先天性肺隔离症的诊断和外科治疗

目的总结小儿先天性肺隔离症的诊断和外科治疗经验。方法对8例经手术、病理证实先天性肺隔离症患儿的临床资料进行回顾性分析。结果8例先天性肺隔离症患儿术前有3例误诊；6例叶内型行肺叶切除术，2例叶外型行单纯隔离肺切除术。术后未发生并发症。结论先天性肺隔离症术前诊断应注意结合胸片、碘油造影、逆行主动脉造影，一经诊断即应择期手术治疗，术中注意异常血管的处理。     

小儿肺隔离症20例临床分析

目的提高对小儿肺隔离症临床特点的认识。方法对本院2008年3月至2014年10月收治的20例小儿肺隔离症病例进行回顾性分析,总结其临床特征,并对其诊断和治疗方法进行探讨。结果小儿肺隔离症由于合并病变的影响,术前确诊率并不高。对所有病例行隔离肺切除或病变所在肺段切除,术后恢复良好,无手术死亡及并发症发生。结论小儿肺隔离症术前极易误诊,一旦确诊,手术切除病变肺组织,同时矫正伴发畸形是治疗小儿肺隔离症的最佳选择。     

肺隔离症12例诊断和治疗分析

目的总结12例小儿肺隔离症的诊断和治疗经验。 方法1994-01—2006-08,对温州医学院附属育英儿童医院收治的12例经手术和病理检查证实为肺隔离症的患儿临床资料进行回顾性分析。 结果12例肺隔离症患儿胸部X线均发现肺部异常阴影,7例呈圆形、类圆形或不规则实性块影,5例呈囊性及多囊性阴影改变;术前确诊5例,1例 经主动脉逆行造影证实,4例经螺旋CT三维重建、MRI检查发现胸主动脉与病灶之间有异常血管相通,术前高度怀疑肺隔离症3例,术前有4例误诊 ;8例叶内型肺隔离症患儿行肺叶切除术,4例叶外型肺隔离症行单纯隔离肺切除术。 结论临床医师应提高对肺隔离症的认知和重视程度。为降低肺隔离症误诊率,对可疑的肺隔离症患儿提倡螺旋CT三维重建和胸部MRI检查。     

小儿先天性肺隔离症的诊断与治疗

目的 探讨小儿先天性肺隔离症的诊断与治疗.方法 回顾性分析2001年3月至2011年3月收治的小儿先天性肺隔离症20例的临床资料.其中,男11例,女9例,年龄4 d～9岁(平均5.3岁),体重4～28 kg(平均11.3 kg).术前20例均行X线平片检查,15例加行普通CT平扫检查.误诊为肺囊肿9例,肺内肿瘤2例,漏诊4例在行其他手术时发现本病;另5例加行64层高速螺旋CT三维血管成像检查,发现有异常体动脉分支血管入隔离肺组织4例,高度疑诊本病1例.结果 全部20例均接受手术治疗,术后病理检查均证实为本病.术中发现病变肺组织(隔离肺)均有起自体动脉分支血管供血.本组均治愈,术后随访15例,随访2～7年,生长发育良好无复发,失访5例.结论 本病较少见,临床表现缺乏特异性,易被误诊和漏诊.高速螺旋CT 三维血管成像检查是术前确诊本病的重要手段.术中准确处理来自体动脉的分支血管是手术成功的关键.     

小儿腹腔内肺隔离症4例并文献复习

目的探讨儿童腹腔内肺隔离症(intra-abdominal extralobar pulmonary sequestration,IEPS)的临床特征、病理类型、诊疗方法及预后情况,以提高对该病的认识及诊治水平。方法回顾性分析2007年6月至2018年6月首都医科大学附属北京儿童医院收治的4例腹腔内肺隔离症患儿临床资料;检索万方、维普、CNKI、Pubmed、Medline数据库截至2018年6月关于小儿腹腔内肺隔离症的相关文献,并进行分析。结果本研究4例腹腔内肺隔离症患儿,合并先天性肺气道畸形Ⅱ型/Ⅲ型(congenital pulmonary airway malformation type 2/type 3,CPAMⅡ/Ⅲ型)各2例,均行手术治疗,分别随访3个月、6个月、3年半、4年,预后良好。通过文献检索,检索到31篇儿童IEPS相关文献,联合本中心4例患儿分析结果如下:共47例患儿,44例产前发现病变时母亲孕龄为25周(IQR:12～37),46例产前经多普勒B超诊断腹腔肿物,10例怀疑神经母细胞瘤(neuroblastoma,NB),7例怀疑IEPS。5例终止妊娠。产后42例患儿中7例(7/42,16.7%)伴发先天畸形。术前主要经B超(30/38,78.9%)、增强CT(27/38,71.1%)明确诊断,其中11例误诊为NB,9例诊断为IEPS。5例行保守治疗,3例保守治疗无效后行手术治疗。共40例患儿行手术治疗,25例(25/40,62.5%)出生后6个月内行手术治疗,主要采用开腹手术(25/40,62.5%),3例行腹腔镜中转开腹手术。肿物主要位于左腹(32/40,80.0%)。手术切除肿物平均长径3.0(IQR:2.8～3.9)cm,供血来源主要为腹主动脉及其分支(16/21,76.2%)。术后病理检查均证实为肺隔离症,22例(22/40,55.0%)伴CPAM,其中12例伴CPAMⅡ型,2例伴CPAMⅢ型。所有患儿预后良好。结论产前超声对于小儿腹腔内肿物具有良好的诊断价值,但明确腹腔内肺隔离症仍需产后随访及综合评估。手术治疗是早期诊断IEPS的唯一方式,应根据病变选择合适的手术方案,完整切除肿物者预后良好。     

肺隔离症

肺隔离症（ＰＳ）是小儿少见的先天性肺发育育异常，其临床表现缺少特异性，术前诊断困难，易误诊为肺炎，肺脓肿及肺囊肿合并感染等。现代影像学技术在本病的诊断中具有重要作用。本文在综合了ＰＳ的临床及病理资料的同时，着重介绍了影像学诊断方面某些新的研究进展，以便为本病的诊断提供列多的信息和帮助。     

全胸腔镜手术与传统开胸手术治疗儿童肺隔离症的对比研究

目的比较电视胸腔镜手术与传统开胸手术治疗儿童先天性肺隔离症的安全性与有效性,探讨全胸腔镜手术治疗儿童先天性肺隔离症的临床价值。方法选择2010年5月至2018年1月于南京医科大学附属儿童医院心胸外科接受手术治疗的66例先天性肺隔离症患儿作为研究对象,其中27例采取全胸腔镜手术,为全胸腔镜手术组; 39例采取传统开胸手术,为开胸手术组。对比两组手术时间、术中出血量、术后引流量与引流管留置时间以及术后住院时间的差异。结果全胸腔镜手术组中有1例中转开胸手术,无一例手术中死亡,手术后均恢复顺利,无严重并发症发生。全胸腔镜手术组和开胸手术组中位随访时间分别为0. 7年、4. 1年。全胸腔镜手术组手术时间(80±5. 6) min,术中出血(20±9. 2) m L,术后住院时间(5. 1±2. 1) d、术后引流量(50±9. 6) m L,术后引流管留置时间(4±1. 3) d,均明显优于传统手术组(P 0. 05)。结论与开放手术相比,全胸腔镜下手术治疗儿童先天性肺隔离症具有手术时间短、出血少、恢复快、切口美观等优点,对患儿心肺功能影响小,临床效果基本满意。     

胸腔镜下婴幼儿隔离肺的处理分析

目的总结分析胸腔镜下婴幼儿隔离肺的手术处理,分析手术的可行性以及风险。方法回顾分析本院2014年3月2015年4月收治的68例隔离肺患儿临床资料,所有隔离肺病例均经产前超声诊断,产前评估及出生后增强CT确诊,包括叶外型隔离肺、叶内型隔离肺、膈肌内隔离肺,分析胸腔镜下操作情况。结果本院共80例产前18~30周B超发现胎儿胸部实性或高回声肿块,经2次以上B超确诊为BPS。所有病例均经专业评估,其中5例因合并胎儿水肿,家属主动中止妊娠;75例继续妊娠至出生并全部存活。64例(85%)出生时无症状,11例(15%)出生时有症状。68例于生后早期增强CT检查进一步确诊为隔离肺,于婴幼儿期行手术治疗,平均手术年龄4.2个月;7例出生后无临床症状,家属拒绝CT检查。68例手术病理分型结果:叶内型30例,叶外型38例,其中叶外型中有9例为膈肌内隔离肺,1例腹腔型隔离肺,28例为胸腔内型隔离肺。手术方式:2例叶内型为全胸腔镜微创手术;28例为胸腔镜辅助手术;9例膈肌内隔离肺中,6例采用全胸腔镜微创手术,3例为胸腔镜辅助手术;1例腹腔型隔离肺采用传统开腹手术;胸腔内型隔离肺28例全部采用全胸腔镜微创手术。所有病例均治愈。均获1个月至1年的随访,CT检查生长发育及肺功能均良好。结论婴幼儿隔离肺在先天性肺部发育异常中相对少见。由于存在感染以及对周围肺组织的压迫风险,建议早期手术。胸腔镜下操作,对于婴幼儿隔离肺,相比传统开胸手术,不会增加手术风险,是一种可行的手术方式。     

儿童肺隔离症的介入治疗

目的用心导管介入方法治疗肺隔离症。方法10例平均年龄(9.9±5.8)岁(1~13岁),临床出现反复呼吸道感染、咯血症状,术前经X线胸片、胸部CT和磁共振基本明确诊断为肺隔离症的儿童,经股动脉插管至主动脉弓降部行造影,了解异常血管的起始、直径、数量及回流的静脉,选择大于血管直径30%~40%的蘑菇伞片或金属弹簧圈在降主动脉起始部行异常血管栓塞。结果介入治疗全部成功,无残余瘘。咯血症状当天消失,原有的肺部感染3~5d内控制;粗大血管堵塞后出现一过性胸痛症状,生命体征稳定,持续2周左右恢复。1~6个月胸片复查,肺部阴影8例完全消退,2例明显缩小仅残留微小淡密度影,且无临床症状。结论肺隔离症介入治疗有效、安全、无手术疤痕,恢复快,可免除开胸肺叶切除手术的损伤。     

胸腔镜手术与开胸手术治疗先天性支气管囊肿的对比分析

目的总结传统开胸手术与胸腔镜手术治疗先天性支气管囊肿的经验,分析两种手术的可行性及优劣。方法回顾性分析本院自2013年1月至2叭5年11月收治的先天性支气管囊肿患儿临床资料。患儿均经产前超声检查获得初步诊断,经产前评估及出生后增强CT检查明确诊断,最后经手术确诊。手术方式分为传统开胸手术及胸腔镜手术,比较两组手术时间、术中出血量、住院时间及胸腔引流管留置情况,探讨小儿胸腔镜手术治疗支气管囊肿的可行性及风险。结果共13例确诊,12例手术,1例家属决定暂不手术。手术病例中,女性6例,男性6例,男:女=1:1。左右侧比为1:1,其中6例手术诊断为支气管囊肿,3例合并肺囊腺瘤,3例合并肺隔离症。5例术前准确诊断为支气管囊肿,7例诊断为肺囊腺瘤或肺隔离症。手术年龄最小5 d,最大1岁,平均手术年龄4.8个月。5例采取传统开胸手术;7例采取胸腔镜手术,其中1例2月龄患儿因术中须行肺叶切除而增加辅助切口。所有手术病例均治愈。结论先天性支气管囊肿属于少见疾病,由于存在感染以及占位的风险,建议早期手术。胸腔镜下操作,对于婴幼儿病例,不会增加手术风险,是一种可行的手术方式。     

Intrapericardial Extralobar Pulmonary Sequestration Presenting as a Prenatal Intrathoracic Mass

An intrathoracic mass, which persisted during the remaining pregnancy, was first seen during routine ultrasound examination performed at 20 weeks gestation. After birth, the child was asymptomatic. Echocardiography showed the mass to be located intrapericardially. The mass was electively resected via sternotomy 3 weeks after the birth. Microscopic examination showed normal lung tissue surrounded by pleura corresponding to the diagnosis of extralobar pulmonary sequestration. To the authors' knowledge, this is the earliest described detection of such a lesion. Furthermore, this article reports the unique finding of a feeding vessel from the right pulmonary artery.     

Radionuclide evaluation of a communicating bronchopulmonary foregut malformation

A boy with a communicating bronchopulmonary foregut malformation is presented. Since arteriography failed to demonstrate systemic or pulmonary blood supply to the lesion,^99MTc-macroaggregated albumin was injected into the aorta at the conclusion of the arteriogram. This procedure documented the existence of a systemic blood supply which at surgery arose from the ninth left intercostal artery. Administration of^99MTc-pertechnetate prior to surgery demonstrated gastric mucosa in the lesion.     

175例小儿连续胸腔镜肺切除术回顾性研究

目的 探讨胸腔镜肺切除术在治疗婴幼儿和儿童肺部病变的安全性和有效性.方法 回顾性研究2014年9月至2016年6月间一组在北京儿童医院收治的需要肺切除的患儿,总结分析175例病例的临床资料、手术方法、手术效果及术后病理.结果 175例病例均顺利完成手术,无严重并发症及二次开胸的病例.175例中,男100例,女75例.年龄2个月至13岁10个月,平均(18.5±41.6)个月;手术时间15～120min,平均(75.0±37.9) min;出血量1～300ml,平均(5.0±26.9)ml.中转开胸7例,中转开腹1例,中转率4.6％.术后病理:先天性肺气道畸形即肺囊性腺瘤样畸形(CPAM) 105例、隔离肺47例、肺部肿瘤9例、肺气肿6例、肺囊肿4例、肺血管淋巴管畸形1例,肺动静脉瘘1例、肺寄生虫性肉芽肿1例.结论 婴幼儿和儿童的肺部病变可以经过胸腔镜治疗,且胸腔镜肺切除术是安全和有效的.腔镜的微创手术可以降低手术创伤,减轻术后疼痛,缩短住院时间,同时减小手术切口,伤口美观.     

Congenital cystic lung disease: contemporary antenatal and postnatal management

Congenital cystic lung disease comprises a broad spectrum of rare but clinically significant developmental abnormalities, including congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, bronchogenic cysts, and congenital lobar emphysema that result from perturbations in lung and airway embryogenesis. As congenital lung lesions are now more commonly recognized antenatally, mothers require accurate prenatal counseling and appropriate perinatal management. In light of long-term complications of infection and malignancy, there is growing consensus that infants with asymptomatic lesions should undergo elective excision of congenital pulmonary adenomatoid malformation (CPAM) or bronchopulmonary sequestration (BPS). This review will focus on advancements and current practice in the diagnosis and management of CPAM and BPS, identifying aspects of the literature that are confusing or controversial. Although our knowledge and pre- and postnatal management of lung lesions will continue to evolve and improve, there is a compelling need for a unified clinical and pathological classification system that creates a common platform for discussion, clinical management, and research.     

Pitfalls in the sonographic diagnosis of juxtadiaphragmatic pulmonary sequestrations

Background. Sonographic evaluation of juxtadiaphragmatic lesions is frequently the initial imaging modality of choice in the pediatric population. The increasing sophistication of sonographic and Doppler technologies has led to the suggestion in the literature that lesions suspected of being juxtadiaphragmatic pulmonary sequestrations can be confidently imaged by ultrasound examination alone. Objective. To present four cases which raise concern over the accuracy of sonographic examination of these lesions. Materials and methods. We present four such lesions for which an initial erroneous diagnosis of pulmonary sequestration was suggested on an initial ultrasound examination. Conclusion. The potential of a missed diagnosis of neuroblastoma has led us to propose that any lesion not demonstrating unequivocal sonographic findings of pulmonary sequestration should undergo further investigation and, if necessary, tissue sampling or excision. Received: 14 June 2000 Accepted: 2 November 2000     

Neonatological and pulmonological management of bilateral pulmonary sequestration in a neonate

Background  Bronchopulmonary sequestration is a lung malformation characterized by nonfunctioning lung tissue without primary communication with the tracheobronchial tree. Intrauterine complications such as mediastinal shift, pleural effusion or fetal hydrothorax can be present. We present the case of a newborn with bilateral intralobar pulmonary sequestration. Methods  Prenatal ultrasonography in a primigravida at 20 weeks of gestation revealed echogenic masses in the right fetal hemithorax with mediastinal shift towards the left side. Serial ultrasound confirmed persistence of the lesion with otherwise appropriate fetal development. Delivery was uneventful and physical examination revealed an isolated intermittent tachypnea. Chest CT scan and CT angiography showed a bilateral intrathoracic lesion with arterial supply from the aorta. Baby lung function testing suggested possible multiple functional compartments. Results  Right and left thoracotomy was performed at the age of 7 months. A bilateral intralobar sequestration with vascularisation from the aorta was resected. Pathological and histological examination of the resected tissue confirmed the surgical diagnosis. At the age of 24 months, the child was doing well without pulmonary complications. Conclusions  Bilateral pulmonary sequestration requires intensive prenatal and postnatal surveillance. Though given the fact of a bilateral pulmonary sequestration, postnatal outcome showed similar favourable characteristics to an unilateral presentation. Baby lung function testing could provide additional information for optimal postnatal management and timing of surgical intervention.     

28例儿童肺部弥漫性疾病的病因和诊断分析

目的 探讨儿童肺部弥漫性疾病的病因和诊断思路。方法 回顾性分析28例肺部弥漫性疾病患儿的诊断以及确诊过程。结果 确定病因25例,包括支原体肺炎1例、沙眼衣原体肺炎2例、巨细胞病毒肺炎2例、EB病毒肺炎1例、血型播散性肺结核3例、金黄色葡萄球菌败血症性肺炎1例、肺隐球菌病1例、侵袭性肺曲霉菌病2例、广泛支气管扩张合并肺部感染2例、特发性肺含铁血黄素沉着症1例、特发性肺纤维化1例、HIV合并淋巴细胞间质性肺炎1例、鸽粪引起的外源性变应性肺泡炎1例、韦格内肉芽肿1例、郎格罕细胞组织细胞增生症2例、恶性淋巴瘤3例。疑似诊断3例,包括奴卡菌感染1例、少年类风湿性关节炎合并肺纤维化1例、HIV合并卡氏肺囊虫1例。18患儿经X线检查、病史和体格检查以及其他非创伤性检查诊断,8例经皮肤活检或肺活检诊断,2例由尸解确诊。结论 儿童肺部弥漫性疾病的病因包括肺部感染性疾病、特发性疾病和全身疾病的肺部表现。影像学表现、病史和体格检查以及其他非创伤性检查能确定多数患儿的病因,少数病例需经创伤性检查诊断。     

Pulmonary Sequestration In A Child With Acute Myelold Leukemia

The article describes a relatively rare congenital anomaly that was difficult to diagnose in a 10–year-old child with acute nonlymphoblastic leukemia. Just at diagnosis of leukemia, the patient showed a pathologic chest radiograph because of a parenchymal thickening at the right lung apex. The presence of bronchopneumonia was suspected, and broad-spectrum antibiotic therapy was started with subsequent antifungal treatment for persistent fever and concurrent chemotherapy-induced marrow aplasia, which did not favor pulmonary infiltrate recovery. Continuous culture tests, including bronchial swab, proved negative for Koch-Weeks bacillus, fungal organisms, and other pathogens. Computed tomography, however, was suggestive of Aspergillus lung involvement, and apical sepmentectomy was performed. The anatomic pathologist suggested the diagnosis of intralobar sequestration. In summary, when pulmonary pathology with an excavation is found in a leukemic child, one must consider the possibility of pulmonary sequestration complicated by an infectious disease.     

Necrotizing fasciitis in a newborn with an enormous cavernous hemangioma

Necrotizing fasciitis (NF) is a rapidly progressive, invasive softtissue infection with a high mortality that is rare in the pediatric population. Of the reported pediatric cases, 50% involved neonates; the most common cause was neonatal omphalitis. The cornerstone of therapy is early, aggressive surgical treatment with boradspectrum antibiotics and nutritional support. In this case report, a large, contaminated cavernous hemangioma in the gluteal area associated with NF is reported. The patient's condition was severely compromised due to toxicity and sequestration of blood in the hemangioma. Effective treatment relied on a multimodal approach.