CPAM Publications

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

Kunisaki SM, Saito JM, Fallat ME, Peter SDS, Lal DR, Karmakar M, Deans KJ, Gadepalli SK, Hirschl RB, Minneci PC, Helmrath MA; Midwest Pediatric Surgery Consortium. Fetal Risk Stratification and Outcomes in Children with Prenatally Diagnosed Lung Malformations: Results from a Multi-Institutional Research Collaborative. Ann Surg. 2022 Nov 1;276(5):e622-e630. doi: 10.1097/SLA.0000000000004566. Epub 2020 Nov 17. PMID: 33214447.

  • Abstract

    Objective: The aim of this study was 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 has not been well defined.

    Methods: A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at 11 children's hospitals from 2009 to 2016. Statistical analyses were performed using nonparametric 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.

    Conclusions: 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.

    Cite

    Kunisaki SM, Saito JM, Fallat ME, Peter SDS, Lal DR, Karmakar M, Deans KJ, Gadepalli SK, Hirschl RB, Minneci PC, Helmrath MA; Midwest Pediatric Surgery Consortium. Fetal Risk Stratification and Outcomes in Children with Prenatally Diagnosed Lung Malformations: Results from a Multi-Institutional Research Collaborative. Ann Surg. 2022 Nov 1;276(5):e622-e630. doi: 10.1097/SLA.0000000000004566. Epub 2020 Nov 17. PMID: 33214447.

Response to the Comment on "Fetal Risk Stratification and Outcomes in Children With Prenatally Diagnosed Lung Malformations: Results From a Multi-institutional Research Collaborative by Kunisaki et al"

Kunisaki SM. Response to the Comment on "Fetal Risk Stratification and Outcomes in Children With Prenatally Diagnosed Lung Malformations: Results From a Multi-institutional Research Collaborative by Kunisaki et al". Ann Surg. 2021 Dec 1;274(6):e891. doi: 10.1097/SLA.0000000000004898. PMID: 33856392.

  • Abstract

    No abstract available.

    Cite

    Kunisaki SM. Response to the Comment on "Fetal Risk Stratification and Outcomes in Children With Prenatally Diagnosed Lung Malformations: Results From a Multi-institutional Research Collaborative by Kunisaki et al". Ann Surg. 2021 Dec 1;274(6):e891. doi: 10.1097/SLA.0000000000004898. PMID: 33856392.

Development of a multi-institutional registry for children with operative congenital lung malformations.

Kunisaki SM, Saito JM, Fallat ME, St Peter SD, Lal DR, Johnson KN, Mon RA, Adams C, Aladegbami B, Bence C, Burns RC, Corkum KS, Deans KJ, Downard CD, Fraser JD, Gadepalli SK, Helmrath MA, Kabre R, Landman MP, Leys CM, Linden AF, Lopez JJ, Mak GZ, Minneci PC, Rademacher BL, Shaaban A, Walker SK, Wright TN, Hirschl RB; Midwest Pediatric Surgery Consortium. Development of a multi-institutional registry for children with operative congenital lung malformations. J Pediatr Surg. 2020 Jul;55(7):1313-1318. doi: 10.1016/j.jpedsurg.2019.01.058. Epub 2019 Feb 28. PMID: 30879756.

  • Abstract

    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.

    Level of evidence: Level II.

    Cite

    Kunisaki SM, Saito JM, Fallat ME, St Peter SD, Lal DR, Johnson KN, Mon RA, Adams C, Aladegbami B, Bence C, Burns RC, Corkum KS, Deans KJ, Downard CD, Fraser JD, Gadepalli SK, Helmrath MA, Kabre R, Landman MP, Leys CM, Linden AF, Lopez JJ, Mak GZ, Minneci PC, Rademacher BL, Shaaban A, Walker SK, Wright TN, Hirschl RB; Midwest Pediatric Surgery Consortium. Development of a multi-institutional registry for children with operative congenital lung malformations. J Pediatr Surg. 2020 Jul;55(7):1313-1318. doi: 10.1016/j.jpedsurg.2019.01.058. Epub 2019 Feb 28. PMID: 30879756.

Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium.

Kunisaki SM, Saito JM, Fallat ME, St Peter SD, Kim AG, Johnson KN, Mon RA, Adams C, Aladegbami B, Bence C, Burns RC, Corkum KS, Deans KJ, Downard CD, Fraser JD, Gadepalli SK, Helmrath MA, Kabre R, Lal DR, Landman MP, Leys CM, Linden AF, Lopez JJ, Mak GZ, Minneci PC, Rademacher BL, Shaaban A, Walker SK, Wright TN, Hirschl RB; Midwest Pediatric Surgery Consortium. Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium. J Pediatr Surg. 2019 Jun;54(6):1138-1142. doi: 10.1016/j.jpedsurg.2019.02.043. Epub 2019 Mar 1. PMID: 30898401.

  • Abstract

    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.

    Level of evidence: Level III.

    Cite

    Kunisaki SM, Saito JM, Fallat ME, St Peter SD, Kim AG, Johnson KN, Mon RA, Adams C, Aladegbami B, Bence C, Burns RC, Corkum KS, Deans KJ, Downard CD, Fraser JD, Gadepalli SK, Helmrath MA, Kabre R, Lal DR, Landman MP, Leys CM, Linden AF, Lopez JJ, Mak GZ, Minneci PC, Rademacher BL, Shaaban A, Walker SK, Wright TN, Hirschl RB; Midwest Pediatric Surgery Consortium. Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium. J Pediatr Surg. 2019 Jun;54(6):1138-1142. doi: 10.1016/j.jpedsurg.2019.02.043. Epub 2019 Mar 1. PMID: 30898401.

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

Engwall-Gill AJ, Chan SS, Boyd KP, Saito JM, Fallat ME, St Peter SD, Bolger-Theut S, Crotty EJ, Green JR, Hulett Bowling RL, Kumbhar SS, Rattan MS, Young CM, Canner JK, Deans KJ, Gadepalli SK, Helmrath MA, Hirschl RB, Kabre R, Lal DR, Landman MP, Leys CM, Mak GZ, Minneci PC, Wright TN, Kunisaki SM; Midwest Pediatric Surgery Consortium. Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children. JAMA Netw Open. 2022 Jun 1;5(6):e2219814. doi: 10.1001/jamanetworkopen.2022.19814. PMID: 35771571; PMCID: PMC9247735.

  • Abstract

    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.

    Cite

    Engwall-Gill AJ, Chan SS, Boyd KP, Saito JM, Fallat ME, St Peter SD, Bolger-Theut S, Crotty EJ, Green JR, Hulett Bowling RL, Kumbhar SS, Rattan MS, Young CM, Canner JK, Deans KJ, Gadepalli SK, Helmrath MA, Hirschl RB, Kabre R, Lal DR, Landman MP, Leys CM, Mak GZ, Minneci PC, Wright TN, Kunisaki SM; Midwest Pediatric Surgery Consortium. Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children. JAMA Netw Open. 2022 Jun 1;5(6):e2219814. doi: 10.1001/jamanetworkopen.2022.19814. PMID: 35771571; PMCID: PMC9247735.

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

Weller JH, Peter SDS, Fallat ME, Saito JM, Burns CR, Deans KJ, Fraser JD, Gadepalli SK, Helmrath MA, Hirschl RB, Kabre R, Lal DR, Landman MP, Leys CM, Mak GZ, Minneci PC, Wright TN, Kunisaki SM; Midwest Pediatric Surgery Consortium. Thoracoscopic versus open lobectomy in infants with congenital lung malformations: A multi-institutional propensity score analysis. J Pediatr Surg. 2021 Dec;56(12):2148-2156. doi: 10.1016/j.jpedsurg.2021.04.013. Epub 2021 Apr 25. PMID: 34030879.

  • Abstract

    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.

    Cite

    Weller JH, Peter SDS, Fallat ME, Saito JM, Burns CR, Deans KJ, Fraser JD, Gadepalli SK, Helmrath MA, Hirschl RB, Kabre R, Lal DR, Landman MP, Leys CM, Mak GZ, Minneci PC, Wright TN, Kunisaki SM; Midwest Pediatric Surgery Consortium. Thoracoscopic versus open lobectomy in infants with congenital lung malformations: A multi-institutional propensity score analysis. J Pediatr Surg. 2021 Dec;56(12):2148-2156. doi: 10.1016/j.jpedsurg.2021.04.013. Epub 2021 Apr 25. PMID: 34030879.

Thoracoscopy Versus Thoracotomy for Esophageal Atresia and Tracheoesophageal Fistula: Outcomes from the Midwest Pediatric Surgery Consortium

Marquart JP, Bowder AN, Bence CM, St Peter SD, Gadepalli SK, Sato TT, Szabo A, Minneci PC, Hirschl RB, Rymeski BA, Downard CD, Markel TA, Deans KJ, Fallat ME, Fraser JD, Grabowski JE, Helmrath MA, Kabre RD, Kohler JE, Landman MP, Lawrence AE, Leys CM, Mak GZ, Port E, Saito J, Silverberg J, Slidell MB, Wright TN, Lal DR; Midwest Pediatric Surgery Consortium. Thoracoscopy versus thoracotomy for esophageal atresia and tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium. J Pediatr Surg. 2023 Jan;58(1):27-33. doi: 10.1016/j.jpedsurg.2022.09.015. Epub 2022 Sep 24. PMID: 36283849.

  • Abstract

    Background/purpose: Controversy persists regarding the ideal surgical approach for repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). We examined complications and outcomes of infants undergoing thoracoscopy and thoracotomy for repair of Type C EA/TEF using propensity score-based overlap weights to minimize the effects of selection bias.

    Methods: Secondary analysis of two databases from multicenter retrospective and prospective studies examining outcomes of infants with proximal EA and distal TEF who underwent repair at 11 institutions was performed based on surgical approach. Regression analysis using propensity score-based overlap weights was utilized to evaluate outcomes of patients undergoing thoracotomy or thoracoscopy for Type C EA/TEF repair.

    Results: Of 504 patients included, 448 (89%) underwent thoracotomy and 56 (11%) thoracoscopy. Patients undergoing thoracoscopy were more likely to be full term (37.9 vs. 36.3 weeks estimated gestational age, p < 0.001), have a higher weight at operative repair (2.9 vs. 2.6 kg, p < 0.001), and less likely to have congenital heart disease (16% vs. 39%, p < 0.001). Postoperative stricture rate did not differ by approach, 29 (52%) thoracoscopy and 198 (44%) thoracotomy (p = 0.42). Similarly, there was no significant difference in time from surgery to stricture formation (p > 0.26). Regression analysis using propensity score-based overlap weighting found no significant difference in the odds of vocal cord paresis or paralysis (OR 1.087 p = 0.885), odds of anastomotic leak (OR 1.683 p = 0.123), the hazard of time to anastomotic stricture (HR 1.204 p = 0.378), or the number of dilations (IRR 1.182 p = 0.519) between thoracoscopy and thoracotomy.

    Conclusion: Infants undergoing thoracoscopic repair of Type C EA/TEF are more commonly full term, with higher weight at repair, and without congenital heart disease as compared to infants repaired via thoracotomy. Utilizing propensity score-based overlap weighting to minimize the effects of selection bias, we found no significant difference in complications based on surgical approach. However, our study may be underpowered to detect such outcome differences owing to the small number of infants undergoing thoracoscopic repair.

    Level of evidence: Level III.

    Cite

    Marquart JP, Bowder AN, Bence CM, St Peter SD, Gadepalli SK, Sato TT, Szabo A, Minneci PC, Hirschl RB, Rymeski BA, Downard CD, Markel TA, Deans KJ, Fallat ME, Fraser JD, Grabowski JE, Helmrath MA, Kabre RD, Kohler JE, Landman MP, Lawrence AE, Leys CM, Mak GZ, Port E, Saito J, Silverberg J, Slidell MB, Wright TN, Lal DR; Midwest Pediatric Surgery Consortium. Thoracoscopy versus thoracotomy for esophageal atresia and tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium. J Pediatr Surg. 2023 Jan;58(1):27-33. doi: 10.1016/j.jpedsurg.2022.09.015. Epub 2022 Sep 24. PMID: 36283849.

Pleuropulmonary Blastoma in Pediatric Lung Lesions

Kunisaki SM, Lal DR, Saito JM, Fallat ME, St Peter SD, Fox ZD, Heider A, Chan SS, Boyd KP, Burns RC, Deans KJ, Gadepalli SK, Hirschl RB, Kabre R, Landman MP, Leys CM, Mak GZ, Minneci PC, Wright TN, Helmrath MA; MIDWEST PEDIATRIC SURGERY CONSORTIUM. Pleuropulmonary Blastoma in Pediatric Lung Lesions. Pediatrics. 2021 Apr;147(4):e2020028357. doi: 10.1542/peds.2020-028357. Epub 2021 Mar 24. PMID: 33762310.

  • Abstract

    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 (P < .0001). Among 177 children without a history of prenatal detection, 15 (8.7%) were classified as having a malignant tumor (type 1 PPB, n = 11; other PPB, n = 3; adenocarcinoma, n = 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 (P = .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; P < .0001] and 42.03 [95% CI, 3.51-995.6; P = .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.

    Cite

    Kunisaki SM, Lal DR, Saito JM, Fallat ME, St Peter SD, Fox ZD, Heider A, Chan SS, Boyd KP, Burns RC, Deans KJ, Gadepalli SK, Hirschl RB, Kabre R, Landman MP, Leys CM, Mak GZ, Minneci PC, Wright TN, Helmrath MA; MIDWEST PEDIATRIC SURGERY CONSORTIUM. Pleuropulmonary Blastoma in Pediatric Lung Lesions. Pediatrics. 2021 Apr;147(4):e2020028357. doi: 10.1542/peds.2020-028357. Epub 2021 Mar 24. PMID: 33762310.