Swyer-James-MacLeod syndrome-a rare diagnosis presented through two adult patients

Mehra, Sumit; Basnayake, Thilini; Falhammar, Henrik; Heraganahally, Subash S.; Tripathi, Suryakant

doi:10.1002/rcr2.245

Cited by 16 publications

(11 citation statements)

References 14 publications

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“…Diffused decrease in ventilation of affected lung is noted in the lung V/Q scan. Angiography shows smaller pulmonary artery and its branches on the affected side (15).…”

Section: Discussionmentioning

confidence: 99%

Isolated unilateral absence of pulmonary artery in adulthood: a clinical analysis of 65 cases from a case series and systematic review

et al. 2017

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Background: Isolated unilateral absence of pulmonary artery (UAPA) in adulthood is a rare congenital anomaly. Although some case reports exist, the clinical symptomatology, lung parenchymal features, collateral circulation and therapeutic approaches in adult patients with isolated UAPA remain unknown. The objectives of this study are to investigate the clinical characteristics, elucidate the correlation between clinical symptomatology and radiology, and summarize treatment of adult patients with isolated UAPA. Methods: Cases of adult patients with isolated UAPA who had been diagnosed at our hospital and identified from PubMed, EMBASE and Web of Science from 1990 to 2016 were analyzed. Results: Hemoptysis was present in 41.5% of patients, exertional dyspnea in 41.5%, and recurrent respiratory infection in 35.4%. Lung parenchymal abnormalities were found on chest computed tomography (CT) scan, including bronchiectasis, which occurred in 30.2% of the patients, interstitial changes in 14.0%, and multiple bullae in 14.0% of the patients. Exertional dyspnea was more frequent in patients with pulmonary hypertension than in those without pulmonary hypertension (P<0.001). Recurrent respiratory infection were more frequent in patients with bronchiectasis than in those without bronchiectasis (P<0.001).Hypertrophic bronchial, phrenic, internal thoracic and intercostal arteries were found in 71.9%, 46.9%, 43.8%, and 43.8% of the patients, respectively. Pneumonectomy reduced hemoptysis in seven cases. Oral phosphodiesterase inhibitors or endothelin receptor antagonist improved exertional dyspnea in three cases with pulmonary hypertension. Conclusions: Clinicians should be aware of undiagnosed cases of isolated UAPA in adults with unexplained hemoptysis or exertional dyspnea. Early recognition and management of isolated UAPA in adult patients are crucial to avoid the devastating effect of massive hemoptysis or severe pulmonary hypertension (PHT) in the long term.

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“…Diffused decrease in ventilation of affected lung is noted in the lung V/Q scan. Angiography shows smaller pulmonary artery and its branches on the affected side (15).…”

Section: Discussionmentioning

confidence: 99%

Isolated unilateral absence of pulmonary artery in adulthood: a clinical analysis of 65 cases from a case series and systematic review

et al. 2017

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“…One study estimates the prevalence of the condition as 0.01% after review of 17,450 chest radiographs [5]. Most of the cases reported who were diagnosed as adult patients are below 60 years of age [6][7][8][9] compared to two reports of elderly patients who were 72 and 73 years at the time of diagnosis [7,10]. The classic triad for diagnosis is unilateral hyperlucent lung, diffusely decreased ventilation, and matching decreased perfusion in the affected lung [11].…”

Section: Discussionmentioning

confidence: 99%

A Rare Presentation of a Rare Case: Acute Respiratory Failure in Swyer-James-Macleod Syndrome

Mechineni¹,

Singh²,

Manickam³

2021

Cureus

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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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“…The differential diagnosis of SJMS include pneumothorax, congenital lobar emphysema, pneumatocele, bronchogenic cyst, bullous lung disease, pulmonary embolism, endobronchial foreign body, or postlobectomy compensatory emphysema, with radiologic studies being of extreme importance for their distinction. [1,6]…”

Section: Discussionmentioning

confidence: 99%

Swyer-James-Macleod syndrome as a rare cause of unilateral hyperlucent lung

Machado¹,

Lima²,

Marques³

2019

Medicine

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Rationale: Swyer-James-Macleod syndrome (SJMS) is a rare lung disorder characterized by unilateral hyperlucent lungs which arises as a complication of bronchiolitis obliterans. It is typically diagnosed during childhood, although some patients may only be diagnosed in adulthood, often as an incidental finding, but others due to recurrent chest infections. Patient concerns: Three patients were referred to our institution with complaints of dyspnea on exertion, chronic productive cough, and recurrent pulmonary infections. Two of them had a history of lower respiratory tract infections during childhood. Diagnosis: A computed tomography scan was performed and showed unilateral reduced density of the lung and bronchiectasis in the 3 patients. Based on the clinical presentation and radiologic features, the diagnosis of SJMS was established. Interventions: Patients started inhaled corticosteroids and long acting beta agonist, vaccines to prevent respiratory infections were administered and airway clearance techniques were taught. Outcomes: Two patients became asymptomatic with inhaled therapy and no recurrent chest infections were observed over a 3-year follow-up period, being discharged from our institution to the general practitioner. The 3rd patient had some improvement in the frequency of pulmonary infections and dyspnea, without improvement in respiratory function tests, maintaining vigilance at our center. Lessons: These cases highlight the importance of being aware of this condition and its frequent association with bronchiectasis, which may adversely affect the prognosis, to manage patients appropriately and prevent recurrent pulmonary infections.

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Swyer-James-MacLeod syndrome-a rare diagnosis presented through two adult patients

Cited by 16 publications

References 14 publications

Isolated unilateral absence of pulmonary artery in adulthood: a clinical analysis of 65 cases from a case series and systematic review

Isolated unilateral absence of pulmonary artery in adulthood: a clinical analysis of 65 cases from a case series and systematic review

A Rare Presentation of a Rare Case: Acute Respiratory Failure in Swyer-James-Macleod Syndrome

Swyer-James-Macleod syndrome as a rare cause of unilateral hyperlucent lung

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