Narrative review of congenital lung lesions

Abstract

This article reviews the contemporary diagnosis and management of antenatally diagnosed congenital lung lesions. These anomalies, which include congenital pulmonary airway malformation (CPAM) (formerly congenital cystic adenomatoid malformation), bronchopulmonary sequestration (BPS), bronchogenic cyst, and congenital lobar emphysema (CLE), are relatively rare but are increasingly encountered by clinicians because of the improved resolution and enhanced sensitivity of fetal ultrasound. Serial assessment of these lesions throughout pregnancy remains the norm rather than the exception. Perinatal management strategies may differ based on initial size and growth patterns of these masses until delivery. Fetal magnetic resonance imaging and other diagnostic testing can sometimes be helpful in providing additional prognostic information. Over the last decade, maternal steroids have become standard of care in the management of larger lesions at risk for nonimmune hydrops. As a result, fetal surgical procedures, including open resection, thoracoamniotic shunting, and ex utero intrapartum treatment (EXIT), are less uncommonly performed. Decisions regarding whether delivery of these fetuses should occur in a tertiary care center with pediatric surgery coverage versus delivery at a local community hospital are now highly relevant in most prenatal counseling discussions with families. Large lung malformations may require urgent surgical removal in the early postnatal period because of respiratory distress. Other complications, such as recurrent pneumonia, pneumothorax, and cancer, are indications for postnatal lung resection on an elective basis. Many children are good candidates for minimally invasive (thoracoscopic) surgical approaches as an alternative to resection by thoracotomy. In the vast majority of cases, the overall prognosis remains excellent.

Keywords: Congenital lung lesion; bronchopulmonary sequestrations (BPS); congenital pulmonary airway malformations (CPAM); fetal surgery.

Figure 1

Graph showing the relative frequency of different types of prenatally diagnosed congenital lung lesions, with permission (1).

Figure 2

Chest CT transverse section (lung window) showing a congenital pulmonary airway malformation involving the right lower lobe, with permission (6).

Figure 3

Chest CT coronal section (abdominal window) demonstrating a left bronchopulmonary sequestration with a large systemic blood supply arising from the aorta, with permission (6).

Figure 4

Plain radiograph of the chest (anteroposterior view) in a neonate showing hyperinflation of the right middle lobe with mass effect and mediastinal shift. The lesion was a congenital lobar emphysema, with permission (6).

Figure 5

Plain radiograph of the chest (anteroposterior view) in a neonate showing hyperinflation of the right middle lobe with mass effect and mediastinal shift. The lesion was a congenital lobar emphysema, with permission (6).

Figure 6

Congenital pulmonary airway malformation volume ratio (CVR)-based management algorithm for prenatally diagnosed lung lesions, with permission (20).

Figure 7

Correlation between the congenital pulmonary airway malformation volume ratio (CVR) and neonatal respiratory symptoms (Sx). Line graph of CVR stratified based on presence or absence of respiratory symptoms (left) and vertical bar graph of CVR based on initial, maximum, and final CVR measurements (right), with permission (5). *P<0.05.

Figure 8

Schematic depiction of ex utero intrapartum treatment, with permission (39).

Figure 9

Fetal left thoracotomy and lobectomy for a congenital pulmonary airway malformation.