Achalasia is a rare motility disorder of the oesophagus, with an incidence rate of 0.38/100,000 children per year in the UK.¹ The characteristics of achalasia are a non-relaxing lower oesophageal sphincter (LOS) and failure of peristalsis. High resolution oesophageal manometry (HROM) is the gold standard for diagnosis and allows achalasia to be divided into three sub-types dependant on the nature of the peristaltic failure. There are a variety of treatment options available for achalasia, including medications, pneumatic dilatation, injection of botulinum toxin and myotomy - all focused on reducing the pressure at the LOS. There is no standard consensus of evidence based for the optimum treatment modality in paediatric achalasia.

Pneumatic dilatations have high success rate on Type I (absent peristalsis) and Type II (pan-oesophageal pressurisations) achalasia.² In our institution, children with achalasia (type I&II) will undergo a series of 3 dilatations (rigid balloons), 4 weeks apart by the interventional radiologist and a regular clinical assessment. A repeat HROM will be considered after the last dilatation to assess the response to the intervention.

We present a series of three patients (2 type I and 1 type II, mean age 9.5 years) who underwent HROM pre and post dilatation. Following dilatation, both Type I achalasia patients reported symptomatic relief (reduction in Eckardt Score) and showed significant reduction in both wet and solid IRP values. The patient with Type II achalasia reported only mild symptomatic relief for a short period and showed progression of their condition on repeat HROM.

It is not uncommon for patients with achalasia to report symptoms post interventions (dilatations or myotomies), so it is salient to ensure the appropriate assessment is undertaken prior to escalation or change of therapy.

In conclusion, we report a small case series of HROM findings in children with achalasia before and after pneumatic dilatation enabling tailor made management intervention.

References

1. Dokal K, Mutalib M. An updated incidence of paediatric achalasia and number of myotomies performed in the United Kingdom. GastroHep. 2021;3:420– 425. doi:10.1002/ygh2.493

2. Oude Nijhuis RAB, Prins LI, et al. Factors associated with achalasia treatment outcomes: systematic review and meta-analysis. Clinical Gastroenterology and Hepatology 18(Issue 7):1442–1453. https://doi.org/10.1016/j.cgh.2019.10.008