The Clinical Characteristics Of Vascular Ring With Tracheal Stenosis

BackgroundPediatric tracheal stenosis (TS) may be acquired or congenital, and most of them are congenital tracheal stenosis (CTS). CTS is frequently associated with other anomalies. The congenital tracheal segmental stenosis is accompanied by cardiovascular anomalies, especially vascular rings (VR). VR is a group of rare congenital anomalies that primarily result from an embryological derangement of the paired aortic arches or branching pulmonary arteries. It is a relatively rare disease of the cardiovascular system in children. The clinical presentation of VR with TS is usually involved respiratory symptoms. Misdiagnosis and missed diagnosis have been reported frequently. With the improvement of the inspection techniques, the diagnosis of VR with TS is obviously increased. As uncharacteristic clinical symptoms and ignorance of clinical doctors, missed diagnosis and misdiagnosis are still very common. There were few studies available on the clinical characteristics of VR with TS.ObjectiveThe objective of this study was to provide the full basis for early diagnosis of VR with TS by analyzing the clinical presentation, imaging characteristics, comparison and correlation of TS and VR.Data and MethodsThe research objects were the119patients who admitted with TS and/or VR with respiratory symptoms in Children’s Hospital of Zhejiang University from January2010to July2014. The patients include114patients with TS and5patients with VR without TS. They were divided into two groups according to whether with VR:(1) TS group:86patients of TS without VR. They were divided into two groups according to whether have a definite cause: Idiopathic TS group:75patients of TS without defined cause; Secondary TS group:11patients of TS secondary to other causes.(2) VR group:33patients of VR. They were divided into two groups according to whether with TS: VR with TS group:28patients of VR with TS; VR without TS group:5patients of VR without TS. The general data, clinical and imaging characteristics, and other detailed records were reviewed. The dates between idiopathic TS group and VR with TS group were analyzed.Result(1) Both TS and VR with TS groups, cough (93.86%and89.28%) and wheeze (71.05%and75.00%) were the most common symptoms. The comparison of ages at onset and diagnosis of VR with TS group and idiopathic TS group indicates that the age of former is younger than that of the latter (P=0.000); The VR with TS group had higher incidence of dyspnea, cyanosis and three concave sign than these of TS group.(all P<0.05).(2) In idiopathic TS group, TS was found in61of63patients (61/63,96.82%) in whom MDCT with3D of airway was performed. Bronchoscopy was performed in51patients and TS was found in47patients (47/51,92.16%). There is no statistical differences of the positive rate between the above two examination methods (P<0.05). In VR group, MDCT with3D of vascular was performed in23patients; MRI and cardiac catheterization angiography were performed in7patients respectively, with diagnosis rate of100%. Among75cases of idiopathic TS group, airway stenosis was mainly in the left main bronchus (36/75,48%) while airway stenosis was mainly distributed in the low1/3segments of trachea (21/28,75%) in28cases of VR with TS. (3) The other cardiovascular anomalies were found in7patients (9.33%) in the idiopathic TS group and19(67.86%) in VR with TS group with significant difference (P<0.05).Conclusion(1) The clinical symptoms of VR with TS were presented early in life and severe. The patients have not only cough, wheezing, shortness of breath, but also usually present dyspnea, cyanosis and three concave sign at the same time.(2) VR with TS has higher rate of other cardiovascular malformations, and cardiovascular malformations is usually more complicated;(3) The preferred choice is MDCT when the patient has suspected of TS according to the clinical characteristics. The bronchoscopy can be performed for the accurate diagnosis of TS and the echocardiography can be performed to find the cardiovascular abnormality. The MDCT with3D of airway and vascular remodeling or MRI should be taken earlier to make accurate diagnosis when the patient is suspected of VR with TS at the same time.