肺部先天性囊性病变的CT诊断

目的:分析肺部先天性囊性病变的CT表现,探讨其CT诊断价值。方法:回顾性分析17例经手术病理证实的肺部先天性囊性病变(先天性肺囊肿9例;先天性大叶性肺气肿2例;先天性囊性腺瘤样畸形3例,其中1例合并漏斗胸;先天性肺隔离症3例,均合并有肺囊肿;)的CT表现,包括病变的位置、大小、形态、边缘、数量、内部结构等。结果:肺囊肿多位于两下肺,单发多见,单发者形态规则,密度均匀,边缘清晰,多发囊肿形态不规则。先天性大叶性肺气肿(CLE)多位于两上肺叶,内含气体和肺纹理,边缘清晰。先天性囊性腺瘤样畸形(CCAM)形态不规则,内含气体、索条状结构。肺隔离症多位于纵隔旁,可含有气体、液体和软组织多种成分。结论:结合肺部先天性囊性病变的部位、大小、囊肿的数目、形态等CT表现,有助于提高病变的术前诊断准确率。     

先天性肺囊肿性病变60例临床病理观察

目的探讨先天性肺囊肿性病变临床病理特点以及与肺癌的关系。方法对60例外科手术切除先天性肺囊肿性病变进行临床病理学分析,并进行文献复习。结果支气管源性肺囊肿49例（81．7％）;先天性囊性腺瘤样畸形5例（8．3％）,其中1例伴发粘液性原位腺癌（国际肺癌研究协会／美国胸科学会／欧洲呼吸学会国际多学科肺腺癌分类2011年版,简称新版）,1例伴发鳞癌;肺隔离症6例（10．0％）。结论支气管源性囊肿是先天性肺囊肿性病变最常见的囊肿类型,先天性囊性腺瘤样畸形（CCAM）最少见。CCAMI型常伴有粘液细胞化生,粘液细胞增生可能属于浸润性粘液腺癌（新版分类．过去的粘液型细支气管肺泡癌）癌前病变。     

先天性肺囊性腺瘤样畸形的CT表现

目的:探讨先天性肺囊性腺瘤样畸形的CT表现。方法:回顾性分析15例先天性肺囊性腺瘤样畸形的CT表现,并与手术、病理结果对照。结果:15例先天性肺囊性腺瘤样畸形CT表现为大囊型10例、小囊型5例。病理检查见囊腔为异常增生的管腔或腺样结构,壁内被覆假复层纤毛柱状上皮8例,纤毛柱状及立方上皮7例;囊周可见平滑肌及弹性纤维环绕,15例囊壁内均未见软骨成分及腺体。结论:CT检查是诊断先天性肺囊性腺瘤样畸形的可靠方法,根据CT表现可推测其病理分型。     

Fluid-filled cystic adenomatoid malformation.

Two infants with congenital cystic adenomatoid malformation of the lung are described. The condition progressed from an unusual solid radiographic appearance to the more classical air-filled cystic appearance. The initial appearance is due to delayed emptying of the alveolar fluid either via the bronchi or the lymphatic and circulatory systems. After the fluid is removed, the typical air-filled cysts become apparent.     

Management of fetal mediastinal shift: a practical approach

The purpose of this article is to review the technique of fetal chest ultrasound screening evaluation, the diagnostic work-up in the presence of fetal mediastinal shift and which ultrasound imaging features to look for. The first step in evaluating the fetal thorax is to confirm situs. Then, a median sagittal line is drawn from a four-chamber view to assist in spatial orientation followed by echotexture analysis of the structures of the thorax in the presence of mediastinal shift. We propose a systematic approach based on the direction of the mediastinal shift and echogenicity of the compressing hemithorax. When the hemithorax contralateral to the mediastinal shift is enlarged, which is the most frequent situation, diaphragmatic hernia and macrocystic congenital cystic adenomatoid malformation are the most likely etiologies when the mass is heterogeneous. Microcystic congenital cystic adenomatoid malformation, sometimes associated with sequestration, is the most frequent etiology when the mass is homogeneous. When the hemithorax ipsilateral to the mediastinal shift is small, which is less frequent, and the contralateral hemithorax is homogeneously isoechoic, then a diagnosis of lung hypoplasia-agenesis-aplasia should be considered.     

Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient

OBJECTIVE: This study was designed to assess the effect of prenatal sonographic diagnosis on the treatment of congenital cystic adenomatoid malformation of the lung. MATERIALS AND METHODS: The medical records of 27 patients with pathologically proven congenital cystic adenomatoid malformations were retrospectively reviewed. Patients were divided into four groups based on mode of presentation: with or without abnormal findings on prenatal sonography and with or without symptoms at birth. Age at diagnosis, age at surgical intervention, complications, and length of hospital stay were recorded for each group. RESULTS: Twenty-seven patients with 31 proven congenital cystic adenomatoid malformations were included. Eleven patients underwent prenatal sonography establishing the diagnosis (6 asymptomatic at birth, 5 symptomatic), and 16 did not have a prenatal diagnosis (10 asymptomatic at birth, 6 symptomatic). In the symptomatic populations, prenatal diagnosis had no impact on age at surgery, length of stay, or surgical complication rate (p = 0.78-0.83). In the asymptomatic population, prenatal diagnosis allowed early diagnosis (p < 0.001) and resection in the asymptomatic period. It was also associated with a shorter length of stay at the time of surgical resection (mean time, 4.2 days for patients with prenatal diagnosis versus 12.9 days for those without it;p < 0.001) and with a trend toward lower serious complication rate (3 patients without prenatal diagnosis versus 1 patient with it). CONCLUSION: Prenatal sonography provides the radiologist a means to identify congenital cystic adenomatoid malformations in a population of infants who are asymptomatic at birth. Surgical intervention in the asymptomatic infant is associated with a shorter length of stay, a trend toward fewer complications, and decreased medical cost compared with intervening after symptoms develop.     

多层螺旋CT及其后处理技术对肺部先天性病变的诊断价值

目的 探讨多层螺旋CT(MSCT)及各种图像后处理技术对肺、支气管及肺血管先天性病变的诊断价值. 资料与方法 回顾性分析34例肺部先天性病变的MSCT表现,所有病例均行MSCT平扫,19例行增强扫描,常规行多平面重组(MPR)、最大密度投影(MIP)及容积再现(VR)等技术重组图像.结合手术和临床资料评价其作用. 结果 34例中,肺发育异常3例,支气管囊肿15例,囊性腺瘤样畸形2例,支气管闭锁3例,气管支气管1例,肺动静脉瘘6例,一侧肺动脉近端缺如1例,肺隔离症3例.MSCT轴位及后处理图像能清楚显示病变的部位、与相应支气管及血管的关系,血管性病变均清楚显示供血动脉及引流静脉. 结论 MSCT常规检查结合各种后处理技术能获得肺部先天性病变诊断的足够信息,对肺血管先天性疾病多能作出定性诊断.     

胎儿胸部异常的MRI诊断

目的:分析9例胎儿胸部异常的MRI表现,探讨MRI在胎儿胸部异常诊断中的补充作用。方法:对9例产前超声检查发现的胎儿胸部异常病例,经孕母及家属同意后行胎儿多平面的MRT2WI扫描,部分病例加做T1WI扫描,对胎儿异常胸部MRI表现进行研究,进一步确定胸部异常类型。结果:9例胸部异常中,6例先天性肺囊腺瘤样畸形(CCAM),3例为右肺CCAM,3例为左肺CCAM,MRI表现为患侧肺内多囊样T2WI高信号改变,病侧肺体积增大,纵隔向对侧移位;1例为左侧先天性膈疝(CDH),MRI示左侧胸腔内正常肺组织消失,呈混杂肠腔信号,肺尖少许正常肺组织受压;2例为胸腔积液,1例表现为双侧胸水、腹水、睾丸鞘膜积液,考虑为胎儿免疫性水肿,另1例为右侧胸水、腹水、皮下软组织水肿。结论:MRI作为产科胎儿超声检查的一种重要补充方法,对病变多方位的显示,具有很高的价值,给临床诊断及治疗予以很大帮助。     

Three-dimensional volume rendering of fetal MR images for the diagnosis of congenital cystic adenomatoid malformation

RATIONALE AND OBJECTIVES: Great expertise is necessary to mentally compile a series of individual two-dimensional image sections into a three-dimensional (3D) composite view that can aid in differential diagnosis. The purpose of this study was to test 3D volume-rendering techniques for differentiating congenital cystic adenomatoid malformation from congenital diaphragmatic hernia. MATERIALS AND METHODS: The authors acquired T2-weighted magnetic resonance (MR) images of a 27-week fetus in the sagittal plane and then applied the 3D volume-rendering method to the MR image data sets to obtain a composite 3D image. RESULTS: It was unclear on the MR images whether the intestines were situated above or below the diaphragm. The composite image showed that the intestines were not herniated into the chest, and this facilitated a diagnosis of congenital cystic adenomatoid malformation rather than congenital diaphragmatic hernia. CONCLUSION: The 3D volume-rendering techniques aided in the assessment of fetal organ structure and could be applied also to preoperative simulation and planning of fetal surgery.     

胎儿先天性肺囊腺瘤样畸形的MRI诊断与鉴别诊断

目的:探讨MRI在胎儿先天性肺囊腺瘤样畸形(CCAM)诊断与鉴别诊断中的应用价值.方法:36例孕妇,年龄21～38岁,平均29岁;孕龄20～31周,平均24周.产前常规行超声(US)检查后24～48h内行MR检查,采用二维快速平衡稳态采集 (2D-FIESTA) 序列、单次激发快速自旋回波(SSFSE)序列,行胎儿颅脑...     

Vermehrte Transparenz in der D��nnschicht-CT des Lungenparenchyms

Areas with reduced density are a common finding with thin-slice computed tomography (CT) examinations of lung parenchyma. Diffuse and localized patterns can be differentiated. The most frequent cause for a diffuse decrease in density is pulmonary emphysema which can be classified into centrilobular, panlobular and paraseptal emphysema based on the distinct anatomical features. A mosaic pattern (due to mosaic perfusion or air trapping) is also characterized by a diffuse decrease in density. Focal cystic changes can be differentiated from emphysematous changes and mosaic patterns which do not reveal wall structures. Langerhans cell histiocytosis and lymphangioleiomyomatosis are the most frequent cystic diseases of the lung parenchyma. Based on the geometry and anatomical distribution of the cysts a differentiation is mostly possible. Rare diseases leading to a reduced lung density include the Birt-Hogg-Dubé syndrome, lymphoid interstitial pneumonia as well as congenital diseases or diseases which are acquired during early childhood, such as cystic adenomatoid malformation or Swyer-James syndrome.     

Increased transparency in thin-slice computed tomography of lung parenchyma

Areas with reduced density are a common finding with thin-slice computed tomography (CT) examinations of lung parenchyma. Diffuse and localized patterns can be differentiated. The most frequent cause for a diffuse decrease in density is pulmonary emphysema which can be classified into centrilobular, panlobular and paraseptal emphysema based on the distinct anatomical features. A mosaic pattern (due to mosaic perfusion or air trapping) is also characterized by a diffuse decrease in density. Focal cystic changes can be differentiated from emphysematous changes and mosaic patterns which do not reveal wall structures. Langerhans cell histiocytosis and lymphangioleiomyomatosis are the most frequent cystic diseases of the lung parenchyma. Based on the geometry and anatomical distribution of the cysts a differentiation is mostly possible. Rare diseases leading to a reduced lung density include the Birt-Hogg-Dubé syndrome, lymphoid interstitial pneumonia as well as congenital diseases or diseases which are acquired during early childhood, such as cystic adenomatoid malformation or Swyer-James syndrome.     

Evaluation of pulmonary parenchymal disease by magnetic resonance imaging

Thirty-eight patients with a wide variety of different disorders of the lung have been imaged using magnetic resonance. The disorders studied include congenital abnormalities, acute and chronic inflammation, vascular disorders, primary and secondary tumours of the lung, haematomas and infarcts. Magnetic resonance imaging identified all lesions seen on chest radiographs, but it is not quite as sensitive as computed tomography is for detection of very small abnormalities. Magnetic resonance may provide information to aid in differentiation of a wide variety of disorders causing diffuse pulmonary abnormality and might also prove helpful in differentiating acute inflammation from pulmonary collapse or fibrosis. In some disorders, such as lobar emphysema, cystic adenomatoid malformation and sequestration, the findings on the magnetic resonance scan alone will permit a correct diagnosis to be made in many cases. It is believed that magnetic resonance imaging has more potential for pulmonary imaging than has previously been recognised.     

Case report: Congenital cystic adenomatoid malformation of an entire lung in a 33-year-old man: a case report and review of the literature

Congenital cystic adenomatoid malformation (CCAM) is a rare developmental, non-hereditary, hamartomatous abnormality of the lung with adenomatoid proliferation of cysts resembling bronchioles. It is usually unilateral with involvement of a single lobe. We report an unusual case of a 33-year-old man who presented with this disease with the involvement of an entire lung. We discuss the patient's pathogenesis, histological findings, clinical presentation, imaging characteristics and treatment, and review the associated literature.     

Prenatal thoracoabdominal tumor mimicking pulmonary sequestration: a diagnosis dilemma

Thoracoabdominal masses are among the many congenital anomalies being detected in fetus. The differential diagnosis of such anomalies include cystic adenomatoid malformation, pulmonary sequestration, neuroblastoma, lymphangioma, congenital diaphragmatic hernia, and bronchial atresia. We report a fetal thoracoabdominal mass that proved to be an angioma that was treated by partial embolization. Complete regression was observed. Received: 2 November 1999 Revised: 3 April 2000 Accepted: 4 April 2000     

Congenital cystic adenomatoid malformation presenting as repeated pneumonia in a young adult

Computed tomography (CT) of an 18-year-old man with a history of repeated episodes of pneumonia demonstrated consolidation containing multiple cysts in the right lower lobe. Additional CT angiography and thin-section CT of the lesion revealed neither a supplying systemic artery nor an abnormal bronchus. Lobectomy was performed, and histological examination showed many cysts resembling enlarged bronchioles, diagnosed as congenital cystic adenomatoid malformation.     

Congenital cystic adenomatoid malformation

Congenital cystic adenomatoid malformation of the lung is a rare lesion that typically manifests as neonatal respiratory distress secondary to progressive expansion of the affected lung. Three distinct types have been described based on the size of the cysts and the microscopic appearance. Type I lesions are characterized by large cysts of varying sizes (measuring more than 2 cm in diameter), type II lesions typically contain cysts of a more uniform size (not exceeding 2 cm in diameter), and type III lesions contain microscopic cysts. Radiologic findings include single or multiple large cysts, multiple small cysts of uniform size, and solid-appearing masses. The treatment of choice is excision of the affected lobe. The prognosis is favorable in the absence of pulmonary hypoplasia, fetal hydrops, or associated congenital anomalies.     

Role of multi slice computed tomography in the evaluation of congenital anomalies of tracheobronchial tree and lungs

ObjectiveTo evaluate the role of MSCT in the evaluation of congenital anomalies of tracheobronchial tree and lungPatients and methodsTwenty nine patients with congenital anomalies of tracheobronchial tree and lung were examined using four and six MDCT.ResultsSeven patients (24%) had congenital cystic adenomatoid malformation where successful differentiation of the type was possible. Three patients (10.3%) with bronchopulmonary sequestration were categorized as two (7%) intralobar and one (3.5%) extralobar sequestrations. Four patients (14%) had congenital lobar overinflation. Four patients had Scimitar syndrome with right pulmonary artery hypoplasia, right sided lung hypoplasia, anomalous arterial supply and anomalous pulmonary venous drainage together with pulmonary hypertension. Abnormal bronchial anatomy was revealed in two patients with pulmonary isomerism and one patient with situs inversus. Bronchogenic cyst, tracheal bronchus, Kertagner's syndrome, pulmonary agenesis and horse shoe lung were seen in one patient each. Dynamic MSCT shows the lunate configuration of the trachea during forced expiration in one patient with tracheomalacia. In one patient with tracheo-esophageal fistula, the extent of fistulous tract and the resulting bronchial aspiration were demonstrated.ConclusionMSCT proved to be indispensable as a preoperative planning modality and an essential investigation for congenital anomalies of the lung and tracheobronchial tree.     

Sudden neonatal death from congenital cystic adenomatoid malformation

We report a case of congenital cystic adenomatoid malformation (CCAM) of the lungs resulting in sudden death immediately after birth. The case is extremely unusual because of the diffuse bilateral involvement. The extensive involvement of both lungs could explain the abrupt onset of the symptoms and the ineffectiveness of resuscitation attempts. The presence of cartilage as a part of the malformation adds interest to the case, since it is seldom found in this malformation and to the best of ¶our knowledge has been reported only exceptionally in a ¶type II CCAM.