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.

Idiopathic tracheal stenosis is uncommon. Herein, we report four cases, all presenting a similar clinical profile: diagnosed through bronchoscopy and having a history of being treated unsuccessfully for bronchospasm. Three of the patients were treated with dilatation and an oral corticosteroid. One of those three underwent tracheoplasty. In the remaining patient, the stenosis was more extensive (2 cm, with a 70% reduction in the size of the lumen), and dilatation was not an option. Therefore, that patient underwent laryngotracheal resection. In all four patients, the evolution was favorable. Idiopathic tracheal stenosis should be contemplated in cases of 'bronchitis' that are not resolved using conventional treatments. Bronchoscopy and dilatation have provided satisfactory results. Occasionally, laryngotracheal reconstruction is necessary.

Keywords: Trachea/surgery; Constriction; Pathologic; Case reports [publication type].

Necrotizing pneumonia is a serious complication of lobar pneumonia. Four children aged between ten months and three years were admitted with acute bacteremic pneumonia and developed sepsis, pleural effusion, and respiratory distress despite adequate antibiotic treatment and chest tube drainage. Decortication and pulmonary resection were performed in all of them. The observed complications were bronchopleural fistula and one death. The emergency pulmonary resection is indicated when pulmonary necrosis is associated to sepsis and massive bronchopleural fistula. In such circumstances, morbidity and mortality are higher than in other conditions.

Keywords: staphylococcal pneumonia; thoracotomy; lung, surgery

Lymphangiomatosis, a rare diseases of controversial origin, occurs in individuals of any age, regardless of gender, but is predominantly seen in younger individuals. It often presents with thoracic involvement, although, the bones, spleen and liver can also be affected. Histologically, the pulmonar involvement includes proliferation, complex anastomoses and secondary dilatation of the lymphatic vessels. Clinically, the presentation is variable. Although radiographic findings can be suggestive of the disease, the final diagnosis is made histologically. We report two cases of lymphangiomatosis, both in females: one was oligosymptomatic and is being treated for the disease; the other had a more progressive form, was diagnosed quite late and ultimately died of the disease.

Keywords: Lymphangioma; Lymphatic System; Pleural Effusion; Lymphatic Diseases; Lymphatic System/abnormalities; Lung Diseases, Interstitial.

Objective: The aim of this study was to present the evolution of four patients presenting pulmonary alveolar proteinosis and treated at the State University of Londrina School of Medicine. We focus on the importance of whole-lung lavage as the treatment of choice. Methods: A retrospective study of four patients, three females and one male, 22 to 34 years old, presenting similar histories of progressive dyspnea and dry cough. The final diagnosis was established through open-lung biopsy. Three of the patients underwent whole-lung lavage in the Department of Surgery. The procedures were performed under general anesthesia and using a double-lumen endotracheal tube. Results: One patient presented spontaneous regression of the pulmonary alveolar proteinosis without the need for whole-lung lavage. In the other three cases, the number of lavages varied: in one patient, a single unilateral lavage resulted in complete remission of the bilateral process; in another patient, three lavages yielded no significant improvement; in the remaining patient, four lavages provided intervening periods of transient improvement. Conclusion: In the cases evaluated, whole-lung lavage proved an efficient treatment for pulmonary alveolar proteinosis. Although some patients presented a certain resistance to the procedure, it might lead to complete remission of the disease in others.

Keywords: Pulmonary alveolar proteinosis /diagnosis; Bronchoalveolar lavage; Macrophages, alveolar;

Rhodococcus equi, the principal agent of rhodococcosis, is a pleomorphic, gram-positive, aerobic coccus bacillus that infects humans by inhalation or through a transcutaneous route. It is clinically manifested as a pulmonary abscess. The first two Brazilian cases of rhodococcosis are reported on. Both patients were immunocompromised and showed pulmonary infection. The first patient had AIDS and cavitating pneumonia in the left upper lobe, that was fatal. The second case presented Goodpasture syndrome and was under chronic corticotherapy. He displayed a cavitating nodular lesion in the right upper lobe, that was successfully treated with sulfametoxazol-trimethoprim.

Keywords: Actinomycetal infections. Pulmonary tuberculosis. Acquired immunodeficiency syndrome. Goodpasture syndrome. Brazil.

Objective: To report the results obtained in three patients with diffuse pulmonary emphysema during the pre- and post-operative periods following a new surgical technique: collateral ventilation with lung parenchyma drainage. Methods: Patients suffering from pulmonary failure and disabling dyspnea, despite having received the gold standard treatment, including pulmonary rehabilitation, were selected for the evaluation of pulmonary drainage. During the pre- and post-operative periods, patients were submitted to plethysmography and six-minute walk tests, as well as completing the following quality of life questionnaires: Medical Outcomes Study 36-item Short-Form Health Survey, Saint George's Respiratory Questionnaire, Eastern Cooperative Oncology Group Performance Status and Medical Research Council Scale. In all three cases, the post-operative follow-up period was at least 300 days. The tests were performed at the following time points: during the pre-operative period; between post-operative days 30 and 40; and on post-operative day 300. Data were analyzed using profile plots of the means. Results: When the results obtained in the pre-operative period were compared to those obtained at the two post-operative time points evaluated, improvements were observed in all parameters studied. Conclusions: The results suggest that the surgical technique proposed for the treatment of patients suffering from severe diffuse emphysema successfully reduces the debilitating symptoms of these patients, improving their quality of life considerably.

Keywords: Pulmonary emphysema; Lung diseases, obstructive; Lung, hyperlucent.

A 23 female patient presented with productive cough, vomica and hair elimination in the sputum. Chest X-ray and CT showed a pulmonary abscess in the right lower lobe. The clinical treatment failed. A lobectomy was done and showed a benign lung teratoma, a very rare disease.

Keywords: Teratoma. Thoracic neoplasms.

Paragonimiasis is a chronic infection caused by a trematode of the genus Paragonimus, the Paragonimus westermani being the one that most infects man. It is an endemic disease in East Asia, however, it is also seen in America. The authors report two cases of paragonimiasis, in which a patient showed a past history and clinical and radiological characteristics suggestive of this disease, diagnosed by the presence of eggs of P. westermani in the sputum, and another patient, a thoracic post-traumatism empyema whose paragonimiasis diagnosis was a histopathological finding in the pleura after a decortication. This diagnosis was confirmed by the presence of eggs of P. westermani in the sputum.

Keywords: paragonimiasis

Human pulmonary dirofilariasis is a rare disease caused by the parasite Dirofilaria immitis. It is usually seen as a solitary pulmonary nodule that mimics lung cancer. Although this disease is considered benign, its diagnosis often requires an excisional lung biopsy. Herein we report the epidemiological, clinical and radiological features observed in seven cases of human pulmonary dirofilariasis from Florianópolis. Six of our seven patients, showed a radiological finding of pulmonary nodule and underwent excisional lung biopsy for diagnosis. In one case, the radiological image was unavailable for review. Therefore, it was not described in this work, and the diagnosis was established through transbronchial biopsy.

Keywords: Dirofilariasis. Dirofilaria immitis. Coin lesion pulmonary.

Pulmonary hypertension occurs when the pressure of the pulmonary artery is disproportionally high for a certain level of pulmonary blood flow. Values of mean pulmonary artery pressure of more than 25 mm Hg at rest or 30 mm Hg during exercise allow the diagnosis of pulmonary hypertension. Sustained or chronic pulmonary hypertension may be secondary to known diseases, mainly to those of cardiac or pulmonary nature, or may be a primary abnormality of the pulmonary circulation, with or without identification of associated conditions. Advances in the knowledge of the mechanisms of vasoconstriction and vascular remodeling have brought better prospects for the treatment of the disease. The correct use of vasodilators and anticoagulants, the new vasodilators, as epoprostenol and its analogs, and surgical techniques have increased the survival of many patients. Pneumologists can view cases of pulmonary hypertension as complications of pulmonary diseases or as a result of dyspnea investigation. Despite its etiology, pulmonary hypertension represents a clear abnormality that affects the right ventricle and can be potentially fatal to patients. Image methods have made the diagnosis of pulmonary hypertension more accessible and non-invasive. Six cases of patients with pulmonary hypertension of different causes are presented and discussed. In conclusion, according to new concepts, idiopathic pulmonary hypertension is no longer an irreversible condition, and the identification of associated conditions with potential treatments can be favorable in the management of the patients with pulmonary hypertension.

Keywords: pulmonary hypertension, vascular resistance, lung diseases, vasodilator agents

Post-intubation tracheal injury is a rare and potentially fatal complication. Among the most common causes, cuff overinflation and repetitive attempts of orotracheal intubation in emergency situations are paramount. Diagnosis is based on clinical and radiological suspicion, confirmed by fiberoptic bronchoscopy. Both conservative and surgical management apply, and the decision-making process depends on the patient profile (comorbidities, respiratory stability), characteristics of the lesion (size and location) and the time elapsed between the occurrence of the injury and the diagnosis. We report the cases of three patients presenting tracheal laceration due to traumatic orotracheal intubation, two submitted to surgical treatment and one submitted to conservative treatment.

Keywords: Tracheal diseases; Rupture; Intubation.

Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease. It can be idiopathic or associated with any one of various conditions. To our knowledge, this is the first report of two cases of PPFE in Brazil. Our first patient presented with pleural and subpleural fibrosis in the upper lobes; a spiculated nodule in the left upper lobe; and a mild reticular pattern in the lower lobes. Surgical lung biopsy revealed PPFE in the upper lobes, including the nodule, and unclassified interstitial pneumonia in the left lower lobe. Our second patient had a history of exposure to domestic birds, indicating a risk of hypersensitivity pneumonitis, and presented with advanced lung disease, predominantly in the upper lobes, together with subpleural fibrosis.That patient underwent lung transplantation. In the explant specimen, PPFE and granulomas were identified, suggesting hypersensitivity pneumonitis as an associated cause.

Keywords: Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/etiology; Alveolitis, extrinsic allergic.

Here, we report the cases of three patients diagnosed with extensively drug-resistant tuberculosis and admitted to a referral hospital in the state of São Paulo, Brazil, showing the clinical and radiological evolution, as well as laboratory test results, over a one-year period. Treatment was based on the World Health Organization guidelines, with the inclusion of a new proposal for the use of a combination of antituberculosis drugs (imipenem and linezolid). In the cases studied, we show the challenge of creating an acceptable, effective treatment regimen including drugs that are more toxic, are more expensive, and are administered for longer periods. We also show that treatment costs are significantly higher for such patients, which could have an impact on health care systems, even after hospital discharge. We highlight the fact that in extreme cases, such as those reported here, hospitalization at a referral center seems to be the most effective strategy for providing appropriate treatment and increasing the chance of cure. In conclusion, health professionals and governments must make every effort to prevent cases of multidrug-resistant and extensively drug-resistant tuberculosis.

Keywords: Tuberculosis, multidrug-resistant; Extensively drug-resistant tuberculosis; Antitubercular agents; Antibiotics, antitubercular.

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

Gastrobronchial fistula is a rare condition as a complication following bariatric surgery. The management of this condition requires the active participation of a pulmonologist, who should be familiar with aspects of the main types of bariatric surgery. Herein, we report the cases of two patients who presented recurrent subphrenic and lung abscess secondary to fistula at the angle of His for an average of 19.5 months. After relaparotomy was unsuccessful, cure was achieved by antibiotic therapy and, more importantly, by stenostomy and endoscopic dilatation, together with the use of clips and fibrin glue in the fistula. These pulmonary complications should not be treated in isolation without a gastrointestinal evaluation since this can result in worsening of the respiratory condition, thus making anesthetic management difficult during endoscopic procedures.

Keywords: Fistula; Lung abscess; Subphrenic abscess; Obesity, surgery; Endoscopy.

Objective: To evaluate the impact of the elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) combination on the small airways in adults with cystic fibrosis (CF), a genetic disorder that primarily affects the respiratory system, leading to progressive lung disease. In CF, the small airways play a critical role, contributing to chronic symptoms such as cough, sputum production, and dyspnea. Methods: This was a single-center, retrospective observational study of adults with CF treated with ELX/TEZ/IVA for 12 months. We compared the patients who were homozygous for the F508del mutation of the CF transmembrane conductance regulator (CFTR) gene with those who were heterozygous for that mutation, in terms of lung function outcomes (FEV1, FEF25-75%, and the RV/TLC ratio) and the extent of non-homogeneous ground-glass opacity. Among the patients within the cohort, the same parameters were evaluated separately in those who had advanced lung disease and in those who had previously undergone CFTR modulator therapy. Results: There was a significant post-treatment improvement in lung function, with a median increase of 0.42 L/s in the FEF25-75% (p < 0.001) and a 5% reduction in the mean RV/TLC ratio (p < 0.001). There was a trend toward a higher improvement the F508del homozygous patients. A significant reduction in non-homogeneous ground-glass opacity was observed in 79.5% of the patients. Among the patients with advanced lung disease, there were notable post-treatment improvements in all of the parameters assessed. Conclusions: Our results highlight the positive impact that ELX/TEZ/IVA treatment can have on small airway function in patients with CF, with potential benefits even for those with advanced lung disease. Further research is needed in order to evaluate the long-term effects of this treatment and its relationship with patient-reported outcomes.

Keywords: Cystic fibrosis/diagnostic imaging; Lung/physiopathology; Respiratory function tests; Lung/drug effects; Lung/diagnostic imaging; Cystic fibrosis/drug therapy.