Unilateral lung agenesis--detrimental roles of surrounding vessels.
Unilateral lung agenesis is a rare congenital defect and could be associated with multiple abnormalities. The patients usually have poor long-term outcomes especially in those with right lung agenesis. We reviewed the 10-year experience in our hospital to describe special clinical features and try to delineate the causes of poor outcomes. From 1995 to 2005, 14 patients less than 18 years of age with unilateral lung agenesis (4 with left agenesis, 10 with right agenesis) were enrolled. Medical records reviewed included diagnosis, presentation, chromosome anomalies, cardiovascular anomalies and interventions, outcomes. We found that the mechanisms of severe airway disease in right lung agenesis included (1) trachea compression by the aortic arch, (2) the presence of "pseudo-ring-sling complex," (3) distended pulmonary artery due to left to right shunt which impinged the only bronchus, and finally (4) the persistent LSVC that restricts the growth of trachea. The etiologies of airway complication in left lung agenesis included anomalous aortic arch compression on trachea and the coexisting heart disease with significant left to right shunt, which impinged on the bronchus. In conclusion, unilateral lung agenesis has frequently associated airway problems due to its surrounding vessels. Satisfactory airway intervention remains challenging. This disease still requires great effort to improve patient outcomes.
Chou
An-Kou
AK
Department of Pediatrics, National Taiwan University Hospital, School of Medicine, National Taiwan University, Taipei, Taiwan.
Huang
Shu-Chien
SC
Chen
Shyh-Jye
SJ
Huang
Pei-Ming
PM
Wang
Jou-Kou
JK
Wu
Mei-Hwan
MH
Chen
Yih-Sharng
YS
Chang
Chung-I
CI
Chiu
Ing-Sh
IS
Wu
En-Ting
ET
D000015:Abnormalities, Multiple; D001808:Blood Vessels / Q000002:abnormalities*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities*; D008297:Male; D014135:Tracheal Stenosis / Q000209:etiology*