Publications by authors named "Dave Lal"

70 Publications

Gastric-Type Enteric Duplication Cyst Communicating with an Accessory Pancreatic Duct.

Pediatr Ann 2022 Aug 1;51(8):e324-e327. Epub 2022 Aug 1.

Enteric duplication cysts are rare congenital anomalies defined by the location along the gastrointestinal tract from which they communicate as well as the epithelial lining they contain. Enteric duplication cysts in communication with the pancreas are an even rarer subset that are often difficult to diagnose due to nonspecific presenting symptoms. In a pediatric patient with a history of recurrent pancreatitis episodes, a pancreatic duplication should be on the differential. High clinical suspicion and specific imaging characteristics can aid in the diagnosis. The management of pancreatic duplication cysts requires surgical excision or drainage procedures to alleviate symptoms and prevent associated complications such as recurrent pancreatitis, bleeding, bowel obstruction, or malignancy. Here we present a case of a gastric duplication cyst in communication with an accessory pancreatic lobe with special focus on the preoperative workup, intraoperative findings, and histopathologic examination. .
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http://dx.doi.org/10.3928/19382359-20220606-01DOI Listing
August 2022

Disparities in the Management of Pediatric Breast Masses.

J Surg Res 2022 Aug 3;279:648-656. Epub 2022 Aug 3.

Center for Surgical Outcomes Research, Abigail Wexner Research Institute and Department of Surgery, Nationwide Children's Hospital, Columbus, Ohio.

Introduction: Disparities in surgical management have been documented across a range of disease processes. The objective of this study was to investigate sociodemographic disparities in young females undergoing excision of a breast mass.

Methods: A retrospective study of females aged 10-21 y who underwent surgery for a breast lesion across eleven pediatric hospitals from 2011 to 2016 was performed. Differences in patient characteristics, workup, management, and pathology by race/ethnicity, insurance status, median neighborhood income, and urbanicity were evaluated with bivariate and multivariable regression analyses.

Results: A total of 454 females were included, with a median age of 16 y interquartile range (IQR: 3). 44% of patients were nonHispanic (NH) Black, 40% were NH White, and 7% were Hispanic. 50% of patients had private insurance, 39% had public insurance, and 9% had other/unknown insurance status. Median neighborhood income was $49,974, and 88% of patients resided in a metropolitan area. NH Whites have 4.5 times the odds of undergoing preoperative fine needle aspiration or core needle biopsy compared to NH Blacks (CI: 2.0, 10.0). No differences in time to surgery from the initial imaging study, size of the lesion, or pathology were observed on multivariable analysis.

Conclusions: We found no significant differences by race/ethnicity, insurance status, household income, or urbanicity in the time to surgery after the initial imaging study. The only significant disparity noted on multivariable analysis was NH White patients were more likely to undergo preoperative biopsy than were NH Black patients; however, the utility of biopsy in pediatric breast masses is not well established.
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http://dx.doi.org/10.1016/j.jss.2022.06.049DOI Listing
August 2022

Assessment of MRI and US Screening for Tethered Cord Syndrome in Patients Diagnosed With Esophageal Atresia/Tracheoesophageal Fistula.

J Surg Res 2022 Jun 29;279:193-199. Epub 2022 Jun 29.

Division of Pediatric Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin.

Introduction: Infants with esophageal atresia and/or tracheoesophageal fistula (EA/TEF) undergo screening for tethered cord syndrome (TCS) via ultrasound and magnetic resonance imaging. Existing literature lacks data to guide optimal timing of screening and magnetic resonance imaging (MRI) is often delayed until 3-6 mo of age, when it is frequently forgotten. Detethering surgery has a high rate of success in patients with TCS and is often performed prophylactically due to potential irreversible deficits. This study aims to improve screening procedure for infants with EA/TEF.

Methods: A retrospective chart review was done of all EA/TEF patients treated over 6 y (n = 79). The study examined how often each imaging modality was performed and identified a TCS lesion, as well as age of screening/surgical intervention.

Results: Screening for TCS was done with MRI 58% of the time and US 15% of the time. However, 38% of patients did not undergo any screening. Out of the patients with TCS on MRI (n = 19, 41.3%), 73.7% had neurosurgery. Of patients who underwent US (n = 12), nine patients also had MRI later: two reported TCS lesions and subsequently had neurosurgery. Surgical infection rates and complications were 0/14.

Conclusions: MRI demonstrated a higher rate of detecting TCS lesions than US, and patients with TCS frequently had detethering. Patients with ≥3 VACTERL or vertebral anomalies had a higher incidence of TCS on MRI. Patients with vertebral anomalies reported false negative ultrasounds in two cases, suggesting the potential superiority of MRI screening in this subgroup. A third of children did not undergo any imaging and this will require a process improvement.
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http://dx.doi.org/10.1016/j.jss.2022.06.004DOI Listing
June 2022

Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children.

JAMA Netw Open 2022 06 1;5(6):e2219814. Epub 2022 Jun 1.

Division of General Pediatric Surgery, Johns Hopkins Children's Center, Johns Hopkins University School of Medicine, Baltimore, Maryland.

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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http://dx.doi.org/10.1001/jamanetworkopen.2022.19814DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9247735PMC
June 2022

Incidence and management of pleural effusions in patients with Wilms tumor: A Pediatric Surgical Oncology Research Collaborative study.

Int J Cancer 2022 Jun 24. Epub 2022 Jun 24.

Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA.

Wilms tumor (WT) is the most common renal malignancy in children. Children with favorable histology WT achieve survival rates of over 90%. Twelve percent of patients present with metastatic disease, most commonly to the lungs. The presence of a pleural effusion at the time of diagnosis of WT may be noted on staging imaging; however, minimal data exist regarding the significance and prognostic importance of this finding. The objectives of our study are to identify the incidence of pleural effusions in patients with WT, and to determine the potential impact on oncologic outcomes. A multi-institutional retrospective review was performed from January 2009 to December 2019, including children with WT and a pleural effusion on diagnostic imaging treated at Pediatric Surgical Oncology Research Collaborative (PSORC) participating institutions. Of 1259 children with a new WT diagnosis, 94 (7.5%) had a pleural effusion. Patients with a pleural effusion were older than those without (median 4.3 vs 3.5 years; P = .004), and advanced stages were more common (local stage III 85.9% vs 51.9%; P < .0001). Only 14 patients underwent a thoracentesis for fluid evaluation; 3 had cytopathologic evidence of malignant cells. Event-free and overall survival of all children with WT and pleural effusions was 86.2% and 91.5%, respectively. The rate and significance of malignant cells present in pleural fluid is unknown due to low incidence of cytopathologic analysis in our cohort; therefore, the presence of an effusion does not appear to necessitate a change in therapy. Excellent survival can be expected with current stage-specific treatment regimens.
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http://dx.doi.org/10.1002/ijc.34188DOI Listing
June 2022

Inflammatory myofibroblastic tumor: A multi-institutional study from the Pediatric Surgical Oncology Research Collaborative.

Int J Cancer 2022 Oct 15;151(7):1059-1067. Epub 2022 Jun 15.

Division of Pediatric Surgery, Medical College of Wisconcon, Milwaukee, Wisconsin, USA.

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy. We describe the largest cohort of IMT patients to date, aiming to further characterize this rare, poorly understood tumor. This is a multi-institutional review of IMT patients ≤39 years, from 2000 to 2018, at 18 hospitals in the Pediatric Surgical Oncology Research Collaborative. One hundred and eighty-two patients were identified with median age of 11 years. Thirty-three percent of tumors were thoracic in origin. Presenting signs/symptoms included pain (29%), respiratory symptoms (25%) and constitutional symptoms (20%). Median tumor size was 3.9 cm. Anaplastic lymphoma kinase (ALK) overexpression was identified in 53% of patients. Seven percent of patients had distant disease at diagnosis. Ninety-one percent of patients underwent resection: 14% received neoadjuvant treatment and 22% adjuvant treatment. Twelve percent of patients received an ALK inhibitor. Sixty-six percent of surgical patients had complete resection, with 20% positive microscopic margins and 14% gross residual disease. Approximately 40% had en bloc resection of involved organs. Median follow-up time was 36 months. Overall 5-year survival was 95% and 5-year event-free survival was 80%. Predictors of recurrence included respiratory symptoms, tumor size and distant disease. Gross or microscopic margins were not associated with recurrence, suggesting that aggressive attempts at resection may not be warranted.
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http://dx.doi.org/10.1002/ijc.34132DOI Listing
October 2022

Histologic type predicts disparate outcomes in pediatric hepatocellular neoplasms: A Pediatric Surgical Oncology Research Collaborative study.

Cancer 2022 07 13;128(14):2786-2795. Epub 2022 May 13.

Division of Pediatric Surgery, Children's Hospital, London Health Sciences Center, London, Ontario, Canada.

Background: Hepatocellular carcinoma (HCC) is a rare cancer in children, with various histologic subtypes and a paucity of data to guide clinical management and predict prognosis.

Methods: A multi-institutional review of children with hepatocellular neoplasms was performed, including demographic, staging, treatment, and outcomes data. Patients were categorized as having conventional HCC (cHCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), and hepatoblastoma with HCC features (HB-HCC). Univariate and multivariate analyses identified predictors of mortality and relapse.

Results: In total, 262 children were identified; and an institutional histologic review revealed 110 cHCCs (42%; 69 normal background liver, 34 inflammatory/cirrhotic, 7 unknown), 119 FLCs (45%), and 33 HB-HCCs (12%). The authors observed notable differences in presentation and behavior among tumor subtypes, including increased lymph node involvement in FLC and higher stage in cHCC. Factors associated with mortality included cHCC (hazard ratio [HR], 1.63; P = .038), elevated α-fetoprotein (HR, 3.1; P = .014), multifocality (HR, 2.4; P < .001), and PRETEXT (pretreatment extent of disease) stage IV (HR, 5.76; P < .001). Multivariate analysis identified increased mortality in cHCC versus FLC (HR, 2.2; P = .004) and in unresectable tumors (HR, 3.4; P < .001). Disease-free status at any point predicted survival.

Conclusions: This multi-institutional, detailed data set allowed a comprehensive analysis of outcomes for children with these rare hepatocellular neoplasms. The current data demonstrated that pediatric HCC subtypes are not equivalent entities because FLC and cHCC have distinct anatomic patterns and outcomes in concert with their known molecular differences. This data set will be further used to elucidate the impact of histology on specific treatment responses, with the goal of designing risk-stratified algorithms for children with HCC.

Lay Summary: This is the largest reported granular data set on children with hepatocellular carcinoma. The study evaluates different subtypes of hepatocellular carcinoma and identifies key differences between subtypes. This information is pivotal in improving understanding of these rare cancers and may be used to improve clinical management and subsequent outcome in children with these rare malignancies.
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http://dx.doi.org/10.1002/cncr.34256DOI Listing
July 2022

Demographic and Clinical Characteristics Associated With the Failure of Nonoperative Management of Uncomplicated Appendicitis in Children: Secondary Analysis of a Nonrandomized Clinical Trial.

JAMA Netw Open 2022 05 2;5(5):e229712. Epub 2022 May 2.

Center for Surgical Outcomes Research, Abigail Wexner Research Institute at Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus.

Importance: The factors associated with the failure of nonoperative management of appendicitis and the differences in patient-reported outcomes between successful and unsuccessful nonoperative management remain unknown.

Objectives: To investigate factors associated with the failure of nonoperative management of appendicitis and compare patient-reported outcomes between patients whose treatment succeeded and those whose treatment failed.

Design, Setting, And Participants: This study was a planned subgroup secondary analysis conducted in 10 children's hospitals that included 370 children aged 7 to 17 years with uncomplicated appendicitis enrolled in a prospective, nonrandomized clinical trial between May 1, 2015, and October 31, 2018, with 1-year follow-up comparing nonoperative management with antibiotics vs surgery for uncomplicated appendicitis. Statistical analysis was performed from November 1, 2019, to February 12, 2022.

Interventions: Nonoperative management with antibiotics vs surgery.

Main Outcomes And Measures: Failure of nonoperative management and patient-reported outcomes. The relative risk (RR) of failure based on sociodemographic and clinical characteristics was calculated. Patient-reported outcomes were compared based on the success or failure of nonoperative management.

Results: Of 370 patients (34.6% of 1068 total patients; 229 boys [61.9%]; median age, 12.3 years [IQR, 10.0-14.6 years]) enrolled in the nonoperative group, treatment failure occurred for 125 patients (33.8%) at 1 year, with 53 patients (14.3%) undergoing appendectomy during initial hospitalization and 72 patients (19.5%) experiencing delayed treatment failure after hospital discharge. Higher patient-reported pain at presentation was associated with increased risk of in-hospital treatment failure (RR, 2.1 [95% CI, 1.0-4.4]) but not delayed treatment failure (RR, 1.3 [95% CI, 0.7-2.3]) or overall treatment failure at 1 year (RR, 1.5 [95% CI, 1.0-2.2]). Pain duration greater than 24 hours was associated with decreased risk of delayed treatment failure (RR, 0.3 [95% CI, 0.1-1.0]) but not in-hospital treatment failure (RR, 1.2 [95% CI, 0.5-2.7]) or treatment failure at 1 year (RR, 0.7 [95% CI, 0.4-1.2]). There was no increased risk of treatment failure associated with age, white blood cell count, sex, race, ethnicity, primary language, insurance status, transfer status, symptoms at presentation, or imaging results. Health care satisfaction at 30 days and patient-reported, health-related quality of life at 30 days and 1 year were not different. Satisfaction with the decision was higher with successful nonoperative management at 30 days (28.0 vs 27.0; difference, 1.0 [95% CI, 0.01-2.0]) and 1 year (28.1 vs 27.0; difference, 1.1 [95% CI, 0.2-2.0]).

Conclusions And Relevance: This analysis suggests that a higher pain level at presentation was associated with a higher risk of initial failure of nonoperative management and that a longer duration of pain was associated with lower risk of delayed treatment failure. Although satisfaction was high in both groups, satisfaction with the treatment decision was higher among patients with successful nonoperative management at 1 year.

Trial Registration: ClinicalTrials.gov Identifier: NCT02271932.
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http://dx.doi.org/10.1001/jamanetworkopen.2022.9712DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9062687PMC
May 2022

Interhospital variability in localization techniques for small pulmonary nodules in children: A pediatric surgical oncology research collaborative study.

J Pediatr Surg 2022 Jun 23;57(6):1013-1017. Epub 2022 Feb 23.

Children's Hospital Colorado, Aurora, CO, United States.

Background: Pulmonary nodules that are deep within lung parenchyma and/or small in size can be challenging to localize for biopsy. This study describes current trends in performance of image-guided localization techniques for pulmonary nodules in pediatric patients.

Methods: A retrospective review was performed on patients < 21 years of age undergoing localization of pulmonary nodules at 15 institutions. Localization and resection success, time in interventional radiology (IR), operating room (OR) and total anesthesia time, complications, and technical problems were compared between techniques.

Results: 225 patients were included with an average of 1.3 lesions (range 1-5). Median nodule size and depth were 4 mm (range 0-30) and 5.4 mm (0-61), respectively. The most common localization techniques were: wire + methylene blue dye (MBD) (28%), MBD only (25%), wire only (14%), technetium-99 only (11%), coil + MBD (7%) and coil only (5%). Localization technique was associated with institution (p < 0.01); technique and institution were significantly associated with mean IR, OR, and anesthesia time (all p < 0.05). Comparing techniques, there was no difference in successful IR localization (range 92-100%, p = 0.75), successful resection (94-100%, p = 0.98), IR technical problems (p = 0.22), or operative complications (p = 0.16).

Conclusions: Many IR localization techniques for small pulmonary nodules in children can be successful, but there is wide variability in application by institution and in procedure time.

Level Of Evidence: Retrospective review, Level 3.
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http://dx.doi.org/10.1016/j.jpedsurg.2022.01.061DOI Listing
June 2022

Acid suppression duration does not alter anastomotic stricture rates after esophageal atresia with distal tracheoesophageal fistula repair: A prospective multi-institutional cohort study.

J Pediatr Surg 2022 Jun 14;57(6):975-980. Epub 2022 Feb 14.

Department of Surgery, Division of Pediatric Surgery, University of Louisville School of Medicine, 530 S. Jackson St, Louisville, KY, 40202, United States of America.

Introduction: Anastomotic stricture is the most common complication after esophageal atresia (EA) repair. We sought to determine if postoperative acid suppression is associated with reduced stricture formation.

Methods: A prospective, multi-institutional cohort study of infants undergoing primary EA repair from 2016 to 2020 was performed. Landmark analysis and multivariate Cox regression were used to explore if initial duration of acid suppression was associated with stricture formation at hospital discharge (DC), 3-, 6-, and 9-months postoperatively.

Results: Of 156 patients, 79 (51%) developed strictures and 60 (76%) strictures occurred within three months following repair. Acid suppression was used in 141 patients (90%). Landmark analysis showed acid suppression was not associated with reduction in initial stricture formation at DC, 3-, 6- and 9-months, respectively (p = 0.19-0.95). Multivariate regression demonstrated use of a transanastomotic tube was significantly associated with stricture formation at DC (Hazard Ratio (HR) = 2.21 (95% CI 1.24-3.95, p<0.01) and 3-months (HR 5.31, 95% CI 1.65-17.16, p<0.01). There was no association between acid suppression duration and stricture formation.

Conclusion: No association between the duration of postoperative acid suppression and anastomotic stricture was observed. Transanastomotic tube use increased the risk of anastomotic strictures at hospital discharge and 3 months after repair.

Level Of Evidence: 2:
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http://dx.doi.org/10.1016/j.jpedsurg.2022.02.004DOI Listing
June 2022

Laparoscopy versus laparotomy for pediatric ovarian dermoids.

J Pediatr Surg 2022 Jun 12;57(6):1008-1012. Epub 2022 Feb 12.

Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, United States.

Background: With increased surgeon comfort using laparoscopy, we hypothesized resection of pediatric ovarian dermoids using laparoscopy would yield a shorter length of stay and no increase in morbidity or recurrence compared to laparotomy.

Methods: A retrospective review was performed amongst eleven pediatric hospitals. Patients aged 2 to 21 who underwent resection of an ovarian dermoid from 2010 to 2020 were included. Patient characteristics, operative details, and outcomes by approach were evaluated using Chi-squared and Wilcoxon-Mann tests.

Results: 466 patients were included, with a median age of 14.4 and median follow-up of 4.0 months. 279 patients underwent laparoscopy (60%), 139 laparotomy (30%), and 48 laparoscopy converted to laparotomy (10%). There were no differences in rates of tumor spillage by approach (p = 0.15). 65% underwent ovarian-sparing surgery and 35% underwent oophorectomy. Length of stay was significantly shorter amongst patients who underwent laparoscopy (1 day versus 2 days for laparotomy and converted, p<0.0001). There were no differences in rates of suspected recurrence or reoperation (p = 0.19 and p = 0.57, respectively).

Conclusion: Patients who underwent laparoscopy experienced no differences in the rates of tumor spillage, recurrence, or reoperation and had a shorter length of stay compared to laparotomy. Laparoscopy is an acceptable approach for resection of pediatric ovarian dermoids.
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http://dx.doi.org/10.1016/j.jpedsurg.2022.01.053DOI Listing
June 2022

Multi-Institutional Review of the Preoperative Diagnostic Accuracy for Pediatric Ovarian Mature Cystic Teratomas.

J Pediatr Adolesc Gynecol 2022 Aug 4;35(4):478-485. Epub 2022 Feb 4.

Center for Surgical Outcomes Research, Abigail Wexner Research Institute and Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio.

Study Objective: To assess the preoperative imaging impression and surgeon diagnostic accuracy for pediatric ovarian mature cystic teratomas (MCTs) DESIGN: Retrospective review SETTING: Eleven pediatric hospitals PARTICIPANTS: Patients ages 2 to 21 who underwent surgical management of an ovarian neoplasm or adnexal torsion with an associated ovarian lesion INTERVENTION: None MAIN OUTCOME MEASURES: Preoperative imaging impression, surgeon diagnosis, tumor markers, and pathology RESULTS: Our cohort included 946 females. Final pathology identified 422 (45%) MCTs, 405 (43%) other benign pathologies, and 119 (12%) malignancies. Preoperative imaging impression for MCTs had a 70% sensitivity, 92% specificity, 88% positive predictive value (PPV), and 79% negative predictive value (NPV). For the preoperative surgeon diagnosis, sensitivity was 59%, specificity 96%, PPV 92%, and NPV 74%. Some measures of diagnostic accuracy were affected by the presence of torsion, size of the lesion on imaging, imaging modality, and surgeon specialty. Of the 352 masses preoperatively thought to be MCTs, 14 were malignancies (4%). Eleven patients with inaccurately diagnosed malignancies had tumor markers evaluated and 82% had at least 1 elevated tumor marker, compared with 49% of those with MCTs.

Conclusions: Diagnostic accuracy for the preoperative imaging impression and surgeon diagnosis is lower than expected for pediatric ovarian MCTs. For all ovarian neoplasms, preoperative risk assessment including a panel of tumor markers and a multidisciplinary review is recommended. This process could minimize the risk of misdiagnosis and improve operative planning to maximize the use of ovarian-sparing surgery for benign lesions and allow for appropriate resection and staging for lesions suspected to be malignant.
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http://dx.doi.org/10.1016/j.jpag.2022.01.009DOI Listing
August 2022

Advances in the Surgical Management of Esophageal Atresia.

Adv Pediatr 2021 08 16;68:245-259. Epub 2021 Jun 16.

Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, 999 North 92nd Street, Suite 320, Milwaukee, WI 53226, USA. Electronic address:

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http://dx.doi.org/10.1016/j.yapd.2021.05.004DOI Listing
August 2021

Thoracoscopic versus open lobectomy in infants with congenital lung malformations: A multi-institutional propensity score analysis.

J Pediatr Surg 2021 Dec 25;56(12):2148-2156. Epub 2021 Apr 25.

Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Johns Hopkins Children's Center, Baltimore, MD, United States. Electronic address:

Purpose: The impact of thoracoscopic surgery on outcomes in children with congenital lung malformations (CLM) remains controversial. The purpose of this study was to determine the effect of operative approach on perioperative outcomes in infants undergoing lobectomy for an asymptomatic CLM.

Methods: After IRB approval, a retrospective cohort study was conducted on 506 children with a CLM resected at one of eleven children's hospitals over a seven-year period. Infants undergoing elective lobectomy were identified, and covariates were balanced based on operative approach using propensity scores with full matching. Outcomes were analyzed based on intention to treat with weighted conditional regression.

Results: One hundred seventy-five infants met inclusion criteria. There were 67 (38.3%) open, 89 (50.9%) thoracoscopic, and 19 (10.9%) thoracoscopic-converted-to-open lobectomies. Thoracoscopic lobectomy was associated with significantly longer operative times (26 min, 95% CI 6-47 min, p = 0.012) but used less epidural anesthesia (OR 0.02, 95% CI 0.004-0.11, p<0.001) when compared to open lobectomy. There were no significant differences in intraoperative blood loss, postoperative complications, chest tube duration, or length of stay.

Conclusions: Thoracoscopy has become the most common operative approach for elective lobectomy in infants with asymptomatic CLMs. The non-inferiority of thoracoscopic lobectomy in postoperative outcomes supports its continued use as an alternative to open lobectomy.

Level Of Evidence: Treatment study, Level III.
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http://dx.doi.org/10.1016/j.jpedsurg.2021.04.013DOI Listing
December 2021

Management of Pediatric Breast Masses: A Multi-institutional Retrospective Cohort Study.

J Surg Res 2021 08 10;264:309-315. Epub 2021 Apr 10.

Center for Surgical Outcomes Research, Nationwide Children's Hospital, Columbus, Ohio; Department of Surgery, Division of Pediatric Surgery, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, Ohio.

Background: The objective of our study was to describe the workup, management, and outcomes of pediatric patients with breast masses undergoing operative intervention.

Materials And Methods: A retrospective cohort study was conducted of girls 10-21 y of age who underwent surgery for a breast mass across 11 children's hospitals from 2011 to 2016. Demographic and clinical characteristics were summarized.

Results: Four hundred and fifty-three female patients with a median age of 16 y (IQR: 3) underwent surgery for a breast mass during the study period. The most common preoperative imaging was breast ultrasound (95%); 28% reported the Breast Imaging Reporting and Data System (BI-RADS) classification. Preoperative core biopsy was performed in 12%. All patients underwent lumpectomy, most commonly due to mass size (45%) or growth (29%). The median maximum dimension of a mass on preoperative ultrasound was 2.8 cm (IQR: 1.9). Most operations were performed by pediatric surgeons (65%) and breast surgeons (25%). The most frequent pathology was fibroadenoma (75%); 3% were phyllodes. BI-RADS scoring ≥4 on breast ultrasound had a sensitivity of 0% and a negative predictive value of 93% for identifying phyllodes tumors.

Conclusions: Most pediatric breast masses are self-identified and benign. BI-RADS classification based on ultrasound was not consistently assigned and had little clinical utility for identifying phyllodes.
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http://dx.doi.org/10.1016/j.jss.2021.01.041DOI Listing
August 2021

Pleuropulmonary Blastoma in Pediatric Lung Lesions.

Pediatrics 2021 04 24;147(4). Epub 2021 Mar 24.

Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Background: Pediatric lung lesions are a group of mostly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. Our objective was to evaluate the characteristics of children undergoing resection of a primary lung lesion and to identify preoperative risk factors for malignancy.

Methods: A retrospective cohort study was conducted by using an operative database of 521 primary lung lesions managed at 11 children's hospitals in the United States. Multivariable logistic regression was used to examine the relationship between preoperative characteristics and risk of malignancy, including pleuropulmonary blastoma (PPB).

Results: None of the 344 prenatally diagnosed lesions had malignant pathology ( < .0001). Among 177 children without a history of prenatal detection, 15 (8.7%) were classified as having a malignant tumor (type 1 PPB, = 11; other PPB, = 3; adenocarcinoma, = 1) at a median age of 20.7 months (interquartile range, 7.9-58.1). Malignancy was associated with the DICER1 mutation in 8 (57%) PPB cases. No malignant lesion had a systemic feeding vessel ( = .0427). The sensitivity of preoperative chest computed tomography (CT) for detecting malignant pathology was 33.3% (95% confidence interval [CI]: 15.2-58.3). Multivariable logistic regression revealed that increased suspicion of malignancy by CT and bilateral disease were significant predictors of malignant pathology (odds ratios of 42.15 [95% CI, 7.43-340.3; < .0001] and 42.03 [95% CI, 3.51-995.6; = .0041], respectively).

Conclusions: In pediatric lung masses initially diagnosed after birth, the risk of PPB approached 10%. These results strongly caution against routine nonoperative management in this patient population. DICER1 testing may be helpful given the poor sensitivity of CT for identifying malignant pathology.
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http://dx.doi.org/10.1542/peds.2020-028357DOI Listing
April 2021

Factors Associated with Torsion in Pediatric Patients with Ovarian Masses.

J Surg Res 2021 07 26;263:110-115. Epub 2021 Feb 26.

Center for Surgical Outcomes Research, Nationwide Children's Hospital, Columbus, Ohio; Department of Surgery, Division of Pediatric Surgery, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, Ohio. Electronic address:

Background: Management of ovarian torsion has evolved toward ovarian preservation regardless of ovarian appearance during surgery. However, patients with torsion and an ovarian neoplasm undergo a disproportionately high rate of oophorectomy. Our objectives were to identify factors associated with ovarian torsion among females with an ovarian mass and to determine if torsion is associated with malignancy.

Methods: A retrospective review of females aged 2-21 y who underwent an operation for an ovarian cyst or neoplasm between 2010 and 2016 at 10 children's hospitals was performed. Multivariate logistic regression was used to assess factors associated with torsion. Imaging data were assessed for sensitivity, specificity, and predictive value in identifying ovarian torsion.

Results: Of 814 girls with an ovarian neoplasm, 180 (22%) had torsion. In risk-adjusted analyses, patients with a younger age, mass size >5 cm, abdominal pain, and vomiting had an increased likelihood of torsion (P < 0.01 for all). Patients with a mass >5 cm had two times the odds of torsion (odds ratio: 2.1; confidence interval: 1.2, 3.6). Imaging was not reliable at identifying torsion (sensitivity 34%, positive predictive value 49%) or excluding torsion (specificity 72%, negative predictive value 87%). The rates of malignancy were lower in those with an ovarian mass and torsion than those without torsion (10% versus 17%, P = 0.01). Among the 180 girls with torsion and a mass, 48% underwent oophorectomy of which 14% (n = 12) had a malignancy.

Conclusions: In females with an ovarian neoplasm, torsion is not associated with an increased risk of malignancy and ovarian preservation should be considered.
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http://dx.doi.org/10.1016/j.jss.2020.12.058DOI Listing
July 2021

Appendectomy Versus Observation for Appendicitis in Neutropenic Children With Cancer.

Pediatrics 2021 02;147(2)

Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Seattle, Washington.

Background: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration.

Methods: Sites from the Pediatric Surgery Oncology Research Collaborative performed a retrospective review of children with NA over a 6-year period.

Results: Sixty-six children, with a median age of 11 years (range 1-17), were identified with NA while undergoing cancer treatment. The most common cancer diagnoses were leukemia (62%) and brain tumor (12%). Upfront appendectomy was performed in 41% of patients; the remainder had initial nonoperative management. Rates of abscess or perforation at diagnosis were equivalent in the groups (30% vs 24%; = .23). Of patients who had initial nonoperative management, 46% (17 of 37) underwent delayed appendectomy during the same hospitalization. Delayed appendectomy was due to failure of initial nonoperative management in 65% ( = 11) and count recovery in 35% ( = 6). Cancer therapy was delayed in 35% ( = 23). Initial nonoperative management was associated with a delay in cancer treatment (46% vs. 22%, = .05) and longer LOS (29 vs 12 days; = .01). Patients who had initial nonoperative management and delayed appendectomy had a higher rate of postoperative complications ( < .01).

Conclusions: In pediatric patients with NA from oncologic treatment, upfront appendectomy resulted in lower complication rates, reduced LOS, and fewer alterations in chemotherapy regimens compared to initial nonoperative management.
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http://dx.doi.org/10.1542/peds.2020-027797DOI Listing
February 2021

Novel placement of an esophageal wound vacuum for a persistent anastomotic leak.

Endoscopy 2021 10 17;53(10):E388-E389. Epub 2020 Dec 17.

Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

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http://dx.doi.org/10.1055/a-1308-1007DOI Listing
October 2021

Fetal Risk Stratification and Outcomes in Children with Prenatally Diagnosed Lung Malformations: Results from a Multi-Institutional Research Collaborative.

Ann Surg 2020 Nov 17. Epub 2020 Nov 17.

Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Objective: To assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes.

Summary Background Data: Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery have not been well defined.

Methods: A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at eleven children's hospitals from 2009-2016. Statistical analyses were performed using non-parametric bivariate or multivariable logistic regression.

Results: Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection (p < 0.001). An initial CVR ≤ 1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87-1.00]. A maximum CVR < 0.9 (AUC, 0.72; 95% CI, 0.67-0.85) was associated with a low risk for respiratory symptoms at birth.

Conclusion: In this large, multi-institutional study, an initial CVR ≤ 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.
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http://dx.doi.org/10.1097/SLA.0000000000004566DOI Listing
November 2020

Clinical outcomes following implementation of a management bundle for esophageal atresia with distal tracheoesophageal fistula.

J Pediatr Surg 2021 Jan 6;56(1):47-54. Epub 2020 Oct 6.

Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Louisville, KY.

Background/purpose: This study evaluated compliance with a multi-institutional quality improvement management protocol for Type-C esophageal atresia with distal tracheoesophageal fistula (EA/TEF).

Methods: Compliance and outcomes before and after implementation of a perioperative protocol bundle for infants undergoing Type-C EA/TEF repair were compared across 11 children's hospitals from 1/2016-1/2019. Bundle components included elimination of prosthetic material between tracheal and esophageal suture lines during repair, not leaving a transanastomotic tube at the conclusion of repair (NO-TUBE), obtaining an esophagram by postoperative-day-5, and discontinuing prophylactic antibiotics 24 h postoperatively.

Results: One-hundred seventy patients were included, 40% pre-protocol and 60% post-protocol. Bundle compliance increased 2.5-fold pre- to post-protocol from 17.6% to 44.1% (p < 0.001). After stratifying by institutional compliance with all bundle components, 43.5% of patients were treated at low-compliance centers (<20%), 43% at medium-compliance centers (20-80%), and 13.5% at high-compliance centers (>80%). Rates of esophageal leak, anastomotic stricture, and time to full feeds did not differ between pre- and post-protocol cohorts, though there was an inverse correlation between NO-TUBE compliance and stricture rate over time (ρ = -0.75, p = 0.029).

Conclusions: Compliance with our multi-institutional management protocol increased 2.5-fold over the study period without compromising safety or time to feeds and does not support the use of transanastomotic tubes.

Level Of Evidence: Level II.

Type Of Study: Treatment Study.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.09.049DOI Listing
January 2021

Appendectomy in pediatric patients with synchronous oncologic diagnosis is safe: an analysis using the national surgical quality improvement project, pediatric.

Pediatr Surg Int 2020 Nov 11;36(11):1333-1338. Epub 2020 Sep 11.

Department of Surgery, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY, 14214, USA.

Background: Optimal treatment of children who develop appendicitis while undergoing treatment for an oncologic diagnosis has not been defined, in part due to theoretical concerns for an increased risk of postoperative wound complications. We hypothesized that synchronous oncologic diagnosis conferred no increased odds of developing a wound complication in pediatric patients undergoing appendectomy.

Methods: Retrospective cohort study using the National Surgical Quality Improvement Program, Pediatric (2012-2017) of patients < 18 years of age undergoing appendectomy. The main exposure variable was active treatment for an oncologic diagnosis. The primary outcomes of interest were 30-day wound complications (superficial or deep infections or dehiscence, and abscess). For univariate analysis comparison of baseline differences between patients with/without a cancer diagnosis we employed Pearson's χ and two sample t tests. Multivariate logistic regression was used to evaluate which covariates were independently associated with our outcome.

Results: We identified 28,219 patients who had undergone appendectomy; 95 (0.3%) were undergoing oncologic treatment at the time of surgery. Patients in the cancer group were more likely to be receiving steroids, have lower white blood cell counts and have higher American Society of Anesthesiology classes as compared to the noncancer patients. Age, gender, rates of perforation, and laparoscopy were similar between the two groups. Patients with an active cancer diagnosis suffered wound complications (measured individually and as an aggregate) at no higher odds than those without a cancer diagnosis.

Conclusion: Pediatric patients undergoing treatment for cancer do not have increased odds of suffering postoperative wound complications following appendectomy as compared to the general population.
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http://dx.doi.org/10.1007/s00383-020-04743-8DOI Listing
November 2020

Association of Nonoperative Management Using Antibiotic Therapy vs Laparoscopic Appendectomy With Treatment Success and Disability Days in Children With Uncomplicated Appendicitis.

JAMA 2020 08;324(6):581-593

Center for Surgical Outcomes Research, Abigail Wexner Research Institute at Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio.

Importance: Nonoperative management with antibiotics alone has the potential to treat uncomplicated pediatric appendicitis with fewer disability days than surgery.

Objective: To determine the success rate of nonoperative management and compare differences in treatment-related disability, satisfaction, health-related quality of life, and complications between nonoperative management and surgery in children with uncomplicated appendicitis.

Design, Setting, And Participants: Multi-institutional nonrandomized controlled intervention study of 1068 children aged 7 through 17 years with uncomplicated appendicitis treated at 10 tertiary children's hospitals across 7 US states between May 2015 and October 2018 with 1-year follow-up through October 2019. Of the 1209 eligible patients approached, 1068 enrolled in the study.

Interventions: Patient and family selection of nonoperative management with antibiotics alone (nonoperative group, n = 370) or urgent (≤12 hours of admission) laparoscopic appendectomy (surgery group, n = 698).

Main Outcomes And Measures: The 2 primary outcomes assessed at 1 year were disability days, defined as the total number of days the child was not able to participate in all of his/her normal activities secondary to appendicitis-related care (expected difference, 5 days), and success rate of nonoperative management, defined as the proportion of patients initially managed nonoperatively who did not undergo appendectomy by 1 year (lowest acceptable success rate, ≥70%). Inverse probability of treatment weighting (IPTW) was used to adjust for differences between treatment groups for all outcome assessments.

Results: Among 1068 patients who were enrolled (median age, 12.4 years; 38% girls), 370 (35%) chose nonoperative management and 698 (65%) chose surgery. A total of 806 (75%) had complete follow-up: 284 (77%) in the nonoperative group; 522 (75%) in the surgery group. Patients in the nonoperative group were more often younger (median age, 12.3 years vs 12.5 years), Black (9.6% vs 4.9%) or other race (14.6% vs 8.7%), had caregivers with a bachelor's degree (29.8% vs 23.5%), and underwent diagnostic ultrasound (79.7% vs 74.5%). After IPTW, the success rate of nonoperative management at 1 year was 67.1% (96% CI, 61.5%-72.31%; P = .86). Nonoperative management was associated with significantly fewer patient disability days at 1 year than did surgery (adjusted mean, 6.6 vs 10.9 days; mean difference, -4.3 days (99% CI, -6.17 to -2.43; P < .001). Of 16 other prespecified secondary end points, 10 showed no significant difference.

Conclusion And Relevance: Among children with uncomplicated appendicitis, an initial nonoperative management strategy with antibiotics alone had a success rate of 67.1% and, compared with urgent surgery, was associated with statistically significantly fewer disability days at 1 year. However, there was substantial loss to follow-up, the comparison with the prespecified threshold for an acceptable success rate of nonoperative management was not statistically significant, and the hypothesized difference in disability days was not met.

Trial Registration: ClinicalTrials.gov Identifier: NCT02271932.
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http://dx.doi.org/10.1001/jama.2020.10888DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7385674PMC
August 2020

Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study.

Pediatr Blood Cancer 2020 09 13;67(9):e28425. Epub 2020 Jul 13.

Division of Pediatric Surgery, CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada.

Background: To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD).

Methods: Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected.

Results: Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival.

Conclusion: This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology.
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http://dx.doi.org/10.1002/pbc.28425DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7674210PMC
September 2020

Multimodal Therapy Including Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Can Result in Long-term Disease-free Survival in Pediatric Desmoplastic Small Round Cell Tumor With Extraperitoneal Disease.

J Pediatr Hematol Oncol 2021 08;43(6):228-231

Department of Surgery, Division of Pediatric Surgery.

Desmoplastic small round cell tumor is a rare sarcoma with 5-year overall survival of 15%. An 8-year-old female presented with diffuse abdominal/pelvic desmoplastic small round cell tumor including numerous liver metastasis. She underwent neoadjuvant chemotherapy followed by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Residual disease was found shortly after CRS/HIPEC which was resected, followed by whole abdomen/pelvic radiation and autologous hematopoietic cell transplant. Previous papers have reported dismal survival in patients with liver metastasis and residual disease arguing against CRS/HIPEC. Our patient remains disease-free over 6 years after completing therapy indicating long-term survival is achievable with aggressive multimodal therapy.
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http://dx.doi.org/10.1097/MPH.0000000000001861DOI Listing
August 2021

Pneumonectomy for Pediatric Tumors-a Pediatric Surgical Oncology Research Collaborative Study.

Ann Surg 2021 12;274(6):e605-e609

Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

Objective: To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer.

Summary Background Data: Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors.

Methods: The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients <21 years of age who underwent pneumonectomy from 1990 to 2017 for primary or metastatic tumors at 12 institutions. Clinical information was collected; outcomes included operative complications, long-term function, recurrence, and survival. Univariate log rank, and multivariable Cox analyses determined factors associated with survival.

Results: Thirty-eight patients (mean 12 ± 6 yrs) were identified; median (IQR) follow-up was 19 (5-38) months. Twenty-six patients (68%) underwent pneumonectomy for primary tumors and 12 (32%) for metastases. The most frequent histologies were osteosarcoma (n = 6), inflammatory myofibroblastic tumors (IMT; n = 6), and pleuropulmonary blastoma (n = 5). Median postoperative ventilator days were 0 (0-1), intensive care 2 (1-3), and hospital 8 (5-16). Early postoperative complications occurred in 10 patients including 1 death. Of 25 (66%) patients alive at 1 year, 15 reported return to preoperative pulmonary status. All IMT patients survived while all osteosarcoma patients died during follow-up. On multivariable analysis, metastatic indications were associated with nonsurvival (HR = 3.37, P = 0.045).

Conclusion: This is the largest review of children who underwent pneumonectomy for cancer. There is decreased procedure-related morbidity and mortality than reported for adults. Survival is worse with preoperative metastatic disease, especially osteosarcoma.
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http://dx.doi.org/10.1097/SLA.0000000000003795DOI Listing
December 2021

Optimization of percutaneous biopsy for diagnosis and pretreatment risk assessment of neuroblastoma.

Pediatr Blood Cancer 2020 05 19;67(5):e28153. Epub 2020 Feb 19.

Division of Pediatric Surgery, Department of Surgery, C. S. Mott Children's Hospital, The University of Michigan, Ann Arbor, Michigan.

Background: Image-guided percutaneous core needle biopsy (PCNB) is increasingly utilized to diagnose solid tumors. The objective of this study is to determine whether PCNB is adequate for modern biologic characterization of neuroblastoma.

Procedure: A multi-institutional retrospective study was performed by the Pediatric Surgical Oncology Research Collaborative on children with neuroblastoma at 12 institutions over a 3-year period. Data collected included demographics, clinical details, biopsy technique, complications, and adequacy of biopsies for cytogenetic markers utilized by the Children's Oncology Group for risk stratification.

Results: A total of 243 children were identified with a diagnosis of neuroblastoma: 79 (32.5%) tumor excision at diagnosis, 94 (38.7%) open incisional biopsy (IB), and 70 (28.8%) PCNB. Compared to IB, there was no significant difference in ability to accurately obtain a primary diagnosis by PCNB (95.7% vs 98.9%, P = .314) or determine MYCN copy number (92.4% vs 97.8%, P = .111). The yield for loss of heterozygosity and tumor ploidy was lower with PCNB versus IB (56.1% vs 90.9%, P < .05; and 58.0% vs. 88.5%, P < .05). Complications did not differ between groups (2.9 % vs 3.3%, P = 1.000), though the PCNB group had fewer blood transfusions and lower opioid usage. Efficacy of PCNB was improved for loss of heterozygosity when a pediatric pathologist evaluated the fresh specimen for adequacy.

Conclusions: PCNB is a less invasive alternative to open biopsy for primary diagnosis and MYCN oncogene status in patients with neuroblastoma. Our data suggest that PCNB could be optimized for complete genetic analysis by standardized protocols and real-time pathology assessment of specimen quality.
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http://dx.doi.org/10.1002/pbc.28153DOI Listing
May 2020

Changing the Paradigm for Management of Pediatric Primary Spontaneous Pneumothorax: A Simple Aspiration Test Predicts Need for Operation.

J Pediatr Surg 2020 Jan 24;55(1):169-175. Epub 2019 Oct 24.

Department of Surgery, Phoenix Children's Hospital, Phoenix, AZ.

Purpose: 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.

Type Of Study: Prospective pilot study LEVEL OF EVIDENCE: Level III.
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http://dx.doi.org/10.1016/j.jpedsurg.2019.09.043DOI Listing
January 2020

Understanding the Value of Tumor Markers in Pediatric Ovarian Neoplasms.

J Pediatr Surg 2020 Jan 25;55(1):122-125. Epub 2019 Oct 25.

Department of Surgery, Division of Pediatric Surgery, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, OH. Electronic address:

Purpose: The purpose of this study was to determine the diagnostic accuracy of tumor markers for malignancy in girls with ovarian neoplasms.

Methods: A retrospective review of girls 2-21 years who presented for surgical management of an ovarian neoplasm across 10 children's hospitals between 2010 and 2016 was performed. Patients who had at least one concerning feature on imaging and had tumor marker testing were included in the study. Sensitivity, specificity, and negative and positive predictive values (PPV) of tumor markers were calculated.

Results: Our cohort included 401 patients; 22.4% had a malignancy. Testing for tumor markers was inconsistent. AFP had high specificity (98%) and low sensitivity (42%) with a PPV of 86%. The sensitivity, specificity, and PPV of beta-hCG was 44%, 76%, and 32%, respectively. LDH had high sensitivity (95%) and Inhibin A and Inhibin B had high specificity (97% and 92%, respectively).

Conclusions: Tumor marker testing is helpful in preoperative risk stratification of ovarian neoplasms for malignancy. Given the variety of potential tumor types, no single marker provides enough reliability, and therefore a panel of tumor marker testing is recommended if there is concern for malignancy. Prospective studies may help further elucidate the predictive value of tumor markers in a pediatric ovarian neoplasm population.

Type Of Study: Retrospective Cohort Review.

Level Of Evidence: Level III.
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http://dx.doi.org/10.1016/j.jpedsurg.2019.09.062DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7181461PMC
January 2020

Esophagojejunal Anastomosis by Circular Stapler in Pediatric Patients: Size Minima Defined by Experience and Geometry.

J Laparoendosc Adv Surg Tech A 2019 Oct 24;29(10):1311-1314. Epub 2019 Sep 24.

Pediatric General and Thoracic Surgery, Children's Hospital of Wisconsin, Milwaukee, Wisconsin.

Esophagojejunostomy is facilitated by use of a circular stapler, particularly when performed laparoscopically. The minimum patient size that will allow use of circular staplers in the small intestine is unknown. Retrospective review of esophagogastric dissociations performed at a single tertiary care institution for 48 months. This was combined with a geometric derivation of a size-estimation formula. From the 7 cases identified, patients weighing >16 kg easily accommodated the 21 mm stapler. There was a narrow fit in the patient weighing 13.6 kg, and the 6 kg patient was too small for the stapler. Through a combination of clinical observation and physical reasoning, circular stapler applicability in small intestine is predicted by patient weight or intestinal measurement. Patients weighing >16 kg will accept the stapler, whereas patients <13 kg are likely too small. Alternately, on the basis of a geometric derivation, if the width of the flat intestine is >1.6 × the device diameter, the device will fit. This calculation can be applied broadly (e.g., incision length for laparoscopic ports or single-port access devices).
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http://dx.doi.org/10.1089/lap.2019.0092DOI Listing
October 2019
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