Aim To determine the incidence and examine the epidemiology of achalasia before the age of 16 years in the UK from 1998 to 2008.

Methods 25 regional paediatric surgery referral centres were asked to provide demographic and epidemiological data on cases of childhood achalasia from 1998 to 2008. Incidence rates were calculated from national population estimates. The data collection method was validated in one centre.

Results 228 patients from 24 centres were diagnosed with achalasia before 16 years in the UK from 1998 to 2008. The mean incidence from 1998 to 2008 was 0.18/10⁵ children/year. Where additional data was provided (69–81% of cases) 56% of children were male and the mean age of diagnosis was 10.9 years. Logistic regression analysis showed a rising incidence, with an OR of 1.12 (95% CI 1.06 to 1.16) for having achalasia in each successive year. The validation of this methodology showed that 95% of true cases and no false cases were identified.

Conclusions The mean incidence of childhood achalasia in the UK from 1998 to 2008 is at least 0.18/10⁵ children/year; this has risen over the last 11 years and compared to the only other study published in 1988.