The superior vena cava is formed by the union of the right and left brachiocephalic veins. It is located in the middle mediastinum, to the right of the aorta and anterior to the trachea. Superior vena cava syndrome consists of a group of signs (dilation of the veins in the neck, facial swelling, edema of the upper limbs, and cyanosis) and symptoms (headache, dyspnea, cough, orthopnea and dysphagia) caused by the obstruction of blood flow through the superior vena cava to the right atrium. This obstruction can be caused by extrinsic compression, tumor invasion or thrombosis. Such obstruction may also occur as a result of insufficient venous return secondary to intra-atrial or intraluminal diseases. From 73% to 93% of all cases of superior vena cava syndrome occur during the development of an intrathoracic tumor. Most patients presenting superior vena cava syndrome secondary to malignant neoplasms are treated without surgery, through radiotherapy, chemotherapy or the use of intraluminal stents. When the etiology of superior vena cava syndrome is benign, it can be treated with clinical measures (anticoagulation, raising the head, etc.) or, in refractory cases, with angioplasty, stents or surgery.

Keywords: Superior vena cava syndrome/therapy; Superior vena cava syndrome/surgery; Superior vena cava syndrome/etiology; Superior vena cava syndrome /drug therapy; Superior vena cava syndrome /radiotherapy; Superior vena cava

Lung cancer is now the main cause of blood flow obstruction in the superior vena cava and of its clinical manifestations. History tells that in the 18th Century, syphilis and tuberculosis were responsible for 40% of the known cases. The knowledge of hemodynamic changes seen in this syndrome and the improvement of diagnostic and cytopathologic techniques allow for a safer and more comfortable treatment of the patient than 10 years ago. Contrast CT and MR added to the identification and location of the obstruction, and older techniques such as cavography could be abandoned. SVC obstruction diagnosis and Doppler studies carried out at the bed of the patient contribute to deobstruction procedures such as transluminal percutaneous angioplasty in cases of thrombosis and venous stenosis. In addition, the use of prostheses is of great importance in the management of cases utilization of traumatic lesion to the SVC during lung or mediastinal cancer surgeries. In the realm of radiotherapy, the fractionating technique allows for the administration of high doses of irradiation to cancer patients with benefits to 70% of the cases on the average.

Sclerosing mediastinitis is a rare disorder characterized by an extensive fibrotic reaction involving the mediastinum. Due to the compression or invasion of mediastinal structures, the disorder mimics neoplasia. We present three cases of superior vena cava syndrome in which sclerosing mediastinitis was confirmed. The pathophysiological process is related to enlarged mediastinal lymph nodes, fibroblast proliferation and collagen deposition. The main causes of sclerosing mediastinitis are histoplasmosis and tuberculosis, both of which are prevalent in Brazil. It is difficult to make an accurate histopathological diagnosis using minimally invasive methods, and there is no effective treatment for this condition. In order to make a definitive diagnosis and resolve the aerodigestive tract obstruction, exploratory surgery is indicated.

Keywords: Mediastinitis/physiopathology; Mediastinum; Pulmonary fibrosis; Mediastinal neoplasms; Diagnosis, differential; Case reports [Publication type].

Chylothorax as a complication of the surgical treatment of thoracic outlet syndrome is a quite rare event. We report a case of right-sided chylothorax and present a brief review on the treatment of postoperative chylothorax.

Keywords: Chylothorax; Thoracic outlet syndrome; Cervical rib syndrome; Postoperative complications.

Fibrosing mediastinitis is an uncommon disease associated with a multiplicity of clinical syndromes. The pathophysiology of this disease is predicated on the encasement of the mediastinal vital organ structures within a dense fibrotic tumor. This tumor appears to emanate from an invasive chronic inflammatory process causing erosion and external compression of these structures. Two cases of this disease are reported: one case of superior vena cava syndrome and one case of esophageal obstruction which evolved satisfactorily with conservative treatment.

Keywords: Sclerosing mediastinitis. Fibrosing mediastinitis. Superior vena cava syndrome.

Severe pulmonary hypertension is a debilitating disease with short life expectancy that often affects young people. Pleuropulmonary complications of systemic lupus erythematosus occur in 50-70% of patients. Severe symptomatic pulmonary hypertension in systemic lupus erythematosus is rare and carries a bad prognosis because a fatal outcome can occur within months. The authors describe, for the first time, a patient with systemic lupus erythematosus with severe pulmonary hypertension and secondary antiphospholipid syndrome who responded favorably to oral sildenafil, after unsuccessful use of prednisone, intravenous cyclophosphamide, warfarin and diltiazem.

Keywords: Vasodilator agents/therapeutic use. Lupus erythematosuos systemic. Antiphospholipid antibodies. Phosphodiesterase inhibitors.

The Chiari malformation type I (CM-I) has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery.

Keywords: Sleep apnea, central; Arnold-Chiari malformation; Noninvasive ventilation.

Postemetic rupture of the esophagus, also known as spontaneous rupture or Boerhaave's syndrome, was first described by Herman Boerhaave in 1724. This is a severe disease that causes high mortality rates and is difficult to diagnose not only because it is rare but also because it is frequently confused with other severe clinical conditions, such as acute myocardial infarction, perforated peptic ulcer, and acute pancreatitis. Herein, we describe three cases of patients with this syndrome. Two underwent primary repair of the rupture, and one underwent esophagectomy followed by reconstruction. There was one death due to septic shock in the immediate postoperative period. The other two patients presented favorable long-term evolution.

Keywords: Esophageal diseases; Rupture, spontaneous; Mediastinitis; Esophagectomy.

The physiopathology of obstructive sleep apnea syndrome is multifactorial. Gender and obesity status, as well as genetic, anatomic, and hormonal factors, together with ventilatory drive, interact in a diverse manner in the physiopathology and clinical expression of the disease. Obesity is the main risk factor, since increases in body mass index, visceral fat, and neck circumference are strong predictors of the disease. Progesterone increases the activity of the upper airway dilator muscles and therefore plays a protective role in premenopausal women. This explains the fact that the prevalence of the disease is higher in postmenopausal patients, in patients with polycystic ovary syndrome, as well as in males. Evidence supports the fact that, as individuals grow older, there is a decrease in muscle tonus, with a consequent reduction in the dimensions of the upper airway lumen. Craniofacial anomalies, such as in retrognathia or micrognathia, are accompanied by posterior positioning of the tongue and can result in narrowing of the upper airway lumen. Finally, decreased ventilatory drive has been detected in patients with obstructive sleep apnea syndrome and hypercapnia.

Keywords: Sleep apnea obstructive/physiopathology; Body mass index; Obesity

A síndrome POEMS é uma rara doença de plasmócitos. A ocorrência de hipertensão pulmonar como complicação respiratória da síndrome é pouco frequente e pode estar ligada ao aumento de várias citocinas, quimiocinas e fatores de crescimento como parte dos fenômenos inflamatórios que cercam a fisiopatologia da síndrome POEMS. Descrevemos o caso de uma mulher de 54 anos com síndrome POEMS e hipertensão pulmonar, que foi tratada com corticoide como terapia de primeira linha. Tratava-se de uma paciente com clássicos sintomas dessa síndrome: polineuropatia (confirmada por eletroneuromiografia), organomegalia, hipotireoidismo subclínico, gamopatia monoclonal em dosagem urinária e alterações cutâneas. A cateterização cardíaca direita revelou pressão arterial pulmonar média de 48 mmHg, débito cardíaco de 4,1 L/min e resistência vascular pulmonar de 8,05 Woods. O nível sérico de brain natriuretic peptide (BNP) foi de 150 pg/mL. Nenhuma outra doença foi encontrada durante investigação. Prednisona (1 mg/kg por três meses) foi iniciada, com dramática melhora clínica e funcional, além de normalização dos níveis dos hormônios tireoidianos e de proteína em urina por eletroforese. A pressão arterial pulmonar média caiu para 26 mmHg, o débito cardíaco para 3,8 L/min e a resistência vascular pulmonar para 2,89 Woods. O nível sérico de BNP caiu para 8pg/mL. Nossos achados indicam o potencial papel da corticoterapia como primeira linha de tratamento na hipertensão pulmonar associada à síndrome POEMS. Diante da raridade dessa apresentação, um registro multicêntrico deveria ser desenvolvido para permitir a aquisição de mais dados que suportem essa conduta.

Keywords: Síndrome POEMS; Hipertensão pulmonar; Glucocorticoides.

A case of a patient with Pancoast's syndrome associated with acquired immunodeficiency syndrome is reported. This association is rare and Kaposi's sarcoma and non-Hodgkin lymphoma are the most recurrent tumors in AIDS. The patient was an IV drug user and was admitted to the hospital for diagnosis of apex lung mass with signs of Pancoast's syndrome. HIV serology was positive and pathology of lung mass showed non-small cell lung cancer.

Keywords: Pancoast's syndrome. HIV. Bronchogenic carcinoma. Lung neoplasms. Acquired immunodeficiency syndrome.

Objective: To analyze the treatment strategies that influence the effectiveness of tuberculosis treatment at primary care clinics (PCCs) in Brazil. Methods: This was a descriptive, retrospective epidemiological survey based on the medical records of 588 tuberculosis patients enrolled in the tuberculosis control programs at two PCCs located in the city of Belém, Brazil: Centro de Saúde Escola do Marco (CSEM) and Unidade Básica de Saúde da Pedreira (UBSP). The survey was limited to patients enrolled between January of 2004 and December of 2008. We included only patients between 18 and 59 years of age, and we excluded those who were transferred or were found to have been misdiagnosed. We collected data regarding age, gender, type of treatment (self-administered or supervised), co-infection with HIV, and treatment outcome. The health professionals involved in the tuberculosis control program at the two PCCs were interviewed regarding the strategies used for tuberculosis control and regarding routine clinical care for tuberculosis patients. Results: There were no significant differences between the CSEM and UBSP patients regarding age, gender, or co-infection with HIV. Supervised treatment was used significantly more frequently and the rate of cure was higher at the CSEM than at the UBSP, whereas the rate of treatment noncompliance was higher at the UBSP than at the CSEM. Conclusions: For patients enrolled in tuberculosis control programs at PCCs in Brazil, supervised treatment appears to be an extremely important strategy for reducing the rate of treatment noncompliance.

Keywords: Tuberculosis; Patient dropouts; Patient care planning; Treatment outcome.

Approximately seven million Brazilians over 40 years of age have COPD. In recent years, major advances have been made in the pharmacological treatment of this condition. We performed a systematic review including original articles on pharmacological treatments for COPD. We reviewed articles written in English, Spanish, or Portuguese; published between 2005 and 2009; and indexed in national and international databases. Articles with a sample size < 100 individuals were excluded. The outcome measures were symptoms, pulmonary function, quality of life, exacerbations, mortality, and adverse drug effects. Articles were classified in accordance with the Global Initiative for Chronic Obstructive Lung Disease criteria for the determination of the level of scientific evidence (grade of recommendation A, B, or C). Of the 84 articles selected, 40 (47.6%), 18 (21.4%), and 26 (31.0%) were classified as grades A, B, and C, respectively. Of the 420 analyses made in these articles, 236 were regarding the comparison between medications and placebos. Among these 236 analyses, the most commonly studied medications (in 66, 48, and 42 analyses, respectively) were long-acting anticholinergics; the combination of long-acting 2 agonists and inhaled corticosteroids; and inhaled corticosteroids in isolation. Pulmonary function, adverse effects, and symptoms as outcomes generated 58, 54, and 35 analyses, respectively. The majority of the studies showed that the medications evaluated provided symptom relief; improved the quality of life and pulmonary function of patients; and prevented exacerbations. Few studies analyzed mortality as an outcome, and the role that pharmacological treatment plays in this outcome has yet to be fully defined. The medications studied are safe to use in the management of COPD and have few adverse effects.

Keywords: Pulmonary disease, chronic obstructive/therapy; Pulmonary disease, chronic obstructive/mortality; Review.

Primary ciliary dyskinesia (PCD), previously known as immotile cilia syndrome, is an autosomal recessive hereditary disease that includes various patterns of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS), which accounts for 50% of all cases of PCD. The incidence of PCD ranges from 1:20,000 to 1:60,000. Since PCD causes deficiency or even stasis of the transport of secretions throughout the respiratory tract, it favors the growth of viruses and bacteria. As a result, patients have lifelong chronic and recurrent infections, typically suffering from bronchitis, pneumonia, hemoptysis, sinusitis, and infertility. Bronchiectasis and other chronic conditions infections can be the end result of the irreversible bronchial alterations, leading to chronic cor pulmonale and its consequences. Only half of the patients affected by PDC present all of the symptoms, a condition designated complete KS, compared with incomplete KS, typically defined as cases in which situs inversus does not occur. The diagnosis is made clinically and confirmed through transmission electron microscopy. Since there is no specific therapy for PCD, it is recommended that, upon diagnosis, secondary infections be treated with potent antibiotics and prophylactic interventions be implemented. In this paper, we report six cases of PCD (five cases of complete KS and one case of KS) and review the related literature, focusing on the diagnostic, therapeutic and clinical aspects of this disease.

Keywords: Kartagener syndrome; Ciliary motility disorders; Bronchiectasis; Dextrocardia.

A case of obstructive sleep apnea syndrome (OSAS) with narcoleptic features is described; polysomnographic studies were carried out and revealed a severe nocturnal respiratory impairment due to apneic episodes. A multiple sleep latency test (MSLT) showed early REM sleep onset in two out of five series. Nasal CPAP utilization improved the quality of nocturnal sleep and reverted all narcoleptic features in the next day's MSLT.

Keywords: Obstructive sleep apnea syndrome (OSAS). Narcolepsy. Sleep deprivation. CPAP.

A forty year old woman, 72 hours after liposuction, complained of sudden dyspnea, fever and tachycardia. Thoracic radiography and duplex-scan of deep veins were normal. Pulmonary scintigraphy suggested thromboembolism. The patient had hypoxia, anemia and mental confusion after three days of systemic heparin. Once fat embolism syndrome was suspected, heparin infusion was stopped and corticosteroid therapy was started. After that, the patient was much better and discharged from hospital.

Keywords: Fat embolism syndrome. Pulmonary thromboembolism. Liposuction.

A male farmer, 20 years old, from the countryside of the State of Piauí, developed acute respiratory infection. Despite adequate antimicrobial therapy, there was worsening respiratory manifestations demanding mechanical ventilation. Radiographs showed diffuse pulmonary infiltrates. PaO2/FiO2 was 58. Direct microscopy and culture from tracheal aspirates disclosed Coccidioides immitis. Autochthonous cases of coccidioidomycosis have only recently been described in Brazil, most of them from the State of Piauí. C. immitis has been isolated from humans, dogs and armadillos (Dasypus novemcinctus), and also from soil samples of armadillo's burrows. Therapeutic failure to antimicrobials and patient's origin from recognized endemic areas should alert for the possibility of acute pulmonary coccidioidomycosis.