Completed Studies

Congenital Lung Malformations (CLM) may occur in up to 1 in 2000 live births and include several sub-types such as Congenital Pulmonary Airway Malformations (CPAM), Bronchopulmonary Sequestration (BPS), Congenital Lobar Emphysema (CLE), and Bronchogenic Cysts. They have a range of clinical presentations from lesions that may go undiagnosed until chest imaging is done for other purposes, to recurrent infections, to life-threatening respiratory distress after birth. These malformations have traditionally been managed with surgical resection both to prevent future complications and concern of progression into a race cancer called Pleuropulmonary Blastoma (PPB), however, outcomes from resection and other features of surgical management have not had large patient studies and the MWPSC chose to investigate this.

In 2018 Kunisaki et al. utilized the MWPSC to create a multi-institutional registry of children who underwent surgery for all types of CLM. They were able to study 506 children and studied all aspects of the disease from fetal diagnosis and treatment through post-operative care and follow-up. They were able to describe that 90% of resections occur outside of the neonatal period and were mostly elective. Surgical resections had an all-complication rate of approximately 18% with residual pneumothorax requiring chest tube as the most common occurring in 8% of cases. Complication rates were highest in the neonatal period. This registry also served as a dataset for future studies. In 2019 Kunisaki et al. focused on the operative management of CLE specifically due to the severity of its peri-natal presentation. Using the registry, they were able to find 53 patients with CLE, the largest study of this pathologic sub-type. They found that compared to other congenital lung malformations, prenatal diagnosis was less common for CLE and that ultrasound was not specific for CLE. Neonates with CLE were three times as likely to have respiratory symptoms at birth and to have neonatal resection compared to other CLM lesions. Data on operative management also identified variability in the approach between open and thoracoscopic resections. This led to a study by Weller et al. that looked at outcomes between the two approaches. They found that thoracoscopic approaches were becoming more common and that while they had longer operative times, they required less epidural anesthesia and had no increase in post-operative complications. This has led to an increase in minimally invasive approaches for these children.

Prenatal screening and management of CLM are variables based on institution. Kunisaki et al. focused on this aspect of management in a 2020 study aimed at risk stratification. They were able to show that Congenital Pulmonary Airway Malformation Volume Ratio (CVR) was predictive of postnatal course although CVR measurements were only available for half of the patients identified. The authors found that a cutoff CVR of greater than 2.25 had a 100% sensitivity for detecting fetal hydrops whereas a CVR of below 1.36 had almost a 100% prediction for not developing fetal hydrops. The initial and maximum CVR was found to be associated with respiratory symptoms at birth, need for supplemental oxygen, preoperative neonatal ventilator use, and neonatal lung resection, although the ideal CVR cutoff is different for each outcome. The authors were able to use this data to generate a decision tree to help patients and providers with prenatal counseling and to decide on a delivery facility.

Lastly, one of the major drivers for elective resection is a concern for PPB, however, its exact risk and prevalence were unknown. Kunisaki et al. looked at the pathology of 521 lung lesions. PPB was identified in 8.7 % of postnatally diagnosed CLM. They found that if CT imaging is suggestive of malignancy, then the lung lesion was likely malignant, however, CT imaging was not sensitive. The higher-than-expected malignancy rate for postnatally diagnosed CLM has led providers to consider operative management for more of these patients. CLM remains an active area of study with ongoing analysis and publications of our registry.