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.

Computed tomography-identified phenotypes of small airway obstructions in chronic obstructive pulmonary disease / 中华医学杂志(英文版)

Chronic obstructive pulmonary disease (COPD) is a heterogeneous disease characteristic of small airway inflammation, obstruction, and emphysema. It is well known that spirometry alone cannot differentiate each separate component. Computed tomography (CT) is widely used to determine the extent of emphysema and small airway involvement in COPD. Compared with the pulmonary function test, small airway CT phenotypes can accurately reflect disease severity in patients with COPD, which is conducive to improving the prognosis of this disease. CT measurement of central airway morphology has been applied in clinical, epidemiologic, and genetic investigations as an inference of the presence and severity of small airway disease. This review will focus on presenting the current knowledge and methodologies in chest CT that aid in identifying discrete COPD phenotypes.

Transmogrificación placentaria de pulmón. Presentación atípica del enfisema bulloso / Placental transmogrification of the lung. Atypical presentation of the bullous emphysema

Resumen La transmogrificación placentaria del pulmón es una lesión extremadamente infrecuente, asociada al enfisema bulloso gigante y considerada por algunos autores una variante histológica del enfisema bulloso gigante unilateral. Su etiología aún es desconocida y representa un desafío tanto para el diagnóstico clínico como anatomopatológico. Presentamos el caso de un paciente joven, que consulta con disnea de un año de evolución, y cuyo diagnóstico postoperatorio incluye esta rara entidad.

Abstract Placental transmogrification of the lung is an extremely rare lesion, associated with giant bullous emphysema and considered by some authors to be a histological variant of unilateral giant bullous emphysema. Its etiology is still unknown and represents a challenge for both clinical and pathological diagnosis. We present the case of a young patient, who consults with dyspnea of one year of evolution, and whose postoperative diagnosis includes this rare entity.

Lobectomy with ecmo support in an infant who developed pulmonary interstitial emphysema following repair of hypoplastic aortic arch

Abstract Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Etiology of primary spontaneous pneumothorax / A etiologia do pneumotórax espontâneo primário

ABSTRACT With the advent of HRCT, primary spontaneous pneumothorax has come to be better understood and managed, because its etiology can now be identified in most cases. Primary spontaneous pneumothorax is mainly caused by the rupture of a small subpleural emphysematous vesicle (designated a bleb) or of a subpleural paraseptal emphysematous lesion (designated a bulla). The aim of this pictorial essay was to improve the understanding of primary spontaneous pneumothorax and to propose a description of the major anatomical lesions found during surgery.

RESUMO Com o advento da TCAR, o pneumotórax espontâneo primário passou a ser mais bem entendido e conduzido, pois sua etiologia pode ser atualmente identificada na maioria dos casos. O pneumotórax espontâneo primário tem como principal causa a rotura de uma pequena vesícula enfisematosa subpleural, denominada bleb ou de uma lesão enfisematosa parasseptal subpleural, denominada bulla. O objetivo deste ensaio pictórico foi melhorar o entendimento do pneumotórax espontâneo primário e propor uma descrição das principais lesões anatômicas encontradas durante a cirurgia.

Pulmonary fibrosis and emphysema.

A 58-year-old male presented with symptoms of progressive dyspnoea on exertion and predominantly dry cough of six months duration. He was a cigarette smoker with a smoking index of 10 pack years. He had no history of fever, exposure to dusts or drug therapy. There was no history of similar illness in the family members or any symptoms suggestive of connective tissue disease (CTD). On physical examination, clubbing was observed and there were bibasilar fine endinspiratory crackles on auscultation. Oxygen saturation by pulse oximetry showed significant exercise desaturation from 94% to 77%.

Reconstruction Algorithms Influence the Follow-Up Variability in the Longitudinal CT Emphysema Index Measurements

OBJECTIVE: We wanted to compare the variability in the longitudinal emphysema index (EI) measurements that were computed with standard and high resolution (HR) reconstruction algorithms (RAs). MATERIALS AND METHODS: We performed a retrospective review of 475 patients who underwent CT for surveillance of lung nodules. From this cohort, 50 patients (28 male) were included in the study. For these patients, the baseline and follow-up scans were acquired on the same multidetector CT scanner and using the same acquisition protocol. The CT scans were reconstructed with HR and standard RAs. We determined the difference in the EI between CT1 and CT2 for the HR and standard RAs, and we compared the variance of these differences. RESULTS: The mean of the variation of the total lung volume was 0.14 L (standard deviation [SD] = 0.13 L) for the standard RA and 0.16 L (SD = 0.15 L) for the HR RA. These differences were not significant. For the standard RA, the mean variation was 0.13% (SD = 0.44%) for EI -970 and 0.4% (SD = 0.88%) for EI -950; for the HR RA, the mean variation was 1.9% (SD = 2.2%) for EI -970 and 3.6% (SD = 3.7%) for EI -950. These differences were significant. CONCLUSION: Using an HR RA appears to increase the variability of the CT measurements of the EI.

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.

Enfisema bulloso bilateral gigante por déficit de alfa1-antitripsina / Giant bilateral bullous emphysema due to deficit of alpha1-antritypsin

Se presenta el caso de una paciente de 48 años de edad con un enfisema bulloso bilateral gigante y niveles bajos de alfa1-antitripsina, determinados en el laboratorio Labcel de la Facultad de Ciencias Médicas Dr Miguel Enríquez. La definición de alfa1-antitriptisina es resultante de un defecto genético y aproximadamente el 75 por ciento de los adultos con una carencia grave de esta proteína desarrollan un enfisema que a menudo comienza antes de los 40 años de edad. Con el objetivo de debatir los resultados de los exámenes complementarios realizados, el tratamiento y la evolución de esta paciente, exponemos este trabajo por considerarlo de importancia para la práctica médico-quirúrgica(AU)

The case of a female patient aged 48 with a giant bilateral bullous emphysema and low levels of alpha1-antitripsine determined at the Labcel laboratory of Dr Miguel Enríquez. Faculty of Medical Sciences was reported. The definition of alpha1-antitrypsin is the result of a genetical defect and approximately 75 percent of the adults with a severe lack of this protein develop an emphysema that often begins before the 40 years of age. In order to discuss the results of the complementary tests, the treatment and evolution of this patient, we present this paper for considering it important for the medicosurgical practice(AU)

Cystic Lung Disease: a Comparison of C ystic Size, as Seen on Expira tory and Inspiratory HRCT Scans

OBJECTIVE: To determine the effects of respiration on the size of lung cysts by comparing inspiratory and expiratory high-resolution CT (HRCT) scans. MATERIALS AND METHODS: The authors evaluated the size of cystic lesions, as seen on paired inspiratory and expiratory HRCT scans, in 54 patients with Langerhans cell histiocytosis (n = 3), pulmonary lymphangiomyomatosis (n = 4),confluent centrilobular emphysema (n = 9), paraseptal emphysema and bullae (n= 16), cystic bronchiectasis (n = 13), and honeycombing (n = 9). Using paired inspiratory and expiratory HRCT scans obtained at the corresponding anatomic level, a total of 270 cystic lesions were selected simultaneously on the basis of five lesions per lung disease. Changes in lung cyst size observed during respiration were assessed by two radiologists. In a limited number of cases (n = 11), pathologic specimens were obtained by open lung biopsy or lobectomy. RESULTS: All cystic lesions in patients with Langerhans cell histiocytosis, lymphangiomyomatosis, cystic bronchiectasis, honeycombing, and confluent centrilobular emphysema became smaller on expiration, but in two cases of paraseptal emphysema and bullae there was no change. CONCLUSION: In cases in which expiratory CT scans indicate that cysts have become smaller, cystic lesions may communicate with the airways. To determine whether, for cysts and cystic lesions, this connection does in fact exist, paired inspiratory and expiratory HRCT scans are necessary.

Cystic Lung Disease: a Comparison of C ystic Size, as Seen on Expira tory and Inspiratory HRCT Scans

OBJECTIVE: To determine the effects of respiration on the size of lung cysts by comparing inspiratory and expiratory high-resolution CT (HRCT) scans. MATERIALS AND METHODS: The authors evaluated the size of cystic lesions, as seen on paired inspiratory and expiratory HRCT scans, in 54 patients with Langerhans cell histiocytosis (n = 3), pulmonary lymphangiomyomatosis (n = 4),confluent centrilobular emphysema (n = 9), paraseptal emphysema and bullae (n= 16), cystic bronchiectasis (n = 13), and honeycombing (n = 9). Using paired inspiratory and expiratory HRCT scans obtained at the corresponding anatomic level, a total of 270 cystic lesions were selected simultaneously on the basis of five lesions per lung disease. Changes in lung cyst size observed during respiration were assessed by two radiologists. In a limited number of cases (n = 11), pathologic specimens were obtained by open lung biopsy or lobectomy. RESULTS: All cystic lesions in patients with Langerhans cell histiocytosis, lymphangiomyomatosis, cystic bronchiectasis, honeycombing, and confluent centrilobular emphysema became smaller on expiration, but in two cases of paraseptal emphysema and bullae there was no change. CONCLUSION: In cases in which expiratory CT scans indicate that cysts have become smaller, cystic lesions may communicate with the airways. To determine whether, for cysts and cystic lesions, this connection does in fact exist, paired inspiratory and expiratory HRCT scans are necessary.