Ma Gui-qin, Li Zhong-zhi, Li Xiao-feng, Peng Yun, DU Zhong-dong, Jin Lan-zhong, Wang Fang-yun, Wei Hai-yan, Zheng Lin, Zhang Xin
Department of Cardiology, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
Chin Med J (Engl). 2007 Aug 20;120(16):1408-12.
Congenital vascular rings may often cause unexplained respiratory symptoms in infants and young children. Their diagnosis and treatment are often delayed. Few studies of vascular rings have been reported in China. The aim of this study was to describe the clinical presentation, diagnosis and surgical management of infants and children with congenital vascular rings.
Clinical histories, physical examinations, investigations, image studies and surgical interventions were retrospectively evaluated in 7 children (age range: 2 months-4 years, mean 7 months) with congenital vascular rings. Chest radiography was performed in all patients. Echocardiography and computed tomography (CT) with 3-dimensional (3D) reconstructions were performed in 6 patients. Esophagography, cardiac catheterization and angiography, and bronchoscopy were performed in 1, 1 and 4 children, respectively.
Six of the 7 patients had respiratory symptoms, including recurrent cough, stridor and wheeze. Age at onset of symptoms ranged from 1 month to 11 months. Chest X-ray showed nothing important on the vascular rings, besides bronchitis and pneumonia. Contrast-enhanced CT diagnosed vascular rings in 6 patients. Four patients had double aortic arches, two had balanced arches and two were right arch dominant. One patient had a right aortic arch with left ligament and 1 patient had a pulmonary artery sling. Echocardiography failed to diagnose vascular rings in 2 patients. The esophagogram of 1 patient showed esophageal compression. Bronchoscopy of 4 patients showed compression of the distal trachea. Five of the 7 patients underwent surgical division of the vascular rings. Surgical observation confirmed the CT findings in each patient.
Patients, especially infants or young children, with recurrent respiratory symptoms such as chronic cough, stridor and wheeze, should be examined for the possible presence of congenital vascular rings. Contrast-enhanced CT can clearly show the anatomy of vascular rings. As a noninvasive technique, echocardiography is helpful for diagnosis. Early surgical management in symptomatic patients is effective.
先天性血管环常可导致婴幼儿出现不明原因的呼吸道症状，其诊断和治疗常被延误。中国关于血管环的研究报道较少。本研究旨在描述先天性血管环患儿的临床表现、诊断及外科治疗。
对7例（年龄范围：2个月至4岁，平均7个月）先天性血管环患儿的临床病史、体格检查、各项检查、影像学检查及外科干预措施进行回顾性评估。所有患者均行胸部X线检查。6例患者行超声心动图及三维（3D）重建计算机断层扫描（CT）检查。分别有1例、1例和4例患儿行食管造影、心导管检查及血管造影和支气管镜检查。
7例患者中有6例有呼吸道症状，包括反复咳嗽、喘鸣和喘息。症状出现的年龄为1个月至11个月。胸部X线除显示支气管炎和肺炎外，未发现血管环的重要异常。增强CT诊断出6例血管环。4例为双主动脉弓，2例为平衡弓，2例以右弓为主。1例为右位主动脉弓伴左韧带，1例为肺动脉吊带。2例患者超声心动图未能诊断出血管环。1例患者的食管造影显示食管受压。4例患者的支气管镜检查显示气管远端受压。7例患者中有5例行血管环手术切断术。手术观察证实了每位患者的CT检查结果。
对于有反复呼吸道症状如慢性咳嗽、喘鸣和喘息的患者，尤其是婴幼儿，应检查是否存在先天性血管环。增强CT能清晰显示血管环的解剖结构。超声心动图作为一种无创技术，有助于诊断。有症状患者早期手术治疗有效。
