Clinical diagnosis
Case 328
【Progress】
His pneumothorax was confirmed to be localized at the left apical region on CT. Then, our physician advised him to stay home without aggressive athletics.
【Discussion】
Swyer James syndrome is known to be hemi lateral hyperlucent on chest radiograph. It is first reported in 1953 by Canadian Paul Robert Swyer and George James, followed by English William Mathiseon MacLeod in 1954, then, called Swyer James MacLeod syndrome (1,2). It appears with incidence of 0.01% in a series of 17450 chest radiographs (3). It occurs in 4% of obstructive bronchiolitis (4). Left lateral hyperlucent is more dominant rather than right whose reason is unclarified.
Both bronchus and bronchioles have cilia on surface to eliminate pathogen. However, bronchioles do not own either cartilage or gland in mural bronchus own. In taking place of cartilage, bronchioles mural is reinforced by elastic fiber. The absence of gland indicates absence of goblet cells which secrete mucin which is one of the barriers for pathogen invasion to bronchial cells. Then, it might refer that bronchioles are a little susceptible to bronchus. Obstructive bronchiolitis sometimes conjugates with bronchiectasis.
The etiology is still unknown: congenital or infection early after birth. However, it is becoming to be believed its cause is severe obstructive bronchiolitis caused by early severe infection after birth by adenovirus, mycoplasma, staphylococcus and so on because the process of forming hyperlucent on series of chest radiograph in at least ten cases (5).
Pathological findings revealed marked dilatation of bronchioles with collapse of alveoli forming cystic air space probably due to check valve mechanism by obstructive bronchioles (6 – 8). Then, hyperlucent of Swyer James syndrome on chest radiography is formed by cystic air space and lowering pulmonary circulation due to secondary narrowing lumen of pulmonary artery as functional vessel.
Based on accumulation of information over Swyer James syndrome, clinicians serve to patients, making advice that because this disease is not genetic, this disease does either not interrupt to make a family plan or not to transmit to offspring. It is important to preserve circumstances without direct or indirect smoking. Vaccination for influenza and pneumococcus is recommended to prevent pneumonia. Antibiotics should be prepared at home or carried portable and operative methods for inspiration apparatus should be used and proficient for steroid air spray.
Although life expectancy of patients with Swyer James syndrome might depend on the presence or absence of bronchiectasis, it is expected the same on life expectancy as ordinary individuals in case of absence of bronchiectasis.
【Summary】
We presented a seventeen-year-old male for follow-up study of pneumothorax. Chest CT showed small pneumothorax at left apical region and map-like hyperlucent occupying lingula and left S6 with small fibrous change, compatible with image of Swyer James syndrome. It is borne in mind that histologic findings of Swyer James syndrome indicates obstructive bronchiolitis inducing marked dilatation of bronchioles and large air space. It probably emerge from severe obstructive bronchiolitis early after birth due to infection by adenovirus, mycoplasma, staphylococcus, et al. The subjects for patients with Swyer James syndrome are listed; this disease is not genetic; being important to preserve circumstances without direct or indirect smoking; Vaccination for influenza and pneumococcus to prevent pneumonia; antibiotics and operative methods for inspiration apparatus for steroid air spray. These advices lead to the same life expects as healthy individuals.
【References】
1.SWYER PR, JAMES GC. A case of unilateral pulmonary emphysema. Thorax. 1953 Jun;8(2):133-6.
2.MACLEOD WM. Abnormal transradiancy of one lung. Thorax. 1954 Jun;9(2):147-53
3.Sen HS, Taylan M, Abakay O, Sezgi C, Cetincakmak MG. Adult diagnosis of Swyer-James-Macleod syndrome: retrospective analysis of four cases. Respir Care. 2014 Apr;59(4):e51-4
4.Abba AA, Al-Mobeireek AF. Clinical spectrum of Swyer-James-Macleod syndrome in adults. Saudi Med J. 2003 Feb;24(2):195-8
5.Kliegman, Robert; Richard M Kliegman (2006). Nelson essentials of pediatrics. St. Louis, Mo: Elsevier Saunders
6.Ohri SK, Rutty G, Fountain SW. Acquired segmental emphysema: the enlarging spectrum of Swyer-James/Macleod's syndrome. Ann Thorac Surg. 1993 Jul;56(1):120-4.
7.Dirweesh A, Alvarez C, Khan M, Shah N. A unilateral hyperlucent lung - Swyer-James syndrome: A case report and literature review. Respir Med Case Rep. 2017;20:104-106.
8.Sulaiman A, Cavaille A, Vaunois B, Tiffet O. Swyer-James-MacLeod syndrome; repeated chest drainages in a patient misdiagnosed with pneumothorax. Interact Cardiovasc Thorac Surg. 2009 Apr;8(4):482-4
2024.3.22