儿童先天性肺囊性疾病诊断和治疗进展

儿童先天性肺囊性疾病属临床少见肺部疾病,是肺组织胚胎发育异常所形成的畸形.根据其起源可以分为支气管源性肺囊肿、囊性腺瘤样畸形、先天性大叶性肺气肿和肺隔离症.该病临床表现无特异性,易与多种疾病相混淆,造成误诊.现对其主要发病机制、临床表现、诊断与鉴别诊断、治疗及干预措施进行阐述.     

小儿先天性肺囊性疾病（附65例分析）

目的探讨先天性肺囊性疾病分类、诊断及治疗。方法回顾分析65例先天性肺囊性疾病的临床资料。结果自1980年1月～2004年12月经手术、病理证实的65例，其中先天性肺囊肿51例，先天性肺隔离症8例，先天性囊性腺瘤样畸形6例。结论先天性肺囊性疾病主要分3类，易与其他疾病混淆，引起误诊。影像学检查发现，明确手术指征者应积极采取外科治疗。     

儿童先天性肺囊性疾病69例诊疗分析

目的：总结儿童先天性肺囊性疾病的诊断和治疗经验,探讨肺囊性疾病的手术治疗时机与手术方式。方法回顾性分析2004年7月至2013年7月我们收治的69例先天性肺囊性疾病患儿临床资料,男39例,女30例,年龄2d至15岁,平均年龄（1．2±0．6）岁。其中先天性肺囊肿48例,先天性肺叶气肿15例,先天性肺囊性瘤样畸形6例。肺囊肿中行囊肿摘除术8例,40例行肺叶切除术;先天性肺叶气肿15例,均行肺叶切除术;先天性肺囊性瘤样畸形6例,均行肺叶切除术。结果患儿均治愈出院,无一例死亡。术后近期并发症包括气胸5例,胸腔积液4例,乳糜胸1例,肺炎4例。随访2个月至9年,均恢复良好。结论儿童先天性肺囊性疾病表现多样,易产生压迫症状或急性呼吸窘迫,应早诊断,早治疗,手术切除是治疗的根本方法,对于有急性呼吸窘迫症状者,应急诊手术治疗。     

先天性肺血管病

先天性肺血管病指在人体胚胎发育过程中,肺动脉、肺静脉和肺血管丛形成或连接异常而引起的结构畸形。近年来,关于先天性肺血管病的病例报告日益增多,但尚无统一分类,影响了该类疾病的临床诊疗。该文介绍了先天性肺血管病的分类、临床表现、诊断方法和治疗进展。     

先天性肺囊性疾病

先天性肺囊性疾病是较少见的先天性肺部疾病,是肺组织胚胎发育异常所形成的畸形。囊性病变包括囊性腺瘤样畸形、支气管源性肺囊肿、先天性大叶性肺气肿、肺隔离症。此类疾病的临床表现相似,但发生机制及病理改变有各自的特点。囊性腺瘤样畸形是由于细支气管发育停滞,以肺泡不发育为代价引起的肺间质大量增生,目前根据病理类型分成5型。支气管源性肺囊肿是胚胎发育时期支气管树某段的异常出芽形成的。先天性大叶性肺气肿是支气管内阻外压以及支气管壁的病变造成的气体陷闭,肺泡腔扩张。肺隔离症是部分肺组织与正常的支气管肺组织无交通,供血来自体循环,病变肺无正常功能,分叶内型和叶外型。     

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.     

经肺门入路胸腔镜肺叶切除术治疗儿童先天性肺畸形伴叶裂发育不全

目的评估经肺门入路胸腔镜肺叶切除术治疗儿童先天性肺畸形伴叶裂发育不全的安全性和有效性。方法纳入2014年1月至2020年12月在四川大学华西医院小儿外科行经肺门入路胸腔镜肺叶切除术的先天性肺畸形患儿共550例, 根据叶裂解剖分型将患儿分为叶裂发育不全组(IF组)和叶裂发育完全组(CF组), IF组共152例患儿, CF组共398例患儿。对两组患儿术中、术后的临床资料和手术结果进行比较与分析。连续参数数据采用t检验, 连续非参数数据采用Wilcoxon检验, 非连续数据采用χ2检验或Fisher确切概率法。结果单因素分析显示两组患儿术中出血(P=0.02)、手术时间(P=0.01)、胸腔引流管放置时间(P<0.01)和术后住院时间(P=0.03)差异存在统计学意义。两组患儿均无术中中转开放手术;IF组有7例患儿术后出现漏气, 均在安放或延长留置胸腔闭式引流管后自愈;两组均无支气管胸膜瘘、大出血、重症肺炎等严重术后并发症发生。结论叶裂发育不全会增加儿童先天性肺畸形胸腔镜肺叶切除的手术难度, 经肺门入路的方式可安全而有效地降低这类患儿的中转开放率和术后并发症发生率。     

先天性肺囊腺瘤样畸形40例诊疗分析

目的 分析先天性肺囊腺瘤样畸形胎儿-新生儿的的围产危险因素,探讨其手术时机以及风险管理方法.方法 回顾性分析自2008年10月至2013年1月,来本院就诊40例先天性肺囊腺瘤样畸形病例资料.结合产前诊断以及出生后的胸片、CT检查,分析围产风险因素和手术时机. 结果临床诊断先天性肺囊腺瘤样畸形40例中,顺产17例,剖腹产23例;早产儿3例.出生后有呼吸道症状11例,占27.5％,新生儿期出现呼吸道症状14例,占35.0％.40例中,手术治疗32例;1例放弃治疗,后来死亡;7例家属选择等待.32例手术患儿均治愈出院.平均手术年龄5.6个月.结论 先天性肺囊腺瘤样畸形在新生儿期存在呼吸道风险,应选择在有新生儿外科手术条件的医院生产,出生后需注意预防呼吸道风险,为手术创造条件.     

先天性肺气道畸形的产前诊断和转归

先天性肺气道畸形(congenital pulmonary airway malformation,CPAM)是产前检查发现最常见的胎儿先天性呼吸道畸形。长期以来对于产前诊断CPAM的疾病转归和无症状患儿是否需要手术治疗的问题一直存在争议。因此为了权衡手术效益和风险,了解CPAM的自然病程发展和转归就显意义重大。本综述结合CPAM新的分类体系、产前和产后评估技术和最新的大样本研究,探讨CPAM的自然预后和治疗策略。     

小儿先天性肺囊肿周边肺发育的观察及其临床价值

目的 探讨先天性肺囊肿(CBC)与肺发育不全的关系及其外科治疗。方法 32例CBC患儿和18例正常对照，分别测量肺泡辐射状计数(RAC值)及单位视野内第6级以上肺小动脉数(A值)。行肺小动脉Gomori醛复红弹力纤维染色；随访病例复查胸片，测量TLC、VC。结果 CBC组RAC值及A值低于正常对照，支气管型CBC的RAC值低于肺泡型；肺小动脉内、外弹性膜可见多处断裂，管壁平滑肌变性及肥厚；随访囊肿摘除及肺叶切除术后TCL、VC分别为对照的97％—98％、85％～90％。结论 CBC导致周边肺发育不全，宜尽早手术摘除。     

Presentation and management of pulmonary sequestration with total visceral inflow and outflow

Two of the most common types of congenital thoracic malformations are congenital pulmonary airway malformations (CPAMs) and bronchopulmonary sequestrations (BPS). Here we present the first known case of a hybrid lesion, with coexisting features of an extralobar sequestration (ELS) and CPAM type 2, with arterial inflow from the celiac trunk and venous outflow to the portal vein. The clinical presentation, investigative imaging and timing of surgery are discussed.     

Redirection of Hepatic Drainage for Treatment of Pulmonary Arteriovenous Malformations Following the Fontan Procedure

The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery. In the subsequent operation, we redirected the extracardiac conduit to the innominate vein. This operation provided the affected lung with hepatic venous blood without exposing the patient to the morbidity associated with cardiopulmonary bypass.     

Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment

Pulmonary lymphatic disorders are characterized by the presence of the abnormal lymphatic tissues in the thoracic cavity, presenting clinically as chylothorax, chylopericardium, chyloptysis, interstitial lung disease and plastic bronchitis. These conditions include: neonatal chylothorax, cardiac and non-cardiac plastic bronchitis, non-traumatic chylothorax, post congenital cardiac surgery chylothorax and complex lymphatic malformations.Recently developed lymphatic imaging techniques, such as intranodal lymphangiography and dynamic contrast enhanced magnetic resonance lymphangiography demonstrated abnormal pulmonary lymphatic flow from thoracic duct into pulmonary parenchyma as a pathophysiological mechanism of these diseases. Novel minimally invasive lymphatic interventions, such as thoracic duct embolization, interstitial lymphatic embolization and surgical lympho-venous anastomosis, provide an effective treatment of these conditions.     

Primary pulmonary rhabdomyosarcoma in childhood: Clinico-biologic features in two cases with review of the literature

The cases of two children under three years of age with primary pulmonary rhabdomyosarcoma and no associated lung malformations are reported and a review of the literature is presented. In both, complete surgical removal of the tumor was performed and histologic examination revealed embryonal subtype. Flow cytometric assessment showed a tumor-cell diploid DNA content. Postoperative radio- and chemotherapy were carried out, but in spite of treatment both girls died because of disease progression, fourteen and nine months after diagnosis. The importance of associated cystic lung malformations and DNA content in predicting clinical outcome of primary pulmonary rhabdomyosarcoma is evaluated. © 1996 Wiley-Liss, Inc.     

Pulmonary venous stenosis in a premature infant with bronchopulmonary dysplasia: clinical and autopsy findings of these newly associated entities

Pulmonary venous stenosis is rare and is most commonly found in association with cardiac malformations. Recent studies have associated pulmonary venous stenosis with prematurity, especially with bronchopulmonary dysplasia, although no such case has been documented at autopsy. We report the case of a 26-week-gestation infant who required ventilation at birth and who, among other complications, developed chronic lung disease of prematurity by the age of 3 months. Imaging showed suprasystemic right-sided pressures and pulmonary venous stenosis. Despite aggressive management of respiratory status and surgical marsupialization of stenoses, the infant expired after 3 weeks. At autopsy, 3 of 4 pulmonary veins showed a fibrous ridge obstructing atrial ostia with otherwise normal anatomy. The lungs showed bronchopulmonary dysplasia, pulmonary hypertensive vascular changes, and features of venous obstruction. Pulmonary venous stenosis and bronchopulmonary dysplasia in premature infants may be pathogenetically related. Coincidence of these diseases is likely underrecognized, and careful cardiac examination in these patients is warranted.     

Hypoplastic left heart syndrome in PAGOD syndrome

Chromosomal abnormalities as well as non‐cardiac anomalies have been identified as independent risk factors for surgical morbidity and mortality in Fontan palliation. The combination of malformations consisting of pulmonary hypoplasia, agonadism (sex reversal), omphalocele, and diaphragmatic defect is compatible with pulmonary artery and lung hypoplasia, agonadism, omphalocele, and diaphragmatic defect (PAGOD). Most cases have been associated with cardiac disease, particularly hypoplastic left heart syndrome (HLHS) that is potentially destined for Fontan palliation. Reported herein is the case of a Japanese female infant diagnosed with PAGOD syndrome along with HLHS (mitral atresia and aortic atresia), in whom intractable respiratory failure manifested as bilateral eventration of the diaphragm and presumed right lung hypoplasia. These characteristic pulmonary lesions associated with the syndrome precluded use of the Fontan pathway.     

A spectrum of bronchopulmonary anomalies associated with tracheoesophageal malformations

The combination of tracheoesophageal and pulmonary malformations is unusual and reportedly carries a high mortality. We have observed six patients with esophageal atresia and tracheoesophageal fistula and one with a bronchoesophageal fistula who had associated bronchopulmonary anomalies ranging from lobar hypoplasia and agenesis to unilateral pulmonary hypoplasia or agenesis. All of the pulmonary malformations were right-sided. Vertebral or rib anomalies were present in five patients, congenital heart disease in two, imperforate anus in one and one patient had radial aplasia and Pierre Robin syndrome. The bronchopulmonary anomalies complicated the surgical care of tracheoesophageal malformations and required radiologic differentiation from aspiration pneumonia and atelectasis. Six of the seven patients survived. Mortality and morbidity were related to complications and associated cardiac anomalies as well as severity of the lung anomaly.     

Revisiting the histopathologic spectrum of congenital pulmonary developmental disorders

A retrospective analysis of lobectomy, pneumonectomy, cystectomy specimens (10 years) and stillbirth/neonatal autopsies (5 years) was carried out to analyze the histologic spectrum of congenital pulmonary developmental disorders/malformations. The autopsy data was analyzed to identify the lesions that are more diffuse and not amenable to surgical management. A total of 166 cases of pulmonary developmental disorders/ malformation were found. Out of 2,155 stillbirth/neonatal autopsies, there were 105 cases of pulmonary hypoplasia, 2 cases of congenital pulmonary lymphangiectasis, 2 cases of extralobar sequestration, and 3 cases of congenital pulmonary airway malformation (CPAM). Among the surgical specimens, there were 21 cases of CPAM, 11 cases of intralobar sequestration, 5 cases of congenital lobar emphysema and 17 cases of bronchogenic cyst. All these malformations have been described in reference to the latest updated classification of developmental disorders/malformations.     

Pulmonary sequestration

Pulmonary sequestration refers to the situation whereby a portion of lung tissue receives its blood supply from an anomalous systemic artery. Three main variants exist: intralobar, extralobar and communicating bronchopulmonary foregut malformations. Venous drainage is typically via a systemic vein, although drainage into the pulmonary veins is well documented. Pulmonary sequestrations are the second commonest congenital lung anomaly. Affected individuals often have other anomalies which are responsible for most of the mortality associated with sequestrations. Diagnosis requires a high index of suspicion particularly in any child with a chest x-ray suggesting the presence of a mass, those with recurrent chest infections and those with other anomalies seen with the pulmonary sequestration spectrum. Surgical excision is usually advised, although embolisation of the feeding vessel has a role in selected cases.