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

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...

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Main Authors: Joohee Lim, Jung Ho Han, Jeong Eun Shin, Ho Sun Eun, Soon Min Lee, Min Soo Park, Ran Namgung, Kook In Park
Format: Article
Language:English
Published: Korean Society of Neonatology 2019-08-01
Series:Neonatal Medicine
Subjects:
Online Access:http://www.neo-med.org/upload/pdf/nm-2019-26-3-138.pdf
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author Joohee Lim
Jung Ho Han
Jeong Eun Shin
Ho Sun Eun
Soon Min Lee
Min Soo Park
Ran Namgung
Kook In Park
author_facet Joohee Lim
Jung Ho Han
Jeong Eun Shin
Ho Sun Eun
Soon Min Lee
Min Soo Park
Ran Namgung
Kook In Park
author_sort Joohee Lim
collection DOAJ
description 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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spelling doaj.art-1fcce391f29c4ca999c07d8fc52f5e942022-12-21T23:45:22ZengKorean Society of NeonatologyNeonatal Medicine2287-94122287-98032019-08-0126313814610.5385/nm.2019.26.3.138975Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center ExperienceJoohee Lim0Jung Ho Han1Jeong Eun Shin2Ho Sun Eun3Soon Min Lee4Min Soo Park5Ran Namgung6Kook In Park7 Division of Neonatology, Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea Division of Neonatology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea Division of Neonatology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea Division of Neonatology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea Division of Neonatology, Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea Division of Neonatology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea Division of Neonatology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea Division of Neonatology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, KoreaPurpose 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.http://www.neo-med.org/upload/pdf/nm-2019-26-3-138.pdfcongenital pulmonary airway malformationcystic adenomatoid malformation of lung, congenitalcongenitthoracic surgery, video-assisted
spellingShingle Joohee Lim
Jung Ho Han
Jeong Eun Shin
Ho Sun Eun
Soon Min Lee
Min Soo Park
Ran Namgung
Kook In Park
Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience
Neonatal Medicine
congenital pulmonary airway malformation
cystic adenomatoid malformation of lung, congenital
congenit
thoracic surgery, video-assisted
title Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience
title_full Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience
title_fullStr Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience
title_full_unstemmed Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience
title_short Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience
title_sort evaluation of newborn infants with prenatally diagnosed congenital pulmonary airway malformation a single center experience
topic congenital pulmonary airway malformation
cystic adenomatoid malformation of lung, congenital
congenit
thoracic surgery, video-assisted
url http://www.neo-med.org/upload/pdf/nm-2019-26-3-138.pdf
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