A unilateral hyperlucent lung - Swyer-James syndrome: A case report and literature review

Abstract

Swyer-James-Macleod syndrome (SJMS) is a rare etiology of a unilateral hyperlucent hemithorax but an important one, which should be considered in any individual with such findings. Presentation usually occurs in adulthood with an asymptomatic history in many cases or with a history of childhood infections. Clinically, symptomatic patients may present with productive cough, dyspnea on exertion, hemoptysis, decreased exercise tolerance and recurrent pulmonary infections. Many individuals are asymptomatic only requiring conservative management. However, some patients may require surgical intervention. SJMS can be easily misdiagnosed and must be suspected for example, in any patient diagnosed with asthma who does not respond to therapy. We report a case of a 51-year-old African American male diagnosed with SJMS on the basis of his medical history, clinical presentation and x-rays and computed tomography chest scans findings.

Swyer-James-MacLeod Syndrome: A case report in an adult and review of the literature

“…Non-infective causes of SJMS include foreign body aspiration, inhalation of toxic fumes and hydrocarbon inhalation. [1,2,6] SJMS is characterized by the narrowing and obliteration of the small bronchioles and hypoplasia of the pulmonary arteries leading to hypoperfusion of the pulmonary parenchyma. [1,2] The vascular damage takes place throughout childhood and impedes the expected formation of the alveolar ducts.…”

“…[1,2] The vascular damage takes place throughout childhood and impedes the expected formation of the alveolar ducts. [6] Post-infectious BO results in inflammation and submucosal fibrosis in the airways and leads to airway distortion and occlusion with the diseased distal bronchioles and their lung sacs becoming hyperinflated. [2,6] Destruction of the pulmonary capillary bed and decreased circulation to the principle pulmonary artery segment is caused by the fibrosis of the interalveolar septae.…”

“…[6] Post-infectious BO results in inflammation and submucosal fibrosis in the airways and leads to airway distortion and occlusion with the diseased distal bronchioles and their lung sacs becoming hyperinflated. [2,6] Destruction of the pulmonary capillary bed and decreased circulation to the principle pulmonary artery segment is caused by the fibrosis of the interalveolar septae. [2] Overexpansion of the terminal sacs due to obstruction of the bronchioles of the distal airways increases resistance to blood flow through the alveolar capillaries and leads to deterioration of the vasculature.…”

“…Adult patients present with productive cough, shortness of breath on exertion, hemoptysis, reduced exercise tolerance, and lung infections that can be frequent and recurrent. [6] Diagnosis of SJMS is made incidentally in some cases. [6] Adult patients with SJMS are frequently identified after a radiography of the chest, oftentimes acquired for another reason, demonstrates a unilateral hyperlucency.…”

“…[6] Diagnosis of SJMS is made incidentally in some cases. [6] Adult patients with SJMS are frequently identified after a radiography of the chest, oftentimes acquired for another reason, demonstrates a unilateral hyperlucency. [1] It is essential to exclude other sources of unilateral hyperlucency such as congenital lobar emphysema, bronchogenic cyst, pneumotocele, bronchiectasis with air trapping and emphysema secondary to bronchial stenosis or bronchospasm.…”