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Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

 Joohee Lim  ;  Jung Ho Han  ;  Jeong Eun Shin  ;  Ho Sun Eun  ;  Soon Min Lee  ;  Min Soo Park  ;  Ran Namgung  ;  Kook In Park 
 Neonatal Medicine, Vol.26(3) : 138-146, 2019 
Journal Title
 Neonatal Medicine 
Issue Date
Congenital pulmonary airway malformation ; Cystic adenomatoid malformation of lung, congenital ; Congenit ; Thoracic surgery, video-assisted
Purpose Congenital pulmonary airway malformation (CPAM)-a rare developmental anomaly-affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. Methods We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children’s Hospital between January 2005 and July 2017. Results We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. Conclusion Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.
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1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Namgung, Ran(남궁란) ORCID logo https://orcid.org/0000-0001-7182-9535
Park, Kook In(박국인) ORCID logo https://orcid.org/0000-0001-8499-9293
Park, Min Soo(박민수) ORCID logo https://orcid.org/0000-0002-4395-9938
Shin, Jeong Eun(신정은) ORCID logo https://orcid.org/0000-0002-4376-8541
Eun, Ho Seon(은호선) ORCID logo https://orcid.org/0000-0001-7212-0341
Lee, Soon Min(이순민) ORCID logo https://orcid.org/0000-0003-0174-1065
Lim, Joo Hee(임주희) ORCID logo https://orcid.org/0000-0003-4376-6607
Han, Jung Ho(한정호) ORCID logo https://orcid.org/0000-0001-6661-8127
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