Robert Baird1, Dave R Lal2, Robert L Ricca3, Karen A Diefenbach4, Cynthia D Downard5, Julia Shelton6, Stig Sømme7, Julia Grabowski8, Tolulope A Oyetunji9, Regan F Williams10, Tim Jancelewicz10, Roshni Dasgupta11, L Grier Arthur12, Akemi L Kawaguchi13, Yigit S Guner14, Ankush Gosain15, Robert L Gates16, Juan E Sola17, Lorraine I Kelley-Quon18, Shawn D St Peter19, Adam Goldin20. 1. Department of Pediatric General and Thoracic Surgery, BC Children's Hospital, University of British Columbia, 4480 Oak, Vancouver V6H3V4, British Columbia. Electronic address: robert.baird@cw.bc.ca. 2. Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin. 3. Division of Pediatric Surgery, Naval Medical Center, Portsmouth, Virginia. 4. Department of Pediatric Surgery, Nationwide Children's Hospital, The Ohio State University, Columbus, OH. 5. Hiram C. Polk Jr, MD Department of Surgery, University of Louisville, Louisville, KY. 6. University of Iowa Stead Family Children's Hospital, Iowa City, IA. 7. Division of Pediatric Surgery, Children's Hospital Colorado, University of Colorado, Aurora, CO. 8. Division of Pediatric Surgery, Department of Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL. 9. Department of Surgery, Children's Mercy Kansas City, University of Missouri-Kansas City School of Medicine, Kansas City, MO. 10. Division of Pediatric Surgery, Le Bonheur Children's Hospital, Memphis, TN. 11. Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH. 12. Division of General, Thoracic, and Minimally Invasive Surgery, St. Christopher's Hospital for Children, Drexel University, Philadelphia, PA. 13. Department of Pediatric Surgery, University of Texas Health Science Center at Houston, Houston, TX. 14. Department of Surgery University of California Irvine and Division of Pediatric Surgery Children's Hospital of Orange County. 15. Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Children's Foundation Research Institute, Le Bonheur Children's Hospital, Memphis, TN. 16. Clinical University of South Carolina-Greenville, Division of Pediatric Surgery, Greenville, SC. 17. Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL. 18. Division of Pediatric Surgery, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA. 19. Children's Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64108. 20. Department of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Seattle, WA.
Abstract
BACKGROUND: Treatment of the neonate with long gap esophageal atresia (LGEA) is one of the most challenging scenarios facing pediatric surgeons today. Contributing to this challenge is the variability in case definition, multiple approaches to management, and heterogeneity of the reported outcomes. This necessitates a clear summary of existing evidence and delineation of treatment controversies. METHODS: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted four consensus-based questions regarding LGEA. These questions concerned the definition and determination of LGEA, the optimal method of surgical management, expected long-term outcomes, and novel therapeutic techniques. A comprehensive search strategy was crafted and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were utilized to identify, review and report salient articles. RESULTS: More than 3000 publications were reviewed, with 178 influencing final recommendations. In total, 18 recommendations are provided, primarily based on level 4-5 evidence. These recommendations provide detailed descriptions of the definition of LGEA, treatment techniques, outcomes and future directions of research. CONCLUSIONS: Evidence supporting best practices for LGEA is currently low quality. This review provides best recommendations based on a critical evaluation of the available literature. Based on the lack of strong evidence, prospective and comparative research is clearly needed. TYPE OF STUDY: Treatment study, prognosis study and study of diagnostic test. LEVEL OF EVIDENCE: Level II-V.
BACKGROUND: Treatment of the neonate with long gap esophageal atresia (LGEA) is one of the most challenging scenarios facing pediatric surgeons today. Contributing to this challenge is the variability in case definition, multiple approaches to management, and heterogeneity of the reported outcomes. This necessitates a clear summary of existing evidence and delineation of treatment controversies. METHODS: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted four consensus-based questions regarding LGEA. These questions concerned the definition and determination of LGEA, the optimal method of surgical management, expected long-term outcomes, and novel therapeutic techniques. A comprehensive search strategy was crafted and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were utilized to identify, review and report salient articles. RESULTS: More than 3000 publications were reviewed, with 178 influencing final recommendations. In total, 18 recommendations are provided, primarily based on level 4-5 evidence. These recommendations provide detailed descriptions of the definition of LGEA, treatment techniques, outcomes and future directions of research. CONCLUSIONS: Evidence supporting best practices for LGEA is currently low quality. This review provides best recommendations based on a critical evaluation of the available literature. Based on the lack of strong evidence, prospective and comparative research is clearly needed. TYPE OF STUDY: Treatment study, prognosis study and study of diagnostic test. LEVEL OF EVIDENCE: Level II-V.