Outcomes of Cases of Prenatally-Diagnosed Congenital Pulmonary Airway Malformation

Abstract

Objective To evaluate the outcomes of cases of prenatally-diagnosed congenital pulmonary airway malformation (CPAM). Methods We retrospectively evaluated cases of prenatally-diagnosed CPAM between 2004 and 2018. Ultrasonographic features such as visualization of a fetal lung mass and heterogeneous pulmonary parenchyma were used for CPAM diagnosis. Prenatal and postnatal findings were compared in terms of accuracy regarding the CPAM diagnosis. Results The sample consisted of 27 cases. There were four cases in which the patients opted for the termination of pregnancy due to the severity of the lesion. A total of 23 neonates were delivered, and CPAM was confirmed in 15 cases. The median gestational age at delivery was 37 weeks (28–40 weeks) and the mean birth weight was 2,776 g. There were two neonatal deaths, one due to pneumothorax, and the other due to hypoplastic left heart syndrome (HLHS). A total of five patients with respiratory problems were operated in the postpartum period. There were eight misdiagnosis: bronchopulmonary sequestration (five cases), congenital lobar emphysema (two cases), and congenital diaphragm hernia (one case). Conclusion A precise postnatal diagnosis is very important to organize the proper management of the pregnancies with fetuses with CPAM. The positive predictive value of the prenatal diagnosis of CPAM via ultrasonography is of 70.3%. The differential diagnosis of CPAM may be prolonged to the postpartum period in some cases.

Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature

“…Clinically, CPAMs of all types have a wide range of presentations. CPAMs are often diagnosed only as an incidental radiographic finding in asymptomatic children in 40–60% of cases [ 7 , 22 ]. CPAMs may also clinically present as respiratory distress, pneumothorax, recurrent pneumonias, or wheezing [ 3 – 5 , 7 , 11 , 22 ].…”

Utility of Postnatal Chest X-Ray in Newborns for the Evaluation of Prenatally Suspected Congenital Pulmonary Airway Malformation: A Single-Center Experience

“…Congenital lung malformations are frequently first diagnosed on prenatal ultrasound, often at the time of initial anatomy scan performed between 18-20 weeks gestational age 4 . Though the differential diagnosis for congenital lung malformations is broad, the reported positive predictive value of prenatal ultrasound to diagnose CPAM reaches about 70-90% 3,5 .…”

“…4 Though the differential diagnosis for congenital lung malformations is broad, the reported positive predictive value of prenatal ultrasound to diagnose CPAM reaches approximately 70 to 90%. 3 5…”

Developmental Pathways Underlying Lung Development and Congenital Lung Disorders

“…Fetal intervention is necessary when there is a persistent mediastinal shift and/or hydrops develops. CPAM prenatal interventions include systemic corticosteroid therapy, thoracoamniotic shunts or single-needle thoracentesis, and fetal lobectomy by minimally invasive procedures or open surgery [ 108 , 114 , 143 , 144 ]. Most babies require respiratory support at birth, and postnatal examinations combine several imaging methods such as Computed Tomography (CT) and X-ray.…”

“…There are three general indications to operate in asymptomatic cases: risk of malignancy, risk of complications (such as the risk of infection and pneumothorax), and potential for compensatory lung growth with earlier resection. The optimal operative management methods use minimally invasive approaches, with thoracoscopic techniques adopted over traditional thoracotomy [ 108 , 143 , 144 , 145 ]. According to the existing data, no apparent decrease in lung function is observed after surgery in the short term.…”