Congenital lobar emphysema

Pneumatosis, also known as emphysema, is the abnormal presence of air or other gas within tissues. X-ray of a hip with prosthesis and pneumarthrosis, in this case aseptic.A vacuum sign is a normal finding on shoulder X-rays. Pneumatosis, also known as emphysema, is the abnormal presence of air or other gas within tissues. In the lungs, emphysema involves enlargement of the distal airspaces, and is a major feature of chronic obstructive pulmonary disease (COPD). Pneumoperitoneum (or peritoneal emphysema) is air or gas in the abdominal cavity, and is most commonly caused by a perforated abdominal organ. Pneumatosis is also a frequent result of surgery. The term is from pneumat- + -osis. Pulmonary emphysema, more usually called emphysema, is characterised by air-filled cavities or spaces, (pneumatoses) in the lung, that can vary in size and may be very large. The spaces are caused by the breakdown of the walls of the alveoli and they replace the spongy lung parenchyma. This reduces the total alveolar surface available for gas exchange leading to a reduction in oxygen supply for the blood. It is a typical feature of chronic obstructive pulmonary disease (COPD), a type of obstructive lung disease characterized by long-term breathing problems and poor airflow. Even without COPD, the finding of pulmonary emphysema on chest CT confers a higher mortality in tobacco smokers. In 2016 in the United States there were 6,977 deaths from emphysema – 2.2 per 100,000 of the population. There are three subtypes of pulmonary emphysema – centrilobular or centriacinar, panlobular or panacinar, and paraseptal or distal acinar emphysema, related to the anatomy of the lobules of the lung. These are not associated with fibrosis (scarring). A fourth type known as irregular emphysema involves the acinus irregularly and is associated with fibrosis. Though the subtypes can be seen on imaging they are not well-defined clinically. Centrilobular emphysema also called centriacinar emphysema, affects the centrilobular portion of the lung, the area around the terminal bronchiole, and the first respiratory bronchiole, and can be seen on imaging as an area around the tip of the visible pulmonary artery. Centrilobular emphysema is the most common type usually associated with smoking. The disease progresses from the centrilobular portion, leaving the lung parenchyma in the surrounding (perilobular) region preserved. Usually the upper lobes of the lungs are affected. Panlobular emphysema also called panacinar emphysema can involve the whole lung or mainly the lower lobes. This type of emphysema is associated with alpha-1 antitrypsin deficiency (A1AD or AATD), and is not related to smoking. Paraseptal emphysema also called distal acinar emphysema relates to emphysematous change next to a pleural surface, or to a fissure. The cystic spaces known as blebs or bullae that form in paraseptal emphysema typically occur in just one layer beneath the pleura. This distinguishes it from the honeycombing of small cystic spaces seen in fibrosis that typically occurs in layers. This type of emphysema is not associated with airflow obstruction.