Peroral endoscopic myotomy provides effective and sustained relief for achalasia in Allgrove syndrome: a long-term comparative cohort study
A minimally invasive procedure provides long-lasting relief for achalasia in Allgrove syndrome, offering the best-known treatment for this ultra-rare disorder.
The longest POEM follow-up data for Allgrove syndrome (ultra-rare autosomal recessive disorder, AAA triad) demonstrates durable efficacy (median 5.8 years, Eckardt 5.3→0.8) with outcomes comparable to idiopathic achalasia, while characterizing the distinct earlier onset and lower symptom perception in Allgrove patients. POEM should be considered the treatment of choice for achalasia in Allgrove syndrome based on this 16-year institutional series at PUMCH (China's national rare disease center).
What the study was
- Study design
- Long-term retrospective cohort with matched comparison (Allgrove vs idiopathic achalasia, POEM)
- Population
- Allgrove syndrome patients (n=7) with POEM vs matched idiopathic achalasia (n=12), 16-year institutional series
- Sample size
- 19
- Category
- Treatment Innovation
- Maturity
- Validated
- Journal
- Frontiers in Medicine
Why it surfaced
Longest POEM follow-up in Allgrove syndrome from a dedicated rare disease center; provides treatment evidence for this ultra-rare condition despite small N (n=7).
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