Browsing by Author "Wright, Tiffany N."

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    Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children
    (American Medical Association, 2022-06-01) Engwall-Gill, Abigail J.; Chan, Sherwin S.; Boyd, Kevin P.; Saito, Jacqueline M.; Fallat, Mary E.; St. Peter, Shawn D.; Bolger-Theut, Stephanie; Crotty, Eric J.; Green, Jared R.; Hulett Bowling, Rebecca L.; Kumbhar, Sachin S.; Rattan, Mantosh S.; Young, Cody M.; Canner, Joseph K.; Deans, Katherine J.; Gadepalli, Samir K.; Helmrath, Michael A.; Hirschl, Ronald B.; Kabre, Rashmi; Lal, Dave R.; Landman, Matthew P.; Leys, Charles M.; Mak, Grace Z.; Minneci, Peter C.; Wright, Tiffany N.; Kunisaki, Shaun M.; Midwest Pediatric Surgery Consortium; Surgery, School of Medicine
    Importance: The ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear. Objective: To assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children. Design, setting, and participants: This retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020. Main outcomes and measures: Accuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors. Results: Among 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002). There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P < .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%. Conclusions and relevance: This study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.
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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.