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

Neonatal Medicine 2019년 26권 3호 p.138 ~ 146
 ( Lim Joo-Hee ) - Yonsei University College of Medicine Gangnam Severance Hospital Department of Pediatrics

 ( Han Jung-Ho ) - Yonsei University College of Medicine Severance Children’s Hospital Department of Pediatrics
 ( Shin Jeong-Eun ) - Yonsei University College of Medicine Severance Children’s Hospital Department of Pediatrics
 ( Eun Ho-Sun ) - Yonsei University College of Medicine Severance Children’s Hospital Department of Pediatrics
 ( Lee Soon-Min ) - Yonsei University College of Medicine Gangnam Severance Hospital Department of Pediatrics
 ( Park Min-Soo ) - Yonsei University College of Medicine Severance Children’s Hospital Department of Pediatrics
 ( Namgung Ran ) - Yonsei University College of Medicine Severance Children’s Hospital Department of Pediatrics
 ( Park Kook-In ) - Yonsei University College of Medicine Severance Children’s Hospital Department of Pediatrics

Abstract


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.

키워드

Congenital pulmonary airway malformation; Cystic adenomatoid malformation of lung, congenital; Congenit; Thoracic surgery, video-assisted
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