Swyer–James syndrome

A rare lung disorder characterized by unilateral hyperlucent lung

Overview

Swyer–James syndrome (also known as Swyer–James–Macleod syndrome) is a rare lung disorder characterized by a unilateral hyperlucent lung, which is typically the result of post-infectious obliterative bronchiolitis in childhood. This condition leads to decreased vascularity and air trapping in the affected lung, often resulting in a smaller, less dense lung on chest X-ray.

Pathophysiology

The underlying mechanism of Swyer–James syndrome involves damage to the small airways, or bronchioles, usually following a severe respiratory infection such as adenovirus or measles during childhood. This damage leads to obliterative bronchiolitis, which causes air trapping and reduced perfusion in the affected lung. The result is a lung that appears hyperlucent on imaging due to decreased blood flow and increased air content.

Clinical Presentation

Patients with Swyer–James syndrome may be asymptomatic or present with symptoms such as chronic cough, dyspnea (shortness of breath), and recurrent respiratory infections. The condition is often discovered incidentally during imaging for other reasons.

Diagnosis

Chest X-ray showing unilateral hyperlucent lung characteristic of Swyer–James syndrome.

Diagnosis of Swyer–James syndrome is primarily based on imaging studies. A chest X-ray typically reveals a unilateral hyperlucent lung with reduced vascular markings. Computed tomography (CT) scans can provide more detailed information, showing air trapping and decreased lung volume on the affected side. Pulmonary function tests may show obstructive patterns.

Treatment

There is no specific treatment for Swyer–James syndrome. Management focuses on controlling symptoms and preventing complications. This may include bronchodilator therapy, antibiotics for infections, and vaccination against respiratory pathogens. In severe cases, surgical intervention such as lobectomy may be considered.

Prognosis

The prognosis for individuals with Swyer–James syndrome varies. Many patients lead normal lives with minimal symptoms, while others may experience recurrent infections and progressive respiratory issues. Regular follow-up with a healthcare provider is recommended to monitor lung function and manage any complications.

Related pages

• Bronchiolitis
• Pulmonary function test
• Respiratory infection