Neonatal Med.  2019 Aug;26(3):138-146. 10.5385/nm.2019.26.3.138.

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

Affiliations
  • 1Division of Neonatology, Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
  • 2Division of Neonatology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea. kipark@yuhs.ac

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.

Keyword

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

MeSH Terms

Birth Weight
Bronchopulmonary Sequestration
Cystic Adenomatoid Malformation of Lung, Congenital
Female
Gestational Age
Humans
Infant, Newborn*
Lung
Medical Records
Parturition
Radiography
Respiratory System
Teratoma
Thoracic Surgery, Video-Assisted
Thoracoscopy
Thorax
Tomography, X-Ray Computed
Ultrasonography
Ultrasonography, Prenatal
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