Pneumonectomy in a patient with swyerjamesmacleod syndrome. Treatment of sjms is usually conservative and involves chest physiotherapy. Swyerjamesmacleod syndrome is a rare disease characterized by unilateral pulmonary hyperlucency on chest radiography. It may be caused by the destruction of peripheral capillaries in the affected lung usually as a result of recurring lung infections during childhood. Swyerjames syndrome sjs, also called macleod syndrome is a rare lung disorder found by english chest physician william mathiseon macleod, and simultaneously by physician paul robert swyer and radiologist george james in the 1950s in canada. Pneumonectomy in an older patient with swyerjamesmacleod. Swyerjamesmacleod syndrome sjms is a rare lung disorder. Pdf the aim of this study is to describe the clinical and imaging features of swyerjamesmacleod syndrome sjms in 9 adults. Mycoplasma pneumoniae, parainfluenza virus types, influenza virus types a and b, respiratory syncytial virus. Xy gonadal dysgenesis, also known as swyer syndrome, is a type of hypogonadism in a person whose karyotype is 46,xy.
Pdf a 31yearold woman presented with a history of dyspnea on exertion. A group of symptoms that collectively indicate or characterize a disease, disorder, or other condition considered abnormal. Swyerjamesmacleod 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. Swyerjamesmacleod syndrome sjms or unilateral hyperlucent lung syndrome is a rare entity associated with postinfectious bronchiolitis obliterans occurring in childhood. The treatment of sjms is often conservative, and usually involves managing current and preventing future pulmonary infections. Treatment of sjms is usually conservative and involves chest.
Swyerjamesmacleod syndrome sjms or unilateral hyperlucent lung syndrome is characterized by hypoplasia andor agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion. It is considered to be an acquired disease secondary to viral bronchiolitis and pneumonitis in childhood. Swyerjames syndrome is a rare condition in which the lung or portion of the lung does not grow normally and is slightly smaller than the opposite lung, usually following bronchiolitis in childhood. Two cmes of this entity are reported that dem onstrate its similarity to and differentiation from pnlmo. It is characterized by hypoplasia andor agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion. The aim of this study was to describe the anesthetic management and perioperative outcomes in patients with swyerjames syndrome sjs, a rare acquired pulmonary. Swyerjames macleod syndrome sjms, first reported in 1953 and 1954, is a rare cause of a unilateral hyperlucent hemithorax. In one case, a middle lobe swyerjamesmacleod syndrome caused complete chronic atelectasis of the healthy right upper lobe. We report a case of a previously healthy young woman that presented with insidious progression of exertional\ dyspnea with restrictive lung physiology. The syndrome occurs due to a vascular and parenchymal development impairment in an area affected by bronchiolitis obliterans in childhood, resulting in hypoplastic vascular regions and emphysematous pulmonary areas.
Swyerjamesmacleod syndrome sjms is a rare lung disorder characterized. The swyerjames macleods syndrome or unilateral hyperlucency of the lung frequently presents a diagnostic problem. Swyerjamesmacleod syndrome sjms, also known as swyerjames syndrome or hyperlucent lung syndrome, is an uncommon syndrome of unilateral functional hypoplasia of the pulmonary vasculature and emphysema, with or without associated bronchiectasis. Swyer jamesmacleod syndrome sjms is a rare constrictive. An unusual case of swyerjamesmacleods syndrome with dextrocardia. Currently there are no established treatment recommendations for swyerjames syndrome to guide care. Swyerjames syndrome is a manifestation of postinfectious obliterative bronchiolitis. Mcleod syndrome, also known as mcleod phenomenon is a rare xlinked syndrome caused by a mutation in the xk gene that results in hematological abnormalities and lateonset, slowly progressive degeneration of a variety of tissues including the brain, peripheral nerves, muscle and heart 1 it should not be confused with swyerjamesmacleod syndrome postinfectious obstructive bronchiolitis. Regarding the etiology, swyerjames syndrome is thought to be acquired following infections that occurred during infancy or childhood 3. Anesthetic implications for patients with swyerjames syndrome. The diagnosis is usually made in childhood but sometimes. Clinical, physiologic, and roentgenographic changes after pneumonectomy in a boy with macleodswyerjames syndrome and bronchiectasis 30 september 2002 pediatric pulmonology, vol. A case of unilateral hyperlucent lung ats journals.
There are isolated case reports and small case series describing the diagnosis of swyerjamesmacleod syndrome in adults 49. Swyerjames macleod syndrome is an acquired form of unilateral hyperlucency of the lung and is characterized by the development of severe emphysema, bronchiectasis, andor bronchiolitis obliterans. The progression of swyerjames syndrome american journal of. Swyerjamesmacleod syndrome is often diagnosed in childhood and the longterm clinical course of swyerjamesmacleod syndrome in adults has not been well described. Swyerjamesmacleod syndrome or unilateral hyperlucent lung. In sjs, the involved lung or portion of the lung does not grow normally and is slightly smaller than the opposite lung.
Pdf pneumonectomy in a patient with swyerjamesmacleod. Swyerjamesmacleod syndrome sjms is a rare constrictive. Another patient with swyerjames syndrome, this time of the left lung. Here we report the case of a 53yearold caucasian woman with swyerjamesmacleod syndrome. Computed tomography provides useful additional information. The distinctive radiographic signs of the swyerjames syndrome are a unilateral hyperlucent lung of normal or decreased size with diminished lung markings, a smaller hilar shadow than the opposite lung, and slight displacement of the mediastinum to the affected side. It is typically diagnosed after a chest xray or ct scan which shows unilateral pulmonary hyperlucency one lung appearing less dense and diminished pulmonary arteries. Almost all the reported cases of sjms were managed by conservative therapy, comprising. It is typically diagnosed after a chest xray or ct scanwhich shows unilateralpulmonary hyperlucency one lung appearing less dense caused by overdistention of the alveoli in conjunction with diminished arterial flow. Pdf a case of swyerjamesmacleod syndrome diagnosed in.
It is recognized as one of several causes of unilateral translucent lung. Swyerjames syndrome is a rare condition characterized by a unilateral hyperlucency in the lung secondary to infectious bronchiolitis acquired in childhood. Swyerjamesmacleod syndrome the american journal of. A rare case of swyerjames macleod syndrome and a new clinical presentation. Swyerjamesmacleod syndrome is a rare form of postinfectious bronchiolitis obliterans. Besides the described pathological perfusion changes in the diseased lung sections, their occasionally bizarre hyperinflation results in a compression of healthy lung areas, ultimately causing their atelectases.
Sjms was first described in 1953 by swyer and james and considered to be an acquired disease secondary to viral respiratory infections during childhood. Swyerjamesmacleod syndrome sjms is a rare condition characterized by unilateral hyperlucency of a part of or the entire lung which was first described in 1953 by swyer and james 1,2, and further detailed by macleod. However treatment is usually described as conservative. We report the case of a 54yearold woman who experienced exertional dyspnea owing to swyerjamesmacleod syndrome associ. Swyerjamesmacleod syndrome also known as swyerjames syndrome or unilateral hyperlucent lung syndrome is a rare acquired lung disorder, being first described in the 1950s. This syndrome is most often seen in children, but can rarely present in adults with a. An unusual case of swyerjamesmacleods syndrome with. Swyerjamesmacleod syndrome statpearls ncbi bookshelf. She was diagnosed as having swyer james syndrome from the results of ct scan and ventilation perfusion scintigraphy, which revealed unsuspected bilateral involvement.
Swyerjames syndrome definition of swyerjames syndrome. This disease was first described in 1953 by swyer and james in a 6yearold child who had been treated with pneumonectomy 2. I suffer from swyerjames syndrome mcleod syndrome too, and had a. Case report by journal of evolution of medical and dental sciences. Swyerjamesmacleod syndrome sjms is a rare lung disorder characterized by unilateral hyperlucent lungs. Swyerjamesmacleod syndrome presenting with pulmonary. Patients with sjms may be asymptomatic at diagnosis, but its differential diagnosis with asthma is difficult. They typically have normal female external genitalia, identify as female, and are raised as girls the person is externally female but with functionless gonads, fibrous tissue termed streak gonads, and if left untreated, will not experience puberty. Swyerjamesmacleod syndrome or unilateral hyperlucent lung syndrome is a rare entity associated with postinfectious bronchiolitis obliterans occurring in childhood. Swyerjames or macleod syndrome is a postinfectious constrictive bronchiolitis that is usually the sequelae of severe childhood pneumonia. Six adults with swyerjamesmacleod syndrome european. Swyerjames macleod syndrome sjms is a rare condition characterized by unilateral hyperlucency of part of whole of lung which. Swyerjamesmacleod syndromea rare diagnosis presented.
Original research article anesthetic implications for patients with swyerjames syndrome mark m smith, md, david w barbara, md, benjamin c smith, md, juraj sprung, md, phd, and toby n weingarten, md objective. Presents as an incidental finding on chest xray, with chronic symptoms or. In this case, swyerjamesmacleod syndrome associated with a giant calcified bulla that compressed the patients left lung was treated with right pneumonectomy. Swyerjamesmacleod syndrome sjms is a rare, complex disease characterised by unilateral hyperlucent lung or lobe owing to loss of pulmonary vasculature and alveolar hyperdistention. Adenovirus types 3, 7, and 21 have been implicated most commonly, but other infectious etiologies include. Swyerjamesmacleod syndrome sjms is a rare constrictive bronchiolitis with airflow obstruction and a decreased number and diameter of ipsilateral peripheral pulmonary vessels. Pdf swyerjamesmacleod syndrome sjms is a rare constrictive bronchiolitis with airflow obstruction and decreased number and diameter of ipsilateral.
It is a relatively uncommon entity, occurring in 3. Swyerjamesmacleod syndrome and pulmonary arteriovenous. Swyerjamesmacleod syndrome sjms, or unilateral hyperlucent lung syndrome, is a rare disease thought to be associated with postinfectious bronchiolitis obliterans 1. Swyerjames macleod syndrome, also named unilateral hyperlucent syndrome, is a rare condition which was first described by swyer and james in 1953 followed by macleod in 1954. Adult diagnosis of swyerjamesmacleod syndrome bmj case. Swyer james macleod syndrome congenital heart defect lung. Swyerjames syndrome synonyms, swyerjames syndrome pronunciation, swyerjames syndrome translation, english dictionary definition of swyerjames syndrome. Pdf clinical spectrum of swyerjamesmacleod syndrome in adults. On closer examination, note how many fewer pulmonary blood vessels there are on the left than the right. Swyerjames syndrome sjs is a manifestation of postinfectious obliterative bronchiolitis. Swyerjamesmacleod syndrome is a rare disease that results in parenchymal damage believed to be caused by recurrent lung infections in childhood. Swyerjamesmacleod syndrome as a rare cause of unilateral. Swyerjamesmacleod syndrome as a rare cause of unilateral h.
This syndrome is characterized by unilateral hyperlucency on chest radiography. Swyerjames syndrome american journal of respiratory and. For most people with swyerjames syndrome, treatment aims to control symptoms such as cough, shortness of breath, chest pain, wheezing, and recurring infections. Health, general fever hyperthermia respiratory tract diseases.
The following year, macleod 2 reported nine patients with unilateral pulmonary hyperlucency. In most patients with swyerjamesmacleod syndrome, treatment is managed by chest physiotherapy, lowdose inhaled corticosteroids, and inhaled. Bilateral swyerjames macleods syndrome springerlink. Pulmonary mr angiography in swyerjames syndrome 240 european journal of general medicine introduction swyerjames syndrome sjs or macleod syndrome, which is synonymous, was first described by swyer and james 1953 1. The disorder is the manifestation of postinfectious obliterative bronchiolitis of the lung or a part of the lung 2. Swyerjames 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. Swyer james syndrome sjs, also known as swyer james macleod syndrome and bret syndrome, is a rare lung condition that manifests as unilateral hemithorax lucency as a result of postinfectious obliterative bronchiolitis. This condition should be considered as a differential diagnosis in a patient with swyer james macleods syndrome without an obvious etiology. Sjms treatment includes the early control of lung infections as well as. Notice the left hemithorax is more lucent than the right. Diagnostic and surgical considerations in swyerjamesmacleod syndrome. It is characterized by hypoplasia andor agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion, showing a characteristic radiological pattern, such as translucent or hyperlucent unilateral lung 2. Swyerjamesmacleod syndrome sjms is an uncommon obliterative lung disease that is a rare complication of a lower respiratory tract infection during childhood.
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