Browsing by Subject "congenital pulmonary airway malformation"

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    Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium
    (Elsevier, 2019) Kunisaki, Shaun M.; Saito, Jacqueline M.; Fallat, Mary E.; St. Peter, Shawn D.; Kim, Aimee G.; Johnson, Kevin N.; Mon, Rodrigo A.; Adams, Cheryl; Aladegbami, Bola; Bence, Christina; Burns, R. Cartland; Corkum, Kristine S.; Deans, Katherine J.; Downard, Cynthia D.; Fraser, Jason D.; Gadepalli, Samir K.; Helmrath, Michael A.; Kabre, Rashmi; Lal, Dave R.; Landman, Matthew P.; Leys, Charles M.; Linden, Allison F.; Lopez, Joseph J.; Mak, Grace Z.; Minneci, Peter C.; Rademacher, Brooks L.; Shaaban, Aimen; Walker, Sarah K.; Wright, Tiffany N.; Hirschl, Ronald B.; Surgery, School of Medicine
    Purpose The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium. Methods After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009–2015). Results Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p < 0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p < 0.0001). The most common locations for CLE were the left upper (n = 24, 45.3%), right middle (n = 16, 30.2%), and right upper (n = 10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1–12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0–13.0). Conclusions Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion.
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    Development of a multi-institutional registry for children with operative congenital lung malformations
    (Elsevier, 2019) Kunisaki, Shaun M.; Saito, Jacqueline M.; Fallat, Mary E.; St. Peter, Shawn D.; Lal, Dave R.; Johnson, Kevin N.; Mon, Rodrigo A.; Adams, Cheryl; Aladegbami, Bola; Bence, Christina; Burns, R. Cartland; Corkum, Kristine S.; Deans, Katherine J.; Downard, Cynthia D.; Fraser, Jason D.; Gadepalli, Samir K.; Helmrath, Michael A.; Kabre, Rashmi; Landman, Matthew P.; Leys, Charles M.; Linden, Allison F.; Lopez, Joseph J.; Mak, Grace Z.; Minneci, Peter C.; Rademacher, Brooks L.; Shaaban, Aimen; Walker, Sarah K.; Wright, Tiffany N.; Hirschl, Ronald B.; Surgery, School of Medicine
    Introduction The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection. Methods After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative. Results Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1–12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6–11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%). Conclusion This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients.