https://scholars.lib.ntu.edu.tw/handle/123456789/434138
標題: | Unilateral lung agenesis - Detrimental roles of surrounding vessels | 作者: | Chou A.-K. SHU-CHIEN HUANG SHYH-JYE CHEN PEI-MING HUANG JOU-KOU WANG MEI-HWAN WU YIH-SHARNG CHEN CHUNG-I CHANG ING-SH CHIU EN-TING WU |
公開日期: | 2007 | 卷: | 42 | 期: | 3 | 起(迄)頁: | 242-248 | 來源出版物: | Pediatric Pulmonology | 摘要: | 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. ? 2007 Wiley-Liss, Inc. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-33947269143&doi=10.1002%2fppul.20561&partnerID=40&md5=99c2e40c8076ea4c6128de7100395082 https://scholars.lib.ntu.edu.tw/handle/123456789/434138 |
ISSN: | 8755-6863 | DOI: | 10.1002/ppul.20561 | SDG/關鍵字: | aorta arch; aorta arch anomaly; article; bronchus; cardiovascular malformation; chromosome aberration; clinical article; clinical feature; disease association; disease severity; female; fetus; heart left right shunt; human; infant; lung agenesis; lung blood vessel; lung complication; male; newborn; outcome assessment; priority journal; pulmonary artery malformation; rare disease; respiratory tract disease; trachea compression; Abnormalities, Multiple; Blood Vessels; Female; Humans; Infant; Infant, Newborn; Lung; Male; Tracheal Stenosis |
顯示於: | 醫學系 |
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