A Case Report of Congenitally Absent Pericardium Masquerading as Recurrent Pericarditis.

A 24-year-old female with a history of Swyer-James-MacLeod syndrome presented with acute onset of pleuritic chest pain and was initially diagnosed with acute pericarditis. The 12-lead electrocardiogram demonstrated typical diffuse ST-segment elevation and PR-segment depression. Symptoms resolved rapidly with anti-inflammatory therapy consisting of ibuprofen and colchicine. After completing a 3-month course of the latter, her symptoms rapidly recurred. Workup, including labs and cardiac imaging consisting of a transthoracic echocardiogram and cardiac magnetic resonance imaging, was initially interpreted as normal. Re-review of her cardiac imaging revealed the patient had signs of a congenitally absent pericardium, including a "Snoopy Sign" on her posterior-anterior chest X-ray, which is characterized by levoposition of the cardiac silhouette, a lucent area between the pulmonary artery and aorta because of the presence of lung tissue, a lucent area between the base of the heart and the left hemidiaphragm, loss of the right heart border, a prominent pulmonary artery, and a flattened and elongated left ventricular contour. The patient had a cardiac computed tomography scan, which confirmed the diagnosis. In conclusion, a congenitally absent pericardium is a rare disorder, often undetected or misdiagnosed. There are characteristic findings on imaging such as a "Snoopy Sign" on a posterior-anterior chest X-ray, which can be easily missed because of its rarity. Our goal of this report is to educate health care providers about this rare disorder.

Síndrome de Swyer James-Mcload: relato de casos / Swyer James-Mc Load syndrome: case reports

A síndrome de Swyer James-Mc Load é uma entidade rara descrita pela primeira vez em 1953 sendo caracterizada por hipoplasia ou agenesia das artérias pulmonares, resultando em hiperlucência pulmonar unilateral. Embora a patogênese ainda não tenha sido completamente elucidada, acredita-se que seja uma complicação pós-infecciosa de bronquiolites ocorridas na infância. O objetivo deste estudo estudo foi analisar a melhor conduta terapêutica na síndrome de Swyer James-Mc Load e o impacto na qualidade de vida das pessoas acometidas. Relata-se dois casos desta síndrome diagnosticados em pacientes adultos, sendo que uma delas apresenta asma persistente grave e a outra é ex-tabagista e já realizou cirurgia para ressecção de um nódulo pulmonar no pulmão contralateral ao acometido. A terapêutica conservadora foi capaz de estabilizar a condição clínica dos pacientes, mantendo uma boa qualidade de vida. No entanto, pacientes que não se estabilizam com tal terapêutica provavelmente devem ser incluídos à intervenção invasiva, visto que fornece ao paciente melhora na qualidade e expectativa de vida. Uma breve revisão da literatura pertinente foi realizada para melhor compreensão do assunto...

The Swyer-James Mc Load syndrome is a rare entity first described in 1953 and is characterized by hypoplasia or agenesis of the pulmonary arteries, resulting in unilateral lung hyperlucency. Although the pathogenesis has not yet been fully elucidated, it is believed to be a post-infectious complication of bronchiolitis occurring in childhood. The objective of this article was analyzing the best treatment for SSJM and its impact on affected people's lives. We report two cases of this syndrome diagnosed in adult patients, one of whom has severe persistent asthma and the other is a former smoker and has undergone surgery for resection of a pulmonary nodule in the contralateral lung to the affected. We concluded that conservative therapy is able to stabilize the clinical condition of patients maintaining a good quality of life. However, patients who do not stabilize with such therapy are likely to be included in invasive intervention, since it improves the quality of life and life expectancy of the patient. A brief review of the literature was conducted to better understand the subject...

Unilateral hyperlucent lung in a child caused by a foreign body identified with v/q scintigraphy.

A 5-year-old girl presented with episodes of coughing, inspiratory stridor, and occasionally squeaking breath sounds. There was no history of a foreign body aspiration. Initially, she was diagnosed with allergic asthma. However, signs and symptoms continued despite a trial of inhaled corticosteroids. A chest radiograph showed a hyperlucent right lung, mainly in the upper lobe, with decreased vascular markings compatible with Swyer-James-MacLeod syndrome, or foreign body aspiration. V/Q imaging showed markedly diminished right lung ventilation and perfusion, especially in the upper lobe, with a hot spot in the area of the right bronchus. At bronchoscopy, a pistachio nutshell was removed from the right main bronchus.

Swyer-James syndrome with peculiar course and ipsilateral pulmonary vein defect.

Swyer-James syndrome (SJS) is a rare disease probably resulting from bronchiolitis obliterans. The radiological findings of this entity are characterized by hyperlucent appearance of one or more lobes of a unilateral lung, decreased lung volume, diminished ipsilateral hilar shadow and hardly visible arterial structure on chest radiography. We report a 50-year-old patient, who had unilateral right lower lung hyperlucency on chest radiography. However, the patient reported a history of pulmonary atelectasis of lobus centralis dexter in the course and the corresponding affliction of ipsilateral pulmonary vein, that rendered the diagnosis more complicated. The radiological and pathological features of this syndrome, as well as differential diagnosis were also discussed.

Collateral ventilation to congenital hyperlucent lung lesions assessed on xenon-enhanced dynamic dual-energy CT: an initial experience.

OBJECTIVE: We wanted to evaluate the resistance to collateral ventilation in congenital hyperlucent lung lesions and to correlate that with the anatomic findings on xenon-enhanced dynamic dual-energy CT. MATERIALS AND METHODS: Xenon-enhanced dynamic dual-energy CT was successfully and safely performed in eight children (median age: 5.5 years, 4 boys and 4 girls) with congenital hyperlucent lung lesions. Functional assessment of the lung lesions on the xenon map was done, including performing a time-xenon value curve analysis and assessing the amplitude of xenon enhancement (A) value, the rate of xenon enhancement (K) value and the time of arrival value. Based on the A value, the lung lesions were categorized into high or low (A value > 10 Hounsfield unit [HU]) resistance to collateral ventilation. In addition, the morphologic CT findings of the lung lesions, including cyst, mucocele and an accessory or incomplete fissure, were assessed on the weighted-average CT images. The xenon-enhanced CT radiation dose was estimated. RESULTS: Five of the eight lung lesions were categorized into the high resistance group and three lesions were categorized into the low resistance group. The A and K values in the normal lung were higher than those in the low resistance group. The time of arrival values were delayed in the low resistance group. Cysts were identified in five lesions, mucocele in four, accessory fissure in three and incomplete fissure in two. Either cyst or an accessory fissure was seen in four of the five lesions showing high resistance to collateral ventilation. The xenon-enhanced CT radiation dose was 2.3 ± 0.6 mSv. CONCLUSION: Xenon-enhanced dynamic dual-energy CT can help visualize and quantitate various degrees of collateral ventilation to congenital hyperlucent lung lesions in addition to assessing the anatomic details of the lung.

Pulmón hiperlucente / Lung hiperlucente

El pulmón hiperlucente unilateral también se ha denominado síndrome de Swyer-James, síndrome de Macleod o enfisema lobular o unilateral. Es una enfermedad poco frecuente que se caracteriza por un hiperclaridad pulmonar o lobar unilateral asociada a un atrapamiento aéreo en la espiración. En cuanto a la etiología, existe evidencia que se atribuye a una enfermedad adquirida que aparece tras una infección pulmonar durante la infancia, probablemente una bronquiolitis una neumonía viral. El cuadro clínico es variable: algunos pacientes se encuentran asintomáticos, otros con infecciones de vías respiratorias bajas a repetición, y los que presentan disnea de esfuerzo. El diagnóstico se suele realizar como un hallazgo incidental al hacer una radiografía de tórax a un niño por infecciones respiratorias repetidas, o en la edad adulta en una exploración radiológica de rutina, en una persona por lo demás asintomática. El diagnóstico diferencial de este síndrome se debe hacer con otras entidades torácicas que disminuyen la densidad radiológica pulmonar unilateral. A continuación se describe el caso clínico de un niño que es portador de pulmón hiperlucente.

Implicaciones anestésicas del síndrome de Swyer-James / Anesthetic implications of Swyer-James syndrome

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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 syndrome.

Swyer-James Syndrome is a rare disorder characterized by a unilateral hyperlucent lung. It is thought to be due to sequelae of childhood respiratory infections. It may present with recurrent pulmonary infections, chronic respiratory symptoms or be found incidentally on chest imaging. We report a case of Swyer-James syndrome presenting with recurrent pulmonary infections.

Síndrome de piernas inquietas y neuroacantocitosis / Restless legs syndrome and neuroacanthocytosis

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Hiperclaridad pulmonar: revisión y comentario, a propósito de un caso / Lung hiperlucency: case report and review of the literature

El enfisema bulloso es una forma progresiva de enfisemaque aparece en varones jóvenes. Se realiza una revisión de lasdiferentes causas que se plantean en el diagnostico diferencialde hiperclaridad pulmonar y la aportación de la TAC de alta resoluciónen el estudio radiológico de esta entidad

Bullous emphysema is a progressive variant of emphysemaoccuring in young men. We present a case-report and reviewthe differential diagnosis of lung hyperlucency and the role ofhigh-resolution computed tomography (HRCT) in the radiologicwork-up of this entity

Utility of ventilation and perfusion scan in the diagnosis of young military recruits with an incidental finding of hyperlucent lung.

Swyer-James-MacLeod syndrome (SJMS) is considered to be a relatively uncommon disease presenting with unilateral hyperlucent lung due to hypoplasia of a pulmonary artery and bronchiectasis of the affected lung. In this report, we describe the ventilation-perfusion (V/Q) scan findings of nine male recruits (aged 20-29 years, mean 24.4+/-2.96 years) with SJMS in whom the diagnosis was first established in adulthood. V/Q scan findings of all patients were compared with those on planar radiographs, pulmonary function studies, high resolution computed tomography (HRCT) and digital subtraction angiography (DSA). The ventilation (133Xe) and perfusion (99Tcm-macro-aggregated albumin) scans showed the characteristic pattern of a matched V/Q defect and marked air trapping on the washout phase on 133Xe scintigraphy. HRCT displayed hypodense lung with integrity of main airways, and markedly diminished vasculature on the affected side in all patients. A smaller pulmonary artery on the affected side with poor peripheral vasculature was observed with DSA in all patients. All patients had features of obstructive airway disease in varying degrees on pulmonary function studies. In contrast to other imaging methods, bronchiectasis as an etiological factor was displayed on HRCT. Some pulmonary areas, which were normal on HRCT and planar radiographs, showed air trapping on V/Q scan. Although a V/Q scan was more helpful in determining the extent of the disease and correlates well with conventional imaging methods, HRCT was the most valuable imaging method for the evaluation of aetiology in unilateral hyperlucent lung.

Swyer-James-MacLeod syndrome.

Swyer-James-MacLeod syndrome is a rare complication of respiratory tract infection occurring in early childhood. We report two children with chronic cough and recurrent wheezing who fulfilled the diagnostic criteria for this disorder: 1) Unilateral loss of lung volume with hyperlucency on chest x-ray. 2) Unilateral reduction in vascularity on CT scan of the chest. 3) Unilateral loss of perfusion on Technetium 99c lung scan.

Síndrome de Swyer-James/Macleod: relato de um caso em criança / Swyer-James/MacLeod syndrome: case report in child

Os autores relatam o caso de criança do sexo masculino com hipertransparência pulmonar unilateral e redução do volume do pulmão esquerdo, conseqúente a infecções respiratórias do trato inferior no primeiro ano de vida que configura a síndrome de Swyer-James ou de MacLeod. A broncoscopia foi normal e a arteriografia evidenciou diminuição de calibre da artéria pulmonar esquerda. O paciente foi acompanhado por mais de 10 anos, mantendo-se assintomático e sua função pulmonar, com padrão restritivo, não sofreu alteração dos oito aos 11 anos, época em que deixou de ser seguido.