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Respiratory Symptoms Due to Vascular Ring in Children

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Respiratory Symptoms Due to Vascular Ring in Children HK J Paediatr (new series) 2016;21:14-21 Respiratory Symptoms due to Vascular Ring in Children GM ZHENG, XH WU, LF TANG Abstract Aim: To highlight the clinical features, signs and diagnosis of vascular ring. Methods: A total of 33 patients (22 boys and 11 girls) diagnosed from January 2010 to July 2014 were enrolled. The types of vascular ring, basic demographic data, clinical symptoms, imaging and associated other malformations were analysed. Results: Among these patients, pulmonary artery sling was found in 17, right aortic arch in 9, double aortic arch in 5, and aberrant right subclavian artery in 3 (one with pulmonary artery sling). The median age (min-max) at onset and diagnosis were 1.8 (0.0-8.4) and 6.9 (0.1-149.0) months, respectively. Patients with pulmonary artery sling had higher incidence of stridor and dyspnea than patients with right aortic arch (P=0.028 and P=0.036, respectively). All patients combined with other anomalies, including airway anomalies in 31 (93.94%) and cardiovascular anomalies in 23 (69.70%). The diagnostic accuracy of computed tomography with contrast/angiography, magnetic resonance imaging and cardiac catheterisation angiography were high (up to 100%) and lower with X-ray and endoscopy. Conclusion: Vascular ring should be considered in infants and young children with recurrent or chronic respiratory complaints and other anomalies. Computed tomography with contrast/angiography or magnetic resonance imaging should be performed to confirm the diagnosis. Key words Children; Diagnosis; Pulmonary; Vascular ring Introduction ring" was published by Gross in the New England Journal of Medicine in 1945.3 It was classified into double aortic Vascular ring is a set of aortic arch malformations that arch (DAA), right aortic arch (RAA) with left ligamentum, are the result of abnormal development of the embryologic pulmonary artery sling (PAS), and innominate artery aortic arches. The abnormal development of the fourth and compression by the International Congenital Heart Surgery sixth paired aortic arches forms anatomically complete or Nomenclature and Database Committee.4 The vascular ring incomplete vascular ring around the trachea, esophagus, can cause various respiratory and/or esophageal symptoms or both during fetal life.1,2 The first report named "vascular especially in infants and young children because of compression of the trachea and/or esophagus. However, a few patients with vascular ring do not present symptoms until adolescence or adulthood, or even entire life.5 The Department of Pulmonology, The Children's Hospital of common clinical features were respiratory symptoms, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China including cough, stridor, wheezing, recurrent respiratory tract infections, dyspnoea, respiratory distress, and even GM Zheng MD 6-10 XH WU Postgraduate student respiratory arrest. The oesophageal symptoms have LF TANG MD, PhD occurred in a few cases, including dysphagia, slow feeding, reflux, vomiting, choking and failure to thrive.7,8,11 These Correspondence to: Prof. LF TANG various clinical manifestations mainly depend on the 5 Received December 10, 2014 different types and severity of vascular rings. Other Zheng et al 15 symptoms associated with other cardiovascular anomalies and age among children with different types of vascular ring and tracheal anomalies have also been reported.1,5,8,12 As were not significant, as showed in Table 1. most of these patients presented with respiratory symptoms, In this series, all the patients were admitted with the but not cardiovascular symptoms,1 it is difficult to diagnose respiratory symptoms. Cough was the most common in infants and young children if the medical staff do not pay symptom, which was complained in 28 patients. Moreover, attention or are unfamiliar with this rare condition. recurrent cough was complained in 23 patients and chronic Misdiagnosis and missed diagnosis have been reported cough in 4. Stridor and wheezing were the other common frequently. symptoms and noted in 24 patients, respectively. Serious In this study, we analysed retrospectively the clinical symptoms including dyspnoea and cyanosis were also features, imaging and associated anomalies of 33 paediatric common with the same incidence of 39.39%. All patients patients with respiratory symptoms due to vascular rings in with DAA presented with cough, stridor and wheezing. Two order to highlight the clinical features and diagnosis of this patients had a history of "asthma" without significant rare condition. improvement after receiving the standardised treatment of asthma. Two patients (one PAS and one DAA) were admitted to hospital because of "laryngitis" with acute Patients and Methods laryngeal obstruction. Two patients with RAA and aberrant left subclavian artery had additional complaint of dysphagia Patients as well (Table 1). A total of 33 patients with vascular ring diagnosed in While analysing clinical features among the different our unit from January 2010 to July 2014 were types of vascular ring, we noted that there was significant retrospectively reviewed and analysed. They were 22 boys difference of stridor among the four types (P=0.001), and and 11 girls aged from 3 days to 12 years with a median patients with PAS had a significantly higher rate of stridor (range) age of 6.9 (0.1-149.0) months at the time of than that of patients with RAA (88.24% vs. 44.44%, diagnosis. Their basic demographic data, type of P=0.028). Also, there was a marginal difference of abnormalities, clinical symptoms and signs, imaging, dyspnoea among the four types (P=0.077), and dyspnoea in endoscopic findings, and associated other malformations patients with PAS was more common than that in the patients were recorded for further analysis. with RAA (58.82% vs. 11.11%, P=0.036). No significant Consent was obtained from the parents and the study difference of cough, recurrent cough, chronic cough, or was proved by the Ethical Committee of the Children's cyanosis was found among the four different types of Hospital of Zhejiang University School of Medicine. vascular ring, as shown in Table 1. Among these patients, other malformations, mainly Statistical Analysis airway or cardiovascular anomalies, were found in all Statistical analyses were conducted using SPSS software patients. Airway anomalies were presented in 31 patients (version 11.5). The Fischer's Exact test was used to measure (93.94%), including tracheal or main bronchial stenosis in enumeration data among the groups. The data with skewed 28 (84.85%), tracheal bronchus in 4, laryngomalacia in 3, distributions were presented as median (min-max) analysed tracheomalacia in 2, bridging bronchus in 1 and bronchus by nonparametric test (Kruskal-Wallis test). Significant atresia in one. The tracheal stenosis in 28 patients occurred differences were regarded if a P value≤0.05. almost around the tracheal bifurcation level (22/28, 78.57%). The malformations of lung included circumscribed emphysema in 4, pulmonary hypoplasia in Results 3, pulmonary sequestration and pulmonary cysts in one, respectively. The concurrent rate of different type of airway Among these 33 patients, the ratio of male to female anomalies had no significant difference among the four was 2 to 1. The median age at clinical feature onset was 1.8 types, except for the pulmonary hypoplasia (P=0.022). It (0.0-8.4) months. PAS was predominant type (Figure 1), was notable that tracheal bronchus, tracheomalacia and which was found in 17 patients, followed by RAA with bronchus atresia were found in PAS patients only, but not aberrant left subclavian artery in 9, DAA in 5 (Figures 2a- found in others, as shown in Table 2. 2b), and aberrant right subclavian artery (ARSA) in 3 (one Other cardiovascular anomalies presented in 23 patients with PAS as well) (Figures 2c-2e). The differences of gender (69.70%), which mainly included the ventricular septal 16 Vascular Ring in Children Table 1 The basic demographic data and respiratory symptoms according to type of vascular ring Total PAS RAA DAA ARSA χ2 P value Multiple comparison† Number (%) 33 17 (51.52) 9 (27.27) 5 (15.15) 3 (9.09) Male/female 22/11 14/3 6/3 2/3 1/2 5.068 0.128 N Age at onset (M) 1.8 (0.0-8.4) 1.6 (0.0-8.4) 0.9 (0.0-8.0) 3.1 (0.1-7.8) 1.0 (0.0-7.9) 1.443 0.695 N Age at diagnosis (M) 6.9 (0.1-149.0) 5.6 (0.7-56.0) 9.0 (0.7-149.0) 8.1 (0.1-19.0) 8.0 (2.7-61.0) 2.268 0.519 N Cough (%) 28 (84.85) 13 (76.47) 8 (88.89) 5 (100.00) 2 (66.67) 2.143 0.584 N Recurrent cough (%) 23 (69.70) 11 (64.71) 6 (66.67) 4 (80.00) 2 (66.67) 0.651 1.000 N Chronic cough (%) 4 (12.12) 4 (23.53) 0 0 0 3.009 0.412 N Stridor (%) 24 (72.73) 15 (88.24) 4 (44.44) 5 (100.00) 0 12.876 0.001 PAS vs. RAA (0.028) Wheeze (%) 24 (72.73) 13 (76.47) 5 (55.56) 5 (100.00) 1 (33.33) 4.954 0.154 N Dyspnoea (%) 13 (39.39) 10 (58.82) 1 (11.11) 1 (20.00) 1 (33.33) 6.399 0.077 PAS vs. RAA (0.036) Cyanosis (%) 13 (39.39) 9 (52.94) 2 (22.22) 1 (20.00) 1 (33.33) 3.121 0.377 N M, month; PAS, pulmonary artery sling; RAA, right aortic arch; DAA, double aortic arch; ARSA, aberrant right subclavian artery; †P<0.05. Table 2 The associated malformations according to type of vascular ring Total (%) PAS RAA DAA ARSA χ2 P value Multiple comparison† Number 33 17 9 5 3 Airway Stenosis 28 (84.85) 13 (76.47) 8 (88.89) 5 (100.00) 2 (66.67) 2.143 0.593 N Tracheal bronchus 4 (12.12) 4 (23.53) 0 0 0 3.009 0.415 N Laryngomalacia 3 (9.09) 0 2 (22.22) 1 (20.00) 0 4.842 0.143 N Tracheomalacia 2 (6.06) 2 (11.76) 0 0 0 1.790 0.729 N Bridging bronchus 1(3.03) 1(5.88) 0 0 0 2.329 1.000 N Bronchus atresia 1 (3.03) 1 (5.88) 0 0 0 2.329 1.000 N Emphysema 4 (12.12) 3 (17.64) 0 0 1 (33.33) 3.318 0.302 N Pulmonary hypoplasia 3 (9.09) 1 (5.88) 0 0 2 (66.67) 7.365 0.022 N Others 2 (6.06) 1 (5.88) 1 (11.11) 0 0 1.554 1.000 N Total (%) 31 (93.94) 17 (100.00) 8 (88.89) 5 (100.00) 2 (66.67) 5.024 0.099 N Cardiovascular VSD 13 (39.39) 6 (35.29) 4 (44.44) 1 (20.00) 2 (66.67) 1.965 0.682 N ASD 12 (36.36) 10 (58.82) 1 (11.11) 0 1 (33.33) 8.607 0.021 PAS vs.
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