OBJECTIVETo study the etiology, pathogenesis, clinicopathologic characteristics, prognosis and treatment of congenital pulmonary airway malformation (CPAM).

METHODSEighteen cases of CPAM were enrolled into the study. The clinical history, autopsy findings and immunohistochemical results were evaluated, with review of literature. The pathogenetic mechanism, pathologic features and differential diagnosis of CPAM were studied.

RESULTSHistologic examination showed that 2 cases were classified as Stocker type I, 12 cases as type II, and 4 cases as type III. The lesion was unilateral and involved single lobe in 13 cases. The remaining 5 cases had bilateral diseases. Of the 18 cases studied, 12 cases showed single organ involvement and 6 cases had malformations affecting multiple organs. The associated malformations included cardiac anomalies (4 cases), polycystic kidney with gastrointestinal atresia (1 case) and nuchal cystic hygroma with hydrothorax (1 case).

CONCLUSIONSCPAM is a rare pulmonary disorder. The etiology of this non-neoplastic condition is unknown. Imaging analysis is a valuable tool to suggest CPAM, while definite diagnosis requires pathologic examination. The overall prognosis is determined by the presence of associated malformations, fetal hydrops and pulmonary hypoplasia.

Abnormalities, Multiple ; pathology ; Autopsy ; Fetus ; abnormalities ; Humans ; Hydrops Fetalis ; Lung ; abnormalities

Ehlers-Danlos Syndrome ; pathology ; Humans ; Lung ; abnormalities ; pathology ; Male ; Patella ; abnormalities

OBJECTIVE: To define the MRI findings of congenital high airway obstruction sequence (CHAOS) in a series of fetuses. MATERIALS AND METHODS: Prenatal fetal MR images were reviewed in seven fetuses with CHAOS at 21 to 27 weeks of gestation. The MRI findings were reviewed. The MRI parameters evaluated included the appearance of the lungs and diaphragm, presence or absence of hydrops, amount of amniotic fluid, airway appearance, predicted level of airway obstruction, and any additional findings or suspected genetic syndromes. RESULTS: All the fetuses viewed (7 of 7) demonstrated the following MRI findings: dilated airway below the level of obstruction, increased lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, massive ascites, centrally positioned and compressed heart, as well as placentomegaly. Other frequent findings were anasarca (6 of 7) and polyhydramnios (3 of 7). MRI identified the level of obstruction as laryngeal in five cases and tracheal in two cases. In four of the patients, surgery or autopsy confirmed the MRI predicted level of obstruction. Associated abnormalities were found in 4 of 7 (genetic syndromes in 2). Postnatal radiography (n = 3) showed markedly hyperinflated lungs with inverted or flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces and tracheotomy. Two fetuses were terminated and one fetus demised in utero. Four fetuses were delivered via ex utero intrapartum treatment procedure. CONCLUSION: MRI shows a consistent pattern of abnormalities in fetuses with CHAOS, accurately identifies the level of airway obstruction, and helps differentiate from other lung abnormalities such as bilateral congenital pulmonary airway malformation by demonstrating an abnormally dilated airway distal to the obstruction.
Abnormalities, Multiple ; Airway Obstruction/congenital/*pathology ; Ascites/pathology ; Diaphragm/abnormalities ; Female ; Fetal Diseases/*pathology ; Humans ; Lung/pathology ; *Magnetic Resonance Imaging ; Placenta Diseases/pathology ; Pregnancy ; *Prenatal Diagnosis ; Retrospective Studies

OBJECTIVEThe development of pulmonary vascular bed is strongly flow-dependent. Abnormal pulmonary blood flow leads to pulmonary pathological changes. This study aimed to observe the pathological changes of small pulmonary arteries and alveoli in complex congenital heart defect with diminished pulmonary blood flow but without aortopulmonary collateral artery (APCA) and patent ductus arteriosus (PDA) in infants and young children.

METHODSAutopsy pulmonary specimens obtained from 5 children who died of non-cardiovascular diseases were used as the control group (age: 4-18 months). Fifty-six children (age: 4-36 months) with complex congenital heart defect with diminished pulmonary blood flow but without APCA and PDA served as the study group, including 34 cases of tetralogy of Fallot, 7 cases of double outlet right ventricle with pulmonary stenosis, 9 cases of single ventricle with pulmonary stenosis, 4 cases of tricuspid atresia with pulmonary stenosis and 2 cases of complete atrioventricular septal defect with pulmonary stenosis. Pulmonary specimen sections were stained by hematoxylin-eosin and Weigert-Van Gieson. Percentage of media thickness (MT%), percentage of media section area (MS%), number of small arterial per square centimeter (APSC), mean alveolar number (MAN), mean linear intercept (MLI), proportion of parenchyma area in total area (PPA%) and alveolar to small arterial ratio per unit area (AAR) were measured by morphologic quantitative analysis.

RESULTSMT% (10.93+/-2.87% vs 15.08+/-2.51%), MS% (18.97+/-5.56% vs 25.04+/-3.87%) and APSC (202.43+/-67.45 vs 441.69+/-65.29) decreased significantly in the study group compared with the control group (P<0.01). The internal diameter of small pulmonary artery (80.26+/-21.57 microm vs 58.53+/-10.29 microm; P<0.05), AAR (46.59+/-14.43 vs 34.46+/-4.98; P<0.01) and MLI (144.98+/-44.87 microm vs 108.39+/-20.76 microm; P<0.05) increased significantly compared with the control group.

CONCLUSIONSThe media of small pulmonary arteries becomes thinner, the lumen of small pulmonary arteries becomes larger, and the number of small arterial per square centimeter and the mean alveolar number are reduced in infants and young children with complex congenital heart defect with diminished pulmonary blood flow but without APCA and PDA.

Aorta ; abnormalities ; Child, Preschool ; Collateral Circulation ; Ductus Arteriosus, Patent ; pathology ; Female ; Heart Defects, Congenital ; pathology ; Humans ; Infant ; Lung ; pathology ; Male ; Pulmonary Artery ; abnormalities ; Pulmonary Circulation

We reviewed the data of 38 neonates who died of respiratory failure. Paraffin sections of the autopsy lung samples were examined with HE staining or immunolabeling for CD34, CD68 and CK to observe the development of the pulmonary vessels and detect potential pulmonary vascular diseases (PVDs). Five cases were identified to have PVDs, including pulmonary hypertensive vascular remodeling in 3 cases and alveolar capillary dysplasia in 2 cases. The result indicated that PVD was one of the important reasons for respiratory failure in these neonates.
Death ; Humans ; Infant, Newborn ; Lung ; pathology ; Lung Diseases ; diagnosis ; Persistent Fetal Circulation Syndrome ; pathology ; Pulmonary Alveoli ; abnormalities ; pathology ; Respiratory Insufficiency ; mortality ; Vascular Diseases ; diagnosis ; Vascular Remodeling

OBJECTIVETo investigate the relationship between pulmonary pathological features and clinical physiology of congenital heart defects (CHD) with decreased pulmonary artery blood flow.

METHODSBetween July 2001 and May 2006, 18 patients with CHD with decreased pulmonary artery blood flow undergoing palliative or definitive repair and having lung biopsy intraoperatively were enrolled in this study. The patients' age was 0.4 - 8.0 years, and body weight was 6.0 - 20.0 kg. The method of semi-quantitative morphometric technique and an image analyzer were applied to measure the following indices of pulmonary microvessels: the percentage of media thickness (MT%), the percentage of media section area (MS%) and numbers of microvessels per square centimeter (VPSC). The diameters of left pulmonary artery (LPA) and right pulmonary artery (RPA) were measured with two-dimensional echocardiography. The percutaneous oxygen saturation (SpO(2)), hemoglobin concentration (HB) and hematocrit value (HCT) were examined and recorded preoperatively.

RESULTSThere was a significant negative correlation between SpO(2) and HCT or Hb (R(2) = 0.4914, P = 0.001 and R(2) = 0.5505, P < 0.001), the variation trend of these three variables was linked. There was a negative correlation between SpO(2) and the body weight (R(2) = 0.2208, P = 0.049), which is in accordance with clinical features of aggravated process of cyanosis and hypoxia. The morphological observation of lung biopsy specimens indicated that most of peripheral pulmonary arteries were distended, irregular and their walls were uneven, and "lake" type of pulmonary AV malformations were observed. There was a positive correlation between VPSC and the body weight or BSA (R(2) = 0.5472, P < 0.001 and R(2) = 0.5233, P = 0.001). There was a significant correlation between VPSC and LPA or RPA (R(2) = 0.4312, P = 0.003 and R(2) = 0.2463, P = 0.036). It was shown that the diameter of central pulmonary arteries could be a reflection of peripheral pulmonary artery growth. The diameter of LPA also correlated with the diameter of RPA (R(2) = 0.286, P = 0.022).

CONCLUSIONSFor patients with congenital heart defects with decreased pulmonary blood flow, the pulmonary pathological changes are the bases of their clinical physiologic features. It is suggested that they should be treated in their earlier stage of life.

Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; pathology ; physiopathology ; Humans ; Infant ; Lung ; blood supply ; Male ; Pulmonary Artery ; abnormalities ; pathology ; physiopathology

We report a case of unusual bronchopulmonary foregut malformation composed of a mediastinal bronchogenic cyst with sequestrated lung tissue and communicating tubular esophageal duplication associated with complete pericardial defect. A 18-yrold man, who had suffered from dry cough and mild dyspnea, was admitted because of an incidentally detected chest mass. A computed tomography scan demonstrated a cystic mass with an air fluid level connected with esophagus in the middle mediastinum. The surgically resected mass was a pleural invested accessory lobe of the lung (8.0x7.0x4.5 cm) connected with the esophageal wall by a tubular structure (3.0 cm in length and 2.0 cm in diameter). A complete left pericardial defect was also identified. Histologically, the cystic wall was composed of fibrovascular connective tissue with a smooth muscle layer, mixed seromucous glands and cartilage, and the inner surface of the cyst was lined by ciliated pseudostratified columnar epithelium. The inner surface of the tubular structure was lined by non-keratinizing or keratinizing squamous epithelium, and the wall contained submucosal mucous glands, muscularis mucosa, and duplicated muscularis propria. This case is important in understanding the embryological pathogenesis of the variable spectrum of the bronchopulmonary foregut malformation.
Adolescent ; Bronchogenic Cyst/*complications/*diagnosis ; Digestive System/pathology ; Esophageal Cyst/diagnosis/pathology ; Esophageal Diseases/*complications/*diagnosis ; Esophagus/abnormalities/*pathology ; Humans ; Lung/abnormalities/pathology ; Male ; Pericardium/pathology ; Tomography, X-Ray Computed

Bronchopulmonary Sequestration ; classification ; pathology ; surgery ; Humans ; Infant ; Lung ; diagnostic imaging ; Male ; Radiography ; Respiratory System Abnormalities ; diagnosis ; surgery

Abnormalities, Multiple ; Bronchial Diseases ; complications ; Bronchoscopy ; Child ; Child, Preschool ; Female ; Foreign Bodies ; complications ; Humans ; Infant ; Lung ; diagnostic imaging ; pathology ; Male ; Radiography ; Respiratory System Abnormalities ; complications ; Respiratory Tract Diseases ; etiology ; pathology

Epidermal Growth Factor ; physiology ; Fetal Diseases ; therapy ; Hernia, Diaphragmatic ; complications ; therapy ; Hernias, Diaphragmatic, Congenital ; Humans ; Lung ; abnormalities ; pathology ; Prenatal Diagnosis ; Pulmonary Surfactants ; pharmacology ; Tumor Necrosis Factor-alpha ; physiology