Contemporary Management of Achalasia by Pediatric Surgeons: A Survey of the International Pediatric Endosurgical Group

Introduction: Achalasia is a rare neurodegenerative disorder of the esophagus. Surgical repair consists of esophagomyotomy, often in conjunction with an antireflux procedure. We sought to determine practice patterns in surgical treatment of pediatric achalasia. Methods: Data regarding preferences were collected as part of a comprehensive online-based survey sent to members of the International Pediatric Endosurgery Group (IPEG) completed by 191 surgeons of which 141 performed esophagomyotomies for achalasia. Results: Procedures performed per surgeon were 1-2 (n = 21, 15%); 3-5 (n = 49, 34%); 6-10 (n = 39, 28%); 11-20 (n = 21, 15%); >20 (n = 11, 8%). Most approached the operation laparoscopically (n = 127, 90%). Workup before esophageal myotomy consisted of a diagnostic esophagram (n = 133, 94%) or manometry (n = 102, 73%). Only 60% of surgeons (n = 84) required an EGD. No preference observed in division location of the phrenoesophageal ligament for mobilization of the esophagus. There was a predominant preference for hook cautery (n = 82, 58%) over harmonic shears (n = 30, 21%), heated sealing device LigaSure (TM) (n = 18, 13%), and other devices (n = 11, 8%) for muscle division. Intraoperatively, 57% (n = 80) had endoscopy and 50% (n = 71) had postoperative esophagram before initiation of enteral feeding. For antireflux procedure, Thal/Dor approach was performed most frequently (n = 111, 79%) followed by the Toupet (n = 18, 13%) and Nissen (n = 4, 3%) and none (n = 7, 5%). Diet restrictions were provided in 76% (n = 107) of postoperative patients. Conclusion: Given the infrequency of achalasia in children, there are a range of treatment plans among pediatric surgeons. We have identified current practices as a first step in developing more standard treatment pathways.