Background 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...Background 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. Methods 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. Results 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. Conclusions 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. 展开更多
目的探讨超声心动图对单侧肺动脉起源异常的诊断价值。方法回顾性分析1999年1月至2015年5月在复旦大学附属儿科医院进行超声心动图检查,诊断或疑似诊断为单侧肺动脉起源异常(AOPA)患儿的超声心动图资料。超声心动图结果与多排螺旋CT(MS...目的探讨超声心动图对单侧肺动脉起源异常的诊断价值。方法回顾性分析1999年1月至2015年5月在复旦大学附属儿科医院进行超声心动图检查,诊断或疑似诊断为单侧肺动脉起源异常(AOPA)患儿的超声心动图资料。超声心动图结果与多排螺旋CT(MSCT)和术中诊断结果比较,分析超声心动图对单侧肺动脉异常起源的诊断及随访价值。结果超声心动图共诊断42例单侧肺动脉起源异常,其中确诊36例,疑似诊断6例,男性24例,女性18例,平均月龄(9.76±27.00)个月。36例确诊患儿中,4例患儿首次本院超声心动图误诊为原发性肺动脉高压,再次超声心动图检查确诊为单侧肺动脉起源异常;4例患儿外院误诊或漏诊。6例疑似诊断患儿中,通过MSCT检查,5例明确诊断为单侧肺动脉起源异常,1例为右侧肺动脉缺如。超声心动图直接征象为主肺动脉分叉结构消失,发现一侧肺动脉由主动脉发出;间接征象表现为重度肺动脉高压。诊断准确率为85.7%(36/42),近端型诊断准确率97.2%,远端型诊断准确率2%。20例患儿进行手术治疗后,超声心动图随访,吻合口平均流速为(2.36±0.62)m/s,平均压差为(23±13)mm Hg(1 mm Hg=0.133 k Pa)。结论超声心动图是术前诊断单侧肺动脉起源异常的首选有效方法,可明确诊断近端型,对远端型可提示诊断;同时也是术后评价肺动脉吻合口及肺动脉压力简便、无创的最佳方法。展开更多
Background:The presence of a single umbilical artery(SUA)is a fetal soft marker of congenital abnormalities.Among the most common related malformations,there are cardiological,nephrourological and digestive anomalies,...Background:The presence of a single umbilical artery(SUA)is a fetal soft marker of congenital abnormalities.Among the most common related malformations,there are cardiological,nephrourological and digestive anomalies,most of which are considered to have a vascular etiology.There is an association between increased incidence of intrauterine growth retardation and adverse perinatal indicators,but whether this association is due to related anomalies or isolated SUA(SUA)is controvisal.Methods:We reviewed 96 cases of iSUA and non-isolated SUA(niSUA),diagnosed in a period of two years in a referral hospital for high-risk pregnancies.Data on prenatal explorations,including fetal ultrasonography and karyotyping,were obtained.niSUA was diagnosed when no malformations were found prenatally or in postnatal evaluation.Results:Sixty-six newborns(68.8%)had no other anomalies and 30(31.3%)presented with a variety of malformations including heart diseases,urophaties,digestive,nervous and musculoskeletal disorders,genetic abnormalities and complex malformations.Cardiological and nephrourological abnormalities were found to be the most frequent association with a SUA(both in 23.8%of malformed SUA newborns).Intrauterine growth restriction was not higher in iSUA newborns than in a normal population.Utrasound allowed optimal prenatal diagnosis in most cases.Conclusions:The prognosis of the fetus with a SUA is determined by the presence of other malformations observed by an expert sonographer.If no other findings are made,only a routine physical examination should be performed in newborns,but no other complementary examinations are required.展开更多
文摘Background 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. Methods 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. Results 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. Conclusions 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.
文摘目的探讨超声心动图对单侧肺动脉起源异常的诊断价值。方法回顾性分析1999年1月至2015年5月在复旦大学附属儿科医院进行超声心动图检查,诊断或疑似诊断为单侧肺动脉起源异常(AOPA)患儿的超声心动图资料。超声心动图结果与多排螺旋CT(MSCT)和术中诊断结果比较,分析超声心动图对单侧肺动脉异常起源的诊断及随访价值。结果超声心动图共诊断42例单侧肺动脉起源异常,其中确诊36例,疑似诊断6例,男性24例,女性18例,平均月龄(9.76±27.00)个月。36例确诊患儿中,4例患儿首次本院超声心动图误诊为原发性肺动脉高压,再次超声心动图检查确诊为单侧肺动脉起源异常;4例患儿外院误诊或漏诊。6例疑似诊断患儿中,通过MSCT检查,5例明确诊断为单侧肺动脉起源异常,1例为右侧肺动脉缺如。超声心动图直接征象为主肺动脉分叉结构消失,发现一侧肺动脉由主动脉发出;间接征象表现为重度肺动脉高压。诊断准确率为85.7%(36/42),近端型诊断准确率97.2%,远端型诊断准确率2%。20例患儿进行手术治疗后,超声心动图随访,吻合口平均流速为(2.36±0.62)m/s,平均压差为(23±13)mm Hg(1 mm Hg=0.133 k Pa)。结论超声心动图是术前诊断单侧肺动脉起源异常的首选有效方法,可明确诊断近端型,对远端型可提示诊断;同时也是术后评价肺动脉吻合口及肺动脉压力简便、无创的最佳方法。
文摘Background:The presence of a single umbilical artery(SUA)is a fetal soft marker of congenital abnormalities.Among the most common related malformations,there are cardiological,nephrourological and digestive anomalies,most of which are considered to have a vascular etiology.There is an association between increased incidence of intrauterine growth retardation and adverse perinatal indicators,but whether this association is due to related anomalies or isolated SUA(SUA)is controvisal.Methods:We reviewed 96 cases of iSUA and non-isolated SUA(niSUA),diagnosed in a period of two years in a referral hospital for high-risk pregnancies.Data on prenatal explorations,including fetal ultrasonography and karyotyping,were obtained.niSUA was diagnosed when no malformations were found prenatally or in postnatal evaluation.Results:Sixty-six newborns(68.8%)had no other anomalies and 30(31.3%)presented with a variety of malformations including heart diseases,urophaties,digestive,nervous and musculoskeletal disorders,genetic abnormalities and complex malformations.Cardiological and nephrourological abnormalities were found to be the most frequent association with a SUA(both in 23.8%of malformed SUA newborns).Intrauterine growth restriction was not higher in iSUA newborns than in a normal population.Utrasound allowed optimal prenatal diagnosis in most cases.Conclusions:The prognosis of the fetus with a SUA is determined by the presence of other malformations observed by an expert sonographer.If no other findings are made,only a routine physical examination should be performed in newborns,but no other complementary examinations are required.
