Abstract
Abstract
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™ (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.
Introduction
A
Following diagnosis, treatment options are similar to those offered in adults. Nonoperative management includes botulinum toxin injection, oral calcium channel blockers, and pneumatic dilations (PD). Surgical treatment consists of an esophageal myotomy (Heller myotomy) with or without an antireflux procedure and most recently peroral endoscopic myotomy (POEM).
Given the rarity of achalasia in the pediatric population as well as the variety of technical approaches and management strategies in diagnosis and treatment, we sought to determine the current practice patterns through a survey of the International Pediatric Endosurgery Group (IPEG) membership.
Materials and Methods
The survey was conducted between October and November 2014 on behalf of the IPEG Research Committee using an online provider for Web-based surveys (SurveyMonkey™ [www.SurveyMonkey.com]). All IPEG members (n = 650) were contacted by e-mail and invited to complete an anonymous questionnaire that included personal background and management of achalasia. The invitation was sent out by e-mail with an embedded link to connect to the survey. Two reminder messages were sent during a 4-week period. Trainee/fellows were excluded from the study. A completion of all items was not mandatory if the question did not apply to the respondents' practice.
Data were collected on demographics, specialty board certification, experience as measured by year in practice and number of cases performed if done for achalasia, preoperative workup required, operative approach, muscle division specifics, preference on type and need for wrap, as well as postoperative management.
Statistical analysis
Answers were anonymously collected, converted into a database with Microsoft (Redmond, WA) Office Excel™ (Version 2015), and analyzed using descriptive statistics. Each response was calculated as a percentage to account for the fallout rate per operation.
Results
Demographics
A total of 207 individuals completed the online survey (32%). Of these, 191 commented on performance of esophagomyotomy. More broadly, 202 described themselves as pediatric surgeons and the remainder general surgeons. One-fourth (n = 50) of the participants had more than 20 years of experience, and 30% (n = 60) had between 10 and 20 years of experience. Nearly 74% (n = 149) of the participants had performed over 50 pyloromyotomies.
Preoperative management
Of the 191 surgeons who answered, 141 (74%) perform esophagomyotomies for achalasia. The number of procedures performed per surgeon was as follows: 1–2 (n = 21, 15%); 3–5 (n = 49, 34%); 6–10 (n = 39, 28%); 11–20 (n = 21, 15%); >20 (n = 11, 8%). The majority of responders approach the operation laparoscopically (n = 127, 90%) while robotic and open approaches were used with equal frequencies at 5% (n = 7) each. None of the surgeons used POEM. Workup before esophageal myotomy most frequently consisted of a diagnostic esophagram (n = 133, 94%) or manometry (n = 102, 73%). Only 60% of surgeons (n = 84) required an EGD and few requested Trypanosoma cruzi studies.
Operative management
No preference between circumferential, isolated anterior, or anterior and lateral division of the phrenoesophageal ligament for mobilization of the esophagus was demonstrated; 32% (n = 45) mobilize circumferentially, while 36% (n = 51) mobilize anterior and laterally. The remaining 32% (n = 45) only divide the attachments between the diaphragm/crus and esophagus anteriorly. Placement of anchoring sutures between the crus and esophagus after myotomy was performed equally (n = 68, 48%).
Muscle division was preferentially performed with hook cautery (n = 82, 58%) over harmonic shears (n = 30, 21%), heat sealing device LigaSure™ (Covidien-Medtronic, Minneapolis, MN) (n = 18, 13%), and other devices (n = 11, 8%) for muscle division. Of the 11 surgeons that choose other instruments, five surgeons (3%) used scissors, two (1%) used a scalpel, and the remaining used other forms of electric devices. Standard recommended myotomy length is 5 cm on the esophagus with 2 cm extension onto the gastric wall. In this review, 33% (n = 47) of surgeons surveyed determine length based on endoscopic findings of patency. To determine patency endoscopically, 57% (n = 80) of those surveyed believed it was necessary to conduct intraoperative endoscopy. Those who use a specific length range from 2 to 12 cm with 5–6 cm being the mean. Some of the variation stems from clinical judgment based on the child's size.
Failure of surgical intervention is most commonly secondary to gastroesophageal reflux disorder and recurrent dysphagia. Secondary to this, it is common to perform an antireflux procedure in conjunction with Heller myotomy. Five percent (n = 7) of surgeons surveyed did not perform any type of fundoplication to prevent postoperative gastroesophageal reflux. In the remaining, fundoplication accomplished by the Thal/Dor approach was performed most frequently (n = 111, 79%) followed by the Toupet (n = 18, 13%) and Nissen (n = 4, 3%). Fifty percent of surgeons (n = 70) ordered postoperative esophagram before initiation of enteral feeds, and dietary restrictions were provided for 76% (n = 107) of postoperative patients.
Discussion
Achalasia is a well-known disorder of esophageal motility with an evolving treatment algorithm for pediatric patients. Nearly all data and recommendations are based on adult studies and outcomes highlighting the question of degree of correlation between pediatric and adult management. In light of the difference between pediatric and adult recommendations, this survey aimed to identify the preferred surgical strategy of the IPEG members, a group that was established as a venue to showcase advanced endoscopic techniques and the application of new developments.
Similar to adult populations, laparoscopic Heller myotomy (LHM) with or without an antireflux procedure is increasingly the treatment of choice for children. 5 This is the first survey to examine variability in pediatric surgical management globally and demonstrates that the accepted standard of care is relatively consistent across the ages. The vast majority of the participants from all continents worked in academic institutions and had performed between 3 and 10 or more LHM in their career. The preferred surgical technique among IPEG members is LHM with Thal/Dor fundoplication.
One of the main controversies in achalasia management continues to center around LHM versus PD. While many therapies for achalasia exist in the adult population, mechanical dilation has historically been the gold standard upon which other therapies were compared. Short-term results are proven to be effective; however, long-term results are less promising. Currently, LHM has evolved as the preferred procedure for adults with esophageal achalasia, while medical treatments and/or endoscopic esophageal PDs are usually reserved for those older than 45 years and those with high surgical risk. 6 Previous studies have demonstrated the superior long-term results of myotomy; however, this has been challenged by one large multicenter randomized trial demonstrating lack of LHM superiority to PD. 7 Notably, this study did not define failure when repeat interventions were required. In the pediatric population, a systematic review demonstrated that adequate comparative data are lacking to determine ideal treatment for pediatric achalasia. 5 Additionally across the literature PD, which requires intubation, is often only performed on school aged children. Need for reintervention, similar to the adult literature, is vague with a range of 25%–90% 5 with the period of symptom-free time dropping significantly in those children requiring subsequent treatments. 2
Antireflux procedure following LHM is another source of contention. In a previous comprehensive review, comparative data on type of fundoplication were summarized. Nissen fundoplication (360°) was deemed inappropriate secondary to high progressive esophageal stasis and functional obstruction rates believed to be created by complete wrap. 8 Dor fundoplication (180° anterior) results in abnormal pH seen in 5.7–10% of patients up to 1 year postoperatively. 8 Advocates state that the anterior wrap provides a degree of protection over the myotomy from any subclinical intraoperative injuries. Conversely, those who advocate for a Toupet (270° posterior) describe its ability to hold the myotomy open without actually covering an anterior myotomy. 8 While the majority of IPEG members performed an antireflux operation at the time of myotomy, 6% did not perform an antireflux procedure. The authors who report LHM without antireflux procedure report a high rate of symptom relief and low incidence of postoperative complications, despite the lack of esophageal motility restoration to normal. 9
One potential topic for discussion is the apparent lack of POEM in the pediatric population. It has the combined benefits of an endoscopic procedure with the long-term efficacy of a surgical myotomy. 10 Before a 2015 study done by Caldaro et al., the procedure had only been done in adults with proven safety and efficacy. 10 Emerging data have shown that it has shorter operative times, lower complication rates, faster time to feeding, longer myotomy, and more rapid discharge. 10 A single-center experience of 26 patients with an age range of 6–17 years demonstrated at 2-year treatment success with a 20% development of reflux. 11 Given its recent introduction with children, there may be potential for a shift in standard treatment once more experienced endoscopists are available and trained to provide comparable or improved results for achalasia. 10
Another notable difference is the absence of endoscopic biopsy in preoperative workup. For adults, biopsy is recommended to exclude malignancy before surgical intervention. None of the respondents endorsed biopsy before operative intervention. However, 1.5% of those polled endorsed obtaining Trypanosoma cruzi studies, as secondary achalasia leading to malnutrition can often be the first symptom of digestive pathology in a patient with Chagas disease.
An inherent aspect of being a pediatric surgeon is facing a wide variety of rare conditions. Surgical groups have grown to allow for coverage of the busy call schedules, which creates the scenario of having a diluted experience with already uncommon procedures. If only six cases were used as the threshold for stabilizing on the learning curve, half of the respondents do not have a mature experience. This suggests we should consider concentrating the experience among fewer surgeons at each institution. This lack of individual experience likely explains the fact that despite surveying a minimally invasive organization, 5% of respondents still perform an open myotomy.
Despite offering a general picture of the current practice of achalasia among IPEG members, we are aware of limitations of our study. There could have been a bias in selecting the surgeons as not all members responded. In addition, we can only assume that the participants provided honest answers. Surveys in general are susceptible to recall bias. We also did not ask about long-term follow-up on this cohort of patients or need for reintervention. Finally, certain information such as recurrence rates could have a potential bias in patient selection.
Conclusion
Laparoscopic achalasia repair is currently being performed by 90% of all participating IPEG members. The preferred surgical technique among IPEG members is LHM with Thal/Dor fundoplication. The fact that limited information exists on symptom recurrence or age-specific recommendations, method of achalasia diagnosis, procedure techniques, and follow-up suggests the focus of future studies.
Footnotes
Disclosure Statement
No competing financial interests exist.