A National Experience in Paediatric Achalasia: Symptom Recurrence, Reintervention, and Transition Challenges

Abstract

Aims
To assess the long-term outcomes and quality of life (QOL) of patients who underwent intervention for esophageal achalasia in a national Paediatric centre over an 18-year period.

Methods
All patients who underwent surgical or radiological intervention after a diagnosis of achalasia between 2006 and 2024 were identified. A retrospective review of patient demographics, operative technique and Eckardt score before intervention was undertaken. Patients were contacted via telephone to re-assess QOL using Eckardt and Achalasia Scoring Questionnaire (ASQ) scoring systems.

Results
In total, 31 patients were identified and 28 were included in the analysis (15 female and 13 male). The majority 21(75%) underwent laparoscopic Heller Myotomy. The mean age at intervention was 11.6 years. The mean duration of follow-up was 7.6 years. The mean pre-operative Eckardt score was 6.9. The mean post-operative Eckardt score was 2.9. Twenty-one patients (75%) were initially symptom free for an average of 17.4 months and 7 (25%) remain symptom free to this day. Eleven (39%) required further interventions. In terms of quality of life, the current mean ASQ score is 19.4 (scoring system 10-31, with 10 representing symptom free). Of the patients who are now adults, 12 (57%) had a formal transition of care to adult services.

Conclusion
Although surgery may initially improve achalasia symptoms, it is not curative. Patients require lifelong follow-up and support to manage this challenging chronic condition. Ensuring patients are transitioned from Paediatric to adult services could help improve future QOL and tools such as ASQ could help monitor this in an outpatient setting.