|Year : 2020 | Volume
| Issue : 2 | Page : 199-202
Characterization of pediatric aortic arch vascular rings
Ahmed Fareed Almoazen1, Nader Abd EL-Monem Fasseh2, Hosny M.A ELmasry1
1 Pediatrics Department, Faculty of Medicine, Al-Azhar University, Assuit, Egypt
2 Pediatrics Department, Faculty of Medicine, Alexandria University, Egypt
|Date of Submission||16-Sep-2019|
|Date of Decision||07-Apr-2020|
|Date of Acceptance||04-May-2020|
|Date of Web Publication||24-Jul-2020|
MSc Ahmed Fareed Almoazen
Assistant Lecturer of Pediatrics, Faculty of Medicine, Al-Azhar University, Assuit, 71534
Source of Support: None, Conflict of Interest: None
Background Vascular rings (VRs) are rare congenital anomalies of the aortic arch. Along with clinical data, several imaging techniques help to suspect VR. Flexible fiberoptic bronchoscopy (FFB) is also considered the main tool for diagnosis.
Aim The aim of the study is to describe the clinical, radiological, and bronchoscopic features of pediatric aortic VRs.
Patients and methods A total of 61 patients with VR were included in the study on the basis of computed tomography (CT) angiography. The included children were subjected to history taking, clinical examination, chest radiograph, CT chest with angiography, and FFB.
Results This study included 61 children with vascular aortic rings. The median age of diagnosis in this study was 8.5 months (range, 2–23 months). Recurrent chest infection was the most common clinical manifestation (81.96%) followed by monophonic wheeze (60.65%) and stridor (57.37%). Double aortic arch was the most common type (34.4%) followed by innominate artery compression syndrome (18%) and left arch with aberrant right subclavian artery (14.7%). Chest radiography showed the correct diagnosis in seven (11.4%) cases, while CT angiography proved correct in 50 (81.9%) patients and FFB in 56 (91.8%) patients.
Conclusion Prolonged or recurrent respiratory difficulties and/or dysphagia should alert the pediatrician to the possibility of a VR. Preferably, those children should be submitted to bronchoscopy.
Keywords: computed tomography chest with angiography, flexible fiberoptic bronchoscopy, vascular ring
|How to cite this article:|
Almoazen AF, Fasseh NE, ELmasry HM. Characterization of pediatric aortic arch vascular rings. Al-Azhar Assiut Med J 2020;18:199-202
| Introduction|| |
Vascular rings (VRs) are rare congenital anomalies of the aortic arch that result in compression of the tracheobronchial tree and/or esophagus, leading to respiratory and gastrointestinal symptoms . VR received their name because they partially or totally surround the trachea and/or the esophagus and thus might lead to their extrinsic compression and cause morbidity or even mortality in children .
The clinical presentation of VR is variable and nonspecific and depends on the degree and localization of compression exerted on the airways and/or the esophagus. Patients may be asymptomatic or have respiratory symptoms or dysphagia .
Respiratory symptoms are predominating in the initial presentation including stridor, wheezing, rattling, and recurrent pneumonias but dysphagia are less common and often the diagnosis is made only after solid foods are introduced and dysphagia symptoms are more pronounced. Symptoms also include slow breast or bottle-feeding and fatigue with feeding . VR are classified as either complete ring when both the trachea and the esophagus are fully encircled by a vascular anomaly, or incomplete ring without full encirclement of both structures .
Along with clinical data, several imaging techniques help to suspect VR including chest radiography, barium esophagography, computed tomography (CT), MRI, and echocardiography . Flexible fiberoptic bronchoscopy (FFB) is also considered the main tool for diagnosis . Also, bronchoscopy permits direct visualization of airway dynamics, and may help to define the surgical strategy for correcting the VR .
The only definitive treatment is surgery, which was first described by Gross in 1945, clinical outcome is excellent following surgical correction, with resolution of symptoms in the majority of patients with low risk of morbidity and even rare risk of death . This study aims to describe the clinical, radiological, and bronchoscopic features of pediatric aortic VRs.
| Patients and methods|| |
This is a hybrid retrospective study. In the 2-year retrospective phase, clinical records of patients with VRs from the Pediatric Respiratory Unit, Faculty of Medicine, Alexandria University between January 2015 and December 2016 were revised. The study protocol was approved by the local ethics committee and informed consent was obtained from the legal guardians of the included children before interventions.
In all, 61 patients with VR were included in the study on the basis of CT angiography. The included children were subjected to history taking, clinical examination, chest radiography, CT chest with angiography, and FFB (Olympus, Tuttling, Germany; BF-3C160 video bronchoscope with an external diameter of 3.7 mm and working channel of 1.2 mm or Karl Storz, Tuttling, Germany; with an external diameter of 2.8 mm and working channel of 1.2 mm). All children had FFB under general anesthesia with laryngeal mask ventilation with spontaneous respiration.
Data were collected and processed using SPSS 21 (IBM, Chicago, Illinois, USA). Data were entered as numerical or categorical, as appropriate.
| Results|| |
This study included 61 children with vascular aortic rings. The median age of diagnosis in this study was 8.5 months (range, 2–23 months). The clinical data of the studied patients are shown in [Table 1]. Recurrent chest infection was the most common clinical manifestation (81.96%) followed by monophonic wheeze (60.65%) and stridor (57.37%).
The prevalence of different types of VRs is shown in [Table 2]. Double aortic arch was the most common type (34.4%) followed by innominate artery compression syndrome (18%) and left arch with aberrant right subclavian artery (14.7%).
Chest radiography showed the correct diagnosis in seven (11.4%) cases, while CT angiography proved correct in 50 (88.9%) patients and FFB in 56 (91.8%) patients ([Table 3]).
Among the studied patients, 28 (45.9%) presented with associated congenital anomalies. Pulmonary sling was the pathology with most frequent association with congenital anomalies (83.3%) ([Table 4]).
| Discussion|| |
VRs are rare congenital malformations accounting for about 1–3% of cardiovascular malformations . These abnormalities are caused by developmental failure of parts of the paired aortic arches (fourth and fifth arches) during the embryonic life. A genetic origin has not been ruled out .
Our study included 61 cases of VR. By far the most common variant was the double aortic arch and the least common type was the right-sided aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. In a study by Sharma et al. , double aortic arch was also the most common anomaly in their patients. Similar conclusions were reported by other studies ,. However, these data contradict the findings by Shah et al.  where the right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum was the most common vascular anomaly, while in a study by Rogers et al.  innominate artery compression was the most common vascular anomaly.
The median age of diagnosis in this study was 8.5 months reflecting a delayed presentation when compared with other studies. Zheng et al.  reported a median age of 6.9 months, while in the Turner et al.  study, the median age was 4.5 months. This delay in the age of diagnosis in our study can be attributed to the late referral of cases to our center due to lack of high suspicion of VRs by general pediatricians.
Clinical presentations of a VR occur due to encirclement of the ring to trachea and/or esophagus leading to respiratory manifestations or gastrointestinal tract manifestations; the severity of compression determines the severity of symptoms. Common symptoms of a VR include inspiratory stridor, dyspnea, cough, wheezing, dysphagia, and recurrent respiratory tract infections. These symptoms are common during early childhood, but their persistence should alert the general pediatrician to the possibility of a VR . In our population, the most common presenting symptom was recurrent chest infection (81.96%) in line with other studies ,,,. On the contrary, Menaissy et al.  found stridor to be the most common presenting symptom in their series. It is believed that this contradiction may be due to underappreciation of cough as a presenting symptom of VR by both parents and physicians, and this may lead to delay in diagnosis.
In this study, a high proportion of patients with VR were associated with cardiac or noncardiac anomalies. This is similar to the findings by Zheng et al.  where the rate of cardiovascular anomalies in their series was 69.70%.
In this study, chest radiography showed positive findings in ∼11.4% of cases in the form of tracheal compression or right side aortic arch or signs of severe tracheal obstruction (hyperinflation or atelectasis); therefore, chest radiography was of a limited value in agreement with the studies of Turner et al.  and Zheng et al. .High-resolution CT with angiography was a useful tool in the diagnosis of the anatomy and types of VRs. In our study, it detected 81.9% of cases of complete and incomplete vascular airway compression. Results similar to our study were reported by Turner et al. . In comparison, FFB could diagnose 91.8% of cases. This result shows agreement with the Furuya et al.  study, which reported that FFB is considered a key diagnostic tool to identify the degree, location, and extension of airway obstruction with direct visualization of airway and degree of collapse during the respiratory cycle; but in contrast to our study Leonardi et al.  documented that the severity of tracheal narrowing worked out by virtual CT correlated well with bronchoscopy without difference.
| Conclusions|| |
Prolonged or recurrent respiratory difficulties and/or dysphagia should alert the pediatrician to the possibility of a VR. Preferably, those children should be submitted to bronchoscopy.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Loomba RS. Natural history of asymptomatic and unrepaired vascular rings: is watchful waiting a viable option? A new case and review of previously reported cases. Children (Basel) 2016; 3:pii.
Sebening C, Jakob H, Tochtermann U, Lange R, Vahl CF, Bodegom P et al.
Vascular tracheobronchial compression syndromes − experience in surgical treatment and literature review. Thorac Cardiovasc Surg 2000; 48:164–174.
Backer CL, Ilbawi MN, Idriss FS, DeLeon SY. Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg 1989; 97:725–731.
Kussman BD, Geva T, McGowan FX. Cardiovascular causes of airway compression. Paediatr Anaesth. 2004; 14:60–74.
Backer CL, Monge MC, Popescu AR, Eltayeb OM, Rastatter JC, Rigsby CK. Vascular rings. Semin Pediatr Surg. 2016; 25:165–175.
Smith BM, Lu JC, Dorfman AL, Mahani MG, Agarwal PP. Rings and slings revisited. Magn Reson Imaging Clin N Am 2015; 23:127–135.
Leonardi B, Secinaro A, Cutrera R, Albanese S, Trozzi M, Franceschini A et al.
Imaging modalities in children with vascular ring and pulmonary artery sling. Pediatr Pulmonol 2015; 50:781–788.
Woods RK, Sharp RJ, Holcomb GW, 3rd, Snyder CL, Lofland GK, Ashcraft KW, Holder TM. Vascular anomalies and tracheoesophageal compression: a single institution’s 25-year experience. Ann Thorac Surg 2001; 72:434–438; discussion 438-439.
Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg 2005; 129:1339–1347.
Rogers DJ, Cunnane MB, Hartnick CJ. Vascular compression of the airway: establishing a functional diagnostic algorithm. JAMA Otolaryngol Head Neck Surg 2013; 139:586–591.
Bonnard A, Auber F, Fourcade L, Marchac V, Emond S, Revillon Y. Vascular ring abnormalities: a retrospective study of 62 cases. J Pediatr Surg 2003; 38:539–543.
Sharma S, Dobbs JL, Cobanoglu AJAC, Annals T. Surgical correction of vascular ring anomalies. Asian Cardiovasc Thorac Ann 2000; 8:344–346.
Turner A, Gavel G, Coutts J. Vascular rings − presentation, investigation and outcome. Eur J Pediatr 2005; 164:266–270.
Tola H, Ozturk E, Yildiz O, Erek E, Haydin S, Turkvatan A et al.
Assessment of children with vascular ring. Pediatr Int 2017; 59:134–140.
Shah RK, Mora BN, Bacha E, Sena LM, Buonomo C, Del Nido P, Rahbar R. The presentation and management of vascular rings: an otolaryngology perspective. Int J Pediatr Otorhinolaryngol 2007; 71:57–62.
Zheng G, Wu X, Tang LJHKJOP. Respiratory symptoms due to vascular ring in children. Hong Kong J Paediatr 2016; 21:14–21.
Humphrey C, Duncan K, Fletcher S. Decade of experience with vascular rings at a single institution. Pediatrics 2006; 117:e903–e908.
Gaafar AH, El-Noueam KI. Bronchoscopy versus multi-detector computed tomography in the diagnosis of congenital vascular ring. J Laryngol Otol 2011; 125:301–308.
Xu R, Shi K, Yang ZG, Diao KY, Zhao Q, Xu HY, Guo YK. Quantified evaluation of tracheal compression in pediatric complex congenital vascular ring by computed tomography. Sci Rep 2018; 8:11183.
Menaissy YM, Elgamal MAF, Amin S, Zaki AF. Vascular rings and slings: a challenging diagnostic and therapeutic rare disease entity. J Egypt Soc Cardio-Thorac Surg 2017; 25:349–355.
Furuya ME, Vargas MH, Ramírez‐Figueroa JL, Félix‐Heredia JL, González‐Ortíz B, Rodríguez‐Hernández L, Vera‐Canelo JMJP. Endoscopy for the initial suspicion of vascular rings in tracheoesophageal compressions: correlation with surgical findings. Pediatr Pulmonol 2010; 45:560–565.
[Table 1], [Table 2], [Table 3], [Table 4]