Dysphagia lusoria caused by aberrant right subclavian artery associated with truncus bicaroticus in an 8-month-old girl. Case report and review of literature.

Dysphagia lusoria is a rare pediatric condition caused by extrinsic compression of the esophagus by an abnormal subclavian artery. The most common congenital abnormality in aortic arch development is an aberrant right subclavian artery. The retroesophageal right subclavian artery is typically symptomatic in 10-33% of cases. The patient, an 8-month-old girl with a history of early dysphagia and stridor, was diagnosed with an abnormal right subclavian artery. She was admitted to the pneumology service multiple times due to stridor, vomiting, and failure to thrive. During hospitalization at the gastroenterology service, a barium swallow and an upper digestive endoscopy indicated an abnormal right subclavian artery, which was confirmed by an Angiography CT scan. She underwent surgery at the age of sixteen months. All symptoms are resolved following surgical intervention, and the patient is still asymptomatic and in good clinical condition 12 months later. Every physician should be aware of abnormal right subclavian arteries and their clinical symptoms in children and adults in order to recognize and diagnose them early. Only an early evaluation may reduce complications such as delayed physical growth, dysphagia, and recurrent respiratory infections.

[Right aortic arch with mirror image branching : a rare cause of dysphagia]. / L'image du mois. Arc aortique droit avec image en miroir : une cause rare de dysphagie.

We report the case of a 36-year-old female whose dysphagia revealed a congenital anomaly of the thoracic aorta: the right aortic arch with mirror image branching. This is a rare embryonic developmental anomaly where the aorta wraps around the right bronchus and the supra-aortic trunks emerge from the arch in the opposite order to normal. Most of the patients are asymptomatic unless there is a significant compression of mediastinal structures. Major compression of the esophagus or trachea, aneurysmal disease, dissection of the thoracic aorta, or the presence of a Kommerell diverticulum larger than 2 cm may require a surgical repair. There is no standard treatment and it must be adapted to the clinical presentation and the anatomic configuration of each patient. Our patient did not receive any treatment for her condition.

Nous rapportons le cas d'une patiente de 36 ans dont le tableau de dysphagie a permis de mettre en évidence une anomalie congénitale de l'aorte thoracique : l'arc aortique droit avec image en miroir. Il s'agit d'une anomalie de développement embryonnaire rare où l'aorte s'enroule autour de la bronche souche droite et où les troncs supra-aortiques émergent de la crosse dans l'ordre inverse et opposé à la normale. La grande majorité des patients est asymptomatique, à moins qu'il existe une compression des structures médiastinales. Une compression majeure de l'oesophage ou de la trachée, une maladie anévrismale, une dissection de l'aorte thoracique ou la présence d'un diverticule de Kommerell de plus de 2 cm peuvent justifier une sanction chirurgicale. Il n'y a pas de traitement standard et celui-ci doit être adapté à la présentation clinique et à la configuration anatomique du patient. Notre patiente n'a bénéficié d'aucun traitement pour son affection.

Dysplastic aberrant left subclavian artery originating from a thoracic intersegmental artery associated with a right aortic arch.

PURPOSE: A right aortic arch (RAA) is a rare vascular anomaly that often coexists with an aberrant left subclavian artery (ALSA). Due to the rarity of RAA, the development of an ALSA is not well understood. METHOD: We describe a case in which a 58-year-old man who was scheduled to undergo posterior decompression and fusion surgery for thoracic ossification of the posterior longitudinal ligament from Th1 to Th3 was found to have a RAA and an ALSA. RESULTS: Preoperative computed tomography angiography demonstrated a RAA and an ALSA. The ALSA was extremely tortuous and ran in the paraspinal muscles behind the thoracic laminae, which meant it was in the surgical field. The ALSA arose from the descending aorta and bifurcated into the left segmental arteries of Th1 and Th2, and also bifurcated into the left vertebral artery, which had a normal subsequent course. The dysplastic ALSA was considered to have developed from the thoracic intersegmental artery. Based on preoperative examination findings, we performed spinal surgery without vessel injury. CONCLUSION: We report a rare case of a dysplastic ALSA that developed from the thoracic intersegmental artery with a RAA. The knowledge of this anomaly provides safety in spinal surgery of the cervicothoracic junction.

Neuro-Interventional Diagnosis and Treatment Using the Right Transradial Approach in Patients with Aberrant Right Subclavian Artery.

OBJECTIVE: To assess the effectiveness and safety of neurological interventions using the right transradial approach (R-TRA) in patients with aberrant right subclavian artery (ARSA). METHODS: We retrospectively analyzed cases that underwent cerebral angiography and interventions at Huangpi District People's Hospital from January 2023 to July 2023. Out of 335 cases, 5 patients with ARSA were identified. RESULTS: All 5 cases underwent diagnostic cerebral angiography via R-TRA. Two of the patients received interventions via R-TRA: 1 underwent right internal carotid artery balloon dilation angioplasty, while another underwent left vertebral artery stenting. No surgery-related complications were observed during these procedures. CONCLUSIONS: R-TRA proves to be a safe and effective option for neuro-interventional surgery in patients with ARSA.

Right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery.

PURPOSE: Here, we report a case of the right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery. METHODS: A 42-year-old male patient underwent a whole-body computed tomography angiography (CTA) examination upon the clinical suspicion of vasculitis. RESULTS: CTA revealed a right-sided aortic arch with the isolation of the left innominate artery and hypoplasia of the left internal carotid artery. CONCLUSION: The right-sided aortic arch, with the isolation of the left innominate artery, is a scarce vascular variation that may occur with other cardiovascular anomalies such as ventricular septal defect. It can be asymptomatic or can present with symptoms of subclavian steal syndrome. Although its association with the agenesis of the left internal carotid artery has been reported, its association with the hypoplasia of the left internal carotid artery has not been reported previously to the best of our knowledge.

Surgical treatment for right-side aortic arch concomitant with Kommerell's diverticulum: techniques selection and follow-up results.

BACKGROUND: Right-side aortic arch concomitant with Kommerell's diverticulum (KD) is a rare and complex ailment, and there is no consensus on the optimal strategy to deal with this congenital anomaly. We retrospectively analyzed and summary of the cases treated in our center with individual treatment methods for different situations. METHODS: Between September 2018 and December 2021, 10 patients experienced surgical therapy at our institution who presented with a Kommerell's diverticulum arising from an aberrant subclavian artery from the right-side aortic arch. Four main surgical techniques were applied to those patients: 1. total arch replacement with frozen elephant trunk implantation (n = 2); 2. hybrid procedure combining open arch repair and endovascular intervention (n = 1); 3. total endovascular repair using thoracic endovascular aortic repair (TEVAR) with or without left subclavian artery (LSCA) revascularization (n = 6); 4. direct repair underwent endoaneurysmorrhaphy. Clinical characteristics and outcomes were collected. RESULTS: The mean age of these 10 patients was 56.5 years (range 29-79 years) and only 1 woman. The pathology includes aortic dissection (n = 6) and aneurysm (n = 4). The mean diverticulum size was 41.4 [24.2-56.8] mm. There were no in-hospital deaths, and the median hospital stay was 22 [15-43] days. During the follow-up period (21.4 months, 1-44 months), one died of an unknown cause and one died of esophageal fistula. Two patients underwent second-stage endovascular intervention for distal lesion. And none of the patients had endoleak during the follow-up period. CONCLUSIONS: Each of the procedures we have mentioned here has its advantages and disadvantages; individualized treatment should meet the appropriate indications. A single-branched stent graft is feasible and effective in the treatment of aortic disease combined with Kommerell's diverticulum.

Analysis of the regional anatomy of the retro-oesophageal right subclavian artery and surrounding structures.

BACKGROUND: The retro-oesophageal right subclavian artery (RRSA) is a congenital anomalous branching of the arch of the aorta. Because its incidence is very low, it has not been fully understood how the RRSA develops during embryogenesis, and thus accumulation of observed findings in newly found cases is important to elucidate the aetiology of the RRSA. MATERIALS AND METHODS: We encountered a case of the RRSA during the course of gross anatomy dissection for medical students. RESULTS: The main findings in the present observations are that (a) the RRSA arose from the right side wall of the arch of the aorta as its last branch; (b) the detected RRSA was directed to the right and upward between the oesophagus and vertebral column; (c) the right vertebral artery branched from the RRSA and entered the sixth cervical foramen transversarium; (d) the suprema intercostal artery branched from the costocervical trunk on both sides and its distal branches were distributed to the first and second intercostal spaces; and (e) both sides of bronchial arteries originated from the thoracic aorta. CONCLUSIONS: The present study gives further information about the morphological details of the RRSA leading to better understanding of its developmental process.

Case Report: Successful Treatment of Aberrant Left Subclavian Artery With a Right-Sided Aortic Arch.

Right sided aortic arches with concomitant aberrant left subclavian arteries are exceedingly rare anatomical variants. We present a case of a 45 year old male that presented with symptoms consistent with dysphagia and known right sided aortic arch with an aberrant left subclavian artery. Though previous reports of repair have indeed been reported, we confirm that a hybrid approach to these anatomic variants remain feasible and with symptom resolution for patients.

Aberrant Right Subclavian Artery: Demographics, Morphological Features and Follow up CT Scans Dynamics.

Introduction: Aberrant right subclavian artery (ARSA) is the most common of the aortic arch anomalies, occurring in .5% to 1% of the population. There is no standardized follow up protocol, especially in the asymptomatic cases. The purpose of the present study was to evaluate the natural history of ARSA and the role of serial CT scans. Methods: This is a single-center retrospective study of patients with ARSA depicted on chest computed tomography (CT) scans between February 2013 and July 2022. Data were collected from their medical records. Measurements of the aorta at different segments including the aortic diameter at the orifice of ARSA, and ARSA at ostium followed by 1 cm intervals were collected, as well as for follow-up CT scans. Results: 65 patients were diagnosed with ARSA, 70.8% of whom were women. The average age for the cohort was 58.569 ± 16.99 years. The median follow up time was 4 years (range 0-10 years), KM estimated survival after ARSA diagnosis at 1 and 5 years as 97% and 93%, respectively. Nineteen patients had a second CT scan and were included in the morphological CT dynamic analysis, on average of 29 ± 27 months apart (range 7-108). The mean ARSA diameter at origin was larger in the second scan 16.91 ± 4.31 mm compared to the initial scan 16.31 ± 4.96 mm, (P = .04).The mean aortic arch diameter in the first and second CT were 28.54 ± 4.24 and 29.64 ± 5.14 (P = .10), respectively. All other measurements did not disclose any significant enlargement over time. Conclusions: Our cohort demonstrate a benign natural history of ARSA with slow growth rates. However, due to our small sample size we can't draw a clinically sound recommendation on the need for imaging follow up, and further larger cohort with longer follow up interval are required.

Association and Repair of Right Aortic Arch With Aberrant Left Subclavian Artery With Subclavian Stenosis.

Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well. We present three cases of early repair of this arch anomaly with associated subclavian stenosis repaired successfully in that manner.

Recurrent Inferior Laryngeal Nerve Preservation During Thyroid Surgery in a Patient with Right Aortic Arch: A Case Report.

Patients with aortic arch malformations may present with recurrent inferior laryngeal nerve abnormalities that require special attention. Herein, we reported a case of thyroid surgery in a patient with a right aortic arch. The left inferior laryngeal nerve was presumed to be the right inferior laryngeal nerve by confirming the location of the aortic arch and subclavian artery, and the presence of the ductus arteriosus on preoperative computed tomography. Continuous intraoperative nerve monitoring is useful for safe surgery in patients with possible inferior laryngeal nerve abnormalities. Laryngoscope, 134:1986-1988, 2024.

Incomplete vascular ring of the aortic arch presenting with dysphagia in an adult: case report.

Anomalies of the arterial branches of the arch of the aorta are rare, with the aberrant right subclavian artery being the most common of this anomaly. Majority of the anomalies are asymptomatic and often discovered as incidental findings. In the great majority of the symptomatic cases, the presentation may be either with breathlessness or dysphagia or both. This is in addition to the nature of the intrinsic arterial disease of the aberrant vessel, especially in adult patients; and unless borne in mind, the diagnosis is often missed leading to delays and wrong treatment. In this report we present a case of dysphagia in an adult male Nigerian initially diagnosed as œsophageal stricture from herbal potion ingestion but review of his imaging investigations gave a final diagnosis of dysphagia lusoria from an aberrant right subclavian artery. The difficulty in making a diagnosis and the need for a multidisciplinary review of the imaging investigations are highlighted. The patient was successfully treated by a combined trans-thoracic and cervical approach with division and re-implantation of the aberrant vessel unto the right common carotid artery. He has remained symptom-free for 2 years after surgery. Although the great majority of these anomalies are often asymptomatic, it is important they are borne in mind both in imaging investigations as well as in procedures involving structures in the upper visceral mediastinum. Various surgical approaches have been documented in the management of symptomatic ones; it is however recommended that options that ensure revascularization of the affected limb be selected.

[Dysphagia lusoria treated by surgery]. / «Dysphagia lusoria¼ traitée chirurgicalement.

Aberrant right subclavian artery, also called arteria lusoria, is the most common congenital anomaly of the aortic arch (prevalence 0,5-1.8 %). Patients with trisomy 21 have a higher prevalence (35 %). It takes its origin from the aortic arch after the three others supra-aortic trunks. It may be associated with other cardiovascular anomalies. In more than 80 % of cases, the aberrant artery is positioned behind the oesophagus. It is often asymptomatic. Sometimes it is associated with symptoms after the age of 40 years old, more often among women: dysphagia (dysphagia lusoria), dyspnoea or cough. Diagnosis is made by computed tomography or magnetic resonance imaging. When it is symptomatic, a barium contrast radiography of the oesophagus is indicated. Surgical treatment by transposition of the aberrant vessel is performed only for symptomatic patients. We report the case of a 50-year-old woman with dysphagia lusoria treated by transposition of the aberrant right subclavian artery into the right common carotid through a cervical approach.

L'artère sous-clavière droite aberrante, appelée aussi arteria lusoria, est l'anomalie congénitale de l'arc aortique la plus fréquente (prévalence 0,5-1,8 %). Les patients atteints de trisomie 21 ont une prévalence plus élevée (35 %). Elle prend son origine sur la crosse aortique après les trois autres troncs supra-aortiques. Elle peut être associée à d'autres anomalies cardiovasculaires. Dans plus de 80 % des cas, l'artère aberrante est positionnée derrière l'œsophage. Elle est souvent asymptomatique. Elle est parfois associée à des symptômes après l'âge de 40 ans, plus souvent chez les femmes : dysphagie (dysphagia lusoria), dyspnée ou toux. Le diagnostic se fait par tomodensitométrie ou résonance magnétique nucléaire. Lorsqu'elle est symptomatique, un examen radiographique avec contraste baryté de l'œsophage est indiqué. Le traitement chirurgical par transposition du vaisseau aberrant n'est réalisé que pour les patients symptomatiques. Nous rapportons le cas d'une patiente de 50 ans souffrant de dysphagia lusoria traitée par transposition de l'artère sous-clavière droite aberrante dans la carotide primitive droite par abord cervical.

Arteria Lusoria as a Cause of Dysphagia in an Infant.

The aberrant right subclavian artery (i.e., arteria lusoria) arising from the left part of the aortic arch is a rare congenital anomaly. In some patients, esophageal compression may cause symptoms of dysphagia, also called dysphagia lusoria. It can cause serious feeding disorders and poor weight gain in young children. We present the case of an early onset of dysphagia lusoria in a 1-month-old girl whose clinical diagnosis was confirmed by esophagography and magnetic resonance imaging. This kind of vascular anomaly can present a diagnostic challenge and should be considered in diagnosing dysphagia in childhood.

Left Transradial Access Using a Radial-Specific Neurointerventional Guiding Sheath for Coil Embolization of Anterior Circulation Aneurysm Associated With the Aberrant Right Subclavian Artery: Technical Note and Literature Review.

BACKGROUND: Aberrant right subclavian artery (ARSA) is a rare condition, but the most common anomaly of the aortic arch. Although neurointerventions via transradial access (TRA) are becoming increasingly popular worldwide, transradial carotid cannulation has been extremely challenging in patients with an ARSA. Herein, we present a case of ARSA-associated anterior communicating artery (ACoA) aneurysm that was successfully treated with a radial-specific 6F Simmons guiding sheath via left TRA. We also review the relevant literature. METHODS: A 68-year-old-woman who was diagnosed as having an ARSA-associated ACoA aneurysm underwent simple coiling via left TRA. After the 6F Simmons guiding sheath was engaged into the right common carotid artery using the pull-back-technique, transradial quadraxial system (6F Simmons guiding sheath/6F intermediate catheter/3.2F intermediate catheter/coil-delivery microcatheter) was implemented. RESULTS: Simple coiling of the aneurysm was successfully achieved without catheter kinking or system instability. The postprocedural course was uneventful. A follow-up magnetic resonance angiography showed no evidence of recanalization 1 years 9 months after the procedure. CONCLUSIONS: Transradial anterior circulation intervention has been rarely used for patients with an ARSA due to unfavorable catheter trajectory. Left TRA using the 6F Simmons guiding sheath is a useful treatment option to address anterior circulation interventions for patients with an ARSA. Preoperative diagnosis of ARSA is necessary for the application of our method.

A Rare Case of Giant Atypical Patent Ductus Arteriosus.

A broad window-like patent ductus arteriosus (PDA) arising from the transverse aortic arch proximal to the left subclavian artery and without any associated cardiac anomalies in a one-year-old child is rare. This case is reported for the atypical location and unusually large size of the PDA.

Kommerell's Diverticulum in a Right-Sided Aortic Arch With an Aberrant Left Subclavian Artery Hybrid Repair.

BACKGROUND: Kommerell's diverticulum (KD) with a right aortic arch (RAA) and aberrant left subclavian artery (aLSCA) is a rare congenital anomaly of the aortic arch. Treatment is not well defined due to its uncommon presentation, with rupture and dissection risk rates of up to 53%. CASE SUMMARY: A 54-year-old male with a history of chronic obstructive pulmonary disease (COPD) and hypertension presented with difficulty breathing during exercise without dysphagia. Follow-up computerized tomography angiogram (CTA) revealed the presence of a RAA and aLSCA arising from the descending thoracic aorta with an adjacent 58 × 41-mm KD and tracheal and esophageal displacement. Due to the size of the KD, risk of rupture, unsuitable anatomy for total endovascular aortic repair (EVAR), and high COPD burden, the patient was planned to undergo a hybrid surgical repair. Left common carotid (LCCA) artery to LSCA bypass, full aortic debranching, LSCA embolization and percutaneous thoracic endovascular aortic repair (TEVAR) were performed. Successful device position and exclusion of the diverticulum and aneurysmal aorta were observed after completion thoracic aortogram. 18-month follow-up CTA demonstrated patency of the LSCA to LCCA bypass graft and arch vessel branches, as well as stable exclusion of the KD. Persistence of a type II endoleak originated at the right first posterior intercostal artery has been noted and is being followed conservatively since no sac growth has occurred. CONCLUSION: We highlight the presence of a KD with RAA and aberrant subclavian artery, a rare congenital anatomic variation of the aortic arch with complex anatomy. Surgical planning must be individualized according to comorbidities and anatomical variations identified on imaging and 3D reconstructions.

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.