No size fits all: a systematic review of quality of life outcomes in children and adults with achalasia

Background Oesophageal achalasia (OA) is a rare gastrointestinal motility disorder causing significant impact on health-related quality of life (HRQoL). Treatment focuses on symptom amelioration through interventions including laparoscopic myotomy and peroral endoscopic myotomy. We conducted a systematic review to identify the tools used to assess HRQoL in adult and paediatric patients with OA undergoing intervention, and to characterise the extent of heterogeneity in their application and reporting. Study Ovid was used to search multiple digital databases: Journals@Ovid Full Text Subset, Journals@Ovid, Ovid Medline, Embase, Ovid Emcare, HMIC Health Management Information Consortium. Initial search produced 1746 titles with 46 articles remaining after full text review. Results 43 of the 46 papers included adult cohorts with an average of 54 patients per study. Three included paediatric cohorts. 15 different tools were used to evaluate QoL with sparse use of disease-specific assessment tools suggesting these tools are not widely known or fit for purpose. Conclusions This study highlights significant heterogeneity in methods, variations in QoL tools and selective reporting of HRQoL measures. Though these tools and approaches may confer individual benefit, this heterogeneity renders direct comparison or pooling of data difficult. Additionally, many studies did not report HRQoL outcomes for preintervention and postintervention, thus impeding evaluation of treatment effectiveness across existing studies. Future direction should focus on achieving consensus around a standardised approach to QoL assessment in achalasia, which may involve the adoption, refinement or validation of an existing disease-specific tool rather than development of a new one.