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.

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

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.

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.

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.

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

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.

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.

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.

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].

Objective: To compare two models of pulmonary hypertension (monocrotaline and monocrotaline+pneumonectomy) regarding hemodynamic severity, structure of pulmonary arteries, inflammatory markers (IL-1 and PDGF), and 45-day survival. Methods: We used 80 Sprague-Dawley rats in two study protocols: structural analysis; and survival analysis. The rats were divided into four groups: control; monocrotaline (M), pneumonectomy (P), and monocrotaline+pneumonectomy (M+P). In the structural analysis protocol, 40 rats (10/group) were catheterized for the determination of hemodynamic variables, followed by euthanasia for the removal of heart and lung tissue. The right ventricle (RV) was dissected from the interventricular septum (IS), and the ratio between RV weight and the weight of the left ventricle (LV) plus IS (RV/LV+IS) was taken as the index of RV hypertrophy. In lung tissues, we performed histological analyses, as well as using ELISA to determine IL-1 and PDGF levels. In the survival protocol, 40 animals (10/group) were followed for 45 days. Results: The M and M+P rats developed pulmonary hypertension, whereas the control and P rats did not. The RV/LV+IS ratio was significantly higher in M+P rats than in M rats, as well as being significantly higher in M and M+P rats than in control and P rats. There were no significant differences between the M and M+P rats regarding the area of the medial layer of the pulmonary arteries; IL-1 and PDGF levels; or survival. Conclusions: On the basis of our results, we cannot conclude that the monocrotaline+pneumonectomy model is superior to the monocrotaline model.

Keywords: Monocrotaline; Hypertension, pulmonary; Pneumonectomy; Interleukin-1; Receptor, platelet-derived growth factor beta.

The hemangiopericytoma is a rare malignant neoplasia that involves the lungs in about 10% of the cases. It usually presents as a great encapsulated mass in asymptomatic patients. The treatment of choice is surgical resection. The authors present a case of malignant lung hemangiopericytoma in a 62 year-old woman, followed by discussion and review of literature.

Keywords: Hemangiopericytoma. Lung neoplasms.

Pectus excavatum (PEX) is the most frequent congenital deformity of the anterior chest wall and is defined as the dislocation of the medial or inferior portion of the sternal region toward the spinal column. There are various ways to measure the deformity. In this study, we present an objective method of assessing such deformity, the anthropometric index for PEX (AI-PEX). The AI-PEX was developed in the Thoracic Surgery Department of the Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas. The anthropometric measurements are taken during the physical examination. We herein report two cases involving patients with PEX assessed using the AI-PEX and treated with the minimally invasive Nuss technique. The measurements were always taken at the point of greatest deformity. The patients were assessed on the day of the operation and again at 60 days after the surgery. The AI-PEX allowed us to obtain a satisfactory assessment of the defect. In both patients, the post-operative evolution was favorable.

Keywords: Thoracic wall; Thoracic surgery, Video-assisted; Funnel chest.

Background: Pulmonary sequestration is a congenital anomaly involving the pulmonary parenchyma and its vascularization, and it presents as extralobar or intralobar. Aim: Describe the cases treated at the Institute of Heart (InCor) and Clinical Hospital of the Medical School of the University of São Paulo (FMUSP). Method: Retrospective analyses of the files of the patients treated over the 10-year period taking demographic, clinical and laboratorial data. Results: The patients were recurrent pulmonary infection and hemoptysis were frequent clinical findings in these patients. All cases were intralobar. The main localization was the left lower lobe (66%). Only one diagnosis was made intra-operatively. In the other eight cases, diagnosis was suggested on chest radiographs (100%) and confirmed by angiography (77%) and computed tomography (66%). Lobectomy (77%) was the most common surgical treatment, with low post-operative morbidity, and no mortality. Anatomic and histologic examination was performed in seven cases and confirmed the pathology. Conclusion: Pulmonary sequestration is an uncommon entity, in which the computed tomography and the angiography are the radiological investigations that provide the most information to a definitive and secure diagnosis. Resection of the involved tissue leads to excellent results.

Microsporidiosis is a rapidly emerging protozoon infection that has been reported predominantly in severely immunosuppressed persons with acquired immunodeficiency syndrome (AIDS). Since the first case of human microsporidian infection was reported in 1956, over 400 cases have been documented. The initial description of microsporidian infection in AIDS patients occurred in 1985. Five genera have been identified as the agents of microsporidiosis in immunocompromised persons (Enterocytozoon, Encephalitozoon, Septata, Pleistophora and Nosema). Respiratory tract infection due to microsporidia is associated almost exclusively with disseminated disease produced by members of the genera Encephalitozoon and Septata. Metronidazole and albendazole were recently reported to be responsible for clinical improvement of patients. The authors reported a case of a woman with AIDS and respiratory symptoms related to two pulmonary pathogens: Mycobacterium tuberculosis and Encephalitozoon cuniculi, identified by polymerase chain reaction (PCR) using diagnostic primers targeting the small subunit ribosomal RNA.

Keywords: Microsporidiosis. Respiratory tract infection. Acquired immunodeficiency syndrome.