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We present a rare finding on lung ventilation-perfusion (V/Q) scintigraphy for a woman with longstanding dyspnea. CT of the chest showed volume loss on the right side, which raised concern about possible bronchiolitis obliterans or Swyer-James-MacLeod syndrome; however, the right pulmonary artery could not be visualized. A subsequent V/Q scan showed absence of perfusion and decreased ventilation to the entire right lung, consistent with agenesis of the right pulmonary artery. The patient's clinical course and imaging features mimicked Swyer-James-MacLeod syndrome, which usually presents with a matched perfusion defect in a single lung or lobe on V/Q scanning. This case highlights the importance of a multimodality imaging approach to achieve a diagnosis.

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BACKGROUND: Swyer-James-MacLeod syndrome (SJMS) is a rare lung condition characterized by a unilateral lung hyperlucency and reduction in the pulmonary vasculature, with or without the presence of bronchiectasis. In the 1950s, Swyer, James, and Macleod simultaneously characterized the syndrome for the first time. It is typically diagnosed in childhood. Adult-onset cases are extremely rare, with little literature available on its clinical presentation and diagnostic challenges. Swyer-James-MacLeod syndrome can mimic other lung disorders, resulting in misdiagnosis and improper treatment. CASE PRESENTATION: A 49- year-old woman from Debre Berhan, Ethiopia, presented to the emergency department of Hakim Gizaw Teaching Hospital with symptoms and radiographic findings mimicking acute pulmonary embolism. On the basis of the clinical presentation and radiographic findings, the patient was first treated as a probable case of pulmonary embolism. Anticoagulant therapy and oxygen support were initiated. Nevertheless, additional testing using a chest computed tomography angiography revealed left lung hyperlucency, decreased vascularity, bronchiectasis, and a negative result for pulmonary embolism. As a result, Swyer-James-MacLeod syndrome was diagnosed. CONCLUSION: The symptoms of Swyer-James-MacLeod syndrome can be mistaken for pulmonary embolism, which could lead to ineffective treatment and needless expenses. In individuals presenting with symptoms suggestive of pulmonary embolism, this case emphasizes the significance of considering Swyer-James-MacLeod syndrome as a differential diagnosis, especially in the absence of established risk factors for pulmonary embolism.

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When the chest radiograph of a young patient shows lung hyperlucency, it is important to obtain a detailed clinical history of any previous episodes of childhood infection. Previous chest radiographs should be reviewed to determine whether the condition is congenital or acquired, and thus assist in a diagnosis of SJMS.

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Swyer-James-MacLeod Syndrome is a rare obliterative lung disease typically caused by childhood infection resulting in arrested pulmonary development. Imaging findings include unilateral hyperlucency on chest x-ray, and hyperlucency, hypovascularity and expiratory gas trapping on computed tomography. Recognition of abnormal imaging can lead to earlier diagnosis and institution of appropriate management.

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Asthma is one of the most common diseases. However, in patients with refractory asthma, chest imaging assessment should be performed, bearing in mind the possibility of other diseases.

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Scant literature is available regarding pregnancy outcomes in women with Swyer-James-MacLeod syndrome, a rare obstructive lung disease. We present a case of a woman with this syndrome in pregnancy. Her baseline pulmonary function tests (PFT) demonstrated moderate airflow obstruction however she had excellent functional status and exercise tolerance. Her disease remained clinically stable in pregnancy. PFTs demonstrated slight worsening of her obstruction with forced expiratory volume in one second (FEV1). 59% and FEV1/FVC ratio 64%. She was diagnosed with gestational diabetes requiring metformin and insulin. Her labor and delivery was uncomplicated with vaginal delivery of a live male at term with no maternal respiratory complications. She did have a delayed postpartum hemorrhage requiring a D&C procedure. This case report demonstrates women with Swyer-James-MacLeod syndrome can have a successful pregnancy and need not avoid pregnancy if desired.

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Pediatric pulmonary hypertension (PH) has a similar clinical presentation to the adult disease but is associated with several additional disorders and challenges that require a specific approach for their fulminant course. With improved care for premature infants, various forms of pulmonary vascular disease have been found in children that did not previously exist. Pediatric PH can begin in utero, resulting in pulmonary vascularity growth abnormalities that may persist into adulthood. Here, we retrospectively reviewed several unique pediatric PH cases from 2000 to 2020 at Kaohsiung Medical University Hospital, Taiwan, a tertiary teaching hospital. Their comorbidities varied and included surfactant dysfunction, bronchopulmonary dysplasia, premature closure of the ductus arteriosus, high levels of renin and aldosterone, and Swyer-James-Macleod syndrome. Their clinical profiles, radiological characteristics, echocardiography, pulmonary angiogram, and therapeutic regimens were recorded. Further, because the underlying causes of pediatric PH were complex and markedly different according to age, adult PH classification may not be applicable to pediatric PH in all settings. We also classified these cases using different systems, including the Panama classification and the Sixth World Symposium on PH, and compared their advantages and disadvantages.

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Coronavirus disease 2019 (COVID-19) exerts differential effects on various individuals. The majority of infected individuals experience mild-to-moderate disease and usually recover, without requiring hospitalization. It has been reported that those who have underlying chronic diseases are more susceptible to infection and may thus develop significantly more serious illness. As a result, COVID-19 may aggravate pre-existing respiratory illnesses, such as interstitial lung disease, chronic obstructive pulmonary disease and asthma. Swyer-James-MacLeod syndrome is an uncommon clinical condition marked by post-infectious infantile bronchiolitis obliterans. Traditionally, the diagnosis is made in infancy following an investigation for reoccurring respiratory infections, although in rare cases, the diagnosis is made in adulthood. The present study describes the case of a 45-year-old patient with Swyer-James-MacLeod syndrome hospitalized due to COVID-19, which is the first one to be reported. To the best of our knowledge, there are currently no data available on the effects of COVID-19 in these individuals, stheir optimal therapy, or the impact of COVID-19 vaccination on their clinical course. Thus, it is hoped that the present study sheds some light into this condition.

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Swyer-James-MacLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by obliteration of the small bronchioles, hypoplasia/or absence of pulmonary artery and peripheral vascular bed. It is most commonly diagnosed in childhood. Patients are often asymptomatic or they could suffer from symptoms of recurrent pulmonary infections. Spontaneous pneumothorax is a rare presentation of this syndrome. We report a case of a 42-year old female patient presented at our Emergency Department with complaints of dyspnea and pleuritic chest pain with a diagnosis of spontaneous pneumothorax in emphysematous disease. The diagnosis of SJMS is usually based on imaging and clinical findings rather than on the results of pathologic examination; indeed, asymptomatic adult patients with SJMS are often diagnosed after a chest radiograph obtained for another reason. High-resolution computed tomography (HRCT) seems to be the most appropriate technique for the diagnosis. SJMS can be associated with spontaneous pneumothorax which represent an emergency condition due to the underlying pathological disease. The surgical treatment of the affected lung should be considered when conservative approach is ineffective.

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Swyer-James-Macleod syndrome (SJMS) is a rare clinical entity acquired during childhood due to a respiratory infection leading to bronchiolitis obliterans. This inciting event is hypothesized to cause structural and functional changes of the developing alveoli, terminal bronchioles, and the corresponding pulmonary vasculature, resulting in emphysematous changes and a matched ventilation-perfusion defect. We present a 67-year-old male patient with hypercapnic respiratory failure requiring invasive mechanical ventilation, who had typical features of SJMS undiagnosed before this admission. He was extubated successfully, discharged home, and continues to be stable at a 90-day follow-up period. This marks one of the rare accounts where a patient with SJMS was given ventilatory support emergently, and one of the oldest patients reported. SJMS is under-reported due to its indolent clinical course and misdiagnosed as some other pulmonary abnormality. The clinical course progression and prognosis are unclear and variable in many affected patients due to this condition's rarity.

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Unilateral hyperlucent lung was firstly described by Swyer and James 1950s. After that, some patients with same disease were detected by Macleod . Then this syndrome was named as Swyer- James-Macleod syndrome (SJMS), and this syndrome includes a smaller or normal sized unilateral hyperlucent lung. The diagnosis of SJMS includes a detailed evaluation and the exclusion of other reasons of unilateral hypertranslucency. In literature, small groups of patients with this syndrome have been described. This paper reports a 45-year-old male presented to our emergency department with chest pain after fall from height 24 h ago. In his computed tomography no rib fracture, pneumothorax and hemothorax. But an abnormal image (5.5 cm hyperlucent area) was seen in his left lung. This report aims to present one of incidentally diagnosed rare case of SJMS.

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BACKGROUND AND AIM: In patients with pulmonary embolism (PE), a pulmonary radiograph may reveal oligemic fields (the Westermark sign) associated with sites of occlusion of the pulmonary arteries, interruption or loss of the artery line (the knuckle sign), and even unilateral hyperlucency attributable to reduced overall lung vascularity. In Swyer-James-Macleod syndrome (SJMS), which develops as a result of bronchiolitis obliterans, unilateral hyperlucency is evident because of emphysema and hypoplasia of the pulmonary artery and its branches. Therefore, SJMS cases with clinical and laboratory data compatible with PE may in fact be confused with PE. The cases of six adult patients who were initially presumed to have PE but on further investigation were diagnosed with SJMS are presented in this report, which thus can serve as a guide for diagnosis of similar cases in future. METHODS: We studied six adult patients who presented with dyspnea. Their pulmonary radiographs revealed lobar/unilateral hyperlucency and PE was initially suspected. The pulmonary artery and branches thereof exhibited parenchymal emphysema and hypoplasia, and we thus diagnosed SJMS. RESULTS: We studied 4 males and 2 females with a mean age of 51 years (range, 20-73 years). Left lung involvement was evident in five cases. CONCLUSION: Unilateral hyperlucency may be a feature of both PE and SJMS. Although these conditions are very different, both present similarly in radiographic terms and may be easily confused when the clinical data and the anamnesis raise a suspicion of PE, causing unnecessary testing and treatment.

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Swyer-James-MacLeod syndrome (SJMS) is an uncommon obliterative lung disease that is radiologically characterized by hyperlucency of a part of or the entire lung. A 33-year-old man presented to our hospital for chest tightness. A chest X-ray revealed unilateral hyperlucency of left lower lung, and contrast-enhanced computed tomography (CT) of the chest disclosed a hyperlucent left lung without vascularity. Three-dimensional CT reconstruction and ventilation-perfusion scan findings were concordant with SJMS. We herein report a case of SJMS in a patient who showed the characteristic multimodal imaging findings.

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Swyer-James-Macleod syndrome (SJMS) is a rare etiology of a unilateral hyperlucent hemithorax but an important one, which should be considered in any individual with such findings. Presentation usually occurs in adulthood with an asymptomatic history in many cases or with a history of childhood infections. Clinically, symptomatic patients may present with productive cough, dyspnea on exertion, hemoptysis, decreased exercise tolerance and recurrent pulmonary infections. Many individuals are asymptomatic only requiring conservative management. However, some patients may require surgical intervention. SJMS can be easily misdiagnosed and must be suspected for example, in any patient diagnosed with asthma who does not respond to therapy. We report a case of a 51-year-old African American male diagnosed with SJMS on the basis of his medical history, clinical presentation and x-rays and computed tomography chest scans findings.

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Swyer-James-Macleod syndrome (SJMS) is a rare disorder characterized by infective bronchiolitis. It typically presents with a history of recurrent chest infections during childhood. Cardinal sign of the disease includes the hyperlucency of the lung fields. Till date, only a few cases have been reported of this syndrome. We report a very unusual case of SJMS along with comorbid schizophrenia in a 20-year-old female and the successful management using clozapine.

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Swyer-James (Macleod) syndrome was first defined in the 1950s by Swyer, James and Macleod in patients with unilateral hyperlucent lungs. Coronary artery anomalies are congenital anomalies that affect a small part of the population. They constitute about 1-2% of congenital heart diseases. The incidence of a left coronary artery arising from the right coronary sinus Valsalva has been reported as 0.017%, and 1.3% among coronary artery anomalies. We hereby present this case since the case was diagnosed in adult age and was accompanied by a rare congenital heart disease.

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A case of a 19-year-old with severe chronic obstructive pulmonary disease is presented. This case illustrates genetic (severe alpha-1 antitrypsin deficiency) and host factors (such as developmental diaphragmatic hernia and the innate response to injury), and environmental (high oxidative stress and lung injury) interactions that lead to severe chronic obstructive lung disease. The development of chronic lung disease was caused by lung injury under high oxidative and inflammatory conditions in the setting of a diaphragmatic hernia. In the absence of normal alpha-1 antitrypsin levels, a pro-elastolytic environment in the early period of lung growth enhanced the development of severe hyperinflation and precocious airflow obstruction.

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