Akemi L Kawaguchi1, Yigit S Guner2, Stig Sømme3, Alexandria C Quesenberry4, L Grier Arthur5, Juan E Sola6, Cynthia D Downard7, Rebecca M Rentea8, Patricia A Valusek9, Caitlin A Smith10, Mark B Slidell11, Robert L Ricca12, Roshni Dasgupta13, Elizabeth Renaud14, Doug Miniati15, Jarod McAteer16, Alana L Beres17, Julia Grabowski18, Shawn D St Peter19, Ankush Gosain20. 1. Department of Pediatric Surgery, University of Texas Health Science Center at Houston, Houston, TX, USA. Electronic address: akemi.kawaguchi@uth.tmc.edu. 2. Department of Surgery University of California Irvine and Division of Pediatric Surgery Children's Hospital of Orange County, USA. 3. Division of Pediatric Surgery, Children's Hospital Colorado, University of Colorado, Aurora, CO, USA. 4. Health Sciences Library, University of Tennessee Health Science Center, USA. 5. Division of Pediatric General, Thoracic, and Minimally Invasive Surgery, St. Christopher's Hospital for Children, Philadelphia, PA, USA. 6. Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA. 7. Division of Pediatric Surgery, Hiram C. Polk, Jr, MD Department of Surgery, University of Louisville, Louisville, KY, USA. 8. Department of Pediatric Surgery, Children's Mercy-Kansas City, Kansas City, MO, USA. 9. Pediatric Surgical Associates, Children's Minnesota, Minneapolis, MN, USA. 10. Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA. 11. Section of Pediatric Surgery, Comer Children's Hospital, The University of Chicago Medicine, Chicago, Illinois, USA. 12. Division of Pediatric Surgery, Naval Medical Center, Portsmouth, VA, USA. 13. Division of Pediatric General and Thoracic Surgery, Cincinnati Childrens Medical Center, University of Cincinnati, Cincinnati OH, USA. 14. Division of Pediatric Surgery, Alpert Medical School of Brown University, Hasbro Children's Hospital, Rhode Island Hospital, Providence, RI, USA. 15. Division of Pediatric Surgery, Kaiser Permanente Roseville Women and Children's Center, Roseville, California, USA. 16. Alaska Pediatric Surgery, Anchorage, AK, USA. 17. Division of Pediatric General, Thoracic and Fetal Surgery, University of California, Davis, Sacramento CA, USA. 18. Division of Pediatric Surgery, Ann and Robert H. Lurie Children's Hospital, Northwestern University, Chicago, IL long, USA. 19. Department of Surgery, Children's Mercy Hospital, Kansas City, MO, USA. 20. 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, USA. Electronic address: agosain@uthsc.edu.
Abstract
OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD. METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed. RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder. CONCLUSIONS: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.
OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD. METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed. RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder. CONCLUSIONS: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.
Authors: Ruth A Lewit; Laura V Veras; Robert A Cowles; Kathryn Fowler; Sebastian King; Eveline Lapidus-Krol; Jacob C Langer; Christine J Park; Fouad Youssef; Sergey Vavilov; Ankush Gosain Journal: J Surg Res Date: 2021-01-15 Impact factor: 2.192
Authors: Wendy Yang; Jenny Pham; Sebastian K King; Donald F Newgreen; Heather M Young; Lincon A Stamp; Marlene M Hao Journal: Biomolecules Date: 2022-08-10