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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., Competing Interests: Conflicts of interest We have no competing interests to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Double aortic arch (DAA) is a type of vascular ring, causing stridor and dysphagia owing to compression of the trachea and esophagus. In contrast, double innominate veins, which is a relatively rare venous anomaly, usually does not cause symptoms or require intervention., Case Presentation: A 2 month-old boy presented with mild stridor and a hoarse voice while crying, and was diagnosed as having DAA, small atrial septal defects and double innominate vein. The double innominate veins, crossing over and underneath the ascending aorta, did not contribute to airway compression. To release the narrow vascular ring caused by the DAA, surgical resection of the atretic side of the arch was performed by lateral thoracostomy at 4 months of age, before the appearance of signs of bronchomalacia. The double innominate veins were left untouched, and open-heart surgery was avoided., Conclusions: DAA should be suspected if airway symptoms are present in early infancy, particularly if a right aortic arch is noted. It is important not to hesitate to perform diagnostic imaging and release the DAA, as this might prevent or minimize airway complications. However, for double innominate veins, operative release is not necessary unless it interferes with surgical procedures or is causing symptoms., (© 2024. The Author(s).)

Background: Few studies describe outcomes after complete vascular ring surgery in a comprehensive manner., Objectives: This study sought to describe the clinical presentation, diagnostic work-up, operative approach, and outcomes in children undergoing surgery for complete vascular rings., Methods: This single-center retrospective cohort study includes consecutive patients (January 1990 through September 2023) undergoing primary surgery for complete vascular rings, or rerepair after primary surgery elsewhere. The primary outcome of interest was complete (as distinct from partial) symptom resolution at latest clinic follow-up. Our current preference is to pursue a comprehensive initial operation including adjunctive vascular and airway procedures targeting common causes of residual aerodigestive symptoms, such as Kommerell diverticulum resection and tracheobronchopexy. Preoperative work-up routinely involved computed tomographic angiography, dynamic bronchoscopy, and laryngoscopy., Results: Of 515 patients (including 39 rerepairs), the most common diagnoses were right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum (n = 323, 62.7%) and double aortic arch (n = 174, 33.8%). There was no perioperative mortality. Chylothorax occurred in 28 patients (5.4%), vocal cord dysfunction in 22 patients (4.3%), and diaphragm paralysis in 2 patients (0.4%). Follow-up was available on 453 patients (88.0%) with a median duration of 3.0 years (Q1-Q3: 0.6-9.2 years). At latest clinic follow-up, 429 patients (94.7%) reported complete symptom resolution. The risk of reoperation for residual or recurrent aerodigestive symptoms was 9.6% (95% CI: 5.7%-13.5%) at 10 years and 12.4% at 20 years (95% CI: 6.9%-17.8%)., Conclusions: Surgery for complete vascular rings provides good symptomatic relief with low risk of complications, whereas reoperations for aerodigestive symptoms are infrequent., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Introduction: Vascular Rings (VRs) are congenital malformations of the aortic arch that can compress the trachea and esophagus producing symptoms. Approximately, 5%-10% of patients experience persistent symptoms postrepair and 9% require reintervention. There are various approaches to repair-simple ligation and division of the ring or more complex procedures. Our objective was to describe outcomes for VR repair using a "simple" surgical approach., Methods: We identified patients who underwent VR repair from 2012 to 2022 at our institution. Clinical and surgical data, demographics, intraoperative, and postoperative outcomes were extracted from their electronic medical records. Outcomes were analyzed and regression analysis was used to identify risk factors for residual symptoms after repair., Results: Ninety three patients with VR repair were included. Prevalence of symptoms were reduced following surgery (80% preoperative versus 13% postoperative, P 0.03). Tracheomalacia also decreased from 16% to 7% postrepair. No deaths were reported in our cohort and 2% of our patients required reintervention. Multivariable regression analysis showed that preoperative tracheomalacia was associated with having persistent symptoms after VR repair (odds ratio 6.2, 95% CI 1.02-37.6)., Conclusions: Our institutional experience using a simple surgical approach showed a significant decrease in symptoms, a very low reintervention rate and no mortality. Preoperative tracheomalacia can be a risk factor for persistent symptoms postrepair. We believe a simple surgical approach for children with complete VR repair can be effective in relieving symptoms., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Circumflex aorta is described as a retroesophageal aortic arch, with opposite-sided descending aorta forming a true vascular ring with ligamentum arteriosum. We report two cases of right-sided circumflex aorta with varied clinical presentation. Computed tomography diagnosed this vascular ring anomaly. The patients were managed with the aortic uncrossing procedure as a primary surgical strategy. Preoperative diagnosis and thorough planning were essential for the successful outcome. The patients did not have any residual symptoms of tracheal and esophageal compression on follow up., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Vascular rings are unusual congenital malformations. Among them, double aortic arch (DAA) is often difficult to diagnose due to its low incidence of symptoms. DAA can be associated with tracheal or esophageal compression and, in severe cases, could require tracheal intubation or chronic use of a nasogastric tube. This scenario favors the development of aortotracheal fistulas (ATF) or aortoe-sophageal fistulas (AEF)., Objective: To present a clinical case with an unusual association of DAA with ATF and to reinforce the importance of maintaining high diagnostic suspicion in patients with massive aerodigestive bleeding without an obvious source., Clinical Case: A 32-week preterm newborn who required prolonged mechanical ventilation and presented intermittent episodes of massive oropharyngeal bleeding with hemodynamic compromise associated with lower airway obstruction without pulmonary hemorrhage. The patient underwent upper endoscopy and exploratory laparotomy without evidence of bleeding. Flexible nasopharyngolaryngoscopy and direct laryngoscopy also showed no abnormalities. A CT angiography showed complete DAA with indentation of the left dominant arch over the trachea, without severe stenosis or evidence of a fistula. AEF was suspected, so exploratory surgery was considered. However, the patient died before surgery due to a massive pulmonary hemorrhage. The autopsy revealed the presence of ATF., Conclusions: In patients with massive aerodigestive bleeding without an obvious source, the presence of DAA and possible AEF/ ATF should be considered. Imaging studies have a poor performance for this diagnosis, so surgery should be considered for diagnosis and treatment in these patients.

Objectives: Right aortic arch (RAA) is a rare anomaly with an incidence of 0.1 % in the adult population and low-risk fetuses. Our aim in this study was to evaluate associated anomalies and conditions according to subtypes., Methods: This was a retrospective study examining consecutive pregnancies diagnosed with RAA in our hospital between 2018 and 2022. Fetuses with RAA were divided into three groups, RAA with right-sided ductus arteriosus (RAA-RDA), RAA with left-sided ductus arteriosus (RAA-LDA), and RAA with a double aortic arch (RAA-DAA)., Results: A total of 81 fetuses were diagnosed as having RAA during the study period. The rate of cardiac anomalies (82.8 %) in the RAA-RDA group was higher than in the RAA-LDA (17.6 %) and RAA-DAA (22.2 %) groups (p<0.001). No statistically significant difference was found between the groups in terms of maternal age, diagnosis week, pregnancy outcome, extracardiac anomalies, and genetic anomalies. Three (8 %) of 36 fetuses with isolated RAA who resulted in live birth developed symptoms related to the vascular ring, and one (2.7 %) newborn with RAA-DAA underwent surgery., Conclusions: The incidence of cardiac anomalies is high in fetuses with RAA-RDA. Ultrasound examinations should be performed for cardiac anomalies and additional structural anomalies. Vascular ring formation is a rare but important complication due to compression risk to the trachea and esophagus., (© 2024 Walter de Gruyter GmbH, Berlin/Boston.)

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., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

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., Competing Interests: The authors declare no competing interests., (Copyright: Ndubueze Ezemba et al.)

Background: Secondary hyperparathyroidism (SHPT) is a common complication of end-stage renal disease. Parathyroidectomy (PTx) is often employed for treatment of severe SHPT. However, PTx may cause hypotension via unknown mechanisms. COMM domain-containing protein 5 (COMMD5) in the parathyroid glands has been linked to blood pressure regulation of spontaneously hypertensive rats., Objective: To explore the relationship between COMMD5 levels and reduced BP after PTx in patients receiving hemodialysis (HD)., Methods and Results: (1) The study cohort included 31 patients receiving HD who underwent PTx. Serum COMMD5 levels were higher post-PTx vs. pre-PTx. (2) Sprague-Dawley rats (n = 22) were assigned to a 5/6 nephrectomy group or sham surgery group, vascular rings of the thoracic aorta from rats with CKD were incubated with COMMD5, and changes in vascular tension were compared. COMMD5 inhibited vasoconstriction of vascular rings with intact endothelium, but had no effect on vascular rings without the endothelium. (3) Human umbilical vein endothelial cells were stimulated with COMMD5 or small interfering RNA (siRNA). The expression levels of atrial natriuretic peptide (ANP) and endothelial nitric oxide synthase (eNOS) were up-regulated and down-regulated, respectively., Conclusions: Serum COMMD5 levels were increased after PTx in SHPT patients. COMMD5 promoted high expression of ANP and eNOS in endothelial cells, leading to vasodilation and resulting in hypotension., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest. Some of the data of this article have been reported in Chinese., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

To evaluate prenatal findings of the right aortic arch (RAA), associated cardiac, extracardiac, and genetic anomalies, its perinatal outcomes and the need for postnatal interventions in cases of isolated RAA with a view to facilitating appropriate counseling. This was a multicenter, cohort study, that was undertaken in two international major cardiac centers between 2009 and 2020. The study subjects were prenatally diagnosed RAA cases with and without other structural cardiac defects. A RAA was identified in 137 fetuses. There were 84 cases of isolated RAA. Associations with additional intracardiac malformations were found in 53 (38.7%) cases. An extracardiac anomaly was observed in 26/137 (18.9%) fetuses, 11/84 (13.0%) fetuses with isolated RAA, and 15/53 (28.3%) fetuses with an additional intracardiac anomaly. The incidence of extracardiac and chromosomal anomalies was significantly higher in cases of RAA with abnormal intracardiac anatomy (28.3-18.8%, respectively), compared with RAA with normal intracardiac anatomy (13.0-5.9%, respectively) (p < 0.05). 22q11.2 microdeletion was found higher in RAA with CHD (4/18 fetuses) than isolated RAA (2/24 fetuses) (22.2% vs. 8.3% respectively). ALSA was present in 19.3% of cases. ALSA was more frequently observed in cases of isolated RAA (23.6%), than in RAA with structural CHD (7.6%) (p < 0.05). The pregnancy was interrupted in six fetuses, and one died in utero. The mortality rate was higher in fetuses with intracardiac anomaly than RAA without cardiac anomaly (11/49 (22.4%) vs. 2/81 (2.4%). Vascular ring formation was revealed in 21/98 cases. The RAA caused symptoms of a vascular ring in only one patient (0.7%) requiring surgery in the follow-up. Overall survival after initial diagnosis in the total cohort was 85.4% with 38 of 53 (71%) RAA with CHD cases and 79 of 84 (94.0%) isolated RAA cases. Chromosomal and extracardiac anomalies are lower in isolated RAA but not negligible hence amniocentesis should be routinely offered in all cases. The requirement for postnatal intervention in the immediate neonatal period is remote, therefore delivery of these fetuses need not be undertaken at a cardiac or surgical center., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction in childhood. TBM can present with a large spectrum of disease severity and underlying pathologies that may be managed medically and surgically, and it is not always clear which patients would most benefit from surgical intervention. We aim to describe the incidence, patient characteristics, and predictors of surgical intervention in a large cohort of paediatric patients., Methods: We performed a retrospective review of all children diagnosed with TBM to a paediatric Otolaryngology unit in the west of Scotland between 2010 and 2020. Odds ratios for clinical predictors of surgery were calculated using logistic regression with uni- and multivariate analysis., Results: 249 patients were identified of which 219 proceeded to data collection. Primary malacia was noted in 161 (73.5%) and secondary in 58 (26.5%). Causes of secondary malacia included compression by the innominate artery (11%) and vascular rings (7.8%). Surgical interventions were performed in 28 patients (12.8%) including division of vascular ring, aortopexy, and surgical tracheostomy. Multivariate analysis showed secondary TBM, acute life-threatening events, and difficulty weaning from mechanical ventilation were independent risk factors for surgical intervention., Conclusions: TBM can present with a myriad of airway symptoms and is frequently associated with other airway and mediastinal pathologies necessitating multiple interventions. Children aged <1 year present with a more severe form of the disease and the presence of particular independent risk factors may indicate a need for surgical intervention., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome., (© 2022 by the Texas Heart® Institute, Houston.)

The incidence of bilateral ductus arteriosus is sporadically reported in the uterus, and right aortic arch with aberrant of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. We describe a case of right aortic arch and aberrant left subclavian artery associated with bilateral ductus arteriosus. Scanning around the three-vessel and trachea view to search for an "O" shape and a "U" shape vascular ring is essential for the diagnosis, which reveals vascular structures coursing around the trachea. Four-dimensional echocardiography with high-definition flow imaging and spatiotemporal image correlation technique illustrates the spatial relationships of these vessels and the trachea, which should be considered as a complementary modality in fetal cardiac examinations.

Double aortic arch with circumflex aorta is a rare type of vascular ring. Symptoms result from external compression of the trachea and esophagus. The best surgical approach for patients with double arch and circumflex aorta is debated, and options include the highly complex aortic uncrossing procedure. Herein we describe the surgical treatment of a patient with concurrent double aortic arch and circumflex aorta by division of the non-dominant arch and ligamentum arteriosum, plication and posterior tacking of the diverticulum of Kommerell. This left thoracotomy approach provided complete symptom resolution., (© 2022. The Author(s).)

The ring-sling complex is an association between pulmonary artery sling and congenital tracheal stenosis. Pulmonary artery sling is a rare form of vascular ring in congenital heart disease. The clinical case of a child with inspiratory laryngeal stridor associated with respiratory distress is presented, evaluated in another center where respiratory endoscopy was performed, observing extrinsic tracheal compression. Given the clinical suspicion of a vascular ring, CT angiography was requested, confirming the diagnosis of pulmonary artery sling. He was referred to our hospital and during the surgical intervention a new respiratory endoscopy and tracheobronchography were performed, reaching the diagnosis of congenital tracheal stenosis, right accessory bronchus (pig bronchus) and complete vascular rings, both repaired in the same surgical act., Competing Interests: None., (Sociedad Argentina de Pediatría.)

Background: Double aortic arch (DAA) is an extremely rare vascular malformation, even more so when coexisting with esophageal cancer., Methods: We report a new case of DAA with esophageal cancer recently seen at our Thoracic Tumor Clinic and review cases of DAA coexisting with esophageal cancer reported in the literature of English language from 2010 to 2020. The purposes of our literature review were to explore how to best achieve radical esophagectomy while reducing postoperative complications. The clinical manifestations, diagnostic method, surgical approach, reconstruction route, and the extent of lymphadenectomy of esophageal cancer with DAA were analyzed in detail., Results and Conclusion: For such patients, 3D computed tomography is necessary for preoperative diagnosis. The surgical approach should consider factors such as the location of the tumor in the esophagus and whether the tumor is surrounded by DAA, as well as the position of the descending aorta and the requirements for the surgical field for lymphadenectomy. If esophageal reconstruction is required, the retrosternal route is preferred. We recommend that only patients with positive results of intraoperative frozen biopsy of recurrent laryngeal nerve lymph nodes should undergo three-field lymphadenectomy, which may be the best method to achieve radical esophagectomy for middle and lower esophageal cancers with DAA while minimizing postoperative complications., (© 2022. The Author(s).)

Background: Vascular compression of the airway often complicates CHD management. This study evaluated the use of CT in determining cardiovascular causes, clinical manifestations, and outcome of tracheobronchial compression among children with CHD., Methods: A retrospective review of clinical records of all patients with CT scan evidence of tracheobronchial compression from January 2007 to December 2017 at National Heart Institute. Cardiovascular causes of tracheobronchial compression were divided into three groups; group I: vascular ring/pulmonary artery sling, II: abnormally enlarged or malposition cardiovascular structure due to CHD, III: post-CHD surgery., Results: Vascular tracheobronchial compression was found in 81 out of 810 (10%) patients who underwent CT scan. Group I lesions were the leading causes of vascular tracheobronchial compression (55.5%), followed by group II (34.6%) and group III (9.9%). The median age of diagnosis in groups I, II, and III were 16.8 months, 3 months, and 15.6 months, respectively. Half of group I patients are manifested with stridor and one-third with recurrent chest infections. Persistent respiratory symptoms, lung atelectasis, or prolonged respiratory support requirement were clues in groups II and III. Higher morbidity and mortality in younger infants with severe obstructive airway symptoms, associated airway abnormalities, and underlying complex cyanotic CHD., Conclusions: Vascular ring/pulmonary artery sling and abnormally enlarged or malposition cardiovascular structure were the leading causes of cardiovascular airway compression. A high index of suspicion is needed for early detection due to its non-specific presentation. The outcome often depends on the severity of airway obstruction and complexity of cardiac lesions.

Objectives: Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division., Methods: A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed., Results: The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up., Conclusions: The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Objective: To describe the treatment of persistent right aortic arch (PRAA) in dogs with combined ligamentum arteriosum (LA) transection and esophageal diverticulum resection., Animals: Three client owned dogs., Study Design: Short case series., Methods: Medical records were reviewed for clinical signs, diagnostic procedures, surgical treatment, post-operative therapies including medications and feeding regime, outcomes, and follow-up imaging., Results: Esophageal resection was performed using a thoracoabdominal (TA) stapler with suture overlay. All dogs recovered well from surgery and did not experience any peri- or post-operative complications. The last follow-up was performed between 64 and 1004 days post-operatively. In all cases, regurgitation resolved and did not recur in any dogs. No dogs required medical therapy or dietary modifications. In two cases, follow-up imaging was performed that revealed marked improvement of esophageal dilation., Conclusion: Resection of esophageal diverticulum secondary to PRAA utilizing a TA stapler with suture overlay was technically feasible and did not seem associated with early or late complications., (© 2021 American College of Veterinary Surgeons.)

Background: A double aortic arch (DAA) is increasingly identified before birth; however, there are no published data describing the postnatal outcome of a large prenatal cohort., Objective: To describe the associations, symptoms and impact of prenatally diagnosed DAA., Methods: Retrospective review of consecutive cases seen at two fetal cardiology units from 2014 to 2019. Clinical records including symptoms and assessment of tracheobronchial compression using flexible bronchoscopy were reviewed. Moderate-severe tracheal compression was defined as >75% occlusion of the lumen., Results: There were 50 cases identified prenatally and 48 with postnatal follow-up. Array comparative genomic hybridisation (aCGH) was abnormal in 2/50 (4%), aCGH was normal in 33/50 (66%) and of those reviewed after birth, 13 were phenotypically normal. After birth, there was a complete DAA with patency of both arches in 8/48 (17%) and in 40/48 (83%) there was a segment of the left arch which was a non-patent, ligamentous connection.Stridor was present in 6/48 (13%) on the day of birth. Tracheo-oesophageal compressive symptoms/signs were present in 31/48 (65%) patients at median age of 59 days (IQR 9-182 days). Tracheal/carinal compression was present in 40/45 (88%) cases. Seven of 17 (41%) asymptomatic cases demonstrated moderate-severe tracheal compression. All morphologies of DAA caused symptoms and morphology type was not predictive of significant tracheal compression (p=0.3)., Conclusions: Genetic testing should be offered following detection of double aortic arch. Early signs of tracheal compression are common and therefore delivery where onsite neonatal support is available is recommended. Significant tracheal compression may be present even in the absence of symptoms., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Fetal double aortic arch (DAA) malformation is a rare congenital heart disease with few reported cases in the literature. We aimed to investigate the characteristics of prenatal ultrasound and postnatal computed tomography angiography (CTA) of DAA and to describe the associated anomalies and clinical outcomes to improve prenatal diagnosis and assist in perinatal management., Methods: The obstetric ultrasound imaging databases of seven tertiary referral centers were reviewed retrospectively to identify fetuses with a prenatal diagnosis of DAA between January 2013 and December 2018. Ultrasonographic findings, associated anomalies, genetic abnormalities, postnatal CTA images, and long-term postnatal outcomes were evaluated., Results: A total of 36 cases out of 40 prenatally diagnosed DAA fetuses were confirmed by postnatal diagnosis (fetal autopsy, CTA, and surgery). In this cohort of 36 confirmed cases, 24 (67%) were isolated anomalies, while 12 (33%) were associated with intracardiac or extracardiac anomalies, and 2 (6%) had a 22q11.2 chromosome deletion. Among nine cases of pregnancy termination with a fetal autopsy, 7 had other abnormalities. Among the remaining 27 live births, 16 (59%) were asymptomatic and 11 (41%) received surgical treatment due to tracheal or esophageal compression symptoms, all with satisfactory outcomes. Prenatal echocardiography showed that DAA was mainly characterized by a bifurcation of the ascending aorta into the right and left aortic arch and the formation of a complete O-shaped vascular ring around the trachea on the three-vessel tracheal view. A variant in the aortic arch branching pattern was found for the first time. The airway obstruction, branching pattern, and atretic arch of DAA were clearly shown by postnatal CTA., Conclusions: Fetal DAA has unique features on prenatal echocardiography and postnatal CTA, and systematic prenatal examination and timely postnatal CTA evaluation are required. A certain proportion of intracardiac and extracardiac abnormalities are associated with DAA, but the probability of chromosome abnormalities is low, especially for isolated DAA.The clinical outcomes of isolated DAA are favorable, even if surgery is performed due to symptoms. Determining whether other malformations or chromosomal anomalies exist is crucial for prognosis evaluation and prenatal counseling.

A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. Knowledge of the normal embryonic development of the aortic arch and related structures is important for understanding and classifying the various forms of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. With the development of new technologies, multi-detector computed tomography (MDCT) has become a good diagnostic modality for pre- and postoperative evaluation. MDCT provides an excellent image of aortic arch abnormalities and the related anatomy, as well as the tracheal pathology. For patients with symptoms, surgical division of the vascular ring usually achieves excellent outcomes with marked resolution of symptoms and a low risk of morbidity and mortality. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.

Pulmonary artery sling (PAS) is a rare disease frequently associated with severe malacia and stenosis of the trachea. We present a two-day-old newborn that underwent urgent surgery for PAS and needed prolonged respiratory support afterward. Temporary airway stenting above the level of the tracheal bifurcation was performed five days after surgery to overcome severe airway obstruction caused by tracheomalacia and laceration of the tracheal mucosa after diagnostic bronchoscopy. Two days after the procedure, the child could be extubated and after two weeks the stent was removed without complications. Temporary tracheal stenting after PAS repair can be an effective therapeutic strategy in newborns with tracheal obstruction and allows timely weaning from ventilator support.

Introduction: Double aortic arch is a rare congenital malformation of the aortic arch that most frequently presents in childhood. Early surgical intervention typically yields excellent outcomes., Objectives: To describe aortotracheal fistula as a rare, yet serious complication of vascular ring and subsequent aortic aneurysm in an adult patient., Methods: Clinical history, as well as radiographic and endoscopic imaging were obtained to describe the development, diagnosis, and clinical course of this patient's aortotracheal fistula. Additionally, follow up data was obtained to document the healing of this fistula after surgical repair., Results: We describe a case of a 46-year-old male with DiGeorge Syndrome and a double aortic arch, repaired in childhood, which developed into an aortotracheal fistula after tracheostomy placement as an adult., Conclusions: This case demonstrates that dangerous complications of a double aortic arch can persist into adulthood, even after surgical repair in infancy. Each patient's unique anatomy must be considered when thinking about airway management and prevention of complications of this rare congenital anomaly.

Symptoms of airway compression secondary to double aortic arch are relieved by division of one of the two aortic arches. However, in some cases inherent tracheomalacia and other factors may result in persistence of symptoms. We report one such occurrence in our experience and describe the use of aortopexy as a curative procedure to tackle this problem.

Background: In esophageal squamous cell cancer (ESCC) patients, the dissection of the lymph nodes around the recurrent laryngeal nerve (RLN) is essential for curative esophagectomy.1,2 Left pulmonary artery sling (LPAS) is a rare congenital anomaly, in which anomalous left pulmonary artery arises from the right pulmonary artery and reach the left pulmonary hilum.3-5 Because LPAS crosses between esophagus and trachea and the hemodynamics of LPAS could collapse when retracting the trachea anteriorly for left RLN node dissection, esophagectomy for patients with LPAS is technically challenging. In this video, we applied the cervicothoracoscopic approach in a patient with LPAS, in which we performed bilateral RLN node dissection from cervical operation field before thoracoscopic surgery.6,7 METHODS: A 44-year-old woman was diagnosed with stage II ESCC. Following neoadjuvant chemotherapy, we planned to perform a three-stage esophagectomy. Preoperative-enhanced computed tomography revealed LPAS. During the cervical procedure, we identified the RLN, dissected the lymph nodes around the nerve, and mobilized the cervical esophagus. After the cervical procedures, we performed thoracoscopic surgery through the right thoracic cavity with the patient in a prone position., Results: We achieved curative esophagectomy without any intraoperative adverse events. Total operation time was 419 min, with an estimated blood loss of 40 ml. There were no postsurgical complications, including RLN palsy., Conclusions: The presence of LPAS in esophageal cancer surgery makes it difficult to dissect the left RLN nodes. We could safely perform curative esophagectomy for an ESCC patient with LPAS using the cervicothoracoscopic approach.

Objective: Identify incidence and factors associated with respiratory complications after type 1 cleft repair., Methods: Retrospective chart review of patients who underwent cleft repair over a 5-year period performed by a single surgeon. Primary endpoint was respiratory complications (oxygen desaturation <90%). Fisher's exact test was used to identify differences between repair types (endoscopic carbon dioxide laser-assisted repair and injection laryngoplasty). Logistic regression was used to identify predictors of respiratory events., Results: Fifty-five patients were included. Thirty-four (62%) patients underwent endoscopic carbon dioxide laser-assisted repair and 21 (38%) underwent injection laryngoplasty. Average hospital stay for each group was 1.6 days (SD = 3.1) and 0.6 days (SD = 0.9), respectively. Desaturations occurred in three patients (9%) in the laser-assisted repair group and one patient (4%) in the injection group. All occurred within 3 h after surgery and resolved with supplemental oxygen, oral airway placement, and/or mask ventilation. Two affected patients had comorbid diagnosis of asthma (one had poor medication compliance), and one had a history of developmental delay and hypotonia. In the injection group, desaturations occurred in one patient with a history of tracheal stenosis and double aortic arch. No correlation existed between repair type and desaturation (p = 0.57). No variables were significant predictors of events., Conclusions: In this cohort, respiratory events after type 1 laryngeal cleft repair occurred early in the postoperative period, in children with cardiac and pulmonary comorbidities. This suggests postoperative admission may only be necessary for a select group of patients undergoing type 1 cleft repair. However, further research is needed to determine criteria for same-day discharge., Competing Interests: Declaration of competing interest None., (Copyright © 2019. Published by Elsevier B.V.)

Background: This study was intended to determine the intermediate-term outcomes after slide tracheoplasty (STP) in patients with ring-sling complex., Methods: All children undergoing STP between 2009 and 2018 were included. The patients' baseline characteristics, perioperative management details, and follow-up evaluations were reviewed retrospectively., Results: Median age was 1.2 years (range, 2 months to 9.2 years). Seventy-eight patients had additional cardiovascular anomalies and 7 had unilateral pulmonary hypoplasia. Carina involved in the stenotic lesion was present in 42 patients and carinal compression occurred in 58. Twenty-five (21.6%) patients had a stenosis extending into the main bronchus. Type 2, typically having aberrant tracheobronchial patterns, accounted for nearly two-thirds of the patients according to Wells' classification. There were 7 in-hospital deaths and 8 late deaths. Median follow-up was 2.1 years (range, 1.2 months to 9.2 years). Type 2B patients had a higher incidence of malacia and restenosis. In multivariate analysis, the additional cardiovascular anomaly was significantly associated with postoperative tracheomalacia (P = .037). Carinal stenosis (P = .034) was significantly associated with recurrent stenosis. Bronchial stenosis with concomitant carinal stenosis and compression was significantly associated with postoperative tracheomalacia, restenosis, and mortality (for all comparisons, P < .001)., Conclusions: Intermediate outcomes of STP in patients with ring-sling complex is satisfactory. Type 2B is a heterogenous subset patients with more severe tracheobronchial anomalies who will benefit from recognition preoperatively and close surveillance postoperatively., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: None declared.

Coexistence of asymptomatic balanced double aortic arch with tetralogy of Fallot (TOF) is extremely rare and represents a surgical dilemma in decision-making due to the lack of consensus on the management of this subset of patients. We report a case of asymptomatic balanced double aortic arch coexistent with TOF in a two-year-old girl.

We report two cases of prenatally diagnosed double aortic arch with dominant right arch and a left-sided ductus arteriosus, consistent with a complete vascular ring. Postnatal transthoracic echocardiogram and cardiac magnetic resonance imaging demonstrated a spontaneous closure of the ductus arteriosus and obliteration of the left aortic arch distal to the origin of the left subclavian artery in both cases. Spontaneous closure of the ductus arteriosus involving extended ductal tissue in the left aortic arch likely led to obliteration of the distal left arch after birth. One patient presented with recurrent symptoms suggestive of dysphagia and underwent a successful surgical repair of the vascular ring with resolution of symptoms. The other patient has been asymptomatic and is 4 years old at the time of this report.

Background: Limited data exist on the management of vascular rings (VR) in adults. We reviewed our experience of surgical treatment of these patients., Methods: All adult patients with VR (n = 65) who underwent VR repair (mean age, 45 ± 16 years; 33 women [51%]) from January 1972 to January 2018 were retrospectively reviewed. Anatomic variants were right arch with aberrant left subclavian artery (SA) and Kommerell diverticulum (KD) in 22 (34%), left arch with aberrant right SA and KD in 20 (31%), double aortic arch in 12 (18%), right arch with mirror imaging and persistent ligamentum off KD in 7 (11%), and others in 4 (6%). Indications for operation included dysphagia in 43 (63%), respiratory symptoms in 28 (43%), aneurysmal KD in 12 (18%), and dissection/rupture in 7 (11%)., Results: KD was found in 51 patients (78%). The surgical approach included left thoracotomy in 50 (77%), right thoracotomy in 7 (11%), sternotomy in 5 (8%), and hybrid repair in 3 (5%). A 2-stage repair with carotid-SA transposition, followed by transthoracic KD excision, was done in 51% of aberrant SA (n = 23). There was 1 early death. Morbidity included recurrent laryngeal nerve injury in 5 (8%) and chylothorax in 3 (5%). Symptomatic improvement occurred in 97%. Survival was 96.1%, 85.0%, and 73.4% at 1, 5, and 10 years, respectively. Dysphagia recurred in 9 (14%), which included 7 (11%) with esophageal dysmotility., Conclusions: Repair of VR in adults can be performed safely. Dysphagia is the most common symptom and improves in most after repair. Excision of the KD and aberrant vessel is the preferred approach to prevent acute aortic events or recurrent symptoms. Early operation should be considered with esophageal compression to avoid late dysmotility., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Vascular rings account for 1 % of the congenital cardiovascular diseases. They constitute an embryological malformation in which the aortic arch, its branches, or the pulmonary arteries cause pressure on the trachea and/or oesophagus. Anatomically, they are divided into two groups -complete or incomplete- depending on how they surround the trachea and/or the oesophagus. Symptom onset is usually in the first months of life with biphasic or expiratory stridor that increases with crying and feeding, a metallic tracheal cough, recurrent respiratory infections, episodes of apnea and cyanosis, wheezing, cervical hyperextension, sternal and intercostal retraction, and feeding difficulties. A retrospective analysis of 28 patients with vascular rings seen at the Department of Respiratory Endoscopy at Garrahan Paediatric Hospital between January 2015 and September 2017 is presented., Competing Interests: The authors report no conflicts of interest in this work., (Sociedad Argentina de Pediatría.)

Purpose: Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF., Methods: A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up., Results: In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54-2.65) vs. 2.57 kg (2.00-3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32-37) vs. 37 weeks (35-39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29)., Conclusion: RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach., Level of Evidence: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

An 80-year-old woman was transferred to our institution with a subacute type A aortic dissection and a previously undiagnosed double aortic arch. The patient underwent successful repair with aortic valve resuspension, ascending aortic replacement, and repair of the proximal right-sided arch. This is the first reported case of a type A dissection associated with a double aortic arch in the United States., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To define the associations of a prenatally diagnosed, apparently isolated right aortic arch (RAA) with chromosomal or genetic abnormalities and tracheal compression., Methods: This was a retrospective study of apparently isolated RAA assessed by fetal cardiologists and fetal medicine specialists at Kings College Hospital, London between 2000 and 2017., Results: The search identified 138 cases of apparently isolated RAA. Invasive testing was performed in 75, and chromosomal or genetic anomalies were identified in 16 (22%), and the most common was 22q11 microdeletion. An aberrant left subclavian artery was seen in 51% of cases. Symptoms of a vascular ring were present in 24 of 97 (25%) children who were reviewed after birth. Bronchoscopy was performed in 33 children, and significant tracheal compression was diagnosed in 28, including 18 of 19 symptomatic and 10 of 14 asymptomatic children., Conclusions: An apparently isolated RAA is associated with a high incidence of chromosomal or genetic abnormalities and a high incidence of tracheal compression in symptomatic and asymptomatic patients. Prenatal counselling for genetic associations and postnatal airway assessment in the context of the vascular anatomy is recommended., (© 2018 John Wiley & Sons, Ltd.)

We report a case of the repair of an acquired benign tracheoesophageal fistula (TEF) after prolonged mechanical invasive ventilation. Patient had an unknown double incomplete aortic arch determining a vascular ring above trachea and esophagus. External tracheobronchial compression, caused by the vascular ring, increasing the internal tracheoesophageal walls pressure determined by endotracheal and nasogastric tubes favored an early TEF development. The fistula was repaired through an unusual left thoracotomy and vascular ring dissection. TEFs are a heterogeneous group of diseases affecting critically ill patients. Operative closure is necessary to avoid further complications related to this condition. Pre-operative study is mandatory to plan an adequate surgical approach.

Background: The aim of our study was to report our experience in thoracoscopy in infants and neonates for vascular surgical conditions in neonates and infants and to compare our results to open surgery regarding the short-term outcome., Patients and Methods: We retrospectively reviewed all the patients operated in a single institution from 1997 to 2016 for persistent ductus arteriosus (PDA) and vascular ring (VR) anomalies. We compared our thoracoscopic series to a historical control group operated by open surgery. Data collection from charts and office notes included age and weight at surgery, cardiac ultrasound data for PDA, preoperative clinical symptoms for VR, type of surgery, operating time, analgesic treatment requirements, ventilation status during postoperative course, and early complications., Results: The thoracoscopic group included 13 PDA (median age and weight at surgery: 34 days and 1800 g) and 11 VR (median age and weight at surgery: 8 months and 7000 g). The thoracoscopic group did not differ in preoperative symptoms and work-up, operating time, ventilation status, length of hospital-stay, and postoperative complications with the group operated on by thoracotomy, for either PDA or VR., Conclusion: Our short-term results in thoracoscopic PDA closure and VR anomalies surgery in neonates and infants are comparable to open surgery. Thoracoscopy seems to provide less pain especially for neonates and premature babies and allows to decrease the risk for postoperative chest wall deformities. Long-term outcome is mandatory to confirm these preliminary results.

Background: Esophageal cancer frequently metastasizes to lymph nodes along the recurrent laryngeal nerve (RLN),1 therefore it is essential to dissect RLN nodes for curative esophagectomy.2 Right-sided aortic arch (RAA), a rare congenital anomaly, occurs in approximately 0.1% of adults.3 Because RAA forms a vascular ring encircling both the esophagus and the trachea at the cervicothoracic junction, its surgical anatomy is difficult to understand; hence, thoracoscopic dissection of RLN nodes is technically challenging. In this video, we applied the cervicothoracoscopic approach in a patient with esophageal squamous cell carcinoma and RAA, wherein the cervical operation was performed prior to the thoracoscopic operation.4 METHODS: A 60-year-old woman was diagnosed with stage IA esophageal squamous cell carcinoma. During preoperative evaluation, we found that she had RAA with an aberrant left subclavian artery, and therefore decided to perform esophagectomy using the cervicothoracoscopic approach. In the cervical operation, we identified the RLN and dissected the lymph node in the cervicothoracic junction. The thoracoscopic operation was then performed from the left side with the patient in the prone position., Results: The operation was successful. Total operative time was 548 min, with an estimated blood loss of 220 ml. There were no intraoperative and postoperative complications, and RLN palsy did not occur., Conclusions: The vascular ring at the cervicothoracic junction in a patient with RAA made it difficult to dissect the RLN lymph nodes during oncologic esophagectomy. The cervicothoracoscopic approach enables the complete dissection of these lymph nodes and is a useful esophagectomy technique for patients with RAA.