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.

Extracorporeal membrane oxygenation and lung transplantation: initial experience at a single brazilian center

OBJECTIVE: To report initial experience from the use of extracorporeal membrane oxygenation (ECMO) in patients who received lung transplantation. METHODS: Retrospective study of a single tertiary center in the Brazilian state of São Paulo, a national reference in lung transplantation, based on the prospective collection of data from electronic medical records. The period analyzed extended from January 2009 (beginning of the program) until December 2018. RESULTS: A total of 75 lung transplants were performed, with ECMO used in 8 (10.7%) cases. Of the patients, 4 (50%) were female. The mean age was 46.4±14.3 years. The causes of the end-stage lung disease that led to transplantation were pulmonary arterial hypertension in 3 (37.5%) patients, bronchiectasis in 2 (25%) patients, pulmonary fibrosis in 2 (25%) patients, and pulmonary emphysema in 1 (12.5%) patient. In our series, 7 (87.5%) cases were sequential bilateral transplantations. Prioritization was necessary in 4 (50%) patients, and in 1 patient, ECMO was used as a bridge to transplantation. The ECMO route was central in 4 (50%), peripheral venovenous in 2 (25%) and peripheral venoarterial in 2 (25%) patients. The mean length of the intensive care unit (ICU) stay was 14±7.5 days and of the hospital stay was 34.1±34.2 days. The mean ECMO duration was 9.3±6.6 days with a 50% decannulation rate. Three patients were discharged (37.5%). CONCLUSION: Lung transplantation requires complex treatment, and ECMO has allowed extending the indications for transplantation and provided adjuvant support in the clinical management of these patients.

Selective left mainstem bronchial intubation in the neonatal intensive care unit / Intubação seletiva do brônquio principal esquerdo em unidade de terapia intensiva neonatal

Abstract Background: Selective neonatal left mainstem bronchial intubation to treat right lung disease is typically achieved with elaborate maneuvers, instrumentation and devices. This is often attributed to bronchial geometry which favors right mainstem entry of an endotracheal tube deliberately advanced beyond the carina. Case summary: A neonate with severe bullous emphysema affecting the right lung required urgent non-ventilation of that lung. We achieved left mainstem bronchial intubation by turning the endotracheal tube 180° such that the Murphy's eye faced the left instead of the right, and simulated a left-handed intubation by slightly orientating the endotracheal tube such that its concavity faced the left instead of the right as in a conventional right-handed intubation. Conclusion: Urgent intubation of the left mainstem bronchus with an endotracheal tube can be easily achieved by recognizing that it is the position of the endotracheal tube tip and the direction of its concavity that are the chief determinants of which bronchus an endotracheal tube goes when advanced. This is important in critically ill neonates as the margin of safety and time window are small, and the absence of double-lumen tubes. Use of fiberoptic bronchoscope and blockers should be reserved as backup plans.

Resumo Justificativa: A intubação seletiva neonatal do brônquio principal esquerdo para tratar a doença pulmonar direita é tipicamente feita com elaboradas manobras, instrumentação e dispositivos. Isso é frequentemente atribuído à geometria brônquica que favorece a entrada principal direita de um tubo endotraqueal (TET) deliberadamente avançado para além da carina. Resumo do caso: Recém-nascido com enfisema bolhoso grave que afetava o pulmão direito e precisou com urgência da não ventilação desse pulmão. Para conseguir a intubação brônquica esquerda fizemos uma rotação de 180° do TET, de forma que o olho de Murphy ficasse voltado para a esquerda, e não para a direita, e para simular uma intubação à esquerda orientamos ligeiramente o TET, de modo que sua concavidade virasse para a esquerda em vez de para a direita, como em uma intubação convencional à direita. Conclusão: A intubação urgente do brônquio principal esquerdo com um TET pode ser facilmente obtida se reconhecermos que é a posição da ponta do TET e a direção de sua concavidade que determinam para qual brônquio o TET irá quando avançado. Isso é importante em neonatos criticamente doentes diante da margem de segurança e janela de tempo pequenas e na ausência de tubos de duplo lúmen. O uso de broncofibroscópio e bloqueadores deve ser considerado como planos de segurança.

Tratamento Endoscópico com Válvulas Endobrônquicas nos Pacientes com Enfisema Pulmonar / Endoscopic treatment with endobronchial valves in patients with pulmonary emphysema

A doença pulmonar obstrutiva crônica (DPOC) é uma patologia de grande prevalência na sociedade contemporânea, causando grande morbimortalidade e consumo de recursos em saúde. Apesar de flagrante melhora no entendimento de sua fisiopatologia e acréscimo no arsenal terapêutico experimentado nas últimas duas décadas, ainda existe grande parcela de doentes que convivem com limitação funcional extrema a despeito de tratamento clínico otimizado, reabilitação cardiopulmonar e oxigenoterapia suplementar. Tratam-se dos pacientes com doença em estágio avançado, que até pouco tempo só tinham como alternativa terapêutica o transplante pulmonar ou cardiopulmonar. Porém, nos últimos dez anos, esse panorama sombrio vem ganhando mais uma alternativa viável e factível, mesmo em pacientes com comorbidades limitantes e em países como o nosso, onde a realização de transplante pulmonar em larga escala não é uma realidade. Trata-se do tratamento endoscópico do enfisema pulmonar. Dentre as modalidades de terapia disponíveis atualmente, discutiremos o uso de válvulas endobrônquicas, método dos mais estudados, já com diversas publicações importantes e recentemente incluído no documento GOLD como opção terapêutica nos pacientes com doença pulmonar obstrutiva crônica.

Chronic obstructive pulmonary disease is a highly prevalent pathology in contemporary society, causing great morbidity, mortality and consumption of health resources. Despite a striking improvement in the understanding of its pathophysiology and increase in the therapeutic arsenal experienced in the last two decades, there is still a large number of patients living with extreme functional limitation despite optimal clinical treatment, cardiopulmonary rehabilitation and supplemental oxygen therapy. They are those patients with advanced disease that until recently had only as a therapeutic alternative the lung or cardiopulmonary transplantation. However, in the last ten years, this bleak picture has been gaining yet another viable and feasible alternative, even in patients with limiting comorbidities and in countries such as ours, where large-scale lung transplantation is not a reality. It is the endoscopic treatment of pulmonary emphysema. Among the modalities of therapy currently available, we will discuss the use of endobronchial valves, a method most studied, already with several important publications and recently included in the GOLD document as a therapeutic possibility in patients with chronic obstructive pulmonary disease.

Tratamento endoscópico do Enfisema: uma atualização / Bronchoscopic Treatment of Emphysema: an Update

O objetivo desta revisão foi apresentar uma atualização das alternativas endoscópicas utilizadas no tratamento do enfisema pulmonar. Os mecanismos bloqueadores de fluxo (válvulas) permanecem como os únicos dispositivos aprovados para uso clínico no tratamento do enfisema pulmonar na América do Sul. A válvula endobrônquica Zephyr® representa o método broncoscópico para o tratamento do enfisema mais bem estudado até o momento, e seu uso clínico está autorizado em diversos países da Europa, Ásia e América do Sul. A válvula IBV® obteve aprovação para uso no tratamento de fuga aérea prolongada nos EUA. Os critérios mais frequentemente analisados na indicação de tratamento endoscópico, além dos aspectos clínicos, incluem o aprisionamento dinâmico, a heterogeneidade e a ventilação colateral, em especial, a passagem de ar através das cissuras interlobares. Atualmente, há softwares que permitem a medida precisa da heterogeneidade e da integridade da cissura.Os resultados obtidos em diversas séries de casos e alguns ensaios randomizados têm trazido nova luz ao entendimento da fisiopatologia dessa doença. No entanto, ainda há necessidade de mais estudos randomizados utilizando o conhecimento adquirido até o momento

The objective of this review is to present an update on endoscopic alternatives for the treatment of emphysema.One-way endobronchial valves continue to be the only devices approved for clinical use in the treatment of emphysema inSouth America. The use of the Zephyr® endobronchial valve is currently the most widely studied bronchoscopic method fortreatment of emphysema and has been approved for clinical use in several countries in Europe, Asia, and South America. Another valve, the IBV® valve, has been approved for use in the treatment of persistent air leaks in the United States. In additionto clinical features, the criteria most often analyzed for indicating endoscopic treatment are dynamic hyperinflation, heterogeneity, and collateral ventilation, especially the passage of air through the interlobar fissures. Currently, there is softwarecapable of accurately measuring heterogeneity and fissure integrity.The results obtained in various case series and certain randomized trials have shed new light on the pathophysiology of emphysema. However, additional randomized trials using the knowledge gained thus far are warranted in order to furtherevaluate this procedure

La ozonoterapia en pacientes con enfisema pulmonar / Rectal ozone therapy for patients with pulmonary emphysema

Background: Ozone therapy may stimulate antioxidant systems and protect against free radicals. It has not been used formerly in patients with pulmonary emphysema. Aim: To assess the effects of rectal ozone therapy in patients with pul-monary emphysema. Material and Methods: Sixty four patients with pulmonary emphysema, aged between 40 and 69 years, were randomly assigned to receive rectal ozone in 20 daily sessions, rectal medicinal oxygen or no treatment. Treatments were repeated three months later in the frst two groups. At baseline and at the end of the study, spirometry and a clinical assessment were performed. Results: fifty patients completed the protocol, 20 receiving ozone therapy, 20 receiving rectal oxygen and 10 not receiving any therapy. At baseline, patients on ozone therapy had significantly lower values of forced expiratory volume in the frst second (fEV1) and fEV1/forced vital capacity. At the end of the treatment period, these parameters were similar in the three treatment groups, therefore they only improved significantly in the group on ozone therapy. No differences were observed in other spirometric parameters. Conclusions: Rectal ozone therapy may be useful in patients with pulmonary emphysema.

Malformaciones pulmonares: enfisema lobar congénito / Lung malformations: lobar congenital emphysema

Congenital lobar emphysema (CLE) is a rare congenital lung anomaly. Clinical presentation varies from acute neonatal respiratory failure to recurrent episodes of tachypnea or infections. Chest x-ray is often enough to make the diagnosis in newborn, but a normal chest x-ray does not exclude the diagnosis and a CT scan must be done when there is a lung malformation suspect. The better antenatal ultrasound diagnosis has led to detection CLE in asymptomatic and mildly symptomatic infants, and involution of CLE has been demonstrated in some cases. Lobectomy is the treatment of choice for very symptomatic children, but could be expectant in the less symptomatic ones.

El enfisema lobar congénito (ELC), es una malformación pulmonar poco frecuente. Su presentación clínica varía desde la falla respiratoria en el recién nacido (RN) hasta episodios recurrentes de taquipnea o infecciones. La radiografía de tórax, muchas veces es suficiente para realizar el diagnóstico en el RN, pero ante una radiografía normal se debe realizar TAC de tórax si se sospecha malformación pulmonar. El aumento del diagnóstico por ecografía prenatal ha llevado a la pesquisa del ELC en lactantes asintomáticos o levemente sintomáticos, algunos de los cuales se ha demostrado involución de la malformación. El tratamiento de elección en niños francamente sintomáticos es la lobectomía, pudiendo ser conservador en el resto de los pacientes.

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.

Persistent pulmonary interstitial emphysema in a preterm infant.

Persistent pulmonary interstitial emphysema is a chronic disease reported in mechanically ventilated premature newborns. We describe a case of localized persistent pulmonary interstitial emphysema in a preterm infant without mechanical ventilation but on continuous positive airway pressure using nasal prongs. The condition resolved without surgery.

Pulmonary interstitial emphysema and continuous positive airway pressure in a premature infant

Pulmonary interstitial emphysema PIE is a recognized sequel of high pressure mechanical ventilation. Nevertheless, with the improvement in perinatal medical care, PIE started to be seen in spontaneously breathing infants. We present a 29-week-old girl who developed PIE on the first day of life, while she was on nasal continuous positive airway pressure, and was successfully managed with high frequency oscillatory ventilation. There is a paucity of these cases in the literature. We report on the occurrence of this entity, early reorganization, and the possible associated or causation factors

Efeito da atividade física sobre a evolução do enfisema pulmonar: um estudo experimental em ratos Wistar / Effects of physical activity on the development pulmonar emphysema in rats: experimental study in rats Wistar

O propósito da presente investigação foi avaliar o papel da atividade física no desenvolvimento de enfisema induzido por papaina em ratos. Para tanto, ratos Wistar foram radomicamente divididos em quatro grupos (n = 10 para cada grupo) que receberam, respectivamente, infusão intra-traqueal de papaína (6 mg em 1 ml de NaCI 0,9 por cento) ou veículo e foram submetidos ou não ao protocolo de exercício em uma esteira ergométrica. Os ratos exercitaram-se a 13,3 m/min, 6 dias por semana, durante 9 semanas (o tempo de exercício foi aumentado gradualmente, de 10 a 35 min) / The purpose of the present study was to evaluate the role of exercise trainning on the development of papain-induced emphysema in rats. Wistar rats were randomly assigned to four groups (n = 10 for each group) that receiveid, respectively, intratracheal infusion of papain (6 mg in 1 ml NaCI 0,9 per cent) or vehicle and were submitted or not to a protocol of exercise on a treadmill...

Tratamiento del enfisema intersticial pulmonar unilateral con ventilación monopulmonar contralateral. Presentación de 1 caso / Treatment of the unilateral pulmonary interstitial emphysema with contralateral monopulmonary ventilation

Se presenta el caso de un recién nacido de 750 g de peso y 27 semanas de edad gestacional, que durante su evolución en la asistencia respiratoria mecánica, presentó enfisema intersticial pulmonar a tensión en el pulmón derecho, el cual producía desplazamiento mediastinal y pobre aereación del pulmón izquierdo. La subsecuente hipercapnia hizo fracasar todos los intentos de disminuir la presión inspiratoria en la vía aérea proximal y el enfisema intersticial fue empeorando. A los 6 días de vida se decide realizar intubación selectiva del bronquiotronco izquierdo y mantener al paciente con ventilación monopulmonar izquierda durante 36 horas. Al recolocar el tubo en la tráquea y reexpandirse el pulmón derecho, se observó una disminución notable del enfisema intersticial pulmonar y una mejoría gasométrica importante que permitió reducir la intensidad del apoyo ventilatorio. El paciente falleció posteriormente durante una enteritis necrosante

Bronquitis crónica y enfisema: antiguas y modernas terapéuticas / Chronic bronchitis and emphysema: ancient and modern therapies

Los enfermos portadores de Bronquitis Crónica y Enfisema, integrantes del complejo EPOC se caracterizan por su cronicidad, frecuentes descompensaciones y deterioro funcional, progresivo y representan una amenaza de desánimo para los médicos. Sin embargo existen modernas medidas terapéuticas que asociadas a las clásicas, permiten avizorar un futuro mejor para nuestros pacientes. En este artículo se resumen los fundamentos patogénicos de la enfermedad y se revisan los esquemas de tratamiento farmacológico actualmente utilizados. Luego se exponen los avances terapéuticos que implican la oxigenoterapia prolongada, los programas de rehabilitación y las propuestas de cirugía de remodelación pulmonar con sus correspondientes respaldos de la investigación internacional sobre el tema. Se incluye la revisión de la experiencia de rehabilitación respiratoria del CASMU a dos años de su implementación

Oclusäo brônquica seletiva como tratamento de enfisema pulmonar intersitical em recém-nascidos prematuros / Interticial pulmonar emphysema in premature infants-selective bronchial obstruction

Os autores relatam dois casos de enfisema pulmonar intersticial em recém-nascidos prematuros submetidos à asistência ventilatória e tratados com sucesso pela técnica de oclusäo de brônquio fonte por meio de catéter näo balonado

Prótesis expansiva en un lactante neumonectomizado / Expandable prosthesis in a pneumonectomized patient

Una niña de 45 días de vida fue sometida a neumonectomía derecha por daño pulmonar extenso, debido a enfisema pulmonar intersticial persistente generalizado. Para evitar el síndrome postneumonectomía derecha y la torsión del pedículo vascular mediastinal, se colocó en el hemitórax derecho una prótesis de silástico de las que habitualmente se emplean para expander la piel, con buen resultado

Enfisema lobar congénito / Congenital lobe emphysema

Se presenta la experiencia del Hospital Infantil de México Federico Gómez, en el manejo de pacientes con enfisema lobar congénito, en un periodo comprendido entre 1971 a 1989. Hubo 17 casos, de los cuales 13 masculinos y cuatro femeninos; las edades de ingreso variaron de 13 días a 3 años. Como síntomas principales tuvieron insuficiencia respiratoria y cianosis. El estudio que orientó al diagnóstico fué la radiografía de tórax donde se encontró hiperclaridad de un lóbulo, rechazo del mediastino y colapso pulmonar. A todos se les practicó lobectomía. El lóbulo superior izquierdo fue el más afectado. Hubo dos muertos en esta serie. El conocimiento de esta entidad es importante para el diagnóstico temprano y un efectivo tratamiento quirúrgico