Surgical Management of Esophageal Achalasia in Pediatrics: A Systematic Review

Abstract

Introduction There are no evidence-based guidelines on the surgical management of esophageal achalasia (OA) in children. This can be a challenging condition with significant physical and psychological morbidity. Our aim was to identify the most common management modalities and their outcomes. Materials and Methods A systematic review was performed through a literature search of health care databases in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, aiming at identifying pediatric series discussing the diagnosis and management of OA. Duplicates, case series with < 9 patients, and follow-up of < 1 year were excluded. The included papers were analyzed for diagnostic methods, primary treatment method, complications, follow-up duration, outcome measures recorded, and outcome. Results Data from 33 papers for 742 children treated for OA was analyzed. Eleven mentioned multiple management modalities. In summary, 25 described Heller's esophagomyotomy (HM), 13 esophageal dilatation (EOD), and 6 peroral esophageal myotomy (POEM). Mean follow-up was 43.7 months (12–180). Outcome measures were heterogeneous. However, analysis of reported success showed a mean success of 78% for HM (p = 1.79 × 10–7), 44.9% for EOD (p = 0.24), and 99.3% for POEM (p = 0.001). Reported complications were 12.8% for HM, 5% for EOD, and 24.4% for POEM. Further interventions were required for 10.9% of HM, 62.3% of EOD, and 0.01% of POEM patient groups. Conclusion Methods of diagnosis and measures of successful outcomes were heterogeneous, limiting the strength of evidence. HM showed superior short-term success rates to EOD. POEM is a promising modality but requires investment in equipment and training. Information about sustainability of response and long-term outcomes is lacking.

Current management of pediatric achalasia

“…After demonstrating its minimally invasive benefit for adult patients, the POEM procedure has become an important treatment for achalasia in children (30). Overall, the clinical success and safety profile appears to be similar to that described in adults (16,31). Major steps of the POEM are depicted in Figure 4.…”

“…The scoring system ranges from 0 to 12, attributing points to four symptoms: dysphagia, regurgitation, chest pain, and weight loss. Comparisons of post-procedural Eckardt scores have been used to evaluate differences between treatment modalities (15,16). A normal Eckardt score is considered to be £3.…”

“…asymptomatic patients cannot be detected (2). Subclinical symptoms may be treated unsuccessfully for years; by the time achalasia is definitively diagnosed, most patients require an intervention -all of which have an observed recurrence rate (16). Nevertheless, the need for large, prospective trials involving children with achalasia is clear.…”

Achalasia

“…Endoscopic balloon dilatation has been reported to be effective in up to 90% of cases. Most authors, including ourselves, have been unimpressed with its efficacy as a primary treatment in children with achalasia (Goneidy et al 2019). Multiple dilatations are often required with 20-75% of patients needing repeated dilatations.…”

A Case of Peroral Endoscopic Myotomy for Paediatric Oesophageal Achalasia

“…По эффективности POEM сопоставима с лапароскопической операцией Геллера. При этом она менее инвазивна, сохраняет связочный аппарат пищевода и минимизирует риск его перфорации [6,13,14].…”