Marinde van Lennep1, Michiel P van Wijk1,2, Taher I M Omari3,4, Silvia Salvatore5, Marc A Benninga1, Maartje M J Singendonk1. 1. Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Pediatric Gastroenterology. 2. Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Pediatric Gastroenterology, Amsterdam, The Netherlands. 3. Center for Neuroscience. 4. College of Medicine and Public Health, Flinders University, Adelaide, Australia. 5. Pediatric Department, Università dell'Insubria, Varese, Italy.
Abstract
OBJECTIVES: Pediatric achalasia is a rare neurodegenerative disorder of the esophagus that requires treatment. Different diagnostic and treatment modalities are available, but there are no data that show how children can best be diagnosed and treated. We aimed to identify current practices regarding the diagnostic and therapeutic approach toward children with achalasia. METHODS: Information on the current practice regarding the management of pediatric achalasia was collected by an online-based survey sent to members of the European and North American Societies for Pediatric Gastroenterology Hepatology and Nutrition involved in pediatric achalasia care. RESULTS: The survey was completed by 38 centers from 24 countries. Within these centers, 108 children were diagnosed with achalasia in the last year (median 2, range 0-15). Achalasia was primarily managed by a pediatric gastroenterologist (76%) and involved a multidisciplinary team in 84% of centers, also including a surgeon (87%), radiologist (61%), dietician (37%), speech pathologist (8%), and psychologist (5%). Medical history taking and physical examination were considered most important to establish the diagnosis (50%), followed by (a combination of) manometry (45%) or contrast swallow (21%). Treatment of first choice was Heller myotomy (58%), followed by pneumatic dilation (46%) and peroral endoscopic myotomy (29%). CONCLUSION: This study shows a great heterogeneity in the management of pediatric achalasia amongst different centers worldwide. These findings stress the need for well-designed intervention trials in children with achalasia. Given the rarity of this disease, we recommend that achalasia care should be managed in centers with access to appropriate diagnostic and treatment modalities.
OBJECTIVES:Pediatric achalasia is a rare neurodegenerative disorder of the esophagus that requires treatment. Different diagnostic and treatment modalities are available, but there are no data that show how children can best be diagnosed and treated. We aimed to identify current practices regarding the diagnostic and therapeutic approach toward children with achalasia. METHODS: Information on the current practice regarding the management of pediatric achalasia was collected by an online-based survey sent to members of the European and North American Societies for Pediatric Gastroenterology Hepatology and Nutrition involved in pediatric achalasia care. RESULTS: The survey was completed by 38 centers from 24 countries. Within these centers, 108 children were diagnosed with achalasia in the last year (median 2, range 0-15). Achalasia was primarily managed by a pediatric gastroenterologist (76%) and involved a multidisciplinary team in 84% of centers, also including a surgeon (87%), radiologist (61%), dietician (37%), speech pathologist (8%), and psychologist (5%). Medical history taking and physical examination were considered most important to establish the diagnosis (50%), followed by (a combination of) manometry (45%) or contrast swallow (21%). Treatment of first choice was Heller myotomy (58%), followed by pneumatic dilation (46%) and peroral endoscopic myotomy (29%). CONCLUSION: This study shows a great heterogeneity in the management of pediatric achalasia amongst different centers worldwide. These findings stress the need for well-designed intervention trials in children with achalasia. Given the rarity of this disease, we recommend that achalasia care should be managed in centers with access to appropriate diagnostic and treatment modalities.
Authors: Andreas Meinzer; Ibrahim Alkatout; Thomas Franz Krebs; Jonas Baastrup; Katja Reischig; Roberts Meiksans; Robert Bergholz Journal: J Clin Med Date: 2020-12-10 Impact factor: 4.241
Authors: Irina Geiculescu; Jason Dranove; Graham Cosper; Andrew C Edmondson; Eva Morava-Kozicz; Lauren B Carter Journal: Am J Med Genet A Date: 2022-06-04 Impact factor: 2.578