Although thoracoscopic lobectomy is a widely accepted surgical procedure in adult thoracic surgery, its role in small children remains controversial. The purpose of this study was to evaluate perioperative outcomes after thoracoscopic and open lobectomy in infants and young children with congenital lung malformations at a single academic referral center. A cohort study of 62 consecutive children who underwent elective pulmonary lobectomy for a congenital lung lesion between 2001 and 2013 was performed. Patient demographics and perioperative outcomes were evaluated in univariate and logistic regression analyses. Forty-nine patients underwent thoracoscopy and 13 had a thoracotomy. Six children undergoing thoracoscopy required conversion to thoracotomy (conversion 12.2%). Perioperative outcomes, including median blood loss (2.0 vs 1.1 mL/kg; p = 0.34), chest tube duration (3 vs 3 days; p = 0.33), hospital length of stay (3 vs 3 days; p = 0.42), and morbidity as defined by the Accordion Grading Scale (30.6% vs 30.8%; p = 0.73), were similar between thoracoscopy and thoracotomy, respectively. Although thoracoscopy was associated with increased operative duration compared with thoracotomy (239.9 vs 181.2 minutes, respectively; p = 0.03), thoracoscopy operative times decreased with increasing institutional experience (p = 0.048). Thoracoscopic lobectomy infants younger than 5 months of age had a 2.5-fold higher rate of perioperative adverse outcomes compared with older children (p = 0.048). In small children undergoing pulmonary lobectomy, both thoracoscopy and thoracotomy are associated with similar perioperative outcomes. The cosmetic and musculoskeletal benefits of the thoracoscopic approach must be balanced against institutional expertise and a potentially higher risk for complications in younger patients.

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.

Three‐dimensional lung organoids (LOs) derived from pluripotent stem cells have the potential to enhance our understanding of disease mechanisms and to enable novel therapeutic approaches in neonates with pulmonary disorders. We established a reproducible ex vivo model of lung development using transgene‐free human induced pluripotent stem cells generated from fetuses and infants with Bochdalek congenital diaphragmatic hernia (CDH), a polygenic disorder associated with fetal lung compression and pulmonary hypoplasia at birth. Molecular and cellular comparisons of CDH LOs revealed impaired generation of NKX2.1⁺ progenitors, type II alveolar epithelial cells, and PDGFRα⁺ myofibroblasts. We then subjected these LOs to disease relevant mechanical cues through ex vivo compression and observed significant changes in genes associated with pulmonary progenitors, alveolar epithelial cells, and mesenchymal fibroblasts. Collectively, these data suggest both primary cell‐intrinsic and secondary mechanical causes of CDH lung hypoplasia and support the use of this stem cell‐based approach for disease modeling in CDH.

Objective: The aim of this study was to assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes. Summary Background Data: Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery has not been well defined. Methods: A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at 11 children’s hospitals from 2009 to 2016. Statistical analyses were performed using nonparametric bivariate or multivariable logistic regression. Results: Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection (P < 0.001). An initial CVR ≤1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87–1.00]. A maximum CVR <0.9 (AUC, 0.72; 95% CI, 0.67–0.85) was associated with a low risk for respiratory symptoms at birth. Conclusions: In this large, multi-institutional study, an initial CVR ≤ 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.