Clinical and imaging features of pulmonary artery sling in infants without significant hemodynamic changes

Background  Pulmonary artery sling (PAS) is a rare congenital heart anomaly and may cause unexplained respiratory symptoms in infants. Since the non-specific respiratory symptoms of PAS may lead to misdiagnosis, the aim of this study was to clarify the clinical and imaging features of this disease for timely diagnosis and treatment.

Methods  Clinical histories, physical examinations and imaging studies were retrospectively evaluated in nine infants with PAS. Chest X-ray, echocardiography and contrast-enhanced computed tomography (CT) with 3-dimensional reconstructions were performed in all patients and three of them received surgical treatment.

Results  Nine cases included six males and three females with a mean age of (4.3±2.8) months ranging from 2 to 11 months old. All patients had respiratory symptoms including recurrent cough, stridor and wheezing. The onset of symptoms was within 3 months in all cases and three children had symptoms only a few days after birth. The chest X-ray showed pneumonia in all cases. Contrast-enhanced CT showed the tracheal compression at different lengths in every case. The echocardiograph findings of PAS were anomalous origins of the left pulmonary artery from the posterior aspect of the right pulmonary artery. Of the 9 cases, 8 cases were diagnosed correctly by echocardiography. Of the complicated abnormalities, there were one with secundum atrial septal defect, one with patent foramen ovale and three with persistent left superior vena cava. None of them were complicated with significant blood dynamic changes.

Conclusions  Infants with recurrent respiratory symptoms such as chronic cough, stridor and wheezing, should be examined for the possible presence of congenital pulmonary artery sling. As a noninvasive technique, echocardiography is very helpful and should be the first-choice modality for the diagnosis of pulmonary artery sling. Contrast-enhanced CT, clearly demonstrating the anatomy of pulmonary artery sling and the position and extent of trachea compression, is necessary for the final diagnosis and pre-operation evaluation.

     

儿童气管性支气管25例临床分析

目的：通过探讨儿童气管性支气管的临床特征及影像学特点,加深对该病的认识。方法回顾性分析经胸CT加气道三维重建而确诊的25例患儿的临床资料。结果25例患儿均为右侧气管性支气管,移位型20例,额外多支型5例,合并脑瘫及肺纤维化1例,临床予保守治疗后症状缓解。结论临床上多次同一部位肺炎及肺不张,持续咳嗽喘息的患儿,经对症治疗效果不佳时,应注意有否先天气管畸形,需行胸CT加气道三维重建以明确诊断。     

Surgical treatment for vascular anomalies and tracheoesophageal compression

Background  Vascular rings are uncommon anomalies in which preferred strategies for diagnosis and management may vary among institutions. In this study, we reported our approach and a review of our 5-year experience.

Methods  From May 2006 to April 2011, 45 children (31 boys) with vascular rings underwent surgical repair at Beijing Children’s Hospital. Nineteen patients (26%) had associated heart anomalies.

Results  There were two hospital deaths. At follow-up, 11 patients still had intermittent respiratory symptoms, but these symptoms had no effect on growth or physical activities. No patients required reoperation.

Conclusions  The rates of misdiagnosis and missed diagnosis of vascular rings are higher than those of other congenital heart diseases. A high index of clinical suspicion coupled with the use of computed tomography enables early diagnosis. Surgical repair can be performed successfully, although a number of patients will have persistent symptoms.

     

螺旋CT和心脏彩超对新生儿先天性血管环诊断价值分析

目的探讨螺旋CT和心脏彩超对新生儿先天性血管环的诊断价值。方法回顾性分析2006年1月-2012年1月我院收治的40例患有先天性血管环的新生儿的临床资料,所有患儿均接受64层螺旋CT(Multi-slicespiral CT,MSCT)、心脏彩超(transthoracicechocardiogra phy,TTE)检查,所有患者均经手术证实为先天性血管环。将MSCT、TTE的诊断结果与手术诊断结果进行比较,观察并比较MSCT和TTE检查对先天性血管环的确诊率以及对合并心脏畸形、气管狭窄、食管狭窄的确诊情况。结果 TTE和MSCT对先天性血管环的确诊率分别为65.0%、92.5%,TTE的确诊率明显低于MSCT和手术证实结果,比较存在较大的差异(χ2=9.04、16.97,P0.05);而MSCT检查对先天性血管环的确诊率和手术证实结果相近(P0.05)。MSCT对先天性血管环合并心脏畸形、气管狭窄、食管狭窄的诊断准确率分别为40.0%、75.0%、17.5%,明显高于TTE检查的诊断准确率(χ2=4.92、4.38、5.00,P0.05),并与手术证实结果较为相近(P0.05)。结论 MSCT对先天性血管环的诊断准确率较高,与手术诊断基本相符,既能清晰显示血管环的组成,还能明确出血管环与毗邻结构的关系,可联合TTE检查作为先天性血管环的有效诊断手段,值得在临床上推广应用。     

Refractory congestive heart failure after ribavirin in infants with heart disease and respiratory syncytial virus

Although treatment with ribavirin has been known to be associated with a decreased mortality in infants with congenital heart disease (CHD) who have respiratory syncytial virus (RSV), few data are available regarding morbidity. We reviewed records of 10 consecutively hospitalized infants with CHD during a recent RSV epidemic. Despite the presence of left-to-right shunt in each patient, symptoms of RSV were respiratory at presentation. After ribavirin, decreased respiratory symptoms were found in 8 infants but in 2 assisted ventilation were required 1 and 3 days after admission. Congestive heart failure worsened in 8 patients, 6 of whom had improved respiratory status after ribavirin. Of the 8 patients with worse CHF (pulmonary edema), 3 responded to medical management but 5 were refractory and 4 required surgical repair of CHD. One patient died of pulmonary hemorrhage. Medically refractory CHF may develop in infants with CHD who become infected with RSV and are treated with ribavirin. Further studies are needed to determine whether the pulmonary edema is caused by RSV, ribavirin, or combination of effects on pulmonary capillary function or some other unrecognized mechanism.     

Respiratory symptoms in preterm infants: burden of disease in the first year of life

Objective

While respiratory symptoms in the first year of life are relatively well described for term infants, data for preterm infants are scarce. We aimed to describe the burden of respiratory disease in a group of preterm infants with and without bronchopulmonary dysplasia (BPD) and to assess the association of respiratory symptoms with perinatal, genetic and environmental risk factors.

Methods

Single centre birth cohort study: prospective recording of perinatal risk factors and retrospective assessment of respiratory symptoms during the first year of life by standardised questionnaires. Main outcome measures: Cough and wheeze (common symptoms), re-hospitalisation and need for inhalation therapy (severe outcomes). Patients: 126 preterms (median gestational age 28.7 weeks; 78 with, 48 without BPD) hospitalised at the University Children''s Hospital of Bern, Switzerland 1999-2006.

Results

Cough occurred in 80%, wheeze in 44%, rehospitalisation in 25% and long term inhalation therapy in wheezers in 13% of the preterm infants. Using logistic regression, the main risk factor for common symptoms was frequent contact with other children. Severe outcomes were associated with maximal peak inspiratory pressure, arterial cord blood pH, APGAR and CRIB-Score.

Conclusions

Cough in preterm infants is as common as in term infants, whereas wheeze, inhalation therapy and re-hospitalisations occur more often. Severe outcomes are associated with perinatal risk factors. Preterm infants who did not qualify for BPD according to latest guidelines also showed a significant burden of respiratory disease in the first year of life.     

丙种球白联合红霉素治疗毛细支气管炎临床观察

目的 观察丙种球蛋白（IVIG）联合红霉素（EM）治疗毛细支气管炎的疗效。方法 72例毛细支气管炎患儿随机分为对照组36例给予抗感染、抗病毒及吸氧等常规治疗；治疗组36例在常规治疗基础上加用IVIG和EM，观察治疗前后患儿咳嗽、喘憋、呼吸困难、喘鸣音等症状和体征消失时间、住院天数和疗效。结果 治疗组患儿咳嗽、喘憋、喘鸣音等消失天数、住院天数明显短于对照组（P〈0．001），治疗组有效率（97．2％）明显高于对照组（P〈0．001）。结论 用IVIG和EM可快速缓解患儿症状、体征，缩短住院时间，提高治愈率。     

Anomalous origin of left pulmonary artery branch from the aorta with Fallot＇s tetralogy： one case report

We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot＇s tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin in the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged. We present the results of a literature review of the incidence, physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.     

儿童先天性肺囊腺瘤样畸形的影像诊断

目的：描述儿童先天性肺囊腺瘤样畸形的影像特点,提高对该病的认识。方法：对我院1998～2010年14例儿童先天性肺囊腺瘤样畸形患者的临床资料进行分析。结果：经胸片检查发现,见单个或多个含气大囊7例,见多发蜂窝样小囊5例,肿块样改变2例,其中合并肺纵隔疝9例,出现患侧肺气肿10例;经CT检查发现,右肺受累6例,左肺受累8例,其中表现为巨大囊腔5例,类圆形薄壁囊腔3例,蜂窝样小囊5例,含有少量液体3例;病理检查,囊腔壁有异常增生6例。结论：影像学检查是诊断儿童先天性肺囊腺瘤样畸形的可靠方法。     

先天性肺囊性病的诊断和手术治疗

目的总结儿童先天性肺囊性病的诊治经验,探讨手术时机和方式。方法1993年1月至2006年5月,手术治疗52例先天性肺囊性病患儿,男26例,女26例,平均年龄3．5岁,包括新生儿16例。术前诊断主要依靠X线胸片和胸部CT。病种包括：先天性肺囊肿37例,先天性肺大叶气肿6例,肺隔离症10例。肺囊肿行肺叶切除27例、囊肿摘除8例、肺楔形切除2例;肺大叶气肿中5例有呼吸窘迫,均行肺叶切除术。肺隔离症中包括叶内型3例,叶外型7例,均手术切除。结果无手术死亡,近期主要并发症包括反复气胸1例、少量胸腔积液1例、肺炎2例。所有病人均痊愈出院。随访病人中1例肺囊肿复发,术后3年再次行左下肺叶切除术。其余病人均恢复良好,肺功能正常。结论小儿先天性肺囊性病应早诊断、早手术,对有明显压迫症状和呼吸窘迫者应急诊手术。手术是先天性肺囊性病的主要治疗方法,肺叶切除是标准术式,术后肺功能均可恢复。     

Prevalence and spectrum of asthma in childhood

All the 7 year old schoolchildren in North Tyneside were screened for wheeze with a questionnaire followed by selective clinical assessment: 9.3% of the children had had episodic wheeze within the past year and all those followed up subsequently responded to one or more of the drugs used for asthma. A further 1.8% had had similar symptoms since starting school, though they had not wheezed in the past year. Frequently of symptoms in the 11% of children with features of asthma varied widely and correlated with bronchial reactivity on histamine challenge, but it was not possible to separate children with frequent wheeze from asymptomatic controls by their response to histamine. It was concluded that all these wheezy children had symptoms of a common basic disorder and that they should all be treated as asthmatic.     

64层螺旋CT在新生儿及婴儿先天性心脏病诊断中的应用

目的: 探讨64层螺旋CT对新生儿及婴儿先天性心脏病(CHD)的诊断价值.方法: 对55例诊断CHD的新生儿及婴儿进行64层螺旋CT对比增强心脏检查,并用多平面重建、最大密度投影及容积成像进行图像重建.所有病例均做超声心动图(ECG)对照,并经手术证实.结果: 64层螺旋CT、ECG对心血管畸形的诊断准确率分别为96.98%、71.86%,前者诊断准确率明显高于后者(χ2=23.88,P＜0.005).心内畸形68处中,64层螺旋CT、ECG的诊断准确率分别为92.65%、97.06%,两者无统计学差异(χ2=0.68,P＞0.05).心外畸形131处中,64层螺旋CT、ECG的诊断准确率分别为99.24%、58.78%,前者诊断准确率明显高于后者(χ2=32.32,P＜0.005).结论: 64层螺旋CT是诊断CHD可靠的方法,弥补了ECG对心外畸形诊断的不足,尤其能评价主动脉、肺动脉以及冠状动脉,对术前手术方案的制定具有重要价值.     

结节病15例临床分析

目的总结结节病的临床特征,提高临床医生对本病的认识和诊治水平。方法对15例结节病患者的临床资料进行回顾性分析。结果15例结节病患者,以呼吸系统症状为首发表现者7例,以肺外表现为首发症状者6例,无症状者2例。X线及胸CT提示双肺门及纵隔对称性淋巴结肿大者13例,12例患者血管紧张素转化酶升高,血沉加快者10例,结核纯蛋白衍化物试验仅1例阳性。结论肺结节病多见于中青年成人,临床症状不典型,在诊断上应综合其临床表现、胸部X片、CT及实验室检查结果。     

53例婴幼儿先天性心脏病手术治疗分析

目的探讨婴幼儿先天性心脏病的手术治疗经验。方法回顾性分析53例手术纠治的婴幼儿先天性心脏病患者的病例资料,其中2例行姑息手术,51例行解剖矫治（其中2例为急诊手术）。结果 53例患儿中,1例于术后8d肺部感染继发多脏器衰竭死亡;术后发生低心排血量综合征3例,肾功能衰竭1例,延迟性心包填塞1例,胸骨哆开1例。结论婴幼儿先天性心脏病应尽早手术干预,术前诊断、手术时机和方案的选择以及围手术期管理是手术成功的关键。     

53例婴幼儿先天性心脏病手术治疗分析

目的 探讨婴幼儿先天性心脏病的手术治疗经验.方法 回顾性分析53例手术纠治的婴幼儿先天性心脏病患者的病例资料,其中2例行姑息手术,51例行解剖矫治(其中2例为急诊手术).结果 53例患儿中,1例于术后8 d肺部感染继发多脏器衰竭死亡;术后发生低心排血量综合征3例,肾功能衰竭1例,延迟性心包填塞1例,胸骨哆开1例.结论 婴幼儿先天性心脏病应尽早手术干预,术前诊断、手术时机和方案的选择以及围手术期管理是手术成功的关键.     

盘锦市室外空气污染对儿童呼吸系统健康的影响

目的:探讨室外空气污染对儿童呼吸系统健康的危害,对盘锦市不同污染区儿童健康状况及室外环境进行调查.方法:按整群抽样方法,于2002年4月在轻、中、重污染区随机抽取小学和幼儿园各1所,所有儿童作为调查对象.结果:盘锦市儿童持续咳嗽、持续咳痰、哮喘、哮喘现患、喘鸣及喘鸣样症状的发生率分别为4.87%、2.22%、0.95%、0.71%、6.76%、5.82%.幼儿发生率高于学龄儿童;重、中污染区儿童的发生率高于轻污染区.沿街居住、住房与交通干线距离的接近使儿童呼吸系统症状的发生率增高.具有家族哮喘史、家族过敏史或个体过敏体质的儿童呼吸症状发生率较高.易感因素与室外空气污染的联合作用较强,呼吸系统疾病症状的发生率均以受其他因素影响的易感儿童为高.结论:室外空气污染是儿童呼吸系统健康的影响因素.     

多层螺旋CT重组技术对先天性血管环伴气管狭窄的诊断价值

目的 探讨多层螺旋CT(MSCT)重组技术对先天性血管环伴气管狭窄的诊断价值.方法 收集温州医学院附属第二医院2004年10月至2010年4月经手术证实的先天性血管环伴气道狭窄患儿9例,均行心脏大血管薄层CT增强扫描,所得数据传至工作站进行多平面重组(MPR)、容积再现技术(VRT)及VR透明化重组,以手术结果为标准,与超声心动图(UCG)对比分析其影像学特点.结果 9例中,肺动脉吊带4例,右位主动脉弓伴左迷走锁骨下动脉3例,双主动脉弓1例,无名动脉压迫综合征1例,其中5例伴有其他心内外畸形(法洛四联症2例,右室双出口伴动脉导管未闭及室间隔缺损1例,室间隔缺损1例,双上腔静脉1例),1例法洛四联症主动脉周围见多发迂曲侧支动脉,上述畸形VRT及MPR均多角度直观显示;VR透明化技术可立体显示气管及支气管受压情况,其中主气管受压6例,主气管及左主支气管受压2例,主气管及右主支气管受压1例;UCG均检出上述心内畸形,1例肺动脉吊带误诊为动脉导管未闭,余8例血管环、气管及支气管狭窄改变均漏诊.结论 MSCT重组技术是一种无创、快速检查方法,可多方位清晰显示先天性血管环及气管受压狭窄程度,有利于临床减少误诊并及时治疗.

Abstract：

Objective To evaluate the diagnostic value of multi-slice CT (MSCT) reconstructions for congenital vascular rings together with tracheal stenosis. Methods 9 cases of children with congenital vascular ring and tracheal stenosis confirmed by surgery were collected in the study, all cases had undergone thin slice CT contrast enhancement, the MSCT data were transmitted to the workstation for multiplanar reconstruction( MPR), volume rendering technique(VRT) and VR transparency resconstruction. With the surgical results as the gold standard, the imaging characteristics of echocardiography (UCG) and MSCT were comparatively analyzed. Results In 9 cases, there were 4 cases of pulmonary artery sling, 3 cases of right aortic arch combination with left aberrant subclavian artery, 1 case of double aortic arch, Ⅰ case of innominate artery compression syndrome. In this group, 5 cases were accompanied with other cardiac malformations (tetralogy of Fallot in 2 cases, double outlet right ventricle with patent ductus arteriosus and ventricular septal defect in 1 case, ventricular septal defect in 1 case, double superior vena cava in 1 case),1 case of tetralogy of Fallot demonstrated many tortuous collateral arteries around aorta. All malformations were well displayed by VRT, MPR. VR transparency resconstruction can stereoscopically display trachea and bronchial compression condition, the main trachea was compressed in 6 cases, the main trachea and left main bronchus was compressed in 2 cases, the main trachea and left main bronchus was compressed in 1 cases,UCG detected all intracardiac malformations, 1 case of pulmonary artery sling was misdiagnosed as patent ductus arteriosus, 8 cases of vascular rings, tracheal and bronchial stenosis were missed. Conclusion MSCT reconstruction technology is a noninvasive, rapid diagnostic method, it can clearly show the congenital vascular rings abnormalities and the degree of tracheal stenosis, it has important significance for clinic treatment.     

Gastro-oesophageal reflux in children with severe respiratory symptoms--clinical spectrum and management

Respiratory symptoms in children may be associated with underlying gastro-oesophageal reflux (GOR). We reviewed the case notes of 20 children who presented to us from June 1993 to June 1994 with respiratory symptoms and GOR. The patients consisted of 16 Malays, two Chinese and two Indians with equal number of males and females. Their age at diagnosis was less than one year in 17 patients. The earliest age at presentation was at the third day of life. All patients had major respiratory manifestations i.e. recurrent wheezing, recurrent cough and pneumonia. In addition, three patients had stridor and six patients had apparent life threatening episodes (ALTE). Fourteen patients required ventilation because of respiratory failure. Diagnosis of GOR was based on clinical grounds supported by barium oesophagogram in seven patients and ultrasound examination in 11 patients. Eight patients were fundoplicated because of ALTE and recurrent severe bronchospasm. On follow up, 14 patients had hyperactive airways requiring inhaled bronchodilator and steroid therapy.     

General practice audit of asthma in childhood

It has been suggested that asthma is underdiagnosed in children. We studied the records of 52 children with asthma in our group practice. Although asthma had been positively diagnosed in roughly 10% of the children aged 18 months to 11 years, to make the diagnosis required an average of 16 to 20 consultations for respiratory problems. According to the symptoms recorded before the diagnosis was made, only three quarters of the children had presented with wheeze and roughly a third had had difficulty in sleeping. We think that the doctor's time would be better spent teaching patients about their asthma than by repeatedly treating non-existent respiratory infection.     

Asthma and hayfever in Aboriginal and non-Aboriginal children living in non-remote rural towns

OBJECTIVE: To compare the prevalence and risk factors for wheeze, asthma diagnosis and hayfever in Aboriginal and non-Aboriginal children living in rural towns in Australia. DESIGN AND SETTING: Cross-sectional study in two towns in rural NSW, Australia, 1997. PARTICIPANTS: Primary school children (aged 7-12 years) classified by their parents as being of Aboriginal (n = 158) or of non-Aboriginal (n = 1,282) origin. Main outcome measures: Atopy measured by skinprick tests and respiratory symptoms measured by parent-completed questionnaire. RESULTS: Aboriginal children were less likely to be atopic (36.2% v 45.6%; 95% CI for the difference, -17.6 to -1.3) and to have hayfever (23.3% v 35.2%; 95% CI for the difference, -19.1 to -4.6) than non-Aboriginal children, but were equally likely to have had wheeze (31.0% v 27.3%) and asthma (39.4% v 39.3%). Among Aboriginal children, having had bronchitis before age two was a strong risk factor for wheeze (adjusted odds ratio (aOR), 9.3; 95% CI, 2.8-30.2) and asthma (aOR, 19.3; 95% CI, 4.7-79.3) and having a parent with hayfever was a strong risk factor for hayfever (aOR, 17.9; 95% CI, 3.5-90.8), but these risk factors were weaker among non-Aboriginal children. CONCLUSIONS: Asthma and wheeze are equally prevalent in Aboriginal and non-Aboriginal children living in the same towns, but appear to have a different aetiology.