J. Tashiro1, A. Wagenaar1, A. C. Hirzel2, L. I. Rodriguez3, E. A. Perez1, A. R. Hogan1, H. L. Neville1, J. E. Sola1 1University Of Miami,Division Of Pediatric Surgery, DeWitt Daughtry Family Department Of Surgery,Miami, FL, USA 2University Of Miami,Department Of Pathology,Miami, FL, USA 3University Of Miami,Department Of Anesthesiology,Miami, FL, USA
Introduction: Bronchopulmonary Malformations (BPM) are rare conditions affecting the pediatric population. The spectrum of BPM encompasses congenital cystic adenomatoid malformation (CCAM), pulmonary sequestration (PS), congenital lobar emphysema (CLE), bronchogenic cyst (BC), and hybrid lesions. These focal anatomic anomalies typically arise below the carina and can result in significant morbidity (infection/hemorrhage) and mortality (respiratory failure).
Methods: After IRB approval, all children with BPM surgically treated from 2001-2014 at a tertiary care children’s hospital were identified. Patient demographics, surgical indications, procedure type, estimated blood loss (EBL), pathology, perioperative complications, length of stay (LOS), and outcomes were analyzed using standard statistical methods.
Results: Overall, 41 patients with BPM had surgery (39 thoracic/ 2 abdominal) over the study period (CCAM 19, PS 8, CCAM/PS hybrid 6, CLE 6, BC 5). Our cohort was 51% male with a median age (IQR) at resection of 11 (19) months, weight of 9.1 (5.1) kg. Overall survival was 98% (one CDH/ECMO abdominal BPM expired) but 100% for thoracic lesions. Analysis of thoracic lesions revealed a median (IQR) operative time of 140 (45) minutes, EBL 1.47 (1.90) ml/kg, chest tube (CT) days 4 (4), LOS 5 (5) days, and a complication rate of 26% (21% pulmonary). The left and right lower lobes were most commonly resected (39% and 24%, respectively) and 27% of lesions had a systemic artery. Resections were performed thoracoscopically (38%), thoracoscopy converted to open (23%), and via thoracotomy (38%). Conversions to thoracotomy were due to poor visualization (66%) or inability to tolerate single lung ventilation (33%). There were no conversions due to hemorrhage or blood transfusions in thoracoscopy patients. Patients undergoing thoracoscopic surgery were more likely to have a prenatally diagnosed BPM (OR: 18.2) v. open/converted, p=0.002. Open/converted surgery patients had longer CT days (6.2) vs. thoracoscopic (2.9), p=0.048. BPM with a systemic artery (PS/hybrid) were more commonly resected thoracoscopically (OR: 6.1) than open, p=0.047. Additionally, respiratory distress was a more common indication in patients <4 months old (OR: 28.0) vs. ≥4 months, and patients weighing <6 kg (OR: 40.5) vs. ≥6 kg, p<0.05. Similarly, procedures were started as open resections at a higher rate in patients <4 months old (OR: 8.8) and weighing <6 kg (OR: 24.0) vs. ≥4 months and ≥6 kg, respectively, p<0.05. Operative time was lower for <6 kg vs. ≥6 kg, p=0.035.
Conclusion: BPM resections are procedures with high overall survival over a 14-year experience at a large tertiary care children’s hospital. Chest tube days are shorter among thoracoscopic patients but conversion to thoracotomy for poor visualization avoids hemorrhage and need for transfusion. Size and respiratory distress limit use of thoracoscopy in young infants with BPM.