Dave R Lal1, Samir K Gadepalli2, Cynthia D Downard3, Daniel J Ostlie4, Peter C Minneci5, Ruth M Swedler6, Thomas Chelius7, Laura Cassidy8, Cooper T Rapp6, Katherine J Deans5, Mary E Fallat3, S Maria E Finnell9, Michael A Helmrath10, Ronald B Hirschl2, Rashmi S Kabre11, Charles M Leys4, Grace Mak12, Jessica Raque3, Frederick J Rescorla9, Jacqueline M Saito13, Shawn D St Peter14, Daniel von Allmen10, Brad W Warner13, Thomas T Sato6. 1. Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, United States. Electronic address: dlal@chw.org. 2. Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, United States. 3. Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Louisville, KY, United States. 4. Division of Pediatric Surgery, Department of Surgery, University of Wisconsin, Madison, WI, United States. 5. Center for Surgical Outcomes Research, The Research Institute at Nationwide Children's Hospital, Department of Surgery, University of Ohio, Columbus, OH, United States. 6. Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, United States. 7. Division of Epidemiology, Institute for Health and Society, Medical College of Wisconsin, Milwaukee, WI, United States. 8. Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, United States; Division of Epidemiology, Institute for Health and Society, Medical College of Wisconsin, Milwaukee, WI, United States. 9. Division of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, United States. 10. Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States. 11. Division of Pediatric Surgery, Department of Surgery, Feinberg School of Medicine of Northwestern University, Chicago, IL, United States. 12. Section of Pediatric Surgery, Department of Surgery, The University of Chicago Medicine and Biologic Sciences, Chicago, IL, United States. 13. Division of Pediatric Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, MO, United States. 14. Department of Surgery, Children's Mercy Hospital, Kansas City, MO, United States.
Abstract
BACKGROUND/ PURPOSE: Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care. METHODS: A multi-institutional retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals between 2009 and 2014 was performed. Over the 5year period, 396 cases were identified in the 11 centers (7±5 per center per year). All infants with a diagnosis of EA/TEF made within 30days of life who had surgical repair of their defect defined as esophageal reconstruction with or without ligation of TEF within the first six months of life were included. Demographic, operative, and outcome data were collected and analyzed to detect associations between variables. RESULTS: Prenatal suspicion or diagnosis of EA/TEF was present in 53 (13%). The most common anatomy was proximal EA with distal TEF (n=335; 85%) followed by pure EA (n=27; 7%). Clinically significant congenital heart disease (CHD) was present in 137 (35%). Mortality was 7.5% and significantly associated with CHD (p<0.0001). Postoperative morbidity occurred in 62% of the population, including 165 (42%) cases with anastomotic stricture requiring intervention, anastomotic leak in 89 (23%), vocal cord paresis/paralysis in 26 (7%), recurrent fistula in 19 (5%), and anastomotic dehiscence in 9 (2%). Substantial variation in practice across our institutions existed: bronchoscopy prior to repair was performed in 64% of cases (range: 0%-100%); proximal pouch contrast study in 21% (0%-69%); use of interposing material between the esophageal and tracheal suture lines in 38% (0%-69%); perioperative antibiotics ≥24h in 69% (36%-97%); and transanastomotic tubes in 73% (21%-100%). CONCLUSION: Contemporary treatment of EA/TEF is characterized by substantial variation in perioperative management and considerable postoperative morbidity and mortality. Future studies are planned to establish best practices and clinical care guidelines for infants with EA/TEF. LEVEL OF EVIDENCE: Type of study: Treatment study. Level IV.
BACKGROUND/ PURPOSE:Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care. METHODS: A multi-institutional retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals between 2009 and 2014 was performed. Over the 5year period, 396 cases were identified in the 11 centers (7±5 per center per year). All infants with a diagnosis of EA/TEF made within 30days of life who had surgical repair of their defect defined as esophageal reconstruction with or without ligation of TEF within the first six months of life were included. Demographic, operative, and outcome data were collected and analyzed to detect associations between variables. RESULTS: Prenatal suspicion or diagnosis of EA/TEF was present in 53 (13%). The most common anatomy was proximal EA with distal TEF (n=335; 85%) followed by pure EA (n=27; 7%). Clinically significant congenital heart disease (CHD) was present in 137 (35%). Mortality was 7.5% and significantly associated with CHD (p<0.0001). Postoperative morbidity occurred in 62% of the population, including 165 (42%) cases with anastomotic stricture requiring intervention, anastomotic leak in 89 (23%), vocal cord paresis/paralysis in 26 (7%), recurrent fistula in 19 (5%), and anastomotic dehiscence in 9 (2%). Substantial variation in practice across our institutions existed: bronchoscopy prior to repair was performed in 64% of cases (range: 0%-100%); proximal pouch contrast study in 21% (0%-69%); use of interposing material between the esophageal and tracheal suture lines in 38% (0%-69%); perioperative antibiotics ≥24h in 69% (36%-97%); and transanastomotic tubes in 73% (21%-100%). CONCLUSION: Contemporary treatment of EA/TEF is characterized by substantial variation in perioperative management and considerable postoperative morbidity and mortality. Future studies are planned to establish best practices and clinical care guidelines for infants with EA/TEF. LEVEL OF EVIDENCE: Type of study: Treatment study. Level IV.
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Authors: Muhammad Khalid Syed; Ahmad A Al Faqeeh; Alsayed Othman; Talal Almas; Tarek Khedro; Reema Alsufyani; Dana Almubarak; Rehab Al Faqeh; Saifullah Syed; Sabahat K Syed Journal: Cureus Date: 2020-12-04