Abstract
Introduction:
Achalasia among children often fails endoscopic management (e.g., dilation, botulinum toxin). Laparoscopic esophagocardiomyotomy (L-ECM) is a standard intervention to relieve obstruction but can induce gastroesophageal reflux (GER). Concurrent anterior fundoplication (A-fundo) has been evaluated in randomized trials among adults, demonstrating mixed results on controlling postoperative GER without exacerbating dysphagia. Furthermore, evidence for the best approach among children remains sparse. We hypothesized that, among children undergoing L-ECM without mucosal violation, routine A-fundo would not improve postoperative GER control while exacerbating dysphagia.
Materials and Methods:
Observational data of 47 consecutive achalasia patients ≤18 years who received L-ECM (2002–2023) at a single academic institution were collected. Patient records were culled for demographics, achalasia characteristics, and outcomes. Two L-ECM groups were identified: with or without A-fundo. Patients were screened for postoperative dysphagia (additional procedures) and GER (new antireflux medications). Univariate independence testing was conducted to identify statistically significant variables.
Results:
Among 47 patients undergoing L-ECM, 28 (59.6%) received concurrent A-fundo. Compared with patients undergoing L-ECM alone, patients with L-ECM/A-fundo had significantly longer hospital stays (P < .01) without statistically different rates of postoperative dysphagia (P = .81) or GER (P = .51). Five children (10.6%) experienced mucosal injury with L-ECM: 4 recognized intraoperatively received A-Fundo without subsequent leak; 1 mucosal injury was missed and did not receive A-Fundo, which subsequently leaked.
Conclusion:
In this largest observation of pediatric achalasia patients, A-fundo appeared clinically insignificant when determining contributors to control GER or exacerbate postoperative dysphagia. A-fundo should not be routinely adopted in children having L-ECM for achalasia without further multicenter analysis but appears beneficial in cases having inadvertent mucosal violation.
Introduction
Achalasia is a rare pediatric primary motility disorder of the esophagus exacerbated by failure of a high-pressure lower esophageal sphincter to relax. Common symptoms include vomiting, progressive dysphagia, substernal chest pain with deglutition, regurgitation of solid foods, and unintended weight loss. Definitive diagnostic testing involves high-resolution esophageal manometry, often after contrasted swallow studies and failed medical management with antireflux medication trials. 1
Pneumatic therapy is often first-line treatment after failed medical management, sometimes requiring multiple dilations with the possible addition of Botox injections in select patients. Laparoscopic Heller myotomy (or esophagocardiomyotomy; L-ECM) has become the standard of care for pediatric esophageal achalasia patients given its efficacy and relative safety.1,2 Hospital stays average <41 hours and overall recovery times are typically short. 2 The concurrent use of antireflux anterior fundoplication (A-fundo) during myotomy remains controversial, dependent on multiple factors including surgical approach and provider preference. 3
Concurrent A-fundo has been evaluated in randomized trials among adults, demonstrating mixed results on effectively controlling long-term sequelae of postoperative gastroesophageal reflux (GER) without exacerbating dysphagia.4,5 Those studies showed that GER was not consistently eliminated with the addition of a partial fundoplication procedure and can exacerbate the aperistaltic esophagus.3,4 Persistent dysphagia and postoperative GER continue to be credited as the most common reasons for surgical failure after Heller myotomy.
Postoperative GER even after A-fundo continues to have an incidence ranging between 10% and 30%.4,6 It is still a source of debate whether this recurrent GER is pathological. 5 In the background of conflicting viewpoints among the adult population, evidence for the best approach among children remains scarce and anecdotal.
In addition, potential factors influencing likelihood of primary therapy failure, such as achalasia subtype, are poorly studied in children. One challenge is the clinical similarity and frequency of Chicago classification Type 1 and 2 achalasia in children. Limited data exist on the response of these subtypes to pneumatic dilation and Heller myotomy. 7 The purpose of this study was to analyze our large experience with pediatric presentation of achalasia to determine the impact of concomitant A-fundo on outcomes after L-ECM. We hypothesized that, among children undergoing L-ECM without mucosal violation, routine A-fundo would not significantly improve GER control while exacerbating dysphagia.
Materials and Methods
Institutional review board approval No. 222068 was acquired. Applicable study patients were then identified using a combination of searches for International Classification of Diseases (ICD) 9 code 530.0 for achalasia and cardiospasm and ICD 10 code K22.0 for achalasia in the pediatric patient population. This broad search was then narrowed using ICD codes 43330, 43330R, 43331, 32665, and 43279 to identify patients who had undergone Heller myotomy by open, robotic, or laparoscopic technique. Patients who had not yet undergone surgery or had achalasia not limited to the esophagus or secondary to other disease processes were removed from the database.
Demographics including age, gender assigned at birth, race, and ethnicity were culled from patient electronic charts and stored in an appropriately designated RedCap form. Disease process variables collected included achalasia type, preoperative dilation number, preoperative Botox number, and preoperative proton pump inhibitor (PPI)/histamine type 2 (H2) blocker utilization. Operative and hospital admission factors included intraoperative complications, surgeon, length of stay, postoperative day 1 esophagogram, clear documentation of obstruction resolution, hospital readmission numbers, postoperative dilation number, postoperative Botox number, repeat Heller myotomy, and use of postoperative PPI/H2 blockers.
Postoperative dysphagia was defined as recurrence or worsening of dysphagia symptoms postoperatively or the need for additional procedures such as esophageal dilation, Botox injection, and redo Heller myotomy. Postoperative GER was defined by new or worsening onset of reflux symptoms at postoperative visit or continued need or increased dosage of antireflux medications (PPI, H2 blocker, etc.) >2 weeks after myotomy.
Statistical considerations
Statistical analysis was completed using R statistical software. Independence testing was completed using a combination of chi-squared and fisher exact testing for categorical variables. Continuous variables underwent Kruskal Wallace testing. Further statistical analysis with logistic regression, penalized Ridge, Elastic Net, and LASSO logistic models was attempted but inevitably deemed inappropriate due to low power.
Results
Cohort description
Fifty-four refractory achalasia patients between 2002 and 2023 of age 18 years or less were initially identified for this study. Fifty patients underwent L-ECM, 3 of whom were excluded due to the initial L-ECM having been performed at a referring institution for management of refractory symptoms. The remaining 47 patients comprised 28 (59.6%) males and 19 (40.4%) females. Thirty-seven (78.7%) Patients were identified as White/Caucasian, 7 (14.9%) as Black/African American, and 3 (6.4%) of “other/unknown.”
Cohort groups
Twenty-eight (59.6%) patients underwent concurrent A-fundo during their operation, 19 (40.4%) received only laparoscopic esophageal cardiomyotomy (Table 1). Seven (14.9%) experienced intraoperative complications, 5 of whom were identified during the course of the initial Heller operation and included: 1 anesthesia aspiration event and 4 mucosal injuries repaired intraoperatively followed by A-fundo. Two total complications required reoperation during the initial hospitalization including 1 missed mucosal injury that lead to esophageal perforation with takeback repair on postoperative day 3 and 1 left lung pleural tear taken back for repair on postoperative day 1.
Demographics, Characteristics, and Outcomes
Bold values indicates statistically significant P values.
GER, gastroesophageal reflux.
Postoperative dysphagia
Among the 17 (36.2%) patients with findings consistent with postoperative dysphagia, 11 (64.7%) had concurrent A-fundo and 6 (35.3%) had simple L-ECM with no statistically significant association between A-fundo and postoperative dysphagia (P = .82).
The correlation between postoperative dysphagia and postoperative GER was statistically significant (P = .02) across both A-fundo and Heller-only groups. Ten (58.8%) of the 17 patients with the finding of postoperative dysphagia were found to have concomitant postoperative GER.
Nine (52.9%) patients developing postoperative dysphagia had Type 2 achalasia confirmed by esophageal manometry, 3 (17.6%) Type 1, no Type 3, and 6 (29.4%) indeterminate as they were measured before 2014 when high-resolution manometry became widely implemented. 8 The only statistically significant preoperative factor correlated with postoperative dysphagia was the surgeon performing the procedure (P = .01).
Postoperatively, the patients who developed dysphagia were more likely to experience longer hospital stays (49.1 hours versus 36.0 hours, P = .03) and hospital readmissions (0.6 versus 0.07, P = .03) than those without dysphagia.
The mean age at surgery of patients with postoperative dysphagia tended to be slightly younger (13.3 ± 2.2 years old) than participants with normal postoperative findings (14.5 ± 2.6 years old), although this difference was not statistically significant (P = .08).
The number of patients requiring preoperative procedures such as redo Heller myotomy, esophageal dilation, or Botox injection was statistically similar for both groups with 4 (23.5%) patients with postoperative dysphagia requiring procedures and 7 (23.3%) nondysphagia patients. In addition, no statistically significant difference in the number of intraoperative complications emerged. Other demographics such as gender, race, ethnicity, and preoperative PPI use were also statistically insignificant (P > .05, Table 2) between those with postoperative dysphagia and those without.
Postoperative Dysphagia Characteristics and Independence Tests
Bold values indicates statistically significant P values.
GER, gastroesophageal reflux.
Postoperative GER
Among the 16 (34.0%) patients with findings consistent with postoperative GER, 8 (50.0%) had concurrent A-fundo and 8 (50.0%) had simple L-ECM with no statistically significant correlation between postoperative GER and A-fundo (P = .52).
Of the patients with postoperative GER, 7 (43.8%) had Type 2 achalasia, 2 (12.4%) Type 1, no Type 3, and 7 (43.8%) indeterminate, and these findings were not statistically significant (P = .52). A statistically significant correlation between postoperative GER and postoperative dysphagia again was observed (P = .02).
As observed among patients experiencing postoperative dysphagia, the mean age at surgery of patients with postoperative GER also tended to be slightly lower (13.6 ± 2.9 years old) than participants with normal recovery (14.2 ± 2.6 years old); however, this difference was not statistically significant (P = .51).
We noted that the patients with postoperative GER were more likely to have undergone preoperative esophageal dilation procedures (P < .01); however, Botox procedures showed no significant correlation. In addition, the use of preoperative PPI or other antireflux medication did not have a significant correlation with postoperative GER (P = .54).
Operative and hospital admission factors including intraoperative complications, surgeon, length of stay, postoperative day 1 esophagogram, and readmit numbers were not statistically significant. In addition, other demographics such as gender, race, and ethnicity were statistically insignificant (P > .05, Table 3) between those with postoperative GER and those without.
Postoperative Gastroesophageal Reflux Characteristics and Independence Tests
Bold values indicates statistically significant P values.
Discussion
Despite continued debate on the implementation of routine concomitant A-fundo for the management of postoperative GER, laparoscopic Heller myotomy continues to be the standard of care for pediatric esophageal achalasia, especially after failure of pneumatic dilation, botulinum toxin injections, and medical management. This study showed foremost that routine A-fundo did not benefit patients after L-ECM without mucosal violation. We collected a 20-year span of patients making this study one of the highest powered observational single-institution studies on the pediatric achalasia patients undergoing Heller myotomy with an incidentally randomized divide of patients between those who did and those who did not undergo A-fundo.
These data suggest A-fundo to be clinically insignificant when determining contributors to postoperative dysphagia and GER amid the setting of multiple possibly contributory variables such as performing surgeon and need for preoperative dilations. Those with complications were more likely to require longer initial hospital stays and hospital readmissions.
In addition, we found A-fundo did not control GER while also potentially exacerbating dysphagia. Adult studies of achalasia demonstrate similar findings in randomized control trials with long-term GER and dysphagia recurrence being equal in both populations.9,4 However, debate still continues in adult populations given the presence of prior small power studies showing significant improvement in GER after Heller myotomy plus Dor fundoplication. 5 This study in conjunction with the continued debate among higher powered adult studies leads us to conclude that routine A-fundo is not indicated without better Level 1 evidence specifically in children. PPIs can nicely control postoperative GER. Dysphagia rarely occurs after L-ECM alone.
Another significant finding was the association between postoperative GER and postoperative dysphagia. Symptoms associated with each of these disease states appear to be related where it was unlikely one patient would acquire one without the other. Part of this association may in part be due to the qualitative aspect of our study definitions of GER and dysphagia. It is unclear whether patients presenting with postoperative dysphagia were first ineffectively treated for postoperative GER with increasing PPI/H2 blockers before attempting to address persistent dysphagia symptoms. Rarely did a patient receive a repeat postoperative manometry study, limiting the quantitative ability to assess postoperative dysphagia recurrence.
The low power of this is a single-institution study severely limited statistical analysis given the large number of potential variables identified that might contribute to the postoperative course after L-ECM. By identifying in our observational study which variables are most likely to influence outcomes, we can apply targeted statistical analysis to a larger pool of data from multiple pediatric hospitals in future studies. Additional limitations include definitions of postoperative dysphagia and GER, respectively. Most patients did not undergo repeat esophageal manometry, so this unbiased definition could not be broadly utilized.
Patient-reported symptoms and additional postoperative treatments had to be used as surrogates. Length of follow-up for monitoring of postoperative complications was also variable, ranging from a few months to multiple years. Prior studies with follow-up averaging over a decade have found need for repeat operation in almost all their patients with or without fundoplication. 9 Given the natural transition of patients from pediatric surgeons to adult surgeons for late recurrence of symptoms and the method of outcome definitions in this study, a more standardized longitudinal study possibly in conjunction with associated adult hospitals would be necessary to gather a true understanding of symptom recurrence.
In this largest observation of pediatric achalasia patients, A-fundo appeared clinically insignificant when determining contributors to control GER or exacerbate postoperative dysphagia. Patient demographics, hospital course, and disease factors that might influence operative failure rates have not been well defined in this unique population. Multi-institution studies need to be conducted before encouraging routine A-fundo in the setting of mucosal injury given the poor power of prior studies and lack of randomized control trials in children.
Footnotes
Authors' Contributions
M.P.F. carried out the data curation and wrote the original article draft. J.B. and S.A. performed achalasia investigation and validation. H.C. conducted formal analysis. L.S. and I.Z. performed surgical investigation. M.H. conducted validation and review and editing. H.N.L. conceived the original idea, supervised the project, and conducted review and editing of the article.
Disclosure Statement
No competing financial interests exist.
Funding Information
No funding was received for this article.
