Clinical Management of Pediatric Achalasia: A Survey of Current Practice

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

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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.

Original languageEnglish
Pages (from-to)521-526
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume68
Issue number4
Early online date11 Dec 2018
DOIs
Publication statusPublished - 1 Apr 2019

Cite this

van Lennep, M., van Wijk, M. P., Omari, T. I. M., Salvatore, S., Benninga, M. A., Singendonk, M. M. J., & European Society for Paediatric Gastroenterology, Hepatology and Nutrition Motility Working Group (2019). Clinical Management of Pediatric Achalasia: A Survey of Current Practice. Journal of Pediatric Gastroenterology and Nutrition, 68(4), 521-526. https://doi.org/10.1097/MPG.0000000000002221