Congenital lung malformations are rare and vary widely in their clinical presentation and severity, depending mostly on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods. Recently, there has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. The clinical manifestation of these malformations varies from respiratory distress in the immediate postnatal period to an incidental finding on chest X-rays. Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. Lobectomy is the procedure of choice and yields excellent long-term results. This article describes the principal congenital lung malformations, their diagnosis, and the controversies regarding treatment.

Keywords: Cystic adenomatoid malformation of lung, congenital; Bronchopulmonary sequestration; Pulmonary surgical procedures; Diagnosis.

Objective: To determine the main congenital lung malformations treated and the principal diagnostic methods employed, as well as the indications for surgical treatment and the results obtained, at a referral facility for pediatric thoracic surgery. Methods: We reviewed the medical charts of 52 patients anatomopathologically diagnosed with congenital lung malformations and who had been submitted to pulmonary resection between January of 1997 and December of 2006. Exclusion criteria were age ≥ 12 years and incomplete clinical data. The final sample comprised 35 patients. Results: In this sample, the mean age was 31 months, and there was a predominance of males (n = 21). The anatomopathological findings were cystic adenomatoid malformation (n = 14), congenital lobar emphysema (n = 13), pulmonary sequestration (n = 8) and arteriovenous malformation (n = 1). The most common type of lung resection was left lower lobectomy (in 25.71%) followed by different types of segmentectomy (in 22.85%), left upper lobectomy (in 22.85%), right upper lobectomy (in 14.28%), right lower lobectomy (in 8.57%) and middle lobectomy (in 5.71%). Of the 35 patients, 34 (97.14%) were submitted to closed pleural drainage, with a mean duration of thoracic drainage of 3.9 days. Ten patients (28.5%) presented with postoperative complications. There were no deaths in our sample. Conclusions: Pulmonary resection for the treatment of congenital lung malformations is a safe procedure, presenting low morbidity and no mortality at a referral facility for pediatric thoracic surgery.

Keywords: Cystic adenomatoid malformation of lung, congenital; Bronchopulmonary sequestration; Pneumonectomy; Pulmonary surgical procedures; Pulmonary emphysema.

Congenital lobar emphysema is a disease in which symptoms often begin during the first six months of age. In this report, the authors present the case of a twenty-year-old woman whose symptoms started when she had a spontaneous pneumothorax, an exceptional occurrence. The possible etiologies for that congenital lung malformation and the reasons for the performance of a conservative treatment are discussed.

Keywords: Pulmonary emphysema. Pneumothorax. Abnormalities.

Objective: To evaluate clinical features of malformations of lung and mediastinum. Methods: Retrospective analysis of 51 patients with malformations identified in the author's division between 1982 and 1997. They verified the clinical manifestations, diagnostic procedures, and treatment. Results: The most frequent anomaly was esophageal atresia/tracheoesophageal fistula (12), followed by vascular ring (11), congenital lobar emphysema (7), diaphragmatic hernia/eventration (7), bronchogenic cyst (5), pulmonary hypoplasia (4), pulmonary sequestration (3), and arterial-venous fistula (2). Age at diagnosis varied from birth to 10 years. Most of them exhibited symptoms at first month, some even at birth. The main respiratory symptoms were tachypnea, coughing, retractions, dyspnea, wheezing, pneumonia and rales. Chest X-rays with barium swallow were most important for diagnosis. When chest radiology was inconclusive, CT scans were next in achieving diagnosis. Surgical intervention was the effective treatment in 86% of cases. Conclusions: Lung and mediastinal congenital malformations with respiratory symptoms require adequate diagnostic evaluation and immediate intervention. The most frequent respiratory symptoms were coughing and tachypnea and chest X-rays with barium swallow was the main diagnostic method. Survival is good and mortality is secondary to associated malformations.

Keywords: Bronchopulmonary malformations. Respiratory distress. Retrospective studies.

Cystic adenomatoid malformation of the lung is a rare form of congenital pulmonary disease. It has been found in all lobes, it is rarely bilateral, and is associated to hydramnion, placental edema, and fetal hydrops. It is divided into three types, with different clinical and histologic characteristics. Intrauterine diagnosis is possible by means of ultrasound examination, and the treatment of choice is lobectomy. The authors describe four cases seen at their hospital and review the literature.

Keywords: Cystic adenomatoid malformation. Congenital malformation. Lung.

To review the cases of patients with congenital lobar emphysema (CLE) submitted to surgical treatment at two university hospitals over a 30-year period. Methods: We reviewed the medical records of children with CLE undergoing surgical treatment between 1979 and 2009 at the Botucatu School of Medicine Hospital das Clínicas or the Mogi das Cruzes University Hospital. We analyzed data regarding symptoms, physical examination, radiographic findings, diagnosis, surgical treatment, and postoperative follow-up. Results: During the period studied, 20 children with CLE underwent surgery. The mean age at the time of surgery was 6.9 months (range, 9 days to 4 years). All of the cases presented with symptoms at birth or during the first months of life. In all cases, chest X-rays were useful in defining the diagnosis. In cases of moderate respiratory distress, chest CT facilitated the diagnosis. One patient with severe respiratory distress was misdiagnosed with hypertensive pneumothorax and underwent chest tube drainage. Only patients with moderate respiratory distress were submitted to bronchoscopy, which revealed no tracheobronchial abnormalities. The surgical approach was lateral muscle-sparing thoracotomy. The left upper and middle lobes were the most often affected, followed by the right upper lobe. Lobectomy was performed in 18 cases, whereas bilobectomy was performed in 2 (together with bronchogenic cyst resection in 1 of those). No postoperative complications were observed. Postoperative follow-up time was at least 24 months (mean, 60 months), and no late complications were observed. Conclusions: Although CLE is an uncommon, still neglected disease of uncertain etiology, the radiological diagnosis is easily made and surgical treatment is effective.

Keywords: Respiratory system abnormalities; Congenital abnormalities; Pulmonary surgical procedures; Pulmonary emphysema.

Objective: To retrospectively analyze the medical charts of patients with pulmonary malformations submitted to surgical treatment and to investigate the clinical evolution prior to the definitive diagnosis. Methods: We analyzed the medical charts of patients with pulmonary malformations operated on at the São Paulo Hospital-Federal University of São Paulo/Paulista School of Medicine-from 1969 to 2004. Each medical chart was analyzed as to the following aspects: clinical profile; diagnosis; previous treatment; surgical treatment; and nosocomial complications. The inclusion criteria were having received a diagnosis of pulmonary malformation, having undergone pulmonary resection, and chart data being complete. Results: The analysis of the medical charts revealed that 60 patients diagnosed with pulmonary malformations-27 cases of bronchogenic cyst, 14 cases of congenital lobar emphysema, 10 cases of pulmonary sequestration, and 9 cases of cystic adenomatoid malformation-underwent surgery. Ages ranged from 4 days to 62 years (mean, 17.9 years). There was a predominance of males (55%). Ninety-two percent of the patients presented symptoms (mean duration, 15.37 months). Of the 60 patients undergoing surgery, 27 (45%) received preoperative home or hospital treatment with antibiotics. Regarding complications, we observed that morbidity was 23%, and mortality was 3.3%. Surgical times ranged from 1 to 8 h (mean, 3.2 h). Conclusions: Misdiagnosis or delayed diagnosis of pulmonary malformations resulted in unnecessary treatments and hospitalizations, as well as in frequent, recurrent infectious complications. We believe that the definitive treatment is surgery, which is curative and has low morbidity and mortality rates.

Keywords: Bronchogenic cyst; Bronchopulmonary sequestration; Cystic adenomatoid malformation of lung, congenital; Respiratory tract infections; Thoracic surgery.

Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital malformations. When the patient presented symptoms, including cough, wheezing, and dyspnea, with no clinical improvement after a period of 30 days, imaging studies were conducted and the diagnosis was made.

Keywords: Congenital abnormalities; Respiratory tract diseases; Bronchoscopy.

This review aimed to provide an overview of pulmonary arteriovenous malformations, including the major clinical and radiological presentations, investigation, and treatment algorithm of the condition. The primary etiology of pulmonary arteriovenous malformations is hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, with mutations in the ENG gene on chromosome 9 (HHT type 1) or in the ACVRL1/ALK1 complex (HHT type 2). Epistaxis should always be evaluated when repeated, when associated with anemia, and in some cases of hypoxemia. In the investigation, contrast echocardiography and chest CT are essential for evaluating this condition. Embolization is the best treatment choice, especially for correction in cases of hypoxemia or to avoid systemic infections. Finally, disease management was addressed in special conditions such as pregnancy. CT follow-up should be performed every 3-5 years, depending on the size of the afferent and efferent vessels, and antibiotic prophylactic care should always be oriented. Ultimately, knowledge of the disease by health professionals is a crucial point for the early diagnosis of these patients in clinical practice, which can potentially modify the natural course of the disease.

Keywords: Telangiectasia, hereditary hemorrhagic; Arteriovenous malformations; Lung.

Objective: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. Methods: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). Results: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. Conclusions: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.

Keywords: Telangiectasia, hereditary hemorrhagic; Arteriovenous malformations/diagnosis; Arteriovenous malformations/therapy.

Pulmonary sequestration is a rare congenital anomaly, characterized by nonfunctional embryonic pulmonary tissue. Pulmonary sequestration accounts for 0.15-6.40% of all congenital pulmonary malformations. This anomaly, which is classified as intralobar or extralobar, involves the lung parenchyma and its vascularization. We report the case of a 56-year-old male presenting with hemoptysis. A chest X-ray showed an area of opacity behind the cardiac silhouette in the base of the left hemithorax. Chest CT scans with intravenous contrast revealed pulmonary sequestration. The patient underwent surgery, in which the anomalous tissue was successful resected. The postoperative evolution was favorable, and the patient was discharged to outpatient treatment.

Keywords: Hemoptysis; Bronchopulmonary sequestration; Tomography, spiral computed.

Objective: To analyze the outcomes of patients undergoing repair of congenital tracheal stenosis. Methods: This was a retrospective review of congenital tracheal stenosis patients treated between 2001 and 2007 at the University of São Paulo School of Medicine Hospital das Clínicas in São Paulo, Brazil. Results: Six boys and one girl (age at diagnosis ranging from 28 days to 3 years) were included. Five of the patients also had cardiac or major vessel malformations. The stenosis length was short in three patients, medium in one and long in three. The techniques used were pericardial patch tracheoplasty in three patients, resection and anastomosis in two, slide tracheoplasty in one and vascular ring correction in one. One patient died during surgery due to hypoxia and hemodynamic instability, and one died from septic shock on postoperative day 11. Other complications included pneumonia, arrhythmia, stenosis at the anastomosis level, residual stenosis, granuloma formation and malacia. The mean follow-up period was 31 months; four patients were cured, and one required the use of a T-tube to maintain airway patency. Conclusions: Congenital tracheal stenosis is a curable disease. However, its repair is complex and is associated with high rates of morbidity and mortality.

Keywords: Tracheal stenosis/congenital; Tracheal diseases; Surgical procedures, operative.

Bronchial atresia is a rare, congenital anomaly characterized by the presence of bronchocele with distal hyperinflation. The authors report two cases of segmental bronchial atresia and describe the clinical and roentgenographic findings supporting the diagnosis in the absence of other invasive diagnostic modalities or surgical exploration.

Keywords: pulmonary atresia; bronchi; X-ray computed tomography

Objective: Respiratory complications are the main causes of morbidity and mortality in patients with neuromuscular disease (NMD). The objectives of this study were to determine the effects that routine daily home air-stacking maneuvers have on pulmonary function in patients with spinal muscular atrophy (SMA) and in patients with congenital muscular dystrophy (CMD), as well as to identify associations between spinal deformities and the effects of the maneuvers. Methods: Eighteen NMD patients (ten with CMD and eight with SMA) were submitted to routine daily air-stacking maneuvers at home with manual resuscitators for four to six months, undergoing pulmonary function tests before and after that period. The pulmonary function tests included measurements of FVC; PEF; maximum insufflation capacity (MIC); and assisted and unassisted peak cough flow (APCF and UPCF, respectively) with insufflations. Results: After the use of home air-stacking maneuvers, there were improvements in the APCF and UPCF. In the patients without scoliosis, there was also a significant increase in FVC. When comparing patients with and without scoliosis, the increases in APCF and UPCF were more pronounced in those without scoliosis. Conclusions: Routine daily air-stacking maneuvers with a manual resuscitator appear to increase UPCF and APCF in patients with NMD, especially in those without scoliosis.

Keywords: Neuromuscular diseases; Cough; Respiratory function tests; Respiratory therapy.

Lung volume reduction surgery is intended to improve pulmonary function in selected patients with severe emphysema by improving diaphragmatic and chest wall mechanisms. An increasing body of scientific evidence demonstrates that lung volume reduction surgery provides significant palliation, with long-run benefits for at least two years. Measurements of pulmonary function have also confirmed significant improvement in objective physiologic parameters such as airway obstruction, elastic recoil, and gas exchange. However, some questions concerning these issues have not been answered yet: long-term efficacy, patient's outcome predictors, cost-benefit analyses, and clarification of the indication for different surgical approaches.

Keywords: Emphysema. Lung reduction surgery.

The ischemia-reperfusion injury is a common pathophysiologic phenomenon in many diseases seen in daily clinical practice. The lung can be affected both directly, as in pulmonary edema after lung transplant or after resolution of pulmonary thromboembolism, and indirectly, as in shock states or after reperfusion injury in distant organs as intestine or lower extremities. The latter situation can occur secondary to aortic occlusion for surgical procedures. Many mediators have been implicated in the ischemia-reperfusion injury: oxygen free radicals, lipid products as thromboxane, adhesion molecules involved in leukocyte-endothelial interaction, tumor necrosis factor, among others. Therapeutic strategies for the reperfusion injury are used only at the experimental level and in very few clinical studies. Antioxidants, inhibitors of lipid mediators and of the leukocyte-endothelial interaction and promoters of blood flow in post-ischemic vessels have been used.

Keywords: Lung. Injury. Reperfusion. Ischemia.