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Adult Swyer-James-MacLeod Syndrome; a case report with literature review
Abstract
Introduction
Swyer-James-MacLeod syndrome (SJMS) is a rare obliterative pulmonary disease, usually found in children. The current study aims to report a rare adult case of SJMS.
Case report
A 21-year-old married female presented with dyspnea mainly on exertion. Physical examinations were normal and chest examination showed mild decrease in breathing sounds on the right lower zone. High-resolution computed tomography (HRCT) scan of the chest showed hyperlucency of the right lung with the loss of normal lung vasculature. The patient was managed conservatively with antibiotics, bronchodilator inhaler, and influenza vaccination. The patient was in good health upon follow up.
Discussion
SJMS is considered as a type of post infective obliterative bronchiolitis, which is mainly found in children. Only a few cases have been diagnosed in adults. The syndrome usually occurs following lung infections early in childhood, possibly due to infective agents, such as adenovirus.
Conclusion
Diagnosis of SJMS in adulthood is a very rare finding which may be found despite the absence of any history of pulmonary infections during childhood.
Patient: 11 years, male Clinical History: An 11-year-old male child came with a 4-day history of fever, vomiting, abdominal pain, and a recent bout of cough with expectoration. A past history of congenital stridor, which was subsequently diagnosed as laryngomalacia, was managed conservatively. Since then, the child has had frequent hospitalisations due to recurrent lung infections. Imaging Findings: On chest radiograph, unilateral (right) hyperlucent hemithorax with paucity of vessels/oligemia and mild bronchiectasis in the right lower zone. Bilateral costophrenic angles are blunt (Figure 1). HRCT axial and coronal images show a decrease in right lung volume with diffuse decreased attenuation and diminished vascular markings. Collapse of posterior and anterior segments of right upper lobe associated with traction bronchiectasis involving the right middle lobe and basal segments of the right lower lobe. A thin, smooth-walled air-containing cyst is seen in the superior segment of the right lower lobe. Mild to moderate right pleural effusion. The left lung appears normal (Figures 2a and 2b). Past investigation: In view of congenital stridor, laryngoscopy revealed laryngomalacia. 99 Technetium gastro-oesophageal reflux scintigraphy done at 1 year of his age indicated a moderate degree of gastroesophageal reflux. Discussion: The condition known as "unilateral hyperlucent lung" is characterised radiographically by one lung/lobe having a strikingly lower density compared to the other; caused due to hypoperfusion. Owing to potential causes, like technical (most common) and pathological factors. Swyer-James-MacLeod syndrome (SJS) represents a very rare cause of a hyperlucent unilateral hemithorax with a prevalence rate of 0.01% [1]. It is linked to the long-term sequelae of postinfectious bronchiolitis obliterans in childhood, characterised by pulmonary artery hypoplasia and/or agenesis leading to pulmonary parenchyma hypoperfusion, showing a characteristic pattern of hyperlucent unilateral lung [3]. Due to repetitive infection, lung parenchyma shows an obstructive pattern in the bronchioles, leading to structural changes in the form of permanent dilatation and damage to alveolar structures, air-trapping, and lung hypoperfusion [4].
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.
Swyer-James-Macleod syndrome is an infrequent clinical condition characterized by unilateral hyperlucent lung as a complication following infectious bronchiolitis obliterans, typically diagnosed during childhood. However, in some patients, the diagnosis may be confirmed in adulthood. The syndrome can be misdiagnosed with other lung disorders such as asthma, pulmonary embolism, and pneumothorax, leading to inappropriate management and worse outcome. We present a case of Swyer-James-MacLeod syndrome, diagnosed in a 63-year-old man, with frequent hospitalisations due to chronic obstructive pulmonary disease (COPD) exacerbations without a history of significant lung infection in childhood. Complications of Swyer-James-MacLeod syndrome include recurrent infections, lung abscess, pneumothorax, and pulmonary hypertension. The syndrome should always be considered in adults with recurrent respiratory infections or pulmonary hyperlucency on chest imaging to prevent a delay in correct diagnosis and improper treatment.
Introduction: The SCARE Guidelines were first published in 2016 and were last updated in 2018. They provide a structure for reporting surgical case reports and are used and endorsed by authors, journal editors and reviewers, in order to increase robustness and transparency in reporting surgical cases. They must be kept up to date in order to drive forwards reporting quality. As such, we have updated these guidelines via a DELPHI consensus exercise.
Methods: The updated guidelines were produced via a DELPHI consensus exercise. Members were invited from the previous DELPHI group, as well as editorial board member and peer reviewers of the International Journal of Surgery Case Reports. The expert group completed an online survey to indicate their agreement with proposed changes to the checklist items.
Results: 54 surgical experts agreed to participate and 53 (98%) completed the survey. The responses and suggested modifications were incorporated to the 2018 guideline. There was a high degree of agreement amongst the SCARE Group, with all SCARE Items receiving over 70% scores 7-9.
Conclusion: A DELPHI consensus exercise was completed, and an updated and improved SCARE Checklist is now presented.
Swyer–James–MacLeod syndrome (SJMS) is a rare syndrome of acute obliterative bronchiolitis following an early childhood infective insult to the lungs. This causes arrest of alveolarization, affecting lung development with hypoplasia of the ipsilateral pulmonary artery and results in a characteristic radiological pattern, such as a unilateral hyperlucent lung with expiratory air‐trapping and pruned‐tree appearance on pulmonary angiogram. The clinical presentation is either recurrent chest infections, exertional dyspnoea or it may be an incidental finding. Management involves early prevention of infection, airway clearance, and regular vaccinations.
We describe two adult patients with SJMS: A 51‐year‐old female of Indian ethnicity presenting with recurrent haemoptysis and a 40‐year‐old Indigenous male presenting acutely with sepsis and background history of recurrent chest infections.
These cases highlight the importance of being aware of and accurately recognizing this rare condition, to be able to manage patients appropriately and avoid incorrect and unnecessary treatment.
Swyer-James-MacLeod syndrome or unilateral hyperlucent lung syndrome is a rare disease characterised by hypoplasia of the pulmonary artery, unilateral hyperlucent lung and usually bronchiectasis. The syndrome was first described by Swyer and James in 1953 in a child case, and in the following year 9 adult cases were reported by MacLeod. We retrospectively reviewed 6 adult cases, five female and one male, who were followed-up with the diagnosis of Swyer-James-MacLeod syndrome between 2005 and 2012 in our clinic. The clinical and radiological features of these 6 cases are presented in the light of the literature.
Swyer-James-MacLeod Syndrome (SJMS) is a rare constrictive bronchiolitis with airflow obstruction and decreased number and diameter of ipsilateral peripheral pulmonary vessels . This syndrome is characterized by unilateral hyperlucency on chest radiography. Computerized tomography provides useful additional information. The diagnosis is usually made in childhood, but sometimes occur in adulthood. The disease often presents with dyspnea, decreased exercise tolerance, cough, hemoptysis and recurrent pulmonary infections. Swyer-James-MacLeod Syndrome may be confused with asthma or pulmonary embolism due to similar symptoms and may result in inappropriate therapy. This case series examines the clinical and imaging spectrum of four patients who were diagnosed as SJMS in adulthood.
Swyer-James-Macleod syndrome (SJMS) is a rare, complex disease characterised by unilateral hyperlucent lung or lobe owing to loss of pulmonary vasculature and alveolar hyperdistention. Patients with SJMS may be asymptomatic at diagnosis, but its differential diagnosis with asthma is difficult. Almost all the reported cases of SJMS were managed by conservative therapy, comprising prevention and treatment of recurrent pulmonary infections.
Swyer-James-MacLeod syndrome or unilateral hyperlucent lung syndrome is a rare entity associated with postinfectious bronchiolitis obliterans occurring in childhood. It is characterized by hypoplasia and/or agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion.
Here we report the case of a 53-year-old Caucasian woman with Swyer-James-MacLeod syndrome found in the differential diagnosis workup for a new onset of heart failure, secondary to pulmonary arterial hypertension complicated by a patent ductus arteriosus.
Typically, this disorder is diagnosed in childhood after evaluation for recurrent respiratory infections, but sometimes an indolent course means diagnosis is not made until adulthood.
Swyer-James-MacLeod syndrome is a rare pulmonary disorder that exhibits unilateral hyperlucency of a part of or the entire lung and because it is usually asimptomatic the diagnostic of this illness is discovered accidentally after a chest X-ray. Although the cause of this disorder is not completely understood, most authors believe that this is due to late sequela of infectious bronchiolitis that occurs during childhood. The symptoms presented by the individuals are often scarce at the moment of diagnostic, but a history of recurrent episodes of pulmonary infection accompanied by dyspnea, hemoptysis and/or chronic productive cough should raise the suspicion for such a diagnosis. Swyer-James-MacLeod syndrome can be interpreted as asthma or pulmonary embolism and this can result in inappropriate therapy. This case report describes a 38-year-old patient with chronic respiratory symptoms diagnosed on the findings of the chest X-ray and high resolution CT.
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.
Swyer James syndrome (SJS) is a rare disorder. It is generally discovered on a chest radiograph as increased translucency involving one hemithorax with diminished vascular markings. We present a 5-year-old girl admitted for the treatment of recurrent bronchiolitis. She was diagnosed as having Swyer James syndrome from the results of CT scan and ventilation perfusion scintigraphy, which revealed unsuspected bilateral involvement. This condition should be considered as a differential diagnosis in a patient with Swyer James (Macleod's) syndrome without an obvious etiology.
Swyer-James-MacLeod Syndrome (SJMS) is considered to be a relatively uncommon and complex disease characterized by roentgenographic hyperlucency of one lung, lobe, or part of a lobe, due the pulmonary vascular structure and alveolar overdistension. It is sometimes associated with bronchiectasis. This syndrome seems to be an acquired disease that develops after viral bronchiolitis and/or viral pneumonia in early childhood. Microscopically, there is evidence of patchy bronchitis and bronchiolitis.SJMS is usually asymptomatic and discovered accidentally by chest radiography in a child with respiratory symptoms and should be differentiated from other causes of unilateral hyperlucency on chest radiography, such as those related to congenital bronchial and/or vascular abnormalities. Treatment includes early control of lung infections, as well as influenza and pneumococcal vaccination. Few reports of this syndrome in children have been published. We describe the case of a 12-year-old boy with unilateral hyperlucency of the lung and respiratory symptoms of acute pneumonia and discuss the main diagnostic features of this syndrome.