Neonatal Outcomes of Multiple Congenital Thoracic Lesions.
Publication/Presentation Date
1-1-2025
Abstract
INTRODUCTION: Congenital pulmonary airway malformations (CPAM), intra- and extralobar bronchopulmonary sequestrations (iBPS/eBPS), CPAM-BPS hybrid lesions (HL), congenital lobar emphysema (CLE), bronchial atresia (BA), and foregut duplication cysts (FDC), collectively referred to as congenital thoracic lesions (CTL), are mostly solitary. Patients with multiple CTL are rare, and reports on such cases are scarce. To address this dearth, we analyzed a large multifocal CTL patient cohort.
METHODS: Retrospective chart review of patients born between September 1, 2013, and March 31, 2023, who underwent surgery for a CTL at our tertiary center. Patients with radiological and surgical diagnosis of multifocal CTL, defined as ≥2 CTL present in more than one lobe were included to record pre-, peri-, and postnatal patient characteristics.
RESULTS: Among 701 CTL patients, 74 (10.5%) had multiple CTL. CTL multifocality was prenatally recognized correctly in 8 (12.9%) patients. Most multiple CTL were right-sided, unilateral multilobar lesions (n = 33, 44%). Bilateral CTL were found in 9 (12.1%) patients. CPAM-CPAM lesions were the most prevalent CTL types (n = 36, 49%). Genetic syndromes were confirmed in 3 (4%) and additional congenital anomalies in 9 (12.9%) patients, 5 of those had multiple congenital anomalies. Of 49 (65%) patients with multilobar CTL, 25 (51%) underwent bilobectomy and 24 (49%) lung-sparing surgery. Length of stay was similar. Mortality was 5.4%.
CONCLUSION: We report on the largest patient cohort with multiple CTL to date. Multiple CTL occurred in 1/10 patients with CTL, and only 12.9% were recognized prenatally. Lung-sparing surgery can be considered. Multiple additional congenital anomalies and genetic syndromes may be more common and genetic testing should be considered. Overall, outcomes in this patient population are favorable.
Volume
52
Issue
2
First Page
194
Last Page
206
ISSN
1421-9964
Published In/Presented At
di Natale, A., Flohr, S., Mathew, L., Katterman, C., Gallagher, C., Reynolds, T. A., Gebb, J. S., Panitch, H. B., Oliver, E. R., Rintoul, N. E., Peranteau, W. H., Flake, A. W., Adzick, N. S., & Hedrick, H. L. (2025). Neonatal Outcomes of Multiple Congenital Thoracic Lesions. Fetal diagnosis and therapy, 52(2), 194–206. https://doi.org/10.1159/000541319
Disciplines
Medicine and Health Sciences
PubMedID
39245038
Department(s)
Medical Education
Document Type
Article