Swyerjamesmacleod syndrome sjms is a rare constrictive. There are isolated case reports and small case series describing the diagnosis of swyerjamesmacleod syndrome in adults 49. Swyerjamesmacleod syndrome sjms is a rare constrictive bronchiolitis with airflow obstruction and a decreased number and diameter of ipsilateral peripheral pulmonary vessels. A case of unilateral hyperlucent lung ats journals. Swyerjames macleod syndrome sjms is a rare condition characterized by unilateral hyperlucency of part of whole of lung which. Swyerjames or macleod syndrome is a postinfectious constrictive bronchiolitis that is usually the sequelae of severe childhood pneumonia. The aim of this study was to describe the anesthetic management and perioperative outcomes in patients with swyerjames syndrome sjs, a rare acquired pulmonary. I suffer from swyerjames syndrome mcleod syndrome too, and had a. 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.
Mycoplasma pneumoniae, parainfluenza virus types, influenza virus types a and b, respiratory syncytial virus. Swyerjamesmacleod syndrome statpearls ncbi bookshelf. This syndrome is characterized by unilateral hyperlucency on chest radiography. Swyerjamesmacleod syndrome is a rare form of postinfectious bronchiolitis obliterans. Swyerjamesmacleod syndrome is a rare disease that results in parenchymal damage believed to be caused by recurrent lung infections in childhood. Swyerjamesmacleod syndrome presenting with pulmonary. Xy gonadal dysgenesis, also known as swyer syndrome, is a type of hypogonadism in a person whose karyotype is 46,xy. 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. It is recognized as one of several causes of unilateral translucent lung. Swyerjamesmacleod syndrome is a rare disease characterized by unilateral pulmonary hyperlucency on chest radiography.
We report a case of a previously healthy young woman that presented with insidious progression of exertional\ dyspnea with restrictive lung physiology. Swyerjamesmacleod syndrome sjms or unilateral hyperlucent lung syndrome is characterized by hypoplasia andor agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion. The disorder is the manifestation of postinfectious obliterative bronchiolitis of the lung or a part of the lung 2. It is considered to be an acquired disease secondary to viral bronchiolitis and pneumonitis in childhood. Diagnostic and surgical considerations in swyerjamesmacleod syndrome. Swyerjamesmacleod syndrome as a rare cause of unilateral h. 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. Swyerjames syndrome is a rare pulmonary disease characterized by the presence of a unilateral hyperlucent lung 1. Swyerjamesmacleod syndromea rare diagnosis presented. 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 is a rare lung disorder. Swyerjamesmacleod syndrome sjms is a rare lung disorder characterized by unilateral hyperlucent lungs. This syndrome is most often seen in children, but can rarely present in adults with a. Swyerjamesmacleod syndrome also known as swyerjames syndrome or unilateral hyperlucent lung syndrome is a rare acquired lung disorder, being first described in the 1950s. Health, general fever hyperthermia respiratory tract diseases. Pdf a case of swyerjamesmacleod syndrome diagnosed in. Adult diagnosis of swyerjamesmacleod syndrome bmj case. Notice the left hemithorax is more lucent than the right. Pdf a 31yearold woman presented with a history of dyspnea on exertion. 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. Adenovirus types 3, 7, and 21 have been implicated most commonly, but other infectious etiologies include. Currently there are no established treatment recommendations for swyerjames syndrome to guide care. Anesthetic implications for patients with swyerjames syndrome. A rare acquired lung anomaly where one lung is more translucent than the other.
Swyerjamesmacleod syndrome and pulmonary arteriovenous. Computed tomography provides useful additional information. 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. Regarding the etiology, swyerjames syndrome is thought to be acquired following infections that occurred during infancy or childhood 3.
Swyerjames syndrome is a manifestation of postinfectious obliterative bronchiolitis. The treatment of sjms is often conservative, and usually involves managing current and preventing future pulmonary infections. 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. This disease was first described in 1953 by swyer and james in a 6yearold child who had been treated with pneumonectomy 2. We report the case of a 54yearold woman who experienced exertional dyspnea owing to swyerjamesmacleod syndrome associ. 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.
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 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. The following year, macleod 2 reported nine patients with unilateral pulmonary hyperlucency. Presents as an incidental finding on chest xray, with chronic symptoms or. Here we report the case of a 53yearold caucasian woman with swyerjamesmacleod syndrome. Swyer james macleod syndrome congenital heart defect lung. Swyerjamesmacleod syndrome sjms is a rare lung disorder characterized. Another patient with swyerjames syndrome, this time of the left lung. Pdf the aim of this study is to describe the clinical and imaging features of swyerjamesmacleod syndrome sjms in 9 adults. Swyerjamesmacleod syndrome the american journal of. Pneumonectomy in a patient with swyerjamesmacleod syndrome. Swyerjames syndrome definition of swyerjames syndrome. Treatment of sjms is usually conservative and involves chest. Swyerjamesmacleod syndrome sjms is an uncommon obliterative lung disease that is a rare complication of a lower respiratory tract infection during childhood.
On closer examination, note how many fewer pulmonary blood vessels there are on the left than the right. 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. Pdf clinical spectrum of swyerjamesmacleod syndrome in adults. Swyerjamesmacleod syndrome or unilateral hyperlucent lung syndrome is a rare entity associated with postinfectious bronchiolitis obliterans occurring in childhood. It may be caused by the destruction of peripheral capillaries in the affected lung usually as a result of recurring lung infections during childhood. 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 or unilateral hyperlucent lung. However treatment is usually described as conservative. A group of symptoms that collectively indicate or characterize a disease, disorder, or other condition considered abnormal. The progression of swyerjames syndrome american journal of. It is characterized by hypoplasia andor agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion.
Swyerjames syndrome synonyms, swyerjames syndrome pronunciation, swyerjames syndrome translation, english dictionary definition of swyerjames syndrome. 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. Pneumonectomy in an older patient with swyerjamesmacleod. An unusual case of swyerjamesmacleods syndrome with. Swyer jamesmacleod syndrome sjms is a rare constrictive. It is a relatively uncommon entity, occurring in 3. Treatment of sjms is usually conservative and involves chest physiotherapy. Swyerjamesmacleod syndrome sjms, or unilateral hyperlucent lung syndrome, is a rare disease thought to be associated with postinfectious bronchiolitis obliterans 1. In most patients with swyerjamesmacleod syndrome, treatment is managed by chest physiotherapy, lowdose inhaled corticosteroids, and inhaled. In this case, swyerjamesmacleod syndrome associated with a giant calcified bulla that compressed the patients left lung was treated with right pneumonectomy. The swyerjames macleods syndrome or unilateral hyperlucency of the lung frequently presents a diagnostic problem.
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. Swyerjames syndrome american journal of respiratory and. Sjms was first described in 1953 by swyer and james and considered to be an acquired disease secondary to viral respiratory infections during childhood. Sjms treatment includes the early control of lung infections as well as. Swyerjamesmacleod syndrome is often diagnosed in childhood and the longterm clinical course of swyerjamesmacleod syndrome in adults has not been well described. Almost all the reported cases of sjms were managed by conservative therapy, comprising. Swyerjames syndrome is a rare condition characterized by a unilateral hyperlucency in the lung secondary to infectious bronchiolitis acquired in childhood. 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. 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. Bilateral swyerjames macleods syndrome springerlink. Swyerjames macleod syndrome sjms, first reported in 1953 and 1954, is a rare cause of a unilateral hyperlucent hemithorax. 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. An unusual case of swyerjamesmacleods syndrome with dextrocardia.
Six adults with swyerjamesmacleod syndrome european. 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. 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. Swyerjamesmacleod syndrome as a rare cause of unilateral. Swyerjamesmacleod syndrome sjms is a rare, complex disease characterised by unilateral hyperlucent lung or lobe owing to loss of pulmonary vasculature and alveolar hyperdistention. In one case, a middle lobe swyerjamesmacleod syndrome caused complete chronic atelectasis of the healthy right upper lobe. Patients with sjms may be asymptomatic at diagnosis, but its differential diagnosis with asthma is difficult. For most people with swyerjames syndrome, treatment aims to control symptoms such as cough, shortness of breath, chest pain, wheezing, and recurring infections. The diagnosis is usually made in childhood but sometimes.
Two cmes of this entity are reported that dem onstrate its similarity to and differentiation from pnlmo. Clinical, physiologic, and roentgenographic changes after pneumonectomy in a boy with macleodswyerjames syndrome and bronchiectasis 30 september 2002 pediatric pulmonology, vol. Swyerjames syndrome sjs is a manifestation of postinfectious obliterative bronchiolitis. Case report by journal of evolution of medical and dental sciences. 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. This condition should be considered as a differential diagnosis in a patient with swyer james macleods syndrome without an obvious etiology. Pdf swyerjamesmacleod syndrome sjms is a rare constrictive bronchiolitis with airflow obstruction and decreased number and diameter of ipsilateral. Retrospective analysis of four cases hadice selimoglu sen md, mahsuk taylan md, ozlem abakay md, cengizhan sezgi md, and mehmet guli cetincakmak md swyerjamesmacleod syndrome sjms is a.
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