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Journal of Pediatric Surgery Apr 2003The authors analyzed clinical signs of vascular ring anomalies together with appropriate complementary examinations and factors predictive of outcome after surgical... (Review)
Review
BACKGROUND
The authors analyzed clinical signs of vascular ring anomalies together with appropriate complementary examinations and factors predictive of outcome after surgical treatment.
METHODS
The authors reviewed the files of 62 patients with vascular ring abnormalities treated at Necker-Enfants Malades Hospital between January 1990 and January 2000, to analyze age at symptom onset, results of paraclinical examinations, the type of vascular ring abnormality, the surgical indications and type of surgery, and postoperative outcome. Outcomes were divided into 3 categories: cure, partial improvement, and no improvement. The chi2 test corrected with Fischer's Exact test was used for statistical analysis.
RESULTS
Vascular ring abnormalities were diagnosed at birth in 28% of cases and during the first year of life in 68%. Sixteen percent of patients had associated abnormalities. Recurrent pulmonary and bronchial infections occurred after one year of age. An esophagogram was done in 76% of cases and showed impression images. Endoscopy was done in 63% of cases and showed malacia in 41% of patients and stenosis in 51%. Fifty-eight children were treated surgically. The average hospital stay was 7.4 days. The average follow-up was 37.4 months (12 to 159 months). Complete improvement was obtained in 68% of cases, partial improvement in 17%, and no improvement in 15%. Preoperative malacia was more frequent in patients with partial improvement or no improvement.
CONCLUSIONS
Surgical treatment of congenital vascular ring abnormalities is effective and safe. Complications are rare. Prognostic factors include the quality of preoperative preparation of respiratory function, the type of abnormality, and the degree of malacia.
Topics: Abnormalities, Multiple; Airway Obstruction; Aorta, Thoracic; Brachiocephalic Trunk; Child; Child, Preschool; Ductus Arteriosus; Esophageal Stenosis; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Paris; Respiratory Distress Syndrome, Newborn; Retrospective Studies; Subclavian Artery; Tracheal Stenosis; Treatment Outcome; Vomiting
PubMed: 12677561
DOI: 10.1053/jpsu.2003.50117 -
Seminars in Pediatric Surgery Jun 2016The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two... (Review)
Review
The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two structures. The most common vascular rings are double aortic arch and right aortic arch with left ligamentum. Pulmonary artery sling is rare and these patients need to be carefully evaluated for frequently associated tracheal stenosis. Another cause of tracheal compression occurring only in infants is the innominate artery compression syndrome. In the current era, the diagnosis of a vascular ring is best established by CT imaging that can accurately delineate the anatomy of the vascular ring and associated tracheal pathology. For patients with a right aortic arch there recently has been an increased recognition of a structure called a Kommerell diverticulum which may require resection and transfer of the left subclavian artery to the left carotid artery. A very rare vascular ring is the circumflex aorta that is now treated with the aortic uncrossing operation. Patients with vascular rings should all have an echocardiogram because of the incidence of associated congenital heart disease. We also recommend bronchoscopy to assess for additional tracheal pathology and provide an assessment of the degree of tracheomalacia and bronchomalacia. The outcomes of surgical intervention are excellent and most patients have complete resolution of symptoms over a period of time.
Topics: Aorta, Thoracic; Aortic Diseases; Brachiocephalic Trunk; Bronchoscopy; Child; Echocardiography; Esophageal Stenosis; Humans; Infant; Pulmonary Artery; Subclavian Artery; Tomography, X-Ray Computed; Tracheal Stenosis; Vascular Malformations; Vascular Surgical Procedures
PubMed: 27301603
DOI: 10.1053/j.sempedsurg.2016.02.009 -
American Journal of Otolaryngology 2024Vascular rings are often associated with respiratory and swallowing difficulties due to tracheal or esophageal compression. While the results of a vascular ring repair... (Review)
Review
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.
Topics: Child; Humans; Infant; Vascular Ring; Aorta, Thoracic; Retrospective Studies; Trachea; Vascular Surgical Procedures
PubMed: 38101126
DOI: 10.1016/j.amjoto.2023.104147 -
Cardiology in the Young Nov 2021Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this...
BACKGROUND
Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery.
METHODS
Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time.
RESULTS
Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up.
CONCLUSIONS
Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.
PubMed: 34776035
DOI: 10.1017/S1047951121004224 -
BMC Pregnancy and Childbirth May 2023In terms of embryonic origin, vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the...
BACKGROUND
In terms of embryonic origin, vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea or esophagus. Early and accurate diagnosis of a vascular ring is the key to treatment. Prenatal diagnosis mainly relies on fetal echocardiography, but the rate of missed diagnosis and misdiagnosis is still very high, and the prognosis has not been evaluated. The aim of this study was to investigate the accuracy of prenatal diagnosis and to evaluate the prognosis semi-quantitatively according to the shape of the ring and the distance between the vessel and the trachea.
METHODS
From 2019 to 2021, 37,875 fetuses underwent prenatal ultrasound examination in our center. All fetal cardiac examinations were performed using the fetal echocardiography method proposed by the American Institute of Ultrasound in Medicine (AIUM) combined with dynamic sequential cross-sectional observation (SCS). For SCS, the standard abdominal section was taken as the initial section, and the probe was moved cephalically along the long axis of the body until the superior mediastinum had disappeared. If a vascular ring was found, the shape of the ring and the distance of the branch to the airway were observed. The distance relationship with the airway was divided into three grades: I-III; the closer the distance, the lower the grade. The vascular rings were monitored every 4 weeks before birth. All were monitored before surgery or 1 year after birth.
RESULTS
A total of 418 cases of vascular rings were detected. There was no missed diagnoses or misdiagnoses by SCS. The vessels formed different shaped rings according to their origin and route. Grade I, "" and "O" rings have a poor prognosis and are associated with the highest risk of respiratory symptoms.
CONCLUSIONS
SCS can accurately diagnose vascular rings before delivery, evaluate the shape and size of the rings to conduct prenatal monitoring of children until birth, which plays a guiding role in airway compression after birth.
Topics: Female; Humans; Pregnancy; Cross-Sectional Studies; Prenatal Diagnosis; Prognosis; Ultrasonography, Prenatal; Vascular Ring
PubMed: 37131147
DOI: 10.1186/s12884-023-05637-y -
Journal of Paediatrics and Child Health May 2017
Topics: Child, Preschool; Humans; Male; Treatment Outcome; Vascular Ring
PubMed: 28470807
DOI: 10.1111/jpc.13539 -
Journal of the American Society of... Mar 2022The impact of fetal echocardiography on the diagnosis and outcomes of vascular ring has not been well examined. We hypothesized that prenatal detection of vascular ring...
BACKGROUND
The impact of fetal echocardiography on the diagnosis and outcomes of vascular ring has not been well examined. We hypothesized that prenatal detection of vascular ring has improved over time and that prenatal diagnosis of vascular ring is associated with earlier intervention and favorable outcomes.
METHODS
This is a single-center, retrospective study of the evolution and outcomes of prenatal diagnosis of vascular ring from 2000 to 2020. We compared clinical presentation, timing of surgical intervention, and outcomes between the prenatally and postnatally diagnosed cases during the same study period.
RESULTS
A total of 170 patients were included: 50 with prenatal and 120 with postnatal diagnosis of vascular ring. Prenatal diagnoses included 42 patients (84%) with right aortic arch (RAA), aberrant left subclavian artery (ALSCA), and a left-sided ductus arteriosus and eight (16%) patients with double aortic arch (DAA). The postnatal cohort consisted mainly of 90 patients (75%) with DAA and 22 (18%) with RAA-ALSCA. None of the postnatally diagnosed cases had undergone a fetal echocardiogram. Numbers (percentage) of prenatally diagnosed cases of vascular ring compared with the postnatal cases improved from 4/31 (13%), to 10/29 (34%), to 14/25 (56%), and to 22/35 (69%), respectively, during 2000-2005, 2005-10, 2010-15, and 2015-20 (P = .032). Vascular ring was an isolated abnormality in 84% and 85% of the prenatal and postnatal cohorts, respectively. Compared with the prenatal cohort, postnatally diagnosed patients with an isolated vascular ring were more frequently symptomatic (66% vs 48%, P < .03) and underwent cross-sectional imaging (69% vs 44%, P = .009) and surgery more frequently (79% vs 48%, P = .003). Surgery was performed at a later patient age (18 [2-147] months vs 4.8 [0.5-42] months, P = .01) and was more often associated with residual symptoms (27/81 [33%] vs 1/20 [5%], P = .01) in the postnatal cohort than in the prenatal cohort.
CONCLUSIONS
The diagnosis of vascular ring by fetal echocardiography has improved over time. A significantly higher incidence of RAA-ALSCA in the prenatal compared with the postnatal cohort suggests that patients with this form of vascular ring often do not present to medical attention with significant symptoms postnatally. Prenatal diagnosis of vascular ring was associated with a lower incidence of symptoms, less frequent use of cross-sectional imaging, earlier age at surgical intervention, and lower likelihood of residual symptoms.
Topics: Aorta, Thoracic; Female; Humans; Infant; Pregnancy; Prenatal Diagnosis; Retrospective Studies; Ultrasonography, Prenatal; Vascular Ring
PubMed: 34600045
DOI: 10.1016/j.echo.2021.09.010 -
European Journal of Cardio-thoracic... Dec 2021
Topics: Aorta, Thoracic; Aortic Diseases; Esophageal Fistula; Humans; Vascular Fistula; Vascular Ring
PubMed: 34151949
DOI: 10.1093/ejcts/ezab280 -
European Journal of Cardio-thoracic... Nov 2021This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided... (Review)
Review
OBJECTIVES
This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided ligamentum forming a complete vascular ring around the oesophagus and trachea.
METHODS
A systematic database search for appropriate literature was conducted on PubMed/MEDLINE. Articles were considered relevant when providing details on the presentation, diagnosis and surgical treatment of this specific congenital arch anomaly in human beings.
RESULTS
Affected patients present with respiratory and/or oesophageal difficulties due to tracheoesophageal compression. Conservative treatment might be reasonable in asymptomatic or mildly symptomatic cases; however, once moderate-to-severe symptoms develop, surgical intervention is definitely indicated. Surgery is commonly performed through a left thoracotomy or median sternotomy and includes the division of the left ductal ligamentum; if a Kommerell's diverticulum is present that is >1.5 times the diameter of the subclavian artery, then concomitant resection of the large diverticulum and translocation of the aberrant left subclavian artery is also conducted. Postoperative morbidity and mortality are low and are rather related to concomitant intracardiac and extracardiac anomalies than to the procedure itself. In a majority of patients, full resolution of symptoms is seen within months to years from the surgery. Nevertheless, there is also a subset of patients who remain with some tracheobronchial narrowing, sometimes even requiring reintervention during follow-up due to persisting or recurring symptoms.
CONCLUSIONS
Overall, the surgical management of a right aortic arch forming a true vascular ring in infancy, childhood and adulthood seems relatively safe and effective in providing symptomatic relief despite some persistent tracheobronchial and/or oesophageal narrowing in some cases.
Topics: Adult; Aorta, Thoracic; Child; Diverticulum; Heart Defects, Congenital; Humans; Subclavian Artery; Vascular Ring
PubMed: 33970211
DOI: 10.1093/ejcts/ezab225 -
Cardiology in the Young Jul 2022
Topics: Humans; Retrospective Studies; Tracheal Stenosis; Vascular Ring
PubMed: 35581929
DOI: 10.1017/S1047951122000816