Reporte de caso: hemoptisis masiva como manifestación clínica de un síndrome de Swyer-James-Mac Leod / Case report: massive hemoptysis as a clinical manifestation of Swyer-James-Mac Leod syndrome

El síndrome de Swyer-James-Mac Leod es una entidad poco frecuente adquirida en la infancia, generalmente tras una infección pulmonar moderada a grave de tipo bronquiolitis o neumonía, generalmente virales. Ocasionalmente se tiene el antecedente de infecciones repetidas de este tipo. Consiste en el desarrollo de enfisema hipoplásico pulmonar unilateral, que puede a veces relacionarse con bronquiectasias ipsilaterales o bilaterales, obstrucción fija al flujo aéreo y puede también asociarse a reducción del flujo sanguíneo del pulmón hipoplásico, de manera focal o difusa, con o sin tortuosidad de la vascularización proximal y a veces con una compensación del pulmón contralateral, en forma de sobredistensión e hiperflujo vascular relativo. Presentamos el caso de un varón de 79 años de edad con antecedentes de infecciones tipo bronquiolitis virales repetidas en la infancia, obstrucción fija grave al flujo aéreo y hemoptisis masiva secundaria a una infección por Pseudomonas aeruginosa sensible a la terapia antibiótica habitual.

Swyer-James-Mac Leod syndrome is a rare condition acquired in childhood, usually after a moderate to severe lung infection such as bronchiolitis or pneumonia, usually viral. Occasionally there is a history of repeated infections of this type. It consists of the development of unilateral pulmonary hypoplastic emphysema, which can sometimes be related to ipsilateral or bilateral bronchiectasis, fixed airflow obstruction, and may also be associated with reduced blood flow in the hypoplastic lung, with or without tortuosity of the proximal vascular supply and sometimes with compensation from the contralateral lung, in the form of overdistension and relative vascular hyperflow. We present the case of a 79-year-old man with a history of recurrent viral bronchiolitis-type infections in childhood, severe fixed airflow obstruction, and massive hemoptysis secondary to a Pseudomonas aeruginosa infection sensitive to usual antibiotic therapy.

Enfisema pulmonar congênito: relato de caso / Congenital pulmonary enphysema: case report

Introdução: O enfisema pulmonar congênito (EPC) é uma doença rara, possuindo uma incidência de 1:20-30 mil nascimentos, é mais comum no sexo masculino do que no feminino, em uma razão de 3:1 e sua etiologia permanece desconhecida. Um terço dos casos são sintomáticos ao nascer e praticamente todos são diagnosticados nos primeiros seis meses de vida. Relato de Caso: Recém-nascido (RN) do sexo masculino, evoluiu com desconforto respiratório precoce, sendo encaminhado ao centro de terapia intensiva. Após uso de continuous positive airway pressure (CPAP) e cateter nasal de oxigênio de 12 horas, resultou em bom padrão respiratório e boa saturação. Após realização de tomografia computadorizada, foi diagnosticado o EPC. Conclusão: O EPC é uma patologia rara e deve ser suspeitado em RN com desconforto respiratório, atribuindo-se importância aos vários diagnósticos diferenciais possíveis. Apesar da etiologia incerta, é de fácil diagnóstico e possui opções de manejo clínico e cirúrgico.

Introduction: Congenital pulmonary emphysema (EPC) is a rare disease, with an incidence of 1:20-30 thousand births, it is more common in males than in females, in a ratio of 3:1 and its etiology remains unknown. One third of the cases are symptomatic at birth and practically all are diagnosed in the first six months of life. Case Report: Newborn (NB) male, developed early respiratory distress, being referred to the intensive care unit. After using continuous positive airway pressure (CPAP) and a 12-hour oxygen nasal catheter, it resulted in a good breathing pattern and good saturation. After performing computed tomography, EPC was diagnosed. Conclusion: EPC is a rare pathology and should be suspected in newborns with respiratory distress, with importance being given to the various possible differential diagnoses. Despite its uncertain etiology, it is easy to diagnose and has options for clinical and surgical management.

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition / Combinação de fibrose pulmonar e enfisema: uma doença cada vez mais reconhecida

Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

A combinação de fibrose pulmonar e enfisema (CFPE) é cada vez mais reconhecida na literatura. Os pacientes são geralmente fumantes pesados ou ex-fumantes nos quais a TCAR de tórax revela enfisema nos lobos superiores e, concomitantemente, fibrose nos lobos inferiores. Esses pacientes comumente apresentam dispneia grave e baixa DLCO, não obstante os volumes pulmonares relativamente preservados em exames espirométricos. Hipertensão arterial pulmonar de moderada a grave e aumento da incidência de câncer de pulmão também são comuns nesses pacientes. Infelizmente, ainda não existe um tratamento eficaz para a CFPE. O objetivo desta revisão é discutir o que se sabe atualmente a respeito da patogênese, das características clínicas e dos fatores prognósticos da CFPE. Como a maioria dos dados publicados baseia-se em análise retrospectiva, são necessários mais estudos sobre o papel do enfisema e seus subtipos, a progressão da fibrose/enfisema e sua correlação com a inflamação, as opções de tratamento e o prognóstico em pacientes com CFPE.

Quantitative Computed Tomography of Pulmonary Emphysema and Ventricular Function in Chronic Obstructive Pulmonary Disease Patients with Pulmonary Hypertension

OBJECTIVE: This study strived to evaluate the relationship between degree of pulmonary emphysema and cardiac ventricular function in chronic obstructive pulmonary disease (COPD) patients with pulmonary hypertension (PH) using electrocardiographic-gated multidetector computed tomography (CT). MATERIALS AND METHODS: Lung transplantation candidates with the diagnosis of COPD and PH were chosen for the study population, and a total of 15 patients were included. The extent of emphysema is defined as the percentage of voxels below -910 Hounsfield units in the lung windows in whole lung CT without intravenous contrast. Heart function parameters were measured by electrocardiographic-gated CT angiography. Linear regression analysis was conducted to examine the associations between percent emphysema and heart function indicators. RESULTS: Significant correlations were found between percent emphysema and right ventricular (RV) measurements, including RV end-diastolic volume (R2 = 0.340, p = 0.023), RV stroke volume (R2 = 0.406, p = 0.011), and RV cardiac output (R2 = 0.382, p = 0.014); the correlations between percent emphysema and left ventricular function indicators were not observed. CONCLUSION: The study revealed that percent emphysema is correlated with RV dysfunction among COPD patients with PH. Based on our findings, percent emphysema can be considered for use as an indicator to predict the severity of right ventricular dysfunction among COPD patients.

Pulmonary interstitial emphysema complicating pneumonia in an unventilated term infant.

A case of pulmonary interstitial emphysema with pneumothorax and pneumomediastinum complicating pneumonia in a 6-week-old infant is reported. The patient had no history of resuscitation, bag and mask ventilation, nasal continuous positive airway pressure or mechanical ventilation.

Evolución funcional respiratoria en dos pacientes con enfisema y fibrosis pulmonar / Functional respiratory evolution in two patients with emphysema and pulmonary fibrosis

La combinación de enfisema y fibrosis pulmonar es una condición frecuentemente subdiagnosticada. Los estudios funcionales aislados pueden generar interpretaciones inadecuadas. No hemos hallado comunicaciones de casos que documenten la evolución espirométrica de estos pacientes. Se presentan dos casos de fibrosis y enfisema combinados que permiten observar la evolución funcional a largo plazo y comprender los valores espirométricos actuales en forma más precisa. Los hallazgos más relevantes son: 1) espirometría con discretas alteraciones funcionales en presencia de disnea marcada y, en un paciente, necesidad de oxigenoterapia crónica, 2) evolución funcional con seudonormalización del patrón espirométrico obstructivo inicial, posiblemente como consecuencia del desarrollo de fibrosis. Un defecto obstructivo leve en un paciente con diagnóstico de obstrucción crónica al flujo aéreo y marcado deterioro de su condición clínica y clase funcional debería alertar sobre la posibilidad de fibrosis pulmonar asociada. La tomografía y la disponibilidad de estudios funcionales previos permiten comprender esta condición.

Combined pulmonary fibrosis and emphysema (CPFE) is a frequently under-diagnosed condition. Isolated pulmonary function tests (PFT) can give rise to misinterpretations. We have found no reports on these patients’ spirometric progression. We describe two cases of CPFE, showing long-term functional evolution to have a more accurate understanding of current spirometric values. The most relevant findings are: 1) spirometry with discrete functional alterations in the presence of a marked dyspnea and the need, in one patient, for chronic oxygen therapy; and 2) functional evolution reflecting "pseudonormalisation" of the initial obstructive spirometric pattern, possibly as a result of fibrosis development. A mild obstructive defect in a patient with chronic airflow limitation and marked impairment of his/her clinical status and functional class should alert on the possibility of associated pulmonary fibrosis. A computed tomography (CT) and previous PFTs will allow a better understanding of this condition.

Respiratory distress in neonates.

Respiratory distress due to either medical or surgical causes occurs commonly in neonates. It is the most common cause of admission to a neonatal surgical intensive care facility in a tertiary care hospital. The distress can be caused by a variety of clinical conditions; common conditions treated in medical intensive care units are transient tachypnea of the new born, respiratory distress syndrome, pulmonary air leak and pneumothorax. In surgical causes of respiratory distress in neonates the underlying mechanisms include airway obstruction, pulmonary collapse or displacement and parenchymal disease or insufficiency; the common causes are congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, congenital lobar emphysema and esophageal atresia with or without tracheo-esophageal fistula. Obstructive lesions of the new born airway include choanal atresia, macroglossis, Pierre-Robin syndrome, lymphangioma, teratoma or other mediastinal masses, cysts, subglottic stenosis and laryngo tracheomalacia. Imaging plays a very major role in the pre-operative diagnosis of these conditions and proper pre-operative resuscitation helps in improving the results of surgery dramatically.

Tratamiento quirúrgico de la enfermedad vesiculosa del pulmón / Surgical treatment of vesiculose lung disease

Se consideran 22 enfermos con neumotórax secundarios a vesículas y 4 pacientes con enfermedad vesiculosa del pulmón sin neumotórax. El primer grupo eran hombres con una edad media de 45,5 años, y un 73// fumadores importantes. Once enfermos (50//) presentaron neumotórax previos, 11 (50//) EPOC y cuatro (18//) presentaban insuficiencia ventilatoria. En 12 (54//) se debió recurrir a la toracotomía pero en los enfermos con enfisema esta cifra fue inferior (4,36//), pues en ellos predominó el tratamiento con drenaje y pleurodesis con tetraciclina. Según las alteraciones anatopatológicas se diferenciaron cuatro grupos: 1) vesículas en uno o más lóbulos sin enfisema difuso (9,41//); 2) vesiculas asociadas a enfisema difuso (1,50//); 3) distrofia lobal vesiculosa (1,4//); 4) quistes aéreos (1,4//). En los grupos 1,3 y 4 la toracotomía es la terapéutica de elección, mientras que en el grupo a debe favorecerse el drenaje, la toracoscopía o los métodos complementarios. Un paciente falleció en insuficiencia cardiorrespiratoria y neumotórax bilateral con graves alteraciones enfisematosas y múltiples drenajes. Los 4 enfermos con enfermedad vesiculosa del pulmón sin neumotórax consultaron por disnea progresiva y eran severos fumadores. La TAC fue el método más importante en el diagnóstico y la evaluación preoperatoria, junto con la angiografía pulmonar. Se constataron vesículas gigantes bilaterales en 3 pacientes y unilaterales en otro. La cirugía se indicó ante la buena calidad de los vasos del pulmón comprimido. Se propone agrupar a los enfermos en: Grupo 1 o EVP sin enfisema difuso u obstrucción de la vía aérea, y Grupo 2 o EVP con enfisema u obstrucción de la vía aérea. Se favorece el abordaje bilateral de las lesiones, considerándose a la esternotomía como la vía de elección. Los cuatro pacientes mejoraron su calidad de vida, realizan actividades de esfuerzo y dos usan drogas broncodilatadoras después de un año de operados

Paracoccidioidomicose em mulher: ocorrência de pneumotórax espontâneo bilateral: relato de um caso / Paracoccidiomycosis in females: occurrence of bilateal spontaneous pneumothorax: a case report

É relatado o caso de um paciente do sexo feminino com paracoccidioidomicose pulmonar progressiva, que apresentou pneumotórax espontâneo bilateral. Os autores enfatizam a presença do enfisema subpleural como provável fator patogênico do penumotórax, assim como a importância da sua identificaçäo na avaliaçäo radiológica da infiltraçäo pulmonar difusa, e chamam a atençäo para a baixa inciência da paracoccidioidomicose em mulheres

Comprometimento pulmonar letal na neurofibromatose / Compromising lethal pulmonary in neurofibromatosis

É apresentado o caso de um homem de 61 anos, internado com quadro de dispnéia, tosse com pouca expectoraçäo, associado a lesöes em pele e subcutâneo típicas de neurofibromatose tipo I. A radiografia e a tomografia computadorizada de tórax revelaram bolhas enfisematosas pulmonares. O paciente foi submetido a drenagem de hemitórax, porém evoluiu com hemorragia digestiva alta que o levou ao óbito. Embora o comprometimento pulmunar na neurofibromatose varie de 0 a 20%, é possivel que com o aumento da sobrevida dos portadores deste distúrbio a prevalência da doença pulmonar cística aumente