Browsing by Author "Gadepalli, Samir K."
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Item A multi-disciplinary, comprehensive approach to management of children with heterotaxy(BMC, 2022-09-09) Saba, Thomas G.; Geddes, Gabrielle C.; Ware, Stephanie M.; Schidlow, David N.; del Nido, Pedro J.; Rubalcava, Nathan S.; Gadepalli, Samir K.; Stillwell, Terri; Griffiths, Anne; Bennett Murphy, Laura M.; Barber, Andrew T.; Leigh, Margaret W.; Sabin, Necia; Shapiro, Adam J.; Medical and Molecular Genetics, School of MedicineHeterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left-right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.Item 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 MedicineImportance: 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.Item Can Fecal Continence be Predicted in Patients Born with Anorectal Malformations?(Elsevier, 2019) Minneci, Peter C.; Kabre, Rashmi S.; Mak, Grace Z.; Halleran, Devin R.; Cooper, Jennifer N.; Afrazi, Amin; Calkins, Casey M.; Corkum, Kristine; Downard, Cynthia D.; Ehrlich, Peter; Fraser, Jason D.; Gadepalli, Samir K.; Helmrath, Michael A.; Kohler, Jonathan E.; Landisch, Rachel; Landman, Matthew P.; Lee, Constance; Leys, Charles M.; Lodwick, Daniel L.; McLeod, Jennifer; Mon, Rodrigo; McClure, Beth; Rymeski, Beth; Saito, Jacqueline M.; Sato, Thomas T.; St. Peter, Shawn D.; Wood, Richard; Levitt, Marc A.; Deans, Katherine J.; Surgery, School of MedicinePurpose The purpose of this study was to identify factors associated with attaining fecal continence in children with anorectal malformations (ARM). Methods We performed a multi-institutional cohort study of children born with ARM in 2007–2011 who had spinal and sacral imaging. Questions from the Baylor Social Continence Scale were used to assess fecal continence at the age of ≥ 4 years. Factors present at birth that predicted continence were identified using multivariable logistic regression. Results Among 144 ARM patients with a median age of 7 years (IQR 6–8), 58 (40%) were continent. The rate of fecal continence varied by ARM subtype (p = 0.002) with the highest rate of continence in patients with perineal fistula (60%). Spinal anomalies and the lateral sacral ratio were not associated with continence. On multivariable analysis, patients with less severe ARM subtypes (perineal fistula, recto-bulbar fistula, recto-vestibular fistula, no fistula, rectal stenosis) were more likely to be continent (OR = 7.4, p = 0.001). Conclusion Type of ARM was the only factor that predicted fecal continence in children with ARM. The high degree of incontinence, even in the least severe subtypes, highlights that predicting fecal continence is difficult at birth and supports the need for long-term follow-up and bowel management programs for children with ARM.Item Changing the Paradigm for Management of Pediatric Primary Spontaneous Pneumothorax: A Simple Aspiration Test Predicts Need for Operation(Elsevier, 2019) Leys, Charles M.; Hirschl, Ronald B.; Kohler, Jonathan E.; Cherney-Stafford, Linda; Marka, Nicholas; Fallat, Mary E.; Gadepalli, Samir K.; Fraser, Jason D.; Grabowski, Julia; Burns, R. Cartland; Downard, Cynthia D.; Foley, David S.; Halleran, Devin R.; Helmrath, Michael A.; Kabre, Rashmi; Knezevich, Michellle S.; Lal, Dave R.; Landman, Matthew P.; Lawrence, Amy E.; Mak, Grace Z.; Minneci, Peter C.; Musili, Ninette; Rymeski, Beth; Saito, Jacqueline M.; Sato, Thomas T.; St. Peter, Shawn D.; Warner, Brad W.; Ostlie, Daniel J.; Surgery, School of MedicinePurpose Chest tube (CT) management for pediatric primary spontaneous pneumothorax (PSP) is associated with long hospital stays and high recurrence rates. To streamline management, we explored simple aspiration as a test to predict need for surgery. Methods A multi-institution, prospective pilot study of patients with first presentation for PSP at 9 children’s hospitals was performed. Aspiration was performed through a pigtail catheter, followed by 6 h observation with CT clamped. If pneumothorax recurred during observation, the aspiration test failed and subsequent management was per surgeon discretion. Results Thirty-three patients were managed with simple aspiration. Aspiration was successful in 16 of 33 (48%), while 17 (52%) failed the aspiration test and required hospitalization. Twelve who failed aspiration underwent CT management, of which 10 (83%) failed CT management owing to either persistent air leak requiring VATS or subsequent PSP recurrence. Recurrence rate was significantly greater in the group that failed aspiration compared to the group that passed aspiration [10/12 (83%) vs 7/16 (44%), respectively, P = 0.028]. Conclusion Simple aspiration test upon presentation with PSP predicts chest tube failure with 83% positive predictive value. We recommend changing the PSP management algorithm to include an initial simple aspiration test, and if that fails, proceed directly to VATS.Item 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 MedicinePurpose 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.Item 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 MedicineIntroduction 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.Item Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium(Elsevier, 2018) Lal, Dave R.; Gadepalli, Samir K.; Downard, Cynthia D.; Minneci, Peter C.; Knezevich, Michelle; Chelius, Thomas H.; Rapp, Cooper T.; Bilmire, Deborah; Bruch, Steven; Burns, R. Carland; Deans, Katherine J.; Fallat, Mary E.; Fraser, Jason D.; Grabowski, Julia; Hebel, Ferdynand; Helmrath, Michael A.; Hirschl, Ronald B.; Kabre, Rashmi; Kohler, Jonathan; Landman, Matthew P.; Leys, Charles M.; Mak, Grace Z.; Ostlie, Daniel J.; Raque, Jessica; Rymeski, Beth; Saito, Jacqueline M.; St. Peter, Shawn D.; von Allmen, Daniel; Warner, Brad W.; Sato, Thomas T.; Surgery, School of MedicinePurpose Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF. Methods A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up. Results In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54–2.65) vs. 2.57 kg (2.00–3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32–37) vs. 37 weeks (35–39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29). Conclusion RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach.Item The COVID-19 pandemic and associated rise in pediatric firearm injuries: A multi-institutional study(Elsevier, 2022) Collings, Amelia T.; Farazi, Manzur; Van Arendonk, Kyle J.; Fallat, Mary E.; Minneci, Peter C.; Sato, Thomas T.; Speck, K. Elizabeth; Deans, Katherine J.; Falcone, Richard A., Jr.; Foley, David S.; Fraser, Jason D.; Gadepalli, Samir K.; Keller, Martin S.; Kotagal, Meera; Landman, Matthew P.; Leys, Charles M.; Markel, Troy A.; Rubalcava, Nathan; St. Peter, Shawn D.; Flynn-O’Brien, Katherine T.; Midwest Pediatric Surgery Consortium; Surgery, School of MedicineBackground: Firearm sales in the United States (U.S.) markedly increased during the COVID-19 pandemic. Our objective was to determine if firearm injuries in children were associated with stay-at-home orders (SHO) during the COVID-19 pandemic. We hypothesized there would be an increase in pediatric firearm injuries during SHO. Methods: This was a multi institutional, retrospective study of institutional trauma registries. Patients <18 years with traumatic injuries meeting National Trauma Data Bank (NTDB) criteria were included. A "COVID" cohort, defined as time from initiation of state SHO through September 30, 2020 was compared to "Historical" controls from an averaged period of corresponding dates in 2016-2019. An interrupted time series analysis (ITSA) was utilized to evaluate the association of the U.S. declaration of a national state of emergency with pediatric firearm injuries. Results: Nine Level I pediatric trauma centers were included, contributing 48,111 pediatric trauma patients, of which 1,090 patients (2.3%) suffered firearm injuries. There was a significant increase in the proportion of firearm injuries in the COVID cohort (COVID 3.04% vs. Historical 1.83%; p < 0.001). There was an increased cumulative burden of firearm injuries in 2020 compared to a historical average. ITSA showed an 87% increase in the observed rate of firearm injuries above expected after the declaration of a nationwide emergency (p < 0.001). Conclusion: The proportion of firearm injuries affecting children increased during the COVID-19 pandemic. The pandemic was associated with an increase in pediatric firearm injuries above expected rates based on historical patterns.