Swyer–James syndrome: Difference between revisions

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'''String sign''' is a term used in [[radiology]] and [[gastroenterology]] to describe the appearance of a narrowed, elongated channel, often seen in conditions such as [[Crohn's disease]]. The term is derived from the similarity in appearance to a string on imaging studies.
{{Short description|A rare lung disorder characterized by unilateral hyperlucent lung}}
{{Use dmy dates|date=October 2023}}


== Overview ==
== 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]].


The string sign is a radiographic finding that is suggestive of a long, narrow, tubular structure, often seen in the [[small intestine]]. It is typically associated with conditions that cause inflammation and narrowing of the intestinal lumen, such as Crohn's disease. However, it can also be seen in other conditions such as [[intestinal obstruction]] and [[gastrointestinal stromal tumors]] (GISTs).
== 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 Significance ==
== 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.
In the context of Crohn's disease, the string sign is a significant finding as it indicates the presence of [[stricture]]s or narrowing in the small intestine. This can lead to symptoms such as abdominal pain, bloating, and changes in bowel habits. The presence of a string sign on imaging studies may guide the management and treatment of patients with Crohn's disease.
 
In other conditions such as intestinal obstruction or GISTs, the string sign may also be seen. In these cases, it indicates the presence of a blockage or a mass in the intestine, which may require surgical intervention.


== Diagnosis ==
== Diagnosis ==
 
[[File:Swyer2.jpg|thumb|right|Chest X-ray showing unilateral hyperlucent lung characteristic of Swyer–James syndrome.]]
The string sign is typically identified on [[barium swallow]] or [[computed tomography]] (CT) scan of the abdomen. These imaging studies allow for the visualization of the intestinal lumen and can identify areas of narrowing or obstruction.
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 ==
== 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.


The treatment of conditions associated with the string sign depends on the underlying cause. In Crohn's disease, treatment may involve medications to reduce inflammation and prevent further damage to the intestine. In cases of intestinal obstruction or GISTs, surgical intervention may be required.
== 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.
== See Also ==
 
* [[Crohn's disease]]
* [[Intestinal obstruction]]
* [[Gastrointestinal stromal tumor]]
* [[Radiology]]
* [[Gastroenterology]]


[[Category:Medical terminology]]
== Related pages ==
[[Category:Radiology]]
* [[Bronchiolitis]]
[[Category:Gastroenterology]]
* [[Pulmonary function test]]
* [[Respiratory infection]]


{{stub}}
[[Category:Respiratory diseases]]
[[Category:Rare diseases]]

Revision as of 11:17, 15 February 2025

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.

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