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Clinical Management of Pediatric Achalasia: A Survey of Current Practice
Marinde van Lennep1, Michiel P van Wijk1,2, Taher I M Omari3,4
1Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Pediatric Gastroenterology.
Insights
Pediatric achalasia management varies significantly worldwide. This study highlights the need for standardized diagnostic and treatment protocols for children with this rare esophageal disorder.
Area of Science:
- Pediatric Gastroenterology
- Neurodegenerative Esophageal Disorders
- Clinical Practice Management
Background:
- Pediatric achalasia is a rare neurodegenerative esophageal disorder requiring effective treatment.
- Current diagnostic and therapeutic strategies for pediatric achalasia lack comprehensive data for optimal care.
- Understanding current management practices is crucial for improving patient outcomes.
Purpose of the Study:
- To identify and analyze current diagnostic and therapeutic practices for pediatric achalasia.
- To assess the heterogeneity in the management of pediatric achalasia across different healthcare centers.
- To establish a baseline for future intervention trials in pediatric achalasia care.
Main Methods:
- An online survey was distributed to members of the European and North American Societies for Pediatric Gastroenterology Hepatology and Nutrition.
- The survey collected data on diagnostic and treatment approaches for pediatric achalasia from 38 centers in 24 countries.
- Data included diagnostic methods, multidisciplinary team involvement, and primary treatment modalities.
Main Results:
- A total of 108 children were diagnosed with achalasia in the surveyed centers within the last year.
- Pediatric gastroenterologists managed most cases, with multidisciplinary teams involved in 84% of centers.
- Heller myotomy was the most frequent first-choice treatment (58%), followed by pneumatic dilation (46%) and peroral endoscopic myotomy (29%).
Conclusions:
- Significant heterogeneity exists in the global management of pediatric achalasia.
- The findings underscore the urgent need for well-designed clinical trials to establish evidence-based guidelines.
- Centralized care in specialized centers with advanced diagnostic and treatment capabilities is recommended for pediatric achalasia.
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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