Clinical Profile, Surgical Outcomes, and Long-term Follow-up in Children with Esophageal Achalasia: A Retrospective Study of 22 Cases

Abstract

Background: Oesophageal achalasia (EA) is a rare primary motility disorder of the oesophagus characterised by absent oesophageal peristalsis and incomplete relaxation of the lower oesophageal sphincter (LES). In children, diagnosis is frequently delayed because of an atypical and polymorphic clinical presentation. Objectives: To describe the epidemiological, clinical and paraclinical profiles of children operated on for oesophageal achalasia in our department, analyse the outcomes of surgical management, and evaluate long-term outcomes. Materials and Methods: This was a single-centre retrospective study of 22 children who underwent surgery for oesophageal achalasia at the Department of Paediatric Surgery, University Hospital Centre Hassan II, Fez, between January 2009 and December 2023. Of these patients, 18 were included in the actuarial follow-up analysis (median follow-up: 6.7 years; range: 21 days-15 years). All patients underwent Heller seromyotomy combined with anterior Dor-type fundoplication: 11 by laparotomy and 11 by laparoscopy. Results: The median age at surgery was 6.5 years, with a male predominance (sex ratio: 1.4). Six patients (27%) had Allgrove syndrome. Chronic vomiting was the dominant symptom (95%). Oesophageal dilation was confirmed by upper gastrointestinal endoscopy in 95% of patients; EGDT showed the characteristic bird-beak appearance in 64% of cases. All patients underwent Heller seromyotomy with Dor fundoplication. Actuarial analysis showed asymptomatic rates of 76% at 1 year, 58% at 3 years and 52% at 5 years. Two patients required surgical revision, and both achieved complete remission. Conclusion: Heller seromyotomy combined with Dor fundoplication remains the standard surgical treatment for oesophageal achalasia in children. Long-term outcomes support the need for prolonged and structured postoperative surveillance.