Clinical Management of Pediatric Achalasia: A Survey of Current Practice

European Society for Paediatric Gastroenterology, Hepatology and Nutrition Motility Working Group, Marinde van Lennep, Michiel P van Wijk, Taher I M Omari, Silvia Salvatore, Marc A Benninga, Maartje M J Singendonk

Research output: Contribution to journalArticleAcademicpeer-review

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.

LanguageEnglish
JournalJournal of Pediatric Gastroenterology and Nutrition
DOIs
Publication statusE-pub ahead of print - 11 Dec 2018

Cite this

European Society for Paediatric Gastroenterology, Hepatology and Nutrition Motility Working Group. / Clinical Management of Pediatric Achalasia : A Survey of Current Practice. In: Journal of Pediatric Gastroenterology and Nutrition. 2018.
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title = "Clinical Management of Pediatric Achalasia: A Survey of Current Practice",
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.",
author = "{European Society for Paediatric Gastroenterology, Hepatology and Nutrition Motility Working Group} and {van Lennep}, Marinde and {van Wijk}, {Michiel P} and Omari, {Taher I M} and Silvia Salvatore and Benninga, {Marc A} and Singendonk, {Maartje M J}",
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Clinical Management of Pediatric Achalasia : A Survey of Current Practice. / European Society for Paediatric Gastroenterology, Hepatology and Nutrition Motility Working Group.

In: Journal of Pediatric Gastroenterology and Nutrition, 11.12.2018.

Research output: Contribution to journalArticleAcademicpeer-review

TY - JOUR

T1 - Clinical Management of Pediatric Achalasia

T2 - Journal of Pediatric Gastroenterology and Nutrition

AU - European Society for Paediatric Gastroenterology, Hepatology and Nutrition Motility Working Group

AU - van Lennep, Marinde

AU - van Wijk, Michiel P

AU - Omari, Taher I M

AU - Salvatore, Silvia

AU - Benninga, Marc A

AU - Singendonk, Maartje M J

PY - 2018/12/11

Y1 - 2018/12/11

N2 - 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.

AB - 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.

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DO - 10.1097/MPG.0000000000002221

M3 - Article

JO - Journal of Pediatric Gastroenterology and Nutrition

JF - Journal of Pediatric Gastroenterology and Nutrition

SN - 0277-2116

ER -

European Society for Paediatric Gastroenterology, Hepatology and Nutrition Motility Working Group. Clinical Management of Pediatric Achalasia: A Survey of Current Practice. Journal of Pediatric Gastroenterology and Nutrition. 2018 Dec 11. https://doi.org/10.1097/MPG.0000000000002221