestration, including feeding arteries from the thoracic aorta (86.1%), celiac truck (9.3%), abdominal aorta (2.3%) and left gastric artery (2.3%) and venous drainage into inferior pulmonary veins (86.0%) and the azygos vein system (14.0%), was visualized on multidetector computed tomography. Finally, the multidetector computed tomography angiography results of the sequestered lungs and angioarchitectures were surgically confirmed in all the patients. CONCLUSIONS: As a noninvasive modality, multidetector computed tomography angiography is helpful for making diagnostic decisions regarding pulmonary sequestration with high confidence and for visualizing the related parenchymal characteristics, arterial supply, and venous drainage features to help plan surgical strategies.

Pre-aortic inter-azygos veins: two case reports / Venas interácigos pre-aórticas: reporte de dos casos

This report describes two cases of multiple anomalies associated with the azygos venous system. In Case 1, the hemiazygos vein drained the 9th to 11th left posterior intercostal veins. At T9 vertebral level, the interazygos vein passed obliquely and anterior to the aorta to drain into the azygos vein on the right. In Case 2, the common venous trunk formed by the accessory hemiazygos and hemiazygos veins passed anterior to the aorta to drain into the azygos vein at T9 vertebral level. These findings represent pre-aortic inter-azygos veins, which is a rare variation of the azygos venous system. Pre-aortic inter-azygos veins can mimic pathologies such as enlarged lymph nodes, tumors and aneurysms leading to misinterpretation of radiographs, computerized tomography and magnetic resonance scans. Surgeons need to be aware of this type of variation so as to avoid injury of any anomalous passing venous vessels.

Este informe describe dos casos de múltiples anomalías asociadas con el sistema venoso ácigos. En el caso 1, la vena ácigos drena las 9 y 11 venas intercostales posteriores izquierdas. A nivel de la 9 vértebra torácica, la vena interácigos pasó oblicuamente y anterior a la aorta para drenar en la vena ácigos del lado derecho. En el caso 2, el tronco venoso común formado por las venas hemiácigos accesoria y hemiácigos pasaron anterior a la aorta para drenar en la vena ácigos a nivel de la 9 vértebra torácica. Estos resultados representan venas interácigos pre-aórticas, que es una rara variación del sistema venoso ácigos. Las venas interácigos pre-aórticas pueden imitar patologías, como agrandamiento de nódulos linfáticos, tumores y aneurismas que conducen a una mala interpretación de las exploraciones por radiografías, tomografía computarizada y resonancia magnética. Los cirujanos deben ser conscientes de estos tipos de variaciones para evitar lesionarlos.

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.

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.

Persistencia del quinto arco aórtico asociado a persistencia del conducto arterioso / Persistent fifth aortic arch with patent ductus arteriosus

La persistencia del quinto arco aórtico es una rara anomalía congénita vascular que consiste en la presencia de una comunicación entre la aorta ascendente y la aorta descendente a través de un conducto arterial; se diagnostica de manera incidental. Informamos de un caso asociado a persistencia de conducto arterioso.

Persistent fifth aortic arch is a rare congenital vascular anomaly, with no clinical impact, so diagnosis is usually an incidental finding ocasionally associated with other congenital heart defects. We report a case of persistent fifth aortic arch associated with patent ductus arteriosus.

Double aortic arch as a source of airway obstruction in a child.

Double aortic arch (DAA) is a congenital vascular anomaly. The diagnosis was difficult till the child was symptomatic, and other causes were ruled out. We present the interesting images of a child of respiratory distress because of tracheal compression from DAA.