小儿先天性肺腺瘤样畸形的诊断及外科治疗

目的 总结小儿先天性肺腺瘤样畸形(CPAM)诊治经验.方法 回顾2006年11月至2009年11月手术的13例CPAM临床资料,男6例,女7例,手术年龄50d至13岁.多数患儿有临床症状,经胸部X线和CT检查确诊.3例小于2个月者急诊手术,其余患儿择期手术.术式有单肺叶切除10例,肺叶+不规则肺段切除2例,囊肿剥离术1例.病理示Ⅰ型8例,Ⅱ型和Ⅲ型各2例,Ⅳ型1例.结果 无手术死亡.2例术后早期出现支气管胸膜瘘,1例经保守治疗,1例在胸腔镜下瘘口缝合后治愈.术后平均住院9.7 d,平均随访12.3个月,2例肺叶+不规则肺段切除者活动量轻度受限,其余患儿恢复良好.结论 胎儿超声是CPAM产前诊断的主要手段,胸部CT检查对产后诊断有高度特异性.     

全胸腔镜下手术治疗小儿肺隔离症的疗效与安全性分析

目的探讨全胸腔镜下手术治疗小儿肺隔离症的临床疗效与安全性。方法回顾性分析本院2014年2月至2017年8月实施的33例全胸腔镜下肺隔离症手术患儿临床资料,其中男22例,女11例;年龄2个月至12岁1个月。患儿均经术前胸部CT增强明确诊断,手术采取三孔法,根据病变情况选择肺叶切除或不规则切除。结果 33例患儿均在全胸腔镜下顺利完成手术,无一例中转开胸手术,19例行肺叶切除术,6例行不规则肺切除术,1例行肺段切除术,7例行叶外型肺隔离症切除术。结论全胸腔镜手术治疗小儿肺隔离症安全、有效,提高手术技巧可减少术中出血,提高手术的安全性。     

先天性肺囊性病变28例的诊断与治疗

目的 探讨儿童先天性肺囊性病变的诊断、手术时机和方式.方法 对儿童先天性肺囊性病变28例的临床资料进行回顾性分析.男19例,女9例;年龄1～12岁,平均5.4岁,其中1～5岁11例.先天性肺囊肿21例,其中支气管源性囊肿13例;肺源性囊肿6例;混合型2例.肺隔离症7例.术前诊断主要依靠胸部X线片和CT,术前确诊21例,其中先天性肺囊肿17例,肺隔离症4例.术前误诊7例,误诊率25%,其中误诊为肺脓肿、纵隔占位各2例,气胸、肺大疱、支气管扩张症各1例.行肺囊肿摘除术 3例,肺段切除术2例,肺叶切除17例,其中2例为急诊手术,隔离肺切除4例,双肺叶、肺叶加肺段切除术各1例.结果 本组无死亡病例,术后恢复良好,均痊愈出院,随访1～5 a,患儿生长发育及活动正常.结论 儿童先天性肺囊性病变误诊率高,诊断主要依靠影像学检查,确诊后应尽早手术,手术疗效满意,预后良好.     

70例婴幼儿先天性肺囊腺瘤样畸形的围术期分析

目的：总结婴幼儿先天性肺囊腺瘤样畸形的诊断和手术治疗方法。方法回顾性分析自2008年8月至2013年7月于本院就诊的70例先天性肺囊腺瘤样畸形患儿临床资料,比较出生后X线检查、CT扫描检查及手术治疗结果,并进行随访。结果70例患儿中,男39例,女31例。入院时最小年龄为30 min,最大年龄为13岁。手术时期：新生儿期40例,1个月至1岁26例,1～13岁4例,平均手术年龄（4.3±0.6）个月。分型：Ⅰ型18例,Ⅱ型35例,Ⅲ型6例,混合型6例,存在大叶性肺气肿5例。4例合并其他畸形,其中2例合并纵隔囊肿;1例混合型合并骶尾部畸胎瘤;1例左侧Ⅲ型合并左侧膈疝。病变位置：1例为双侧,其余均为单侧。右侧21例,左侧18例。术前X线检查均无特异性。70例均采用开胸手术治疗,其中肺叶切除术59例,肺段切除术6例,肺叶＋肺段切除术3例;2例行右肺两肺叶切除术。术后并发症包括气胸、咳嗽、伤口感染、胸腔粘连、膈肌膨升,经治疗后痊愈。随访2个月至5年,均存活,无一例死亡,且无术后复发现象。结论出生后增强CT检查对于先天性肺囊腺瘤样畸形的确诊及手术方案的制定极其重要,建议确诊后早期手术切除。     

儿童先天性肺囊性腺瘤样畸形四例临床特点分析

目的探讨儿童先天性肺囊性腺瘤样畸形(congenital cystic adenomatoid malformation of lung,CCAM)的临床、影像及组织病理特点,提高临床对该病的认识。方法收集新乡医学院第一附属医院经病理证实为CCAM的4例患儿病例资料,对其临床表现、影像学及病理特点、治疗方法进行分析。结果3例患儿有反复呼吸道感染,1例自发性气胸;2例胸部X线见到囊性改变,2例胸部CT提示存在CCAM,1例提示肺脓肿,1例提示不除外肺隔离症;4例均手术切除病变肺叶,术后病理证实为CCAM,均预后良好。结论反复呼吸道感染者需注意该病可能,需结合临床表现、CT检查、病理结果共同诊断CCAM,手术切除是有效治疗手段。     

儿童先天性肺气道畸形20 例临床分析

目的总结儿童先天性肺气道畸形(CPAM)的临床特点。方法回顾分析2016年1月至2018年12月诊断为CPAM的20例患儿临床资料。结果 20例患儿中,男12例、女8例,平均年龄(30.4±7.6)个月;病程5天～3年。17例表现为反复咳嗽、喘息、发热,1例表现为胸闷,2例表现为呼吸困难。双侧病变4例;单侧病变16例,累及左侧7例,右侧9例。合并心脏畸形2例,1例为三房心。肺高分辨CT检查14例患儿表现为大囊型,均伴有纵隔移位;6例为小囊型。术后病理分型,Ⅰ型14例,Ⅱ型5例,Ⅳ型1例。所有患儿均手术治疗,术后随访时间1～24个月不等,均恢复良好。结论 CPAM少见,可合并其他系统畸形,可通过肺部高分辨CT检查发现,确诊需依据病理组织学检查,经手术治疗后预后较好。     

小儿先天性肺囊性腺瘤样畸形23例诊治分析

目的 总结小儿先天性肺囊性腺瘤样畸形(CCAM)的临床特点及诊治经验.方法 回顾性分析2001年3月至2007年12月在北京儿童医院经手术和病理检查确诊为CCAM的23例患儿的临床资料.所有患儿术前均摄X线胸片并行胸部CT扫描检查.结果 23例患儿中男15例,女8例.进行手术治疗时的平均年龄4.5岁,其中包括新生儿2例.术前诊断主要依靠病史和影像学检查.术前误诊4例,术前影像学检查确诊率为82.6%.依照Stocker组织病理学分型,23例患儿中Ⅰ型15例,Ⅱ型8例,没有恶变.23例均经手术治疗,证实1例为双侧痛变,余均为单侧病变和累及单叶.全部病例痊愈出院,随访结果显示23例均恢复良好.结论 小儿CCAM临床表现无特异性.影像学检查是发现该病的有效手段,确诊依据组织学检查,组织病理学分型可依据胸部CT结果得出大致判断.手术治疗是CCAM主要治疗手段,肺叶切除是标准术式并可取得良好的效果.     

三维CT对支气管异物诊断价值的临床研究

目的 探讨三维CT在支气管异物诊断和鉴别诊断中的作用.方法 对37例可疑支气管异物的患儿行螺旋CT检查,并进行支气管三维CT重建,通过支气管镜检术、保守及手术治疗的结果 判断三维CT检查结果 的准确性.结果 37例可疑支气管异物的患儿中,X线胸部透视提示支气管异物8例(A组);X线胸部透视不提示支气管异物29例(B组).A组中,CT提示肺内感染5例、先天性肺发育异常-肺叶缺如2例、气管食管瘘1例.B组29例患儿中,CT提示支气管异物25例,经支气管镜检证实其中24例存在植物性异物,1例为气管内肿物;CT不提示支气管异物4例,证实为肺内感染、小儿急性喉炎各2例.手术证实存在植物性异物的24例中,CT显示气管支气管内异物影共14例次,不规则狭窄共6例次;阻塞性肺不张、肺气肿、肺炎共7例次;前两项为直接征象,占74%(20/27),后三项为间接征象,占26%(7/27).结论 三维CT检查对支气管异物的诊断及鉴别诊断有重要意义,有助于疑难病例的诊断和鉴别诊断.     

胃管内灌注美兰支气管镜观察法对儿童先天性气管食管瘘诊断价值分析

目的回顾性分析胃管内灌注美兰支气管镜观察法在儿童先天性气管食管瘘中的诊断价值。方法回顾性分析2015年9月至2016年5月深圳市儿童医院呼吸科确诊的2例先天性H型气管食管瘘患儿和3例先天性气管食管瘘术后复发患儿的临床资料,对食管造影、支气管镜常规检查法、管内灌注美兰支气管镜观察法、胸部CT的检查结果进行分析。结果 5例患儿胃管内灌注美兰后支气管镜检查均可观察到气管内美兰渗出;2例气管食管瘘术后复发患儿支气管镜常规检查未发现有瘘口存在;4例患儿行食管造影检查,仅1例诊断出气管食管瘘;4例患儿行胸部多排螺旋CT(MDCT)检查均未见到瘘管,但可见食管下段含气影。结论胃管内灌注美兰支气管镜观察法是诊断儿童先天性H型气管食管瘘和先天性气管食管瘘术后复发值得推荐的检查方法;常规支气管镜检查及食管碘油造影均有漏诊可能;胸部MDCT对于诊断先天性气管食管瘘价值有限,但食管下段含气影提示有气管食管瘘存在的可能。     

儿童胸腔镜下肺叶切除术8例

目的总结本院8例胸腔镜下肺叶切除术患儿的手术经验,探讨儿童胸腔镜下肺叶切除术的可行性。方法回顾性分析2011年7月至2012年12月我们实施的8例儿童胸腔镜肺叶切除术患儿临床资料,对其诊治经过及手术经验进行初步总结。结果8例患儿中,男5例,女3例,年龄4～11岁。切除左肺下叶4例,左肺上叶2例,右肺下叶2例。除1例11岁患儿术中行单肺通气外,其余10岁以下患儿均行双肺通气。2例术中中转开放手术,其余在胸腔镜下顺利完成手术。术中出血10-30mL,手术时间50-90min。术后病理报告：肺腺瘤样囊性病变7例,叶内型隔离肺1例。术后第3～4天拔除胸腔闭式引流管,术后6—7d出院,无严重手术并发症出现。近期随访患侧剩余肺叶均代偿良好。结论胸腔镜肺叶切除术术野显露清楚,创伤小,手术时间短,患儿痛苦小,术后恢复快,呼吸系统并发症少。大龄儿童单肺通气能增加操作空间,同时肺萎陷后也有利于肺门部的解剖,使手术简化。若能掌握指征和操作技巧,即使没有单肺通气情况下,同样具有一定的可行性。     

Lung resections in children for congenital and acquired lesions

We reviewed a single-center experience of pediatric lung resections for various congenital and acquired benign lung conditions. Thirty-five children underwent lung resections between 1998 and 2006, their age ranging from 8 days to 12 years (mean 3 years), with a male:female ratio of 4:1. Twelve patients were neonates. Antenatal diagnosis was available in only one patient. The presenting symptoms were respiratory distress and respiratory tract infections. Imaging with chest X-ray with/without a CT scan picked up the lesion in all cases. Preoperative ventilation was required for five patients. One patient had pneumothorax at presentation; however, ten patients had inadvertent intercostal tube insertion before surgical referral. The surgical procedures performed included lobectomy (28), segmentectomy (3), and pneumonectomy in 4 cases. Twenty-one patients underwent emergency surgery. Six patients required postoperative ventilation. The histopathological diagnosis was congenital lobar emphysema (CLE) (9), congenital cystic adenomatoid malformation (CCAM) (9), bronchiectasis (9), sequestration (3), atelectasis (1), lung abscess (1), unilobar tuberculosis (1), hydatid cyst (1), and foreign body with collapse (1). There was considerable discrepancy between the preoperative diagnosis based on imaging and the postoperative histopathological diagnosis. Postoperative complications included atelectasis (2), pneumothorax (2) and fluid collection (4 cases). Three patients died, one from compromised cardiac function, one from overwhelming sepsis and one from respiratory failure due to severe bilateral CCAM; the rest of the patients made a satisfactory recovery. At short-term follow-up all patients were doing well. Pulmonary resections are necessary for various congenital and acquired lung lesions in children and can be done safely in a pediatric hospital setup. Proper preoperative diagnosis can avoid inadvertent intercostal tube insertion in patients with congenital cystic lung lesions. The histopathological diagnosis often differs from the radiological diagnosis. Emergency lobectomies for acute respiratory distress, even in neonates, result in a satisfactory outcome.     

婴幼儿肺动脉吊带24例临床特征及诊治分析

目的 分析婴幼儿肺动脉吊带（PAS）的临床特征、诊断及治疗。方法 回顾性分析2009年9月至2014年7月在重庆医科大学附属儿童医院诊断为PAS的病例,分析其一般情况、临床特征、影像学检查、诊断、治疗和预后。结果 29例PAS患儿进入分析,男16例,女13例,诊断PAS时年龄2月龄至3.5岁,中位年龄为7月龄。25例有反复喘息症状,26例入院时肺部听诊可闻及哮鸣音。15例为单纯PAS,确诊PAS中位年龄为6月龄;余14例伴有其他心血管病变,确诊PAS的中位年龄为8月龄,与单纯PAS患儿比较差异无统计学意义（P>0.05）。26例患儿行增强CT心血管重建+气道重建明确PAS及合并先天性心脏畸形诊断,余3例心脏超声提示PAS,增强CT仅行气道重建检查;29例CT检查均提示气道狭窄,合并支气管桥7例。25例行心脏彩超检查,其中13例检查时间早于增强CT心血管重建,5例确诊为PAS。13例行纤维支气管镜检查均发现气管狭窄,其中6例存在气管软骨环。16例行手术治疗,其中12例手术成功,4例死亡。单因素分析显示,有机械通气时间>72 h、手术时年龄≤5月龄或体外循环时间>100 min等因素的PAS患儿病死率高（P< 0.05）。结论 对反复喘息发作内科治疗效果不佳的婴幼儿,需警惕PAS的可能,合并心血管畸形并不能帮助早期发现PAS。增强CT心血管重建+气道重建检查可发现PAS及伴随气道畸形,有呼吸道症状者应尽早手术治疗,多数患儿的气道畸形不需手术处理。机械通气时间>72 h、手术时年龄≤5月龄或体外循环时间>100 min与PAS患儿病死率相关。     

混合型完全性肺静脉异位连接17例的诊断与治疗

目的探讨儿童混合型完全性肺静脉异位连接(TAPVC)的诊治要点及影响预后的主要因素。方法近5 a来,在广东省心血管研究所确诊为混合型TAPVC的患儿共17例,收集该17例患儿的临床资料和随访结果,进行回顾性分析。结果 17例混合型TAPVC患儿中心上型+心内型14例,心上型+心下型3例,并肺静脉回流梗阻6例。17例术前全部行超声心动图检查,其中13例行多排螺旋CT扫描,14例行手术治疗。行CT扫描者,10例经手术探查证实此10例患儿CT能够准确显示所有肺静脉回流径路。行超声检查者,12例超声报告了所有4条肺静脉回流径路,5例超声未能报告肺静脉全部回流径路。超声报告所有肺静脉回流径路者,经CT或手术探查,证实其中7例超声显示的所有肺静脉回流径路均准确,4例超声准确显示了3条肺静脉回流径路,1例超声显示的肺静脉回流径路完全错误。并肺静脉回流梗阻的6例患儿,超声心动图发现6例,而CT仅发现1例。14例行手术治疗者,急诊手术6例,择期手术8例。围术期死亡2例,均为急诊手术者。12例康复出院者,随访时间2～53(28.8±17.4)个月,中位随访时间30个月。术后3例发生吻合口狭窄,其中1例术后2个月死于严重吻合口狭窄导致的急性肺水肿。结论混合型TAPVC以心上型+心内型最多见。结合超声心动图和多排螺旋CT扫描是术前评估的最佳策略。对肺静脉回流径路的显示多排螺旋CT优于超声心动图,对肺静脉回流梗阻的显示超声心动图优于多排螺旋CT。急诊手术可能是围术期死亡的危险因素。术后随访主要并发症为吻合口狭窄。     

Gastric necrosis in newborns: a report of 11 cases

Eleven neonates ranging in gestational age from 34 to 40 weeks presented with gastric necrosis. The 4 full-term neonates showed sudden-onset hemorrage and “coffee-ground” vomiting; in the 7 premature babies the initial clinical finding was abdominal distention. The criteria for diagnosis were: perinatal distress in prematures and transient neonatal respiratory distress in full-term babies. Radiographic evidence of gastric distention was typical and preceded clinical signs of hematemesis and gastric perforation. Surgery was performed in 8 patients; 3 received medical treatment. At surgery 1␣total and 3 subtotal gastrectomies and 4 segmental gastric resections were performed. Three of these patients died post-operatively as a consequence of multiorgan failure; a second look was necessary in one patient 1 week after surgery because of prepyloric perforation due to ulcers. Biopsy specimens taken from the site of perforation demonstrated extensive necrosis; ulceration was disseminated in the surrounding gastric mucosa; no signs of phlogosis were detected. The diagnosis, treatment, and physiopathologic considerations are reviewed. Accepted: 6 November 1997     

The role of pulmonary artery anatomy in repair of tetralogy of Fallot

Pulmonary artery anatomy is the key factor that determines the type of surgical treatment required in tetralogy of Fallot. Despite the fact that routine primary repair is now done on infants, inadequate pulmonary artery size can dictate the need for staged surgical repair in even the oldest age groups. From October 1986 to October 1998, 361 patients at our clinic underwent surgery to correct tetralogy of Fallot. A total of 292 cases were treated with primary repair, 69 surgeries were palliative, and 30 of these 69 underwent corrective surgery. The Nakata index was used as a pulmonary artery index (PAI), and PAI< 200 was the criterion for requirement of two-stage repair. Of the 30 patients that underwent staged repair, the Blalock-Taussig shunt (BTS) procedure was used in 24; the remaining six patients had right ventricular outflow tract reconstruction (RVOTR). The mean age of all the palliative surgery patients was 3.4 years (range 6 months to 11 years), and of those who received corrective surgery was 5.5 years (range 2-12 years). These patients' PAI values were 181 +/- 37.5 mm2/m2 and 359 +/- 130.7 mm2/m2, respectively. The period between the two operations ranged from two months to four years. Mortality rates were 2.8 percent for palliative surgery as a whole, 4.1 percent for primary repair, and 16.6 percent for staged repair. Our policy with regard to corrective surgery for tetralogy of Fallot is to do primary repair regardless of a patient's age and weight, except in cases where the pulmonary artery anatomy is appropriate for the patient's body size.     

Radiology, histology and short-term outcome of asymptomatic congenital thoracic malformations

Aim: The aim of this study was to evaluate the safety and short‐term outcome of our management of asymptomatic children with antenatally diagnosed congenital thoracic malformations (CTM), compared with recommendations from a recent review and meta‐analysis. Methods: Twenty‐two asymptomatic children with CTM, born in January 1, 2002 to January 8, 2009 were reviewed. Data on complications and respiratory symptoms were collected. Results: No severe respiratory symptoms were recorded. Seventeen children were referred to surgery. Complications were seen in one child. The final diagnoses were congenital pulmonary airway malformation (CPAM) in 13 children, two had sequestrations, and two had other significant malformations. Five children had minor malformations and did not undergo surgery. No malignancies were reported. Conclusion: Elective surgery at 1 year of age is safe and carries no apparent risk to asymptomatic children with CTM. The rate of complications was equal to that reported in a recent review and meta‐analysis.     

CT of invasive pulmonary aspergillosis in children with cancer

In treating cases of malignancy, the use of chemotherapy carries a high risk of lower respiratory tract infections, especially fungal pneumonopathy. This complication is a major cause of mortality and is often difficult to diagnose because of non-specific clinical or radiological changes, but the early recognition of invasive fungal disease is imperative. CT is an important non-invasive method for the detection and evaluation of opportunistic fungal infections. In these patients an improved survival rate can be achieved when early detection by CT leads to the prompt institution of high-dose antifungal therapy. We illustrate the spectrum of CT findings of invasive pulmonary aspergillosis encountered in children with cancer. These patients had previously been treated with highdose chemotherapy with or without bone marrow rescue, and underwent radiological examinations because of clinical evidence of pneumonopathy. Representative cases demonstrate the clinical applications of CT in the evaluation and management of invasive fungal disease.     

13例小儿横纹肌肉瘤疗效分析

目的：分析小儿横纹肌肉瘤疗效,特别是大剂量化疗的效果。方法：对该院1998年1月至2005年10月收治的13例横纹肌肉瘤患儿临床资料进行分析。男8例,女5例;年龄7个月至12岁;依据美国横纹肌肉瘤研究组(IRS)的分期标准 Ⅰ期2例、Ⅱ期2例、Ⅲ期3例、Ⅳ期6例。所有患儿均经病理活检确诊,其中胚胎型12例,腺泡型1例。1例手术,1例手术加放疗,1例手术加化疗,10例手术加放疗及化疗。2002年前的病例化疗以VDCA、VAC和VadrC为主;2002年后采取了美国COG治疗横纹肌肉瘤的化疗方案,Ⅲ期采取CDV+IE方案治疗,Ⅳ期采用CT+VAC或CT+VAC+VCT方案治疗。结果：10例接受手术加放疗及化疗联合治疗的患儿2年生存率为60%,另外3例没有接受联合治疗的患儿全部死亡。2002年后患儿生存率（60%,3/5）高于2002年前生存率（37.5%,3/8）。患儿2年生存率2002年前为37.5%(3/8),2002年后为60%（3/5）,平均为46.2%(6/13)。结论：儿童横纹肌肉瘤病理类型以胚胎型为主,恶性程度高。晚期病例采取手术联合放、化疗,特别是大剂量化疗可取得较好疗效。