BACKGROUND: Oesophageal achalasia is well-recognized but relatively rare in 
children, occasionally appearing as the "triple A" syndrome (with adrenal 
insufficiency and alacrima). Treatment modalities, as in adult practice, are not 
curative, often needing further interventions and spurring the search for better 
management. The outcome for syndromic variants is unknown. We sought to define 
the efficacy of treatments for children with achalasia with and without triple A 
syndrome.
METHODS: We conducted a retrospective analysis of presentation and outcomes for 
42 children with achalasia presenting over three decades to a major pediatric 
referral center. Long term impact of the diagnosis was assessed by 
questionnaire.
RESULTS: We identified 42 children including six with triple A syndrome. The 
median overall age at diagnosis was 10.8 years and median follow-up 1593 days. 
Initial Heller myotomy in 17 required further interventions in 11 (65%), while 
initial treatment with botulinum toxin (n = 20) was ultimately followed by 
myotomy in 17 (85%). Ten out of 35 patients who underwent myotomy required a 
repeat myotomy (29%). Patients with triple A syndrome developed symptoms 
earlier, but had delayed diagnosis, were more underweight at diagnosis and at 
last follow up. Questionnaire results suggested a significant long term 
deleterious impact on the quality of life of children and their families.
CONCLUSION: Many children with achalasia relapse after initial treatment, 
undergoing multiple, different procedures, despite which symptoms persist and 
impact on quality of life. Symptoms develop earlier in patients with triple A 
syndrome, but the diagnosis is delayed and this has substantial nutritional 
impact.