Pneumonectomy in an Older Patient With Swyer-James-MacLeod Syndrome With a Giant Bulla.

Swyer-James-MacLeod syndrome is a rare lung disease that appears as unilateral pulmonary hyperlucency on chest radiographs and is considered to occur as a result of childhood bronchiolitis obliterans. We report the case of a 54-year-old woman who experienced exertional dyspnea owing to Swyer-James-MacLeod syndrome associated with a giant calcified bulla that occupied the entire hemithorax. Right pneumonectomy was performed because of progressive pulmonary dysfunction caused by the compression of the bulla on the left lung. This treatment dramatically improved both her pulmonary function and symptoms. Swyer-James-MacLeod syndrome is a rare disease characterized by unilateral pulmonary hyperlucency on chest radiography. In this case, Swyer-James-MacLeod syndrome associated with a giant calcified bulla that compressed the patient's left lung was treated with right pneumonectomy.

The vanishing lung: an important cause of hyperlucency on chest radiograph.

Giant bullous emphysema is an uncommon condition characterised by large asymmetric bullae with upper lobe predominance. This condition is most frequent in young male smokers. Patients usually present with progressive breathlessness which is secondary to enlargement of the bullae leading to compression of the lung parenchyma. Large asymmetrical bullae may appear as a unilateral hyperlucency on a plain chest radiograph, and may mimic the appearances of pneumothorax. A computed tomography scan is needed to delineate the lung pathology. We describe two cases with this condition that presented acutely and discuss the management of bullous emphysema.

[Management of a child with Swyer-James syndrome who underwent flexible bronchoscopy under general anesthesia].

Swyer-James syndrome (SJS) is a rare syndrome characterized by hyperlucency of one or more lobes of the unilateral lung and decreased pulmonary vascularity which is considered to be secondary to the childhood obliterative bronchiolitis. We report anesthetic management of a girl with SJS who underwent flexible bronchoscopy. A 15-year-old girl weighing 47 kg was referred to our hospital with suspected SJS. She had a history of bronchiolitis in early childhood. On admission, chest radiograph showed a hyperlucent lung with diminished pulmonary vasculature on the left lung. Pulmonary function test revealed slightly restrictive pattern (%VC 69.3%, %FEVa1.0 77.9%). Ventilation and perfusion scanning demonstrated the markedly decreased ventilation (left : right, 33 : 67) and perfusion (left : right, 10 : 90) of the left lung. To rule out the foreign body and bronchial stenosis, flexible bronchoscopy under general anesthesia was planned. Anesthesia was induced with propofol and maintained with propofol and remifentanil. Spontaneous respiration was maintained by using laryngeal mask airway to prevent the pressure alveolar damage by positive pressure ventilation. Flexible bronchoscopy was performed safely by using topical laryngeal lidocaine administration and additional bolus of propofol. Her postoperative course was uncomplicated and she was discharged on the following day.

Developments in neuroacanthocytosis: expanding the spectrum of choreatic syndromes.

As with other neurodegenerative disorders, research into the group of diseases known under the umbrella term of "neuroacanthocytosis" has greatly benefited from the identification of causative genes. The distinct and unifying aspect of these disorders is the presence of thorny deformations of circulating erythrocytes. This may be due to abnormal properties of red cell membranes, which could lead to insights into mechanisms of neurodegeneration. Research approaches in this field, in addition to examining functions and protein interactions of the affected proteins with particular respect to neurons, have also drawn upon the expertise of hematologists and red cell membrane biologists. In this article, recent developments in the field are presented.

Swyer-James (MacLeod) syndrome with placental transmogrification of the lung: a case report and review of the literature.

Swyer-James (MacLeod) syndrome is an acquired form of unilateral hyperlucency of the lung and is characterized by the development of severe emphysema, bronchiectasis, and/or bronchiolitis obliterans. It may develop as a complication of repeated episodes of pulmonary infection resulting in bronchiolitis obliterans and obstruction of small airways. Most patients with Swyer-James (MacLeod) syndrome can be managed clinically, and the pathologic features of the syndrome have been described in only a few reports. Placental transmogrification of the lung is a rare histopathologic finding that has been described in patients with severe emphysema associated with cigarette smoking, congenital bullous emphysema, and fibrochondromatous hamartomas of the lung and is characterized by the development of peculiar structures in the pulmonary parenchyma that resemble placental villi. To our knowledge, placental transmogrification of the lung has not been previously described in patients with Swyer-James (MacLeod) syndrome. We encountered a 32-year-old man with a history of childhood asthma who presented with progressively severe exertional dyspnea and had unilateral right lung hyperlucency. The patient underwent a right pneumonectomy. Examination of the lung revealed severe mixed centriacinar-panacinar emphysema in all lobes, bullous emphysema in the upper lobe, bronchiectases, mild interstitial pneumonia with fibrosis, and placental transmogrification of the pulmonary parenchyma of all 3 lobes. Here, we review the pathology of Swyer-James (MacLeod) syndrome and the possible pathogenesis of villous-like changes in the lung tissues.

Air-trapping zone surrounding sclerosing hemangioma of the lung.

We present two cases of sclerosing hemangioma of the lung with a peculiar radiologic finding: an air-trapping zone surrounding the tumor. On microscopic examinations, the tumor was of the hemangiomatous subtype, and the radiolucent zone corresponded to enlarged alveoli with septal destruction. A possible mechanism in the production of an air-trapping zone around a sclerosing hemangioma is bleeding from the highly vascular tumor followed by expectoration in communication with an airway. We reviewed the literature on the air meniscus sign in sclerosing hemangioma and concluded that although it is not a common finding, it could be of help in the confident diagnosis of sclerosing hemangioma and in differentiating it from other benign tumors of the lung.