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

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 com prehensive 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 pre dictive 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 videoassisted thoracoscopy. Twentyfive 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 pre natally diagnosed CPAM and to guide appropriate timing of surgery.

Clinical Characteristics of Congenital Pulmonary Airway Malformation of The Lungs: A Single-Center Study

“…This may be due to the inclusion of more severe patients in the university hospital. Previous studies have reported that adult patients with symptoms mainly have lesions in the lower lobe [14] . However, in this study, out of 12 adult patients diagnosed, 3 patients showed symptoms, with 2 cases (67%) involving the upper lobe and 1 case (33%) involving the lower lobe.…”

“…Some patients undergo antenatal interventions such as thoracentesis, thoracocentesis, thoracoamniocentesis, and amniocentesis because fetal death may occur due to hydrops fetalis, hydrothorax, or severe oligohydramnios, and there are studies showing early surgery within the first week of life in neonates who develop symptoms such as severe respiratory distress after birth [12] . However, early planned surgery is sometimes performed in the absence of clinical symptoms after birth, but the appropriate timing of surgery remains controversial [13,14] .…”

“…Korean studies of congenital pulmonary airway malformations in the last decade have included analyses of children diagnosed prenatally [12][13][14][15] and studies of newly diagnosed patients in adulthood [8,16] . However, there is a lack of studies that include all ages of children and adults at the time of diagnosis.…”