Double Aortic Arch: A Comparison of Fetal CMR, Postnatal CT and Surgical Findings.

BACKGROUND: In double aortic arch (DAA) one of the arches can demonstrate atretic portions postnatally, leading to diagnostic uncertainty due to overlap with isolated right aortic arch (RAA) variants. The main objective of this study is to demonstrate the morphological evolution of different DAA phenotypes from prenatal to postnatal life using 3D fetal cardiac magnetic resonance imaging (CMR) and postnatal CT/CMR imaging. METHODS: 3D fetal CMR was undertaken in fetuses with suspected DAA over a six-year period (Jan 2016 - Jan 2022). All cases with surgical confirmation of DAA were retrospectively studied and morphology on fetal CMR was compared to postnatal CT/CMR and surgical findings. RESULTS: 32 fetuses with surgically confirmed DAA underwent fetal CMR. All demonstrated a complete DAA with left-sided arterial duct. The RAA was dominant in 30/32 (94%). Postnatal CT/CMR was undertaken at median age of 3.3months (IQR 2.0-3.9) demonstrating DAA with patency of both arches in 9/32 (28%), with 6 showing signs of coarctation of the left aortic arch (LAA). The LAA isthmus was not present on CT/CMR in 22/32(69%), the transverse arch between left carotid and left subclavian artery was not present in 1 case. CONCLUSIONS: Fetal CMR provides novel insights into perinatal evolution of DAA. The smaller LAA can develop coarctation or atresia related to postnatal constriction of the arterial duct, making diagnosis of DAA challenging with contrast-enhanced CT/CMR. This highlights the potentially important role for prenatal 3D vascular imaging and might improve intepretation of postnatal imaging.

An In Vivo Radiologic Description of Periacetabular Vascularization in a Healthy Subject and a Literature Review of its Clinical Implications.

The aim of this study is to provide a radiologic description of periacetabular vascularization. A computed tomography angiography was used to analyze the vascularization patterns of the periacetabular region, describing for the first time "in vivo" the periacetabular branches of the superior and inferior gluteal artery, obturator artery, and of the medial circumflex femoral artery. The analysis revealed the possibility of visualizing clearly all the previously described vessels of the aforementioned arteries. Both acetabular and supra-acetabular arteries, the rami of the OA directed to the lamina quadrilateral, and the rami of the IGA directed to the posterior wall were identified. In conclusion, understanding the periacetabular vascularization patterns is pivotal for effective clinical decision-making in pelvic trauma, and conservative and reconstructive surgery of the hip. The radiologic description provided in this study, along with the associated literature review, offers valuable insights into the clinical implications of periacetabular vascularization.

Hypertrophic Osteopathy Concurrent with an Aberrant Right Subclavian Artery in a Dog.

A 13-year-old spayed female cocker spaniel was presented with a 2-month history of swelling in several digits and intermittent hindlimb lameness. Radiographs revealed marked soft-tissue swelling and periosteal new bone formation without cortical bone destruction, characteristic of hypertrophic osteopathy (HO), in the distal parts of all extremities except for the right forelimb. However, no notable findings were detected in thoracic radiographs. An ultrasonography indicated cranial bladder wall thickening, which resolved following antibiotic therapy. Computed tomographic angiography identified a potential underlying cause as an aberrant right subclavian artery (ARSA) originating from the aortic arch, compressing the esophagus and causing mild esophageal cranial dilation to the aberrant vessel. No other intrathoracic or neoplastic lesions were observed. Gastrointestinal symptoms, such as regurgitation, were absent. Although an ARSA was likely the cause of HO, surgical correction was declined by the owner. To the best of our knowledge, this is the first reported case of HO concurrent with ARSA in dogs.

Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience.

Background: Vascular rings represent 1% of congenital cardiovascular abnormalities. Phenotypic expression varies from asymptomatic to severe forms related to either oesophageal or tracheal compression. While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate. We report our center experience of vascular ring management over three decades. Methods: In this single-center retrospective study, data were extracted from patient medical records. To obtain information on symptoms/medication at follow-up, clinical records from pediatric clinics were reviewed. For patients followed in other institutions, a web-based questionnaire was sent to referring pediatricians. Results: Out of 82 patients, 69 were symptomatic (84%). Common symptoms included recurrent respiratory tract infections (43%), stridor (32%), gastro-esophageal reflux (33%), and dysphagia (26%). Diagnosis relied on cardiac ultrasound, barium swallow studies, and chest computerized tomography scan. Surgical repair (thoracotomy 91%) was performed in 79 patients (96%). Median age at repair was 13 [interquartile range (IQR), 4.4-48] months. There was no mortality. Minor complications occurred in 14 patients (18%). Median hospital length of stay was 7 (IQR, 6-9) days. In total, 24% of patients remained symptomatic (median follow-up 54 months). Half of those were asthma-related, with nearly 90% freedom from ring-related symptoms. Conclusions: Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. Finally, surgery in asymptomatic patients resulted in low morbidity supporting the current recommendation of early surgical repair. Level of Evidence: III.

Long-Term Outcome of Surgically Repaired and Non-repaired Double Aortic Arch in Children.

Introduction A double aortic arch (DAA) is a rare congenital vascular anomaly that encircles the trachea and esophagus, resulting in compression of both structures and causing variable symptoms of wheezing, stridor, increased work of breathing, or dysphagia. DAA usually presents in infancy but can be incidentally found later in life. The standard management of DAA is surgical repair. However, observation and follow-up have been recommended in asymptomatic or mild cases. The long-term outcome of surgical repair versus observation is not well-reported. We described the long-term clinical outcome of patients with DAA who were surgically repaired versus non-repaired at our institution.  Methods Electronic medical records were searched for the patients diagnosed with DAA before the age of 18 years. Data from clinical, radiological, and bronchoscopic findings, pulmonary function test (PFT), and cardiopulmonary exercise testing (CPET) were extracted. A structured phone questionnaire of patients' parents regarding past and current symptoms was also conducted. Results A total of 12 patients (eight males four females) with DAA were identified. Median age was 8.5 (1.5-17) years. The age at diagnosis was 60 (1-192) months. Post diagnosis follow-up period was 20 (2-156) months. Five patients were surgically repaired, and seven patients were not repaired. The median age of surgery was five (1-15) years in repaired patients. The phone questionnaire was completed in only 10 patients (five repaired and five non-repaired). Respiratory symptoms in infancy were reported in all repaired and non-repaired patients and were resolved in all five repaired patients and in four of the five non-repaired patients. One non-repaired patient complained of intermittent dyspnea on exertion. Gastrointestinal symptoms were present in infancy in three repaired and three non-repaired patients and were improved in two repaired and one non-repaired patient. PFT was performed in five patients (one repaired, four non-repaired) and showed normal forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and FEV1/FVC in all patients. Low peak expiratory flow (PEF) was seen in the repaired patient and in three of the non-repaired patients. CPET was conducted in four non-repaired patients and showed maximal oxygen consumption (VO2-max) of 66% predicted (58-88), maximal ventilation (VE-max) of 75% predicted (70-104), and ventilatory reserve of 55% predicted (48-104).  Conclusion Long-term clinical outcome is favorable in both repaired and non-repaired patients with DAA even though both groups reported respiratory symptoms during infancy. Therefore, clinical observation is a legitimate option in certain DAA patients.

Pediatric Esophageal Dysphagia.

Swallowing is an elaborate process that requires neuromuscular coordination. Pediatric esophageal dysphagia is broadly categorized into structural and nonstructural causes. The structural causes of pediatric esophageal dysphagia are related to processes that narrow the lumen of the esophagus. Esophageal strictures are the result of scar tissue formation within the lumen of the esophagus, leading to stenosis. Vascular rings and slings cause external compression of the esophagus. Diagnosis requires an esophagram and computed tomography or magnetic resonance imaging. Treatment is guided by the patient's symptoms and underlying diagnosis, although it often requires surgical intervention when symptomatic.

[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.

Long-term Outcomes Following Thoracoscopic Division of Vascular Rings.

OBJECTIVES: We evaluate long-term symptomatic improvement in vascular ring patients who underwent thoracoscopic division at a single quaternary pediatric surgery center. METHODS: All pediatric patients who underwent vascular ring division without Kommerell's diverticulum resection between 01/2007-12/2022 were included. Surgeries were performed by pediatric general and thoracic surgeons. Patient demographic and clinical characteristics were obtained from retrospective chart review. Data on long-term symptomatic improvement were collected with structured telephone interviews. RESULTS: 60% of patients were male. Median age at operation was 24 months (IQR: 11, 60 months) with a median weight of 11.3 kg (IQR: 8.7, 19.8 kg). All patients were symptomatic preoperatively with dysphagia being the most frequent complaint (42%), followed by chronic cough (21%). Of 41 patients eligible for the long-term follow-up survey, 8 patients with a primary diagnosis of a double arch with an atretic segment in the non-dominant arch and 9 with a right dominant arch with left ligamentum arteriosum and aberrant left subclavian artery (LSCA) were contacted and consented for participation. Median interval from surgery to survey completion was 95 months (IQR 28, 135 months). Most patients had no, or only minor, symptoms related to breathing and swallowing at the time of long-term follow-up. 88% of patients experienced postoperative symptom improvement, and only one patient reported worsening of symptoms over time. CONCLUSION: Division of an atretic arch and/or ligamentum for patients with an aberrant LSCA without Kommerell's resection may be adequate to ensure long-term improvement of breathing and swallowing problems attributable to vascular rings. LEVEL OF EVIDENCE: Level IV.

Case Report: An unusual case of a transposition of the great arteries with a double aortic arch: a highly complex fetal diagnosis with an unpredictable outcome.

Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of the great arteries has been rarely described in the literature. In this study, we report the prenatal diagnosis of a 28-year-old woman. A fetal echocardiography at a gestational age of 24 weeks + 6 days showed a D-transposition of the great arteries and a double aortic arch with a ventricular septal defect and pulmonary stenosis. On the first night after birth, the baby experienced an increase in lactate levels, with the rate of oxygen saturation consistently below 80%. A few hours after birth, the patient underwent a Rashkind procedure. An echocardiography, CT chest x-ray, and CT angiogram confirmed a diagnosis with a severe reduction of the tracheal lumen (>85%) and bronchomalacia. Then, the patient underwent posterior tracheopexy and aortopexy and later an arterial switch operation, ventricular septal defect closure, and resection of a part of the infundibular septum, accepting the risk of potential neoaortic obstruction. The literature has reported only two cases of patients with a fetal echocardiogram diagnosis. Therefore, our patient is only the third one with a fetal diagnosis and the second one with a complex intracardiac anatomy, characterized not only by a ventricular septal defect but also by two separate components of the obstruction (a bicuspid valve and a dysplastic valve with a posterior deviation of the infundibular septum). In conclusion, a D-transposition of the great arteries with a double aortic arch remains an extremely unusual association. The clinical outcome of these patients presents a high degree of variability and is entirely unpredictable in prenatal life. Our greatest aim as fetal and perinatal cardiologists is to improve the management and outcome of these patients through a fetal diagnosis, recognizing types of congenital heart disease in newborns who require early neonatal invasive procedures.

Variations in the Evaluation and Management of Vascular Rings: A Survey of American Clinicians.

Vascular rings are arterial malformations that lead to the compression of the trachea and/or esophagus. While "tight" rings often produce symptoms and require surgery, "loose" rings rarely produce symptoms. Given advances in fetal echocardiography, this diagnosis is now more often made prenatally. This poses a new conundrum in the management of asymptomatic patients, leading to practice variation and creating a target for clinical system improvement. Hence, we conducted this survey aiming to demonstrate the practice variation existing in current evaluation and management of these patients. An anonymous web-based survey was distributed to several listservs for pediatric cardiologists and pediatric cardiothoracic surgeons. Survey questions targeted respondent practice characteristics, testing obtained, and indications for testing or surgical referral. In total 61 responses were received, predominantly from pediatric cardiologists (95%) in the United States (97%). About 60% of clinicians reported frequently diagnosing patients with vascular rings by fetal echocardiogram, with only about 20% diagnosing them frequently on evaluation of symptoms. Computed tomography angiography and echocardiogram were the most common imaging modalities employed. Most clinicians obtained cross-sectional imaging at the time of diagnosis and referred to surgery once patients had at least occasional symptoms. Respondents demonstrated a low degree of agreement (Krippendorf's alpha 0.48). Few statistically significant patterns were identified between respondents based on their practice characteristics. This study identified significant variation between clinicians regarding the evaluation and management of vascular rings. Further research or expert opinions may help to standardize practice, saving costs and improving the quality of care for affected patients.

Perioperative and Anesthetic Considerations in Vascular Rings and Slings.

Vascular rings represent an increasingly prevalent and diverse set of congenital malformations in which the aortic arch and its primary branches encircle and constrict the esophagus and trachea. Perioperative management varies significantly based on the type of lesion, its associated comorbidities, and the compromise of adjacent structures. Multiple review articles have been published describing the scope of vascular rings and relevant concerns from a surgical perspective. This review seeks to discuss the perioperative implications and recommendations of such pathology from the perspective of an anesthesia provider.

Associated Anomalies and Outcome in Patients with Prenatal Diagnosis of Aortic Arch Anomalies as Aberrant Right Subclavian Artery, Right Aortic Arch and Double Aortic Arch.

We aimed to evaluate retrospectively associated anomalies and outcome in prenatal aortic arch anomalies (AAAs). We included ninety patients with aberrant right subclavian artery (ARSA), right aortic arch (RAA) with mirror image branching (RAA-mirror) or aberrant left subclavian artery (RAA-ALSA) and double aortic arch (DAA) between 2011 and 2020. In total, 19/90 (21.1%) had chromosomal anomalies, the highest rate being within the ARSA subgroup (17/46, 37%). All (13/13) of the RAA-mirror subgroup, 10/27 (37.0%) of RAA-ALSA, 13/46 (28.3%) of ARSA and 0/4 within the DAA subgroup had additional intracardiac anomaly. The rate of extracardiac anomalies was 30.7% in RAA-mirror, 28.3% in ARSA, 25.0% in DAA and 22.2% in the RAA-ALSA subgroup. A total of 42/90 (46.7%) had isolated AAAs: three (7.1%) with chromosomal anomalies, all trisomy 21 (3/26, 11.5%) within the ARSA subgroup. Out of 90, 19 (21.1%) were lost to follow-up (FU). Two (2.2%) intrauterine deaths occurred, and six (6.7%) with chromosomal anomalies terminated their pregnancy. In total, 63 (70.0%) were liveborn, 3/63 (4.8%) with severe comorbidity had compassionate care and 3/60 (5.0%) were lost to FU. The survival rate in the intention-to-treat cohort was 53/57 (93%). Forty-one (77.4%) presented with vascular ring/sling, two (4.9%) with RAA-ALSA developed symptoms and one (2.4%) needed an operation. We conclude that intervention due to vascular ring is rarely necessary. NIPT could be useful in isolated ARSA cases without higher a priori risk for trisomy 21 and after exclusion of other anomalies.

The aortic uncrossing procedure.

OBJECTIVE: Cross-sectional imaging allows identification of rare patients with a vascular ring and circumflex aorta. The key diagnostic feature is crossing of the transverse aortic arch from right to left posterior to the trachea and superior to the carina in a patient with a right aortic arch. We evaluated our patients who received an aortic uncrossing procedure. METHODS: We reviewed all patients who underwent aortic uncrossing from 2002 to 2022. All patients received preoperative computed tomography imaging and bronchoscopy. RESULTS: Eleven patients ranging in age from 1.5 to 10 years (median 4 years) underwent aortic uncrossing. Two patients had prior left ligamentum division, and 3 patients had prior left aortic arch division. All had significant clinical symptoms. Eight patients had deep hypothermic circulatory arrest (mean 34 minutes), and 3 patients had antegrade cerebral perfusion (median, 28 minutes). Patch material was not used for aortic augmentation, and no patient underwent a posterior tracheopexy or rotational esophagoplasty. Postoperative length of stay ranged from 4 to 31 days (median, 5 days). One patient required a temporary tracheostomy for bilateral recurrent laryngeal nerve paresis, which recovered. One patient required an aortic extension graft to alleviate esophageal compression from an unusual ectatic esophageal course. All patients had relief of airway symptoms and dysphagia. CONCLUSIONS: In properly selected patients with a right aortic arch and circumflex aorta, aortic uncrossing is a safe and effective therapy to treat airway and esophageal compression. The procedure can be conducted with deep hypothermic circulatory arrest or antegrade cerebral perfusion. Careful attention to the location of the esophagus and recurrent laryngeal nerves is required.

Management of esophageal cancer in patients with a right aortic arch or double aortic arch: a case series of 34 cases.

BACKGROUND: Few cases describing patients with a right aortic arch (RAA) or double aortic arch (DAA) and esophageal cancer (EC) have been reported. METHODS: We analyzed RAA and DAA cases treated with esophagectomy in our center's database and reported in English-language studies until April 1, 2023. Our study assessed the malformation characteristics and surgical details of EC patients with RAA and DAA. RESULTS: We extracted data of 24 EC patients with RAAs and 10 EC patients with DAAs. In both groups, the patients were more likely to be Japanese and male, to have squamous cell carcinoma and to have tumors located in the upper thoracic esophagus or middle thoracic esophagus. Left thoracotomy was commonly applied for RAA patients. For DAA patients, the proportions of left-sided and right-sided approaches were similar. Esophagectomy under video-assisted thoracoscopic surgery (VATS) in RAA or DAA patients had been performed on a routine basis in recent years. There were two anastomotic leakages in each group. Specifically, Kommerell diverticulum rupture occurred in 1 RAA patient; gastric tube dilation occurred in 1 DAA patient; and recurrent laryngeal nerve (RLN) injury occurred in 2 RAA patients. The postoperative course was uneventful for most patients in both cohorts. CONCLUSIONS: Esophageal carcinoma is rarely seen in patients with an RAA or DAA. To adequately dissect superior mediastinal LNs, an auxiliary incision (such as sternotomy), the left door open method or a preceding cervical procedure should be used appropriately. Esophagectomy, whether via thoracotomy or thoracoscopic surgery, can be performed safely for both RAA and DAA.

Narrowing Down the Symptomatology of Isolated Vascular Rings in Children.

Vascular rings may cause respiratory or gastrointestinal symptoms due to compression of the trachea and/or esophagus. Advances in imaging have enabled early detection in asymptomatic patients posing new management dilemmas. Surgery is expected to relieve symptoms, although this has not been well studied. We sought to evaluate the presence and pattern of symptoms associated with vascular rings before surgical intervention and to detail symptom resolution after surgery. A 10-year retrospective review of patients diagnosed with an isolated vascular ring was performed between January 2010 and December 2019. 100 patients were identified; 35 double aortic arch (DAA) and 65 right aortic arch and left ligamentum arteriosum (RALL). 73 patients were symptomatic on presentation; 47 had respiratory, 5 had gastrointestinal, and 21 had both types of symptoms. Surgical repair was performed in 75 patients; 74 were symptomatic. Respiratory symptoms were more likely in patients with preoperative tracheal narrowing (p < 0.001). Moderate-severe respiratory symptoms led to surgery in RALL patients (OR 10.6, p = 0.0001). DAA patients were more likely to undergo surgery (p < 0.001) irrespective of symptom severity. At a median post-surgical follow-up of 4 months, there was a significant reduction in symptom burden (p < 0.001), except for asthma symptoms (p = 0.131). Symptom resolution was not dependent on the vascular ring anatomy (p = 0.331) or the age at operation (p = 0.158). Vascular rings are typically accompanied by respiratory symptoms and less commonly GI symptoms, both of which resolve in most patients after surgery. Those who present predominantly with asthma-like symptoms may not achieve resolution after surgery.

Persistence of aerodigestive symptoms after vascular ring repair.

OBJECTIVE: Vascular rings are often associated with respiratory and swallowing difficulties due to tracheal or esophageal compression. While the results of a vascular ring repair are considered excellent, the long-term effect of tracheal and esophageal remodeling and the persistence of symptoms have scarcely been reported. Our study aims to evaluate the respiratory and swallowing outcomes of vascular ring repair. STUDY DESIGN AND METHODS: A retrospective cohort study of children who underwent vascular ring repair between 2010 and 2022 in a tertiary-care children's hospital. RESULTS: There were 108 patients enrolled: sixty-three patients (57.41 %) with a right aortic arch, 42 patients (38.89 %) with a double aortic arch, and 3 patients (2.78 %) with other vascular rings. Forty-three (39.81 %) patients were diagnosed prenatally. Of the 65 patients (60.19 %) diagnosed postnatally, 35/65 (53.85 %) had either respiratory or swallowing symptoms as the indication for diagnostic workup. Persistent respiratory and swallowing symptoms were noted in 34/108 (31.48 %) and 30/108 (27.78 %) patients, respectively, within a year of surgical repair. Fourteen patients underwent repeated laryngoscopy and bronchoscopy that demonstrated residual tracheomalacia; however, only 2/14 (1.9 %) patients required tracheostomy tube placement, and 6-out-of-7 patients were weaned off positive pressure airway support. Persistent respiratory symptoms were significantly more common in patients with a double aortic arch compared to a right aortic arch. No differences were noted in demographics, comorbidities, and preoperative aerodigestive symptoms between patients with residual symptoms and patients with no residual symptoms. CONCLUSIONS: Persistent respiratory and swallowing symptoms after vascular ring repair are not uncommon. Postoperative evaluation should be pursued by a dedicated team, and treatment considered as appropriate.

Association Between Airway Stenosis Degree and Respiratory Distress in Infants With a Vascular Ring.

Background Although the number of cases of prenatally diagnosed vascular rings is increasing, some cases may remain asymptomatic, and no indicator of the appearance of dyspnea has been established. Thus, we aimed to determine the relationship between the degree of airway compression by the vascular ring on contrast-enhanced computed tomography (CT) and respiratory distress. Methods This is a retrospective study of nine patients diagnosed with vascular rings at a single hospital from July 2010 to December 2019. Data regarding the patient's clinical characteristics, such as prenatal diagnosis, vascular ring type, complicated cardiac disease, and presence or absence of surgery, were recorded. Airway assessment on contrast-enhanced CT was measured in the axial cross-section. Statistical analysis was performed using Statistical Product and Service Solutions (SPSS) (version 25.0; IBM SPSS Statistics for Windows, Armonk, NY). Results Five of the eight patients had respiratory distress. Patients with respiratory distress were less likely to have been diagnosed prenatally (p = 0.04) and had smaller stenosis degree of anteroposterior diameter (p = 0.03). Conclusion Contrast-enhanced CT is useful in patients with vascular rings. Our study suggests that the stenosis degree of the anterior-posterior diameter of the airway is related to dyspnea.

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.