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 (associated with adenovirus types 3, 7, and 21; paramyxovirus; morbillivirus; Bordetella pertussis; Mycoplasma pneumoniae; Mycobacterium tuberculosis; and influenza A) obliterative bronchiolitis. It is generally characterized on radiographs by a unilateral small lung with hyperlucency and air trapping on expiration. Typically, this disorder is diagnosed in childhood after an evaluation for recurrent respiratory infections but sometimes patients who have little or no sequelae bronchiectasis have minor symptoms or are asymptomatic and may, therefore, miss their diagnosis until adulthood.
Here we report the case of a 24-year-old Turkýsh man with Swyer-James-MacLeod syndrome found in the differential diagnosis workup for a new chief complaint of pain in the chest. Her blood pressure was 124/75 mm Hg, pulse rate of 73 beats per minute, respiratory rate of 14 breaths per minute with an oxygen saturation of 98% while breathing room air. He had a white-cell count of 6800 per mm3, a hematocrit value of 36% and her platelet count was 128,000 per mm3. Levels of serum electrolytes, creatinine and urea nitrogen were normal and levels of hepatic transaminases, serum alkaline phosphatase, bilirubin, total protein and albumin were also normal. Cardiac enzymes were negative and the level of B-type natriuretic peptide was 108 pg per mL (normal range 0 to 100).
The chest radiograph on admission disclosed unilateral hyperlucency and reduced volume in the left lung. Computed tomography (CT) scans demonstrated pulmonary artery hypoplasia, decrease in density, and volume loss in the left lung and unilateral loss of perfusion on technetium Tc 99m lung scan.
Treatment of SJMS is typically conservative and supportive, including close follow-up and management of recurrent pulmonary infections and rarely including lung resections for recalcitrant disease. Our case was treated conservatively, with chest physiotherapy and pulmonary infection prevention.
SJMS can be confused with many chronic lung diseases due to similar symptoms and may result in inappropriate therapy. Physicians should be aware of this condition and its frequent association with bronchiectasis, to manage patients appropriately, prevent recurrent pulmonary infections, and achieve a better prognosis.
Anahtar Kelimeler: Swyer-James-MacLeod Syndrome, Hyperlucency,
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